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Paediatric Heart Journal of Bangladesh (PHJB)

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Editor in Chief : Brig. Gen. Prof. Nurun Nahar Fatema Begum

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Members : Prof. Manzoor Hussain

Prof. Samir K Saha
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Prof. A.F.M. Salim
Prof. Mohd. Zahid Hussain
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Prof. Abdullah-Al-Safi Majumder
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Prof. Chowdhury Yakub Jamal
Prof. Mohammad Sharifuzzaman
Prof. Syed Abdul Quader
Dr. A.B.M. Abdus Salam
Dr. SK-A. Razzaque
Dr. Kazi Abul Hasan
Dr. Md. Tariqul Islam
Dr. Tahera Nazrin
Dr. Md. Abu Syeed
Dr. Mohammad Ataul Haque

Published by Editor, Paediatric Heart Journal of Dhaka, Bangladesh

Published in May 2021
 h
Paediatric Heart Journal of Bangladesh (PHJB)
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 Paediatric Cardiac Society of Bangladesh
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 Editorial
Long term durability of surgical interventions
for complex CHD of the right ventricular outflow
tract (RVOT) is highly variable, depending on
patient age and the type of tissue or material
utilized. Ultimately, these patients are subject
to progressive RVOT dysfunction due to several
mechanisms, including pulmonary
regurgitation (PR), somatic outgrowth,
anastomotic stenosis of the conduit, valvular
stenosis, conduit kinking, sternal
compression, intimal proliferation, conduit
calcification, and aneurysmal degeneration.
Therefore, repeated surgical interventions are
typically required over a lifetime. Although
these procedures have a low mortality rate,
they can be associated with significant
morbidity, particularly with repeated
operations. In this clinical context,
percutaneous pulmonary valve implantation
(PPVI) has been developed as a nonsurgical, less
invasive alternative for the treatment of RVOT
dysfunction which is a minimally invasive
method to treat RVOT/pulmonary trunk
dysfunction in children and adults by restoring
acceptable RV loading conditions, avoiding
open-heart interventions and described
elaborately in leading article section.
Persistent pulmonary hypertension of the
newborn (PPHN) is common and leads to a
major burden of neonatal illness, carries a
significant mortality and morbidity. Although
inhaled NO (iNO) and extracorporeal membrane
oxygenation (ECMO) are the gold standards of
the PPHN therapy, they are expensive
therapeutic modalities associated with
technical difficulties in developing countries
making it necessary to search for cheaper
therapies, assuring quick effectiveness and
stabilization of the patient going through a very
Paed. Heart J of Bangladesh (PHJB) 2019;4(1):1
high-risk situation. For this reason a
randomized clinical trial was conducted in the
Paediatric Cardiac Intensive Care Unit (CICU)
of Dhaka Shishu (Children) Hospital to evaluate
the effect of Sildenafil and Magnesium Sulphate
in PPHN.
Although a median sternotomy was considered
the routine approach for an open-heart
operation, the scar was regarded as unsightly
and displeasing, and may evoke psychological
distress, especially in young, female patients.
To evaluate the outcome and safety of the right
vertical infra-axillary mini incision (RVAI) used
for the repair of ASD a prospective observational
analysis was performed in the department of
cardiac surgery, Ibrahim Cardiac Hospital &
Research Institute.
Transposition of great arteries (TGA) is a
common cyanotic heart disease of paediatric
age group. Wide range of cardiac defects and
anomalies are associated with TGA.
Echocardiography remains the most
confirmatory tool for understanding and
detecting the various presentations of this
cyanotic heart disease. A retrospective study
was conducted to enlist the various
echocardiographic findings of TGA cases
diagnosed by echocardiography at outpatient
settings of paediatric cardiology department of
BSMMU.
These are some of the topics discussed in this
issue of Paediatric Heart Journal of Bangladesh.
Any comments or criticism will be highly
appreciated.
Editor
Paediatric Heart Journal of Bangladesh (PHJB) Volume 4, No. 1, January 2019

2
 Leading Article
Percutaneous Pulmonary Valve Implantation
Mohd. Zahid Hussain1, Somayra Nasreen2
Paed. Heart J of Bangladesh (PHJB) 2019;4(1):3-12
Introduction
The incidence of congenital heart disease
(CHD) varies between 5 and 8 per 1,000 live
births. 1,2 In adulthood, the estimated
prevalence is 1 in 150 people.3 Almost twenty
percent of newborns with CHD that present
complex anomalies such as tetralogy of Fallot,
pulmonary atresia with and without
ventricular septal defect, truncus arteriosus,
transposition of great arteries, some forms of
double outlet right ventricle (DORV), and
congenital aortic valvar anomalies previously
treated with Ross procedure, require surgical
reconstruction using a patch (i.e., infundibular
and transanular), a bioprosthetic valve or a
valved conduit.
Long term durability of surgical interventions
for complex CHD of the RVOT is highly variable,
depending on patient age and the type of tissue
or material utilized.4,5 Ultimately, however,
these patients are subject to progressive RVOT
dysfunction due to several mechanisms,
including pulmonary regurgitation (PR),
somatic outgrowth, anastomotic stenosis of the
conduit, valvular stenosis, conduit kinking,
sternal compression, intimal proliferation,
conduit calcification, and aneurysmal
degeneration.4,6 Therefore, repeated surgical
interventions are typically required over a
lifetime. Although these procedures have a low
mortality rate, they can be associated with
significant morbidity, particularly with
repeated operations.7,8 In this clinical context,
percutaneous pulmonary valve implantation
(PPVI) has been developed as a nonsurgical, less
invasive alternative for the treatment of RVOT
dysfunction.
1. Professor and Chairman, Department of Paediatric Cardiology, BSMMU, Dhaka.
2. MD (Paediatric Cardiology) Student, BSMMU, Dhaka.
Address of correspondence: Prof. Mohd. Zahid Hussain, Professor and Chairman, Department of Paediatric
Cardiology, BSMMU, Dhaka. E-mail: z.hussain71@yahoo.com
Historical over view
The first report about PPVI was published by
Bonhoeffer et al. in August 2000 demonstrating
the feasibility of non-surgical implantation of
a fresh bovine jugular vein containing a native
valve sutured into a vascular stent in pulmonary
position in lambs.9 In October 2000, he reported
the first human PPVI in a 12-year-old boy with
a dysfunctional RV-PA conduit previously
implanted for a pulmonary atresia with a
ventricular septal defect10 and in 2002 the first
clinical series was published showing the acute
success of the procedure.11 The first U.S.
implantation occurred in January 2007 in
Boston Children’s Hospital, and subsequent
trials led to U.S. Food and Drug Administration
(FDA) approval in 2010.12 At this time, the
Melody valve is FDA approved, under
humanitarian device exemption guidelines, for
use only in previously placed surgical conduits
and in patients whose surgical bioprosthetic
pulmonary heart valves have failed. However,
off-label use in native, dysfunctional RV outflow
tracts is possible in certain cases, provided that
the size of the landing zone is adequate.13
Main indication of pulmonary valve
intervention
Current indications for replacing the
pulmonary valve (PV) are symptoms of heart
failure requiring pharmacological therapy,
severe RV hypertension with an RV/LV
pressure >0.7, peak and mean Doppler gradients
across the PV of >50 mmHg and 30 mmHg
respectively, indexed RV end diastolic volume
>160/mL/m2 or RV end systolic volume >80
mL/m2, RV end diastolic volume e”2 times the
LV end diastolic volume, RV ejection fraction
<0.40–0.45, and a QRS duration e”180 ms.
Paediatric Heart Journal of Bangladesh (PHJB)
Adjunctive relevant factors considered when
planning PV replacement are sustained atrial
or ventricular arrhythmias, significant
coexisting lesions (such as significant aortic
regurgitation, tricuspid regurgitation [TR], and
residual ventricular septal defects), and left
ventricular dysfunction. American College of
Cardiology/American Heart Association,
European, and Canadian guidelines have been
published.14-16
In this context, percutaneous PV implantation
(PPVI) offers a minimally invasive method to
treat RVOT/pulmonary trunk dysfunction in
children and adults by restoring acceptable RV
loading conditions, avoiding open-heart
interventions.
Case selection
Once it is clear that the patient needs
intervention for RVOT dysfunction, case
selection for suitability of percutaneous
intervention is important. It is prefered to go
by the following criteria:
1. RV to PA conduit of any type (circumferential
conduit) of a minimum diameter > or equal
to 16 mm and maximum diameter of < or
equal to 22 mm at the time of surgery or at
the time of consideration of the catheter
intervention. There have been successful
implantations in patients with nonconduit
outflow tracts where a good platform is
created by pre-stenting of the outflow tract
to appropriate dimensions that provide a
suitable landing zone for the Melody valve.17
2. Appropriate length to the conduit available
well away from pulmonary artery
bifurcation (Melody valve is mounted on a
34 mm 8 zig CP stent and that shortens by
13.5% (28.8 mm) at 18 mm balloon inflation,
and by 26% (24.6 mm) at 22 mm balloon
inflation).
3. Risk of coronary compression by stent is
ruled out. Anomalous coronary across the
RVOT (LAD from the right sinus or accessory
LAD from the RCA, or single coronary origin
from the left or the right sinus) are not
uncommon indications for use of RV to PA
conduits in repairing certain conotruncal
anomalies. By the nature of the anatomical
4
Volume 4, No. 1, January 2019
substrate, these conduits would lie over the
anomalous coronary artery. For patients
where coronary arteries are reimplanted,
such as Ross operation; although the native
anatomy of coronary origins was normal,
one must be mindful of the changed spatial
orientation of coronaries in relation to the
reconstructed RVOT that could create a risk
of coronary compression.
4. Active infection is an absolute
contraindication for this procedure.
5. Previous history of conduit endocarditis is
a concern. Endocarditis within the conduit
is underdiagnosed and may remain
dormant despite negative blood cultures
and inflammatory markers.
6. Patients less than 20 kg pose a higher risk
of complications, such as vascular injury
or cardiac trauma.
7. Pregnancy is an absolute contraindication
due to the biological component of the
device.
Preoperative evaluation
A complete detailed history and clinical
examination, backed up by investigations for
structural and functional information is
required for planning the procedure.
In the early assessment, history should
include details of all previous catheter
interventions and operative notes. In patients
with stented RVOT conduit, information about
what balloons where used to what final
diameter, gives some idea to choose delivery
systems during PPVI. The surgical notes may
comment on spatial relationship of the coronary
arteries to great arteries or outflow tracts that
may be more helpful than any imaging
technique. Clinical history should include
meticulous questioning to rule out subtle
endocarditis. Examination should include oral
examination to rule out dental caries or in fact
a formal dental referral before the actual
procedure.
To establish indication to PPVI, all patients
should undergo a standardized assessment
protocol:18
• Surface electrocardiogram (EKG) and 24-h
Holter EKG monitoring to detect arrhythmia
and define QRS duration.
Percutaneous Pulmonary Valve Implantation
• Echocardiography as the first screening
tool. On one hand, it allows to determine
the presence of a residual RVOT stenosis
identifying the site, quantifying the
severity and determining the cause of the
stenosis itself. The grade of stenosis is
defined as follow: severe with a peak jet
velocity >4 m/s (peak gradient>64 mmHg),
moderate with a peak jet velocity of 3-4 m/
s (peak gradient 36–64 mmHg), mild with a
peak jet velocity <3 m/s (peak gradient less
than 36 mmHg). 19 On the other hand,
echocardiography allows to assess the
severity of PR according to the regurgitant
jet width (if more than 65% of the RVOT, is
in favour of severe PR) and to the
deceleration of continuous wave signal of
PR jet assessed qualitatively (steep
deceleration is in favour of severe PR).20
Moreover, this non-invasive tool is also
used to evaluate RV dimensions
(measuring RV diameters at the base and
at the mid-level and the long axis in a 4-
chamber view and quantifying the RVOT
diameter in a parasternal short axis view),
RV systolic function (usually through
tricuspid annular plane systolic excursion,
S2 wave peak at the tissue Doppler
imaging or fractional area change) and to
estimate the RV pressure from tricuspid
valve regurgitant jet (TR velocity>2.8-2.9
m/s, assuming an RA pressure of 3–5
mmHg, indicates elevated RV systolic
pressure) and the RV to systemic pressure
ratio. Echocardiography allows also the
assessment of the left chambers end of
associated lesions.21
• Cardiopulmonary exercise testing on a
bicycle provides a comprehensive
assessment of the exercise response, and
reflects the influences and interactions of
the cardiac, respiratory, musculoskeletal
and haematological systems. This test
provides data on respiratory gas exchange,
including oxygen uptake (VO2), carbon
dioxide output (VCO2), tidal volume, minute
ventilation (VE), oxygen pulse, ventilatory
anaerobic threshold, and respiratory
quotient and other variables such as EKG
trace, blood pressure and oxygen
saturation.22,23
Mohd. Zahid. Hussain & Somayra Nasreen
• Cardiovascular magnetic resonance
imaging (MRI), providing that it is not
contraindicated, is the gold standard to
quantify PR and right ventricular volumes
and function. End-diastolic and end-systolic
volumes and derive indices such as stroke
volume, ejection fraction and myocardial
mass are obtained by contouring
endocardial and epicardial contours.24
• Computed tomography (CT) scan is
performed in complex anatomy to evaluate
the relationship between the pulmonary
trunk and the aortic root, to assess coronary
anatomy and RVOT size. Moreover, it is
possible to estimate the risk of coronary
artery compression according to the
distance between the coronary artery and
the landing zone.25
• Three-dimensional (3D) printing.
Candidates to PPVI can have complex post-
surgical anatomies, therefore further
imaging approaches can be necessary in
order to have as many information as
possible prior the percutaneous
intervention. In addition to the standard
imaging techniques previously reported, 3D
printing is a useful tool that can help
planning the PPVI. Fusion of different
modalities (e.g. ventricles from CT, valves
from echocardiography) to create a single
3D model has been reported. Its application
for the study of the RVOT and the simulation
of PPVI preceded by a pre-stenting
intervention, to better understand the
anatomy and the complex relationship with
the coronary arterial course, has already
been reported.26
Currently available valves
Bonhoeffer et al 10 first performed
transcatheter PV implantation in 2000.
Improvements to the device initially used by
Bonhoeffer led to the development of the Melody
transcatheter PV (Medtronic Inc., Minneapolis,
Minnesota)27,28 which consists of a bare-metal
platinum-iridium stent (CP stent, NuMED, Inc.,
Hopkinton, New York) and a manually-sewn
valved segment of bovine jugular vein. The fresh
bovine jugular vessels are fixed in
glutaraldehyde, and then sutured to the frame
5
Paediatric Heart Journal of Bangladesh (PHJB)
using blue suture at the distal end of the valve
to ensure proper orientation of the valve on the
Ensemble transcatheter delivery system,
which has a blue carrot tip on the end. The
initial length of the valve is 28 mm, but it is
shortened in accordance with the final
implanted diameter. The valve can be expanded
from 16 to 22 mm in diameter, and in some
instances up to 24 mm.
Currently, the Melody valve is available in
diameters of 16 and 18 mm, which can be
expanded to 18 or 20 mm, and 18, 20, or 22
mm, respectively. The device is crimped over
a balloon-in-balloon catheter and delivered
through a long 22-F sheathed balloon catheter
(Ensemble Delivery System, Medtronic Inc.).
The balloon-in-balloon technique allows for
stepwise deployment, as the valve can still be
repositioned after the inner balloon is inflated.
The delivery system also includes a sleeve over
the sheath to provide hemostasis at the
insertion site.27,29
The Edwards Sapien Pulmonic transcatheter
heart valve (Edwards Lifesciences,Irvine,
California) is a trileaflet bovine pericardial
tissue valve hand-sutured in a balloon-
expandable, radiopaque, stainless steel stent.
It is available in 23 or 26 mm diameters that
require 22- or 24-F delivery sheaths,
respectively. This device contains a unique
proximal sealing cuff designed to prevent
paravalvular leaks. The valve is implanted
using the Retroflex-3 delivery system (Edwards
Lifesciences), which consists of a guiding
catheter and a single-balloon catheter. A
specialized tool is used to manually crimp the
valve over the valvuloplasty balloon.29-31
The Edwards Sapien 3 THV (Edwards
Lifesciences) is the third-generation Edwards
SAPIEN valve. Similar to the previous
generation valve, in addition it has also an
outer polyethylene terephthalate (PET) cuff,
which is designed to minimize paravalvular
leak. The stent itself has different inflow and
outflow geometry, which causes the valve to
foreshorten more at the inflow portion. There
are three different sizes: 23, 26, and 29 mm.
The expanded valve heights are 18, 20, and 22.5
mm, respectively, but the covered portion (inner
6
Volume 4, No. 1, January 2019
skirt height) is significantly shorter at 9.3, 10.2,
and 11.6 mm, respectively. The valve is
designed to be delivered by the deflectable,
Edwards Commander Delivery System.
Because of the lower profile design, the 23- and
26-mm valves require a 14F and the 29-mm
valve requires a 16F Edwards eSheath. This
low-profile sheath can transiently expand to
accommodate the passage of the valve and
return to the original diameter.3
New devices
A number of innovative strategies have been
studied in an effort to expand the population
eligible for PPVI, particularly in patients with
large RVOT diameters. If effective in oversized
RVOTs, these new techniques and devices
have the potential to expand PPVI indications
to more than 50% of dysfunctional RVOTs.33
Native Outflow Tract device (Medtronic, Inc.),
has completed enrollment in an ongoing
Investigational Device Exemption trial. It
has an hourglass contour with larger
diameters at the proximal and distal ends
and a smaller diameter in the center, where
a porcine pericardial valve sits. The self-
expanding nature of the nitinol stent has the
potential, in theory, to improve valvular
stability in heterogeneous RVOT
morpholologies.34
The Venus P Valve (Venus Medtech,
Shanghai,China) is another novel self-
expanding percutaneous pulmonary device. It
is composed of a trileaflet porcine pericardial
valve mounted on a covered nitinol stent
frame. It is manually crimped onto a delivery
system that ranges from 14- to 22-F,
depending on valvular size, which varies from
20 to 32 mm.35,36 This valve is applicable in
large patch augmented RVOTs, it may play
an important role in the future of PPVI if
favorable results are confirmed in clinical
trials.
Similarly, the Sapien XT (Edwards Lifesciences)
29-mm valve may also become an alternative
for large-diameter RVOTs, although this valve
has not yet been sufficiently studied in the
pulmonary position.
Percutaneous Pulmonary Valve Implantation
RVOT reducers : Patients who underwent
surgical repair of tetralogy of Fallot during
infancy using a transannular patch can have
large pulmonary trunks that often exceed 30
mm in diameter, making PPVI technically
unfeasible with the current valves. To extend
the indications of PPVI, the use of
percutaneously implanted RVOT size reducers
has been described. The device is available with
various external diameters (30-40 mm). This
device was implanted in sheep that had
previously had surgical RVOT enlargement. The
main complications were device embolization
and para-prosthetic leaks.37-39 These devices
seem very promising, but they have not been
tested for human application so far. Indeed, the
applicability of these devices to the human
anatomy with calcified RVOT or non-circular
cross-sectional shape remains to be proven.
Percutaneous pulmonary valve implantation in
small children and those with a low body weight
: The transcatheter valves available currently
(the Melody and SAPIEN valves) require a large
delivery sheath (22 and 24 Fr, respectively),
limiting the use of this technology in patient
weighing >30 kg. Although the optimal timing
for intervention in RVOT conduit dysfunction
remains unclear, there is a trend towards
earlier intervention in the pediatric population
, to minimize deleterious effects of conduit
failure on right ventricular function.40,41 As
technology continues to advance, minimizing
the delivery sheath and valve size, and
developing mini-invasive hybrid approaches
should be prioritized, so that this technology
can be used in younger patient populations.
New devices with reduced delivery systems are
under development at the moment. The most
advanced device is based on a dry valve
mounted on a stent. The whole system comes
prepared, already mounted in the delivery
system and in the same package. The valve
being dry, the delivery system is only 14 Fr,
making the use of this device possible in small
children.
Future developments
OFF-LABEL USE. Currently, indications for
PPVI are limited to dysfunctional surgical
RVOT conduits with dilated diameters between
Mohd. Zahid. Hussain & Somayra Nasreen
18 to 22 mm and 23 to 26 mm for the Melody
and Sapien valves, respectively. 29,30,42
However, it is estimated that only <20% of
patients with CHD and RVOT dysfunction meet
these restricted criteria33,43. The majority of
patients with the potential to benefit from PPVI
have an off-label indication for the procedure,
including native or large patch-augmented
RVOTs, bioprosthetic valves, or small-
diameter conduits(<16 mm) . Boshoff et al43
reported 23 off-label cases of PPVI, including 8
patients with conduit free patch-augmented
RVOTs, 2 native PV stenosis, and 13 undersized
conduits. The use of approved devices for
off-label indications has been associated with
an increased incidence of complications, as
compared with standard indications. 44
Furthermore, there are liability and ethical
concerns with off-label device use . However,
there are instances where devices and
procedures are clinically indicated on the basis
of the published medical data and/ or standards
of practice in the medical community, but
remain off-label according to regulatory
agencies.43,44 In such cases, patients can be
deprived of effective treatment strategies if
device use is strictly restricted to standard “on-
label” indications. Developments in PPVI
technology and larger clinical studies in
patients with native, undersized, or nonconduit
RVOTs will likely expand the “on-label”
indications for PPVI in the near future.
Meanwhile, physicians considering an off-label
procedure must evaluate clinical
appropriateness on a case-by-case basis. This
decision requires a careful assessment of
patient preferences, guided by an informed
decision-making process, as well as a review
of safety and efficacy outcomes data published
in similar patients.
Early and medium-term results
Right ventricular outflow tract gradient
normalization and PR resolution were achieved
in the vast majority of patients after successful
implantation. In isolated cases moderate PR
or residuary pulmonary gradient across the
valve were observed. In a short follow-up (1st
month) improvement in systolic RV volumes
and function as well as left ventricular ejection
7
Paediatric Heart Journal of Bangladesh (PHJB)
fraction has been reported. Moreover, NYHA
class and maximum oxygen consumption
improvement were observed predominantly in
patients with PS. 45 Further observations
confirm that the results are stable in a few
years but do not show subsequent
improvement, indicating that changes in RV
end-diastolic volume and RV ejection fraction
result from normalization of hemodynamic
conditions, not structural myocardial
remodeling.
Lack of continuous improvement beyond the
1st month may be related to late performance
of the intervention.45 Data from 3 prospective
multicenter studies (300 patients) with
transcatheter Melody valve implantation
conducted in Canada and Europe has showed
recently that significant baseline tricuspid
regurgitation (TR), often seen in patients with
RVOT dysfunction, was improved in 65% of
patients. Acute reduction of TR persisted over
5 years of follow-up. This finding may extend
indications for those patients with RVOT
dysfunction suitable for PPVI who were referred
for surgery because of concomitant TR.46
Complications and limitations in
percutaneous pulmonary valve implantation
Acute complication rate reported in the
literature varies from 2% to 6% for the Melody
valve and from 10% to 20% for the Edwards
Sapien Valve.47,48
Approximately 5% of patients that undergo PPVI
are at risk of coronary compression by the stent
or the prosthesis with subsequent acute
myocardial infarction. Therefore, coronary
angiogram with simultaneously balloon
inflation in the landing zone in the RVOT at
the targeted outer valve diameter is
recommended prior to valve implantation. An
abnormal coronary artery anatomy is
considered a possible risk factor.49 With the
advent of bigger prosthesis, aortic root
deformation with consequent severe aortic
regurgitation has emerged as another possible
acute complication that can be identified with
an aortography with a balloon inflated in the
RVOT. It is especially true for patients with
native RVOT. In case of this occurrence, as for
coronary artery compression, the PPVI cannot
8
Volume 4, No. 1, January 2019
be performed and the patient is candidate to
surgical intervention.50
Possible rare acute complications are: partial
or total conduit rupture following balloon pre-
dilation, usually confined using covered stents
(3%); tricuspid valve damage,51 guidewire
related injuries of distal pulmonary artery
branches leading to bronchial bleeding or
haemothorax (0.05%); valve or stent migration
(2.4%); acute flash pulmonary oedema episodes
in high-risk patients with abnormal LV
diastolic function for whom priming diuretics
treatment before the procedure is necessary.
Minor complications can rarely occur at the
vascular access site because of the large size
of the delivery system. 18,47 The overall
periprocedural mortality incidence is around
1.4%.52
Late complication
Stent fractures are the most common
complication detected at follow-up after Melody
valve implantation (12.4%), with a particularly
high incidence in studies that reported lower
rates of pre-stenting of the ROVT prior to PPVI.
They range from minor, hemodynamically
insignificant alterations in stent structure to
complete separation and embolization of stent
segments, with more severe forms of stent
fractures strongly associated with restenosis
and subsequent RVOT reintervention.
However, monitoring may be justified to detect
even minor fractures, as they demonstrated
the propensity to deteriorate and cause
subsequent valve dysfunction. Risk factors
associated with stent fractures include younger
age, higher pre and post procedural RVOT
gradient, smaller angiographic conduit
diameter, stent recoil or compression after
deployment and valve position directly under
the sternum. Pre-stenting has been shown to
decrease the incidence of fractures and prolong
freedom from fracture related reinter-
ventions. 53 No stent fracture has been
described in Edwards Sapien valve.
The second most common complication
identified at follow-up is infective endocarditis
(4.9%). Most of the cases reported involved
Melody valves52 and it seems to be 4.5 times
more frequent after PPVI than after surgical
Percutaneous Pulmonary Valve Implantation
PV replacement. Several factors responsible
for this difference and the possible valvular
damage before percutaneous implantation
during crimping and during balloon expansion
seems to be the more procedure-related. Prior
infective endocarditis increases relative risk
to 3.3. Some authors suggest suboptimal
hemodynamic results (residual gradient,
eccentric turbulence, pockets due to
incomplete apposition, thrombus formation,
asymmetric or incomplete opening with
redundancy of leaflet tissue) as possible risk
elements. Among other traditional factors for
infective endocarditis, poor dental hygiene,
unprotected dental care, piercing, tattoo and
nail biting have been investigated.54
Conclusion
PPVI has been consolidated as a safe and
effective nonsurgical therapeutic alternative
for RVOT dysfunction. In patients with stenotic
or regurgitant surgical RVOT conduits, PPVI
has a high proce-dural success rate, with
immediate and durable resolution of RV-to-
PA gradient as well as a very low incidence of
post-procedure PR. Less than 25% of
patients who undergo PPVI will require repeat
interventions in a 5-year period, most
commonly secondary to a fracture in the
percutaneous valve stent frame. Future
developments in the field aim to reduce the
incidence of complications, improve freedom
from reintervention rates, and, most
importantly, expand the population eligible for
this elegant procedure. Clinical studies in off-
label populations, innovative devices, and new
techniques will likely expand the indications
to native RVOTs, small-diamter conduits, and
over sized patched RVOTs.
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 Original Article
Effect of Sildenafil and Magnesium Sulphate in
Pulmonary Hypertension in Newborn
Mohammad Abdullah Al Mamun1, Manzoor Hussain2, Abdul Jabbar3, KM Sarwar Mahmud4
Abstract
Background: Persistent pulmonary hypertension of Newborn (PPHN) is a severe
event affecting newborn. Recent advancement has improved the therapeutic
approaches to neonates with PPHN.
Objective: To evaluate the effect of Sildenafil and Magnesium Sulphate in
Pulmonary Hypertension of Newborn.
Methodology: This randomized clinical trial was conducted in the Paediatric
Cardiac Intensive Care Unit (CICU) of Dhaka Shishu (Children) Hospital from
August 2015 to July 2017. Neonates having moderate to severe PPHN were
randomized into Sildenafil and Magnesium sulphate group. Outcome measures
include drop of pulmonary vascular resistance measured by right ventricular
systolic pressure, increase PaO2 and time interval to improve O2. Side effects
was observed in the patient and outcome was recorded. Data were analyzed by
using SPSS version 17.
Results: Mean age of neonates in hour in Sildenafil treatment group was
34.36±10.38 hours and Magnesium sulphate treatment group was 29.12±14.26
hours. There was significant improvement of oxygenation at 72 hours after
treatment in both study groups (p<0.05). Statistically significant improvement
was found at 72th hour regarding drop of right ventricular systolic pressure
(RVSP) in both treatment groups (p<0.05). No significant statistical difference
was observed in complications, time taken to improve, hospital stay and outcome
between two study groups (p>0.05).
Conclusion: Sildenafil and Magnesium sulphate both are effective in
improvement of oxygenation and reduction of pulmonary vascular resistance in
newborn. Sildenafil was found more effective than Magnesium sulphate regading
improvement of oxiginaiton.
Key Wards: Sildenafil, Magnesium sulphate, PPHN.
Paed. Heart J of Bangladesh (PHJB) 2019;4(1):13-19

Introduction vascular resistance characteristic of fetal

The incidence of PPHN in term or near-term circulation fails to decrease at birth, resulting
infants is reported to reach around 2-6.8 per in right to left shunting of blood through fetal
1000 live births with a mortality of 10% to channels, diminished pulmonary blood flow,
20%.1,2 It occurs when the high pulmonary and profound hypoxaemia. PPHN can be a

1 Associate Professor, Division of Neonatal Cardiology, Department of Paediatric Cardiology, Bangladesh Institute
of Child Health and Dhaka Shishu (Children) Hospital.
2 Former Head of Paediatric Medicine and Cardiology, Bangladesh Institute of Child Health and Dhaka Shishu
(Children) Hospital.
3 Registrar, Department of Paediatric Cardiology, Dhaka Shishu (Children) Hospital.
4 Assistant Professor of Paediatrics, National Center for Control of Rheumatic Fever and Heart Diseases (NCCRF/
HD), Dhaka.
Address of correspondence: Dr. Mohammad Abdullah Al Mamun, Associate Professor, Division of Neonatal Cardiology,
Department of Paediatric Cardiology, Bangladesh Institute of Child Health and Dhaka Shishu (Children) Hospital.
Cell: 01913475529, E-mail: mamundsh@gmail.com
[Received: 03 March 2020 Accepted: 02 June 2020]
Paediatric Heart Journal of Bangladesh (PHJB)
primary condition or can be secondary to a
variety of disorders causing hypoxic respiratory
failure. It is characterized by increased
pulmonary vascular resistance and right-to-left
shunting through the foramen ovale, with or
without a patent ductus arteriosus, causing
arterial hypoxia even with 100% FiO2.3
Primary treatment of the neonate with PPHN
depends on the underlying disorder. A variety
of treatment options includes surfactant,
sedation, alkalinization, vasodilatation e.g
(tolazoline, inhaled nitric oxide, magnesium
sulfate, bosentan, sildenafil), high frequency
ventilation (HFV) and extracorporeal
membrane oxygenation (ECMO).4 The aim of
treatment is to lower pulmonary vascular
resistance, maintain systemic blood pressure,
reverse right to left shunt, and improve arterial
oxygen saturation.5 There is strong evidence
that use of iNO and ECMO is effective in the
treatment of PPHN. However, many developing
countries and resource limited centers do not
have the funds or the technical expertise
required for these expensive therapies. 6
Although iNO and ECMO are the gold standards
of the PPHN therapy, they are expensive
therapeutic modalities associated with
technical difficulties in developing countries
making it necessary to search for cheaper
therapies, assuring quick effectiveness and
stabilization of the patient going through a very
high-risk situation.
Sildenafil is a potent and selective inhibitor of
cGMP-specific phosphodiesterase 5 (PDE5). This
isoenzyme metabolizes cGMP which is the
second- messenger of NO and a principle
mediator of smooth muscle relaxation and
vasodilatation. By inhibiting the hydrolytic
breakdown of cGMP, sildenafil prolongs the
action of cGMP. This results in augmented
smooth muscle relaxation and cause pulmonary
vasodilatation. 7 Sildenafil decreases
pulmonary vascular resistance in pulmonary
hypertensive neonate.8 Magnesium sulphate
is a natural Ca channel blocker that
antagonizes Ca ion entry into smooth muscle
cell thus promoting vasodilatation. It is safe
and cheaper alternative for first line treatment
in moderate PPHN.9 Raimondi et al10 concluded
14
Volume 4, No. 1, January 2019
that where nitric oxide facilities are not
available, magnesium sulphate and sildenafil
are cheap alternative for first line treatment
of moderate PPHN.
Magnesium sulphate and sildenafil have been
studied independently in the treatment of PPHN
but randomized controlled trials comparing both
are limited. Very few data are available in our
country regarding outcome and treatment of
PPHN in Bangladesh. Current evidence
indicates that different approaches such as
with sildenafil and magnesium sulphate may
reduce pulmonary pressure and improve
oxygenation in PPHN so this study was
conducted to evaluate the effect of Sildenafil
and Magnesium Sulphate in Pulmonary
Hypertension of Newborn.
Materials and Methods
This randomized clinical trial, conducted in the
Cardiac Intensive Care Unit (CICU) of Dhaka
Shishu (Children) Hospital from August 2015
to July 2017. During this period neonates
having respiratory distress and/or cyanosis
were screened. Moderate to severe PPHN were
identified among 50 neonates by
echocardiography and were randomized into
Sildenafil treatment group and Magnesium
sulphate treatment group. Informed written
consent was taken from parents. Sildenafil
treatment group was treated with oral
Sildenafil in a dose of 2 mg/kg/day in three
divided doses by nasogastric tube for 3 days. In
Magnesium Sulphate group Magnesium
sulphate was started with a loading dose of 100
mg/kg over 30 min followed by 20-50 mg/kg/h
for 5 hours for 3 days. Outcome measures
include drop of pulmonary vascular resistance
measured by right ventricular systolic
pressure, increase PaO2 and time interval to
improve O2. Side effect was observed in the
patient and outcome was recorded. Data were
analyzed by using SPSS version 17.
Result
Among 50 PPHN patients severe PPHN were
present in 10(40%) cases in Sildenafil
treatment group and 12(48%) in Magnesium
sulphate treatment group, moderate PPHN were
Effect of Sildenafil & Magnesium Sulphate in Pulmonary Hypertension Mohammad Abdullah Al Mamun et al

present in 15(60%) cases in Sildenafil 15

treatment group and 13(52%) in Magnesium 16 13
12
sulphate treatment group (Fig.-1). Difference 14
12 10
in distribution of severity of PPHN in both
10
treatment groups were not statistically
8
significant (p>0.05). Mean age of neonates in 6
hour in Sildenafil treatment group were 4
34.36±10.38 hours and Magnesium sulphate 2
treatment group 29.12±14.26 hours. There was 0
Moderate PPHN Severe PPHN
no statistically significant difference between
mean age in two groups (p>0.05). Regarding Fig.-1 Distribution of severity of PPHN in both
baseline characteristics like sex and
treatment groups
gestational age there was also no statistical
significant difference was found between two
groups (p>0.05) [Table-I]. among them 5 were in Sildenafil treatment
Among the study population PPNH with PNA group and 8 were in Magnesium sulphate
present in 15 cases, from them 9 were in treatment group. Other cases in both groups
Sildenafil treatment group and 6 were in were PPHN with MAS, PPHN with Pneumonia,
Magnesium sulphate treatment group. PPHN PPHN with congenital diaphragmatic hernia,
with Neonatal sepsis were present in 13 cases, PPHN with CHD, PPHN with RDS [Table-II].

Table-I
Distribution of baseline characteristics of studied newborn
Baseline characteristics Sildenafil Magnesium sulphate p value
treatment group treatment group
(n=25) (n=25)
Age in hour (mean ± SD) 34.36±10.38 29.12±14.26 0.14*
Sex 14 10 14 0.19**
11 15 11
Gestational age in week 4 2 4 0.33**
21 23 21
**Chi-square test & *independent t test

Table-II
Clinical diagnosis in both study groups PPHN
Clinical diagnosis Sildenafil treatment Magnesium sulphate
group (n=25) treatment group (n=25)
PPHN with PNA 9(61.5%) 6(38.5%)
PPHN with MAS 6(60%) 4(40%)
PPHN with Neonatal sepsis 5(38.5%) 8(61.5%)
PPHN with Pneumonia 2(40) 3(60%)
PPHN with PNA with Neonatal sepsis 2(66.67%) 1(33.33%)
PPHN with cong. diaphragmatic hernia 1(50%) 1(50%)
PPHN with CHD (PDA) 0 2(100%)
PPHN with RDS 1(100%) 0
Total 25 25

15
Paediatric Heart Journal of Bangladesh (PHJB) Volume 4, No. 1, January 2019

There was significant improvement of There was statistically significant

oxygenation in each study group from diagnosis
to 72 hour after treatment (p<0.05) [Table-III].
SpO2 at 72th hour of treatment was 96.39±1.58
in Sildenafil treatment group and 91.95±7.96
in Magnesium sulphate treatment group.
improvement of oxygenation was found in
Sildenafil group than magnesium sulphate at
72th hour of treatment (p<0.05). At 72th hour
of treatment regarding ABG & Echocardio-
graphy findings, there were no statistically
significant differences (p>0.05) [Table-IV].

Table-III
Assessment of improvement of oxygenation in each study group from diagnosis to 72th hour after
treatment
Sildenafil treatment Magnesium sulphate treatment
group(n=25) group (n=25)
SpO 2 (mean ±SD) p value SpO 2 (mean ±SD) p value*
At diagnosis 66.08±7.34 0.04 At diagnosis 64.56±7.04 0.08
At 6th hour 75.44±11.43 At 6th hour 70.72±8.49
At diagnosis 66.08±7.34 0.000 At diagnosis 64.56±7.04 0.000
At 12th hour 79.08±9.88 At 12th hour 72.84±6.37
At diagnosis 66.08±7.34 0.000 At diagnosis 64.56±7.04 0.000
At 24th hour 84.32±8.59 At 24th hour 79.12±6.53
At diagnosis 66.08±7.34 0.000 At diagnosis 64.56±7.04 0.000
At 36th hour 89.36±7.82 At 36th hour 82.64±5.83
At diagnosis 66.08±7.34 0.000 At diagnosis 64.56±7.04 0.000
At 48th hour 93.65±3.32 At 48th hour 87.80±7.91
At diagnosis 66.08±7.34 0.000 At diagnosis 64.56±7.04 0.000
At 72th hour 96.39±1.58 At 72th hour 91.95±7.96
SpO2 - Saturation of arterial oxygen, *Paired samples t test

Table-IV
Assessment of improvement at 72th hour in both study groups
Assessment at 72 hrs Sildenafil treatment Magnesium sulphate p
group (n=23) treatment group (n=21) value
SpO2 (mean ± SD) at 72th hr (mean ± SD) 96.39±1.58 91.95±7.96 0.01*
ABG at 72th hour pH (mean ± SD) 7.35±0.09 7.34±0.08 0.61*
PCO2 (mean ± SD) 30.52±7.21 32.25±5.37 0.37*
PO2 (mean ± SD) 101.29±70.23 73.77±46.88 0.13*
HCO3 (mean ± SD) 16.97±5.20 20.63±8.60 0.09*

Echocardiographic RVSP (mean ± SD) 34.65±9.88 34.51±7.60 0.94*

findings at 72 hour
SpO 2 - Saturation of arterial oxygen, ABG - Arterial blood gas analysis, RVSP - Right ventricular systolic
pressure, PDA - Patent ductus arteriosus, PFO - Patent foramen ovaly. Here in Sildenafil treatment group and
Magnessium sulphate treatment group at 72th number of patients were 23 and 21 respectively as 2 patient in
Sildenafil treatment group and 4 patients Magnessium sulphate treatment group died. *Independent t test.

16
Effect of Sildenafil & Magnesium Sulphate in Pulmonary Hypertension Mohammad Abdullah Al Mamun et al

Table-V
Assessment of comparison in improvement regarding RVSP after treatment in both groups at 72th
hour
Drug RVSP (mean ± SD) p value*
Sildenafil treatment group (n=21) At diagnosis = 53.75±7.90 0.000
At 72th hour = 34.51±7.60
Magnessium sulphate treatment group (n=23) At diagnosis = 53.03±9.20 0.000
At 72th hour = 34.69±9.88
RVSP - Right ventricular systolic pressure, Here in Sildenafil treatment group and Magnessium sulphate treatment
group at 72 th number of patients were 23 and 21 respectively as 2 patient in Sildenafil treatment group and 4
patients Magnessium sulphate treatment group died. *Paired Sample Test

Table-VI
Complication of drug among study groups
Complications Sildenafil Magnesium sulphate
treatment group (n=25) treatment group (n-25)
Hypotension - 2(8%)
Urinary retention 3(12%) 3(12%)
Altered GIT function 3(12%) 2(8%)

Table-VII
Comparison of outcome between two study groups

treatment Sildenafil Magnesium sulphate

group (n=25) treatment group (n=25) p value*
Complications Present 6 7 0.44
Absent 19 18
Time taken to improve (hours) 56.57±12.66 47.56±18.38 56.57±12.66
[mean ± SD]
Hospital Stay (days) 7.32±2.54 6.68±1.81 7.32±2.54
[mean ± SD]
Outcome Improved 23 21 0.33
Died 2 4
*Chi-square test

Statistically significant improvement was No significant statistical difference in

found at 72th hour regarding drop of right
ventricular systolic pressure (RVSP) in both
treatment groups [Table-V].
Complications were present in 6(24%) cases
in Sildenafil treatment group (urinary retention
in 3 and altered GIT function in 3 cases). In
Magnesium sulphate group complication was
present in 7(28%) cases (hypotension in 2,
urinary relation in 3 and altered GIT function
n 2 cases) (Table-VI).
comparison of complications, time taken to
improve, hospital stay and outcome between
two study groups (p>0.05) [Table-VII].
Discussion
There was significant improvement of
oxygenation in each study group from diagnosis
to 72 hour after treatment. Oxygenation was
significantly improved in Sildenafil study group
at 72th hour of treatment than Magnesium
sulphate group. Statistically significant

17
Paediatric Heart Journal of Bangladesh (PHJB)
improvement was found at 72th hour regarding
drop of right ventricular systolic pressure
(RVSP) in both treatment groups. No significant
statistical difference in comparison of
complications, time taken to improve, hospital
stay and outcome between two study groups.
Our primary outcome measure was drop in
right ventricular systolic pressure (RVSP) by
echocardiographic evaluation which drops
remarkably in both Sildenafil and Magnesium
sulphate group. Shaltouta et al11 found both
Sildenafil and Magnesium sulphate group
showed a significant improvement in their
pulmonary artery pressure at 48 hours after
therapy as compared to their baseline
measurements. Blood pressure was monitored
and hypotension was observed in two patients
(8%) in Magnesium sulphate group, but no
hypotension was found in Sildenafil group in
this study. In response to hypotension
Magnesium sulphate infusion was temporarily
discontinued and saline infusion was given.
Shaltouta et al11 reported 20% hypotension in
their study in case of Magnesium sulphate.
However, other side effects of Magnesium
sulphate (flaccidity, hypocalcaemia and GIT
disturbance) were not found in the present
study. As for safety profile, systemic reviews of
49 studies concerning the safety of Sildenafil
administration also revealed no evidence of
serious adverse events in infants. 12
Considering Sildenafil’s mechanism of action,
which decreases pulmonary arterial pressure,
concerns have been raised that Sildenafil could
cause serious systemic hypotension and severe
haemodynamic instability. So one must watch
the systemic blood pressure closely, although
this has been rarely a problem. Secondary
outcome measure was improvement of
oxygenation measured by changes in partial
pressure of oxygenation where a significant
improvement of patients’ oxygenation
parameters was noted at 72 hours which also
shown by previous studies.11,13 One Cochrane
collaboration, published in 2011 showed
improvement in oxygenation after use of
sildenafil in PPHN.14 Khorana et al15 in 2011
also reported improvement of oxygenation in
18
Volume 4, No. 1, January 2019
full term babies with the use of Sildenafil.
However, hypotension was a concern in their
study and it was not found on Sildenafil in the
present study. In a randomized clinical trial
Sildenafil was found more effective in the
treatment of PPHN than Magnesium sulphate
with regard to time to adequate clinical
response, duration of mechanical ventilation
with fewer requirements for inotropic
support. 16 This study also found that
oxygenation was more improved in Sildenafil
group. No significant statistical difference in
comparison of time taken to improve, hospital
stay and outcome between two study groups
were found in present study.
Conclusions
Sildenafil and Magnesium sulphate both are
safe and effective in improvement of
oxygenation and reduction of pulmonary
vascular resistance. Sildenafil was more
effective than Magnesium sulphate in the
treatment of PPHN regarding improvement of
oxygenation.
Acknowledgement
This study was funded by Ministry of Science
and Technology, Government of People’s
Republic of Bangladesh.
References
1. Walsh-Sukys MC, Tyson JE, Wright LL,
Bauer CR, Korones SB, Stevenson DK, et
al. Persistent pulmonary hypertension of
the newborn in the era before nitric oxide:
practice variation and outcomes.
Pediatrics. 2000;105:14-20.
2. Perez KM, Laughon M. Sildenafil in Term
and Premature Infants: A Systematic
Review. Clinical Therapeutic. 2015;37:
2598-2607.
3. Askin DF. Fetal-to-neonatal transition-
what is normal and what is not? Neonatal
Netw. 2009;28:e33-40.
4. Konduri GG, Kim UO. Advances in the
diagnosis and management of persistent
pulmonary hypertension of the newborn.
Pediatr Clin North Am. 2009;56:579-600.
5. Kinsella JP, Abman SH. Inhaled nitric
oxide therapy in children. Paediatr Respir
Rev. 2005;6:190-98.
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6. Chambers CD, Hernandez-Diaz S, Van Pulmonary Hypertension of the Newborn.

Marter LJ, Werler MM, Louik C, Jones KL, Int J Clin Pediatr. 2012;1:19-24.
et al. Selective serotonin-reuptake
12. Samiee-Zafarghandy S, Smith PB, van den
inhibitors and risk of persistent pulmonary
Anker JN. Safety of sildenafil in infants.
hypertension of the newborn. N Engl J
Pediatr Crit Care Med. 2014;15:362-68.
Med. 2006;354 :579-87.
13. Chandran S, Haqueb ME, Wickramasinghe
7. Reffelmann T, Kloner RA. Therapeutic
HT, Wint Z. Use of magnesium sulphate
potential of phosphodiesterase 5 inhibition
in severe persistent pulmonary
for cardiovascular disease. Circulation.
hypertension of the newborn. J Trop
2003;108 :239-244.
Pediatr. 2004;50:219-23.
8. Humbert M, Sitbon O, Simonneau G.
14. Shah PS, Ohlsson A. Sildenafil for
Treatment of pulmonary arterial
pulmonary hypertension in neonates.
hypertension. N Engl J Med. 2004;351:
Cochrane Database Syst Rev. 2011(8):
1425-36.
CD005494.
9. Shaltout F, Hegazy R, Aboulghar H, Motelb
15. Khorana M, Yookaseam T, Layangool T,
LA. Magnesium sulphate versus sildenafil
Kanjanapattanakul W, Paradeevisut H.
in the treatment of persistent pulmonary
Outcome of oral sildenafil therapy on
hypertension of the newborn. Int Clin
persistent pulmonary hypertension of the
Pediatr. 2012;1:19-24.
newborn at Queen Sirikit National
10. Raimondi F, Migliaro F, Capasso L. Institute of Child Health. J Med Assoc Thai.
Intravenous magnesium sulphate versus 2011;94 Suppl. 3:S64-73.
inhaled nitric oxide for moderate
16. Uslu S, Kumtepe S, Bulbul A, Comert S,
persistent pulmonary hypertension of
Bolat F, Nuhoglu A. A comparison of
newborn. A multicentre, retrospective
magnesium sulphate and sildenafil in the
study. J Trop Pediatr. 2008;54:196-99.
treatment of the newborns with persistent
11. Shaltouta F, Hegazya R, Aboulghara H, pulmonary hypertension: a randomized
Motelb LA. Magnesium Sulphate Versus controlled trial. J Trop Pediatr. 2011; 57:
Sildenafil in the Treatment of Persistent 245-50.

19
 Original Article

Right Vertical Infra-axillary Mini-incision for

Repair of Simple Congenital Heart Defects: A
Single Center Study
Mohammad Rokonujjaman1, Shaheedul Islam2, Nusrat Ghafoor3, Nowshin Siraj4, Syed
Tanvir Ahmad5, Ibrahim Khalilullah6, Abdullah Al Shoyeb7, Atiqur Rahman8, ZA
Faruquee9, Sirajul Islam10

Abstract
Background: Although a median sternotomy was considered the routine
approach for an open-heart operation, the scar was regarded as unsightly and
displeasing, and may evoke psychological distress, especially in young, female
patients.
Objectives: To evaluate the outcome and safety of the right vertical infra-axillary
mini incision (RVAI) used for the repair of ASD.
Methods: We performed a prospective observational analysis on the patients
with simple congenital heart defects (e.g. ASD) who were being operated from
December 2013 to December 2020. All the recruited patients were treated through
RVAI as per patient’s choice. A total of 50 patients were included who meet the
selection criteria’s. All varieties of ASD were managed including all sizes which
were not amenable to device closure.
Results: Mean age was 11.4±6.4 years, 18(36%) were male and 32(64%) were
female. Body weight ranged from 10 to 65 kg. Mean length of incision was
6.2±0.8 cm. Mean aortic occlusion time was 42±14 min. Separate incision was
used for aortic and inferior venous cannula insertion in 18 cases which facilitated
a comfortable working field. ASD closed directly, using autologous treated
pericardial patch or dacron patch. Mean total operation time was 4.08±0.6 hours
and mean mechanical ventilation time was 8.3±5 hours. Average ICU stay was
35.6±6 hours and total hospital stay was 7.2±0.9 days. There was no significant
blood loss. Three patients developed small bronchopleural fistula, required re-
insertion of chest drain. Only 10 patients required IV analgesics in the post-
operative period and other 40 were managed with only oral NSAIDs. One patient

1. Associate Professor & Consultant, Department of Cardiac surgery, Ibrahim Cardiac Hospital & Research Institute.
2. Associate Professor & Consultant, Department of Cardiology, Ibrahim Cardiac Hospital & Research Institute.
3. Associate Professor & Consultant, Department of Radiology, Ibrahim Cardiac Hospital & Research Institute.
4. Associate Professor & Consultant, Department of Radiology, Ibrahim Cardiac Hospital & Research Institute.
5. Assistant Professor & Associate Consultant, Department of Cardiac surgery, Ibrahim Cardiac Hospital &
Research Institute.
6. Assistant Professor & Associate Consultant, Department of Cardiac Anesthesiology, Ibrahim Cardiac Hospital
& Research Institute.
7. Registrar & Specialist, Department of Cardiac surgery, Ibrahim Cardiac Hospital & Research Institute.
8. Registrar & Specialist, Department of Cardiac surgery, Ibrahim Cardiac Hospital & Research Institute.
9. Associate Professor & Consultant, Department of Cardiac Anesthesiology, Ibrahim Cardiac Hospital & Research
Institute.
10. Professor & Senior Consultant, Department of Cardiac Anesthesiology, Ibrahim Cardiac Hospital & Research Institute.
Address of correspondence: Dr. Mohammad Rokonujjaman, Associate Professor & Consultant, Department of
Cardiac surgery, Ibrahim Cardiac Hospital & Research Institute. Cell: 01711311200, E-mail: drselim74@gmail.com
[Received: 01 Fabruary 2021 Accepted: 30 March 2021]
Right Vertical Infra-axillary Mini-incision for Repair of Simple Congenital Mohammad Rokonujjaman et al

needed re exploration, one needed conversion to median sternotomy and one

suffered from superficial skin infection. There were no operative or late
mortalities.
Conclusions: The RVAI surgical approach to simple congenital heart defects
revealed a safe procedure and could be performed with excellent cosmetic and
clinical outcomes. It provided a good alternative to the standard MSI for simple
congenital heart defects.
Keywords: Minimally invasive cardiac surgery, Right vertical infra-axillary mini-
incision, Congenital heart diseases.
Paed. Heart J of Bangladesh (PHJB) 2019;4(1):20-26

Introduction cardiography, and were operated on by the

Congenital heart surgery has constantly
developed and matured since the ûrst
intracardiac repair of an atrial septal defect by
Lewis and Varco in 1952.1 Although a median
sternotomy was considered the routine
approach for an open-heart operation, the scar
was regarded as unsightly and displeasing, and
may evoke psychological distress, especially in
young, female patients.2 Nowadays, increasing
attention is paid to the cosmetic results of
surgery. During the past decades, minimally
invasive cardiac surgery techniques, such as
a right posterolateral thoracotomy2,3, right
anterolateral (submammary) thoracotomy4,5,
partial sternotomy 6,7 and right axillary
transverse incision1,8, have been increasingly
used in both adult and pediatric patients.
In recent years, the right axillary straight
incision approach has been employed for both
paediatric and adult patients with simple
congenital heart diseases at Ibrahim Cardiac
Hospital & Research Institute. In this study,
we have explored the surgical and cosmetic
results of right vertical infra-axillary mini-
incision (RVAI) approaches for simple
congenital heart diseases.
Materials and Methods
This was a prospective observational study,
taken place in the department of cardiac
surgery, Ibrahim Cardiac Hospital & Research
Institute. Patient selection: All patients
included in this study suffered from simple
congenital heart diseases diagnosed by physical
examination, chest X-ray, electrocardiogram
and transthoracic colour Doppler echo-
same group of surgeons. From December 2013
to December 2020, 50 patients underwent
RVAIs. This study was designed for evaluating
the efûcacy and safety of the RVAI approach.
Patients or their parents were explained about
the procedure before surgery. Cases who met
the inclusion criteria were accepted for the
study. The pathology types of heart defects in
either group included 40 ostium secundum
ASD, 3 sinus venosus ASD and 7 primum ASD.
The median weight of the patients was 30 kg
(range from 10 to 65kg) and median age of the
patients was 10 years (ranged from 2 years to
27 years). Exclusion criteria’s were body weight
less than 10 kg and more than 70 kg. Presence
of other cardiac, thoracic or systemic
abnormalities, history of TB, history of pleural
or pericardial effusion, history of endocarditis
or rheumatic fever and patient not willing to
accept two incisions in case of failed procedure.
In RVAI approach, after the induction of
general anaesthesia, the patient was placed
in the left lateral decubitus position with the
right side elevated 45-60°, and the right arm
was wrapped and suspended over the head or
placed on opposite side. A vertical incision was
made on the right midaxillary line skin from
the second to the fifth ribs, and the length of
the incision was 4-5 cm in children and 8–11
cm in adults. Two sternal retractors were used
to gain a better exposure. The surgical route
was generally through the fourth intercostal
space. Pre-operative chest X-ray was used as a
guide in all cases to select the intercostal space
of entry.

21
Paediatric Heart Journal of Bangladesh (PHJB) Volume 4, No. 1, January 2019

The thymus was partially blunt dissected, and Operating time (skin to skin), CPB and aortic
the pericardium was opened 1-2 cm parallel cross clamp time, ventilation time; intensive
and anterior to the phrenic nerve. After care unit (ICU) stay, requirement of IV
heparinization, the ascending aorta was first analgesics, RE exploration, conversion to
cannulated, followed by both venae cavae, which midline incision drainage, duration of
were cannulated with a right-angled cannula. postoperative hospitalization, postoperative
Cannulating of the superior vena cava was not complications, mortality and satisfaction with
in the right atrium but in the superior vena the cosmetic results were noted.
cava. The interatrial septum was incised in
Results
the fossa ovalis and a left ventricular vent was
inserted via the incision. Separate incision In our study, a total of 50 patients were
was used for aortic and inferior venous cannula included. 18(36%) were male and 32(64%) were
insertion in 18 cases to get a wide and female. Regarding Blood group distribution, 18
comfortable working field. Under a mildly patients were A+ve, 11 patients B+ve, 10
hypothermic (32°C) cardiopulmonary bypass patients O+ve, 3 patients AB+ve, 2 patients B-
(CPB), the ascending aorta was cross clamped, ve, 3 patients O Negative, 1 patient A–ve and 2
and cardioplegia was achieved by infusing a patients AB–ve. The median weight of the
cold blood, Delnido cardioplegic solution into the patients was 30 kg (range from 10 to 65 kg).
ascending aorta root. A right atriotomy was Mean body weight was 31.8±13.8 kg. Median
performed for the closure of the heart defect, age of the patients was 10 years (ranged from
and according to the size of the defect, closure
2 years to 27 years). Mean age was 11.4±6.4
was by direct suture, using a Dacron patch or
autologous gluteraldehyde treated pericardium. years and mean BSA was 1.01±.29 m2. 40
For the correction of sinus venosus atrial septal patients had secundum , 7 patients had primum
defects, a double-patch technique was used. The ASD and only 3 patients had sinus venosus ASD
incision of the right atriotomy was extended to in the series. Mean area of ASD was 4.8±3.4
the superior vena cava (inclined to right side), cm2 (Table-I).
and then the ASD was closed with a patch (the
defect was expanded when necessary) and the
right pulmonary vein was separated to the left Table-I
atrium. An autologous pericardial patch was Base line characteristics of study population
used to close the incision from the superior
vena cava to the anterior wall of the right Variable Mean±SD No(%)
atrium. After the air in the heart was Age (yrs) 11.4±6.4
eliminated and the cross-clamp was removed,
Sex
the patient was rewarmed and CPB was
discontinued gradually. A right pleural drain Male 20(40)
was inserted and the thorax closed in layers. Female 30(60)
In patients with PLSVC an extra sucker was Body weight (kg) 31.8±13.8
inserted to the coronary sinus for blood less PASP (mm Hg) 32.18±14
field. CO2 was in all cases for deairation.
BSA (m2) 1.01±0.29
Congenital Type of ASD
After discharge, clinical data and colour Doppler Primum 7(14)
echocardiography were evaluated after 3 Secundum 40(80)
month, 6 months and yearly thereafter, and
Sinusvenosus 3(6)
special regard was given to the cosmetic results
of the scar. Each patient or their parents Area of ASD(cm2) 4.8±3.4
completed a self- designed questionnaire,
which included only three simple questions, Mean length of incision was 6.2±0.8 cm. Mean
used to investigate the satisfaction with aortic occlusion time was 42±14 min. Mean
cosmetic results. CPB time was 100±29 min. 25 patients had mild

22
Right Vertical Infra-axillary Mini-incision for Repair of Simple Congenital Mohammad Rokonujjaman et al

pulmonary hypertension, 14 patients had incision and recovered without any

moderate PAH and rest 11 patients had severe complication. Superficial skin infection
PAH. Mean PASP was 32.18±14 mm Hg. Delnido occurred in one case and recovered normally
cold blood cardioplegia was used in all the cases. (Table-III).
Two patients required retrograde cardioplegia.
Eight patients had PLSVC opening to the right Table-III
atrium. Maximum patients cooled down upto Post-operative issues and complications
320C and one patient required to cool down to
Variable No(%)
240C and very low flow to get IVC margin in
view properly. In this case IVC cannula was Blood transfusion Yes 6(12)
removed for some time. No 44(88)
Separate incision was used for aortic and Re exploration Yes 1(2)
inferior venous cannula insertion in 18 cases No 49(98)
which facilitated a comfortable working field.
Requirement of IV analgesics Yes 10(20)
4th intercostal space was used for entry of
pleural cavity in 42 cases and 3rd space in eight No 40(80)
cases. ASD closed directly in 4 cases as those Convertion Yes 1(2)
were small. 22 cases closed with autologous No 49(98)
treated pericardial patch and rest 24 cases
Infection Yes 1(2)
were closed with Dacron patch. But all 7
primum, 3 sinus venosus and 12 secundum No 49(98)
ASD were closed with treated autologous Mortality Yes 1(2)
pericardiam. No 49(98)
Mean total operation time was 4.08±0.6 hours. CD removal (hours) Mean±SD 32.3±19
Mean mechanical ventilation time was 8.3±5
hours (Table-II). Average ICU stay was 35.6±6 hours. Mean total
hospital stay was 7.2±0.9 days. There were no
operative or late mortalities and no special care
Table-II
in the intensive care unit (ICU) or re
Operative variable hospitalization. Mean duration of return to
Variable Mean±SD normal life was 39±7.8 days. No significant
residual defects were found in followup. No
Aortic Occlusion time (min) 42±14
asymmetrical development of the breast,
ECCT(min) 100±29
thoracic deformity or scoliosis has been found
Total operation time (hours) 4.08±0.6 during the follow-up. All the patients or the
Mechanical Ventilation time (hours) 8.3±5 parents of young children (100%) were satisfied
with the cosmetic results (Table-IV). Even the
Mean blood loss was 182±118 ml and blood midline conversion patient was also satisfied
transfusion required in 6(12%) cases. because our team has tried for them.
Intercostal tube was removed in 32.3±10 hours. Table-IV
3 patients developed small bronchopleural Outcome variable
fistula and required re insertion of chest drain.
Only 10 patients required IV analgesics in the Variable Mean±SD
post-operative period and other 40 were Total ICU stay (hours) 35.6±6
managed with only oral NSAIDs. Due to Total hospital stay (days) 7.2±0.9
excessive blood loss re exploration was done in
one case and revealed bleeding from a small Length of Incision (cm) 6.2±0.8
branch of intercostal artery. It was also Time to return to normal 39±7.8
managed through axillary incision. Conversion activity (days)
to midline incision required in one case due
to failed aortic cannulation through axillary Satisfaction 100%

23
Paediatric Heart Journal of Bangladesh (PHJB)
Fig.-1 A clinical picture of a child 3 months after surgical repair of ASD
Discussion
Median sternotomy has been the standard
approach used to correct non-complex
congenital heart defects with excellent effects.1
However, deep sternal wound infection, which
contributes to signiûcant morbidity, mortality
and increased medical cost, is a rare but
worrisome complication with this approach and
remains a major challenge to cardiac
surgeons. 9,10 Besides, it is much more
important that the mid-sternotomy scar is a
timeless reminder of a ‘heart disease’ for the
patients and may evoke psychological distress.2
It is beyond all doubt that the cosmetic result
was crucial for the patients who underwent
closure of simple congenital heart defects. Non
surgical percutaneous transcatheter closure
of simple congenital heart defects has been
used for more than 20 years and has been
24
Volume 4, No. 1, January 2019
performed with excellent cosmetic results.11
However, its safety still remains in debates
because it might induce significant
complications such as device malposition,
thrombosis, embolization, arrhythmias, cardiac
perforation, haemopericardium with
tamponade, signiûcant residual shunt, atrial
and/or aortic injury or erosion.12-14
Some of them require surgical treatment,
which would lead to greater operative mor-
tality than that of primary surgical ASD
closure. 14 Both right anterolateral
minithoractomy and partial sternotomy can
prevent femoral cannulation and be performed
with acceptable clinical results. But the former
might induce thoracic deformity or
asymmetrical development of the breast,15 and
the latter is not popular because of the midline
scar, which would prevent some children from
Right Vertical Infra-axillary Mini-incision for Repair of Simple Congenital
going swimming for fear of others’ stares and
sympathy.1
There were several advantages with the RVAI
approach. First of all, the cosmetic result was
excellent. The vertical incision of RVAI located
on the right midaxillary line was small and in
conspicuous (Fig-1). During the follow-up, all
patients in our study group were satisfied with
the cosmetic and no asymmetrical
development of the breast, thoracic deformity
or scoliosis has been found. It is worth noting
that all the patients or the parents of young
children, who underwent median sternotomy,
told us that, if they had a chance to choose
again, they would rather choose the RVAI
approach. Furthermore, the surgical route
through the fourth intercostal space and
appropriate pericardial suspension provide
enough exposure to the ascending aorta, both
venae cavae and the operating field of closure
of the ASD through a right atriotomy. With
neither operative nor late mortality, nor
neither ICU nor hospital readmission in the
RVAI group, and no significant residual defects
were found during follow-up. The RVAI
procedure did not increase the con- suming
time for CPB, aortic cross-clamp and hospital
stay. Besides, the RVAI procedure did not need
femoral cannulation and could prevent femoral
artery stenosis in the future, and it not require
any specialized or expensive equipment.
It was worth emphasizing that the safety of the
RVAI procedure was based on experienced
cardiac surgeons, successful cannulation,
smooth CPB, effectual myocardial preservation
and choosing suitable cases. In this study, all
VSDs were perimembra-neous. Due to poor
exposure of the operating field with the RVAI
approach, subpulmonic ventricular septal
defects and muscular ventricular septal
defects were still repaired with median
sternotomy in our centre. The weight of the
patients in the RVAI group ranged from 10 to
65 kg.
Although all the patients were satisfied with
the cosmetic results, the RVAI approach was
more suitable for the patients with a body
weight <30 kg according to our experience, and
was not suitable for the patients with body mass
Mohammad Rokonujjaman et al
index ³30 kg/m2 .16 Because of the deeper
thoracic cavity, the exposure of the operating
field would be poorer.
Complications included small bronchopleural
fistula in three cases which was treated with
thoracentesis. Although all three patients were
cured and discharged, we should take
particular care of the right pleural drain and
the right lung of the patients after the RVAI
procedure.
Our study may have potential limitations. As
an observational prospective study and a single-
institution survey, it needs to be conûrmed by
expanding the sample and multicenter trials.
Shorter followup time is a major limitation of
the study. Infact most of the patients were not
interested for followup as they became
asymptomatic after 3 months. In addition, the
lack of data about the level of pain was another
limitation.
Conclusion
The RVAI surgical procedure for simple
congenital heart defects is a safe procedure
with excellent cosmetic and clinical results,
conferring psychological and social satisfaction
upon patients. It is a good alternative to
standard median sternotomy for all ASDs which
are not amenable to device closure. Even rare
blood group patients can be managed safely
with this technique. Of course learning curve
is a major deal of the technique.
Conflict of interest: None declared
References
1. Dave HH, Comber M, Solinger T, Bettex D,
Dodge-Khatami A, Prêtre R. Mid-term
results of right axillary incision for the
repair of a wide range of congenital cardiac
defects. Eur J Cardiothorac Surg. 2009;
35:864-70.
2. Mohamed KS. Minimally invasive right
posterior minithoracotomy for open-heart
procedures. Asian Cardiovasc Thorac Ann.
2007;15:468-71.
3. Yoshimura N, Yamaguchi M, Oshima Y,
Oka S, Ootaki Y, Yoshida M. Repair of atrial
septal defect through a right posterolateral
thoracotomy: a cosmetic approach for
25
Paediatric Heart Journal of Bangladesh (PHJB)
female patients. Ann Thorac Surg. 2001;
72:2103-05.
4. Däbritz S, Sachweh J, Walter M, Messmer
BJ. Closure of atrial septal defects via
limited right anterolateral thoracotomy as
a minimal invasive approach in female
patients. Eur J Cardiothorac Surg 1999;
15:18-23.
5. Formigari R, Di Donato RM, Mazzera E,
Carotti A, Rinelli G, Parisi F, et al.
Minimally invasive or interventional
repair of atrial septal defects in chil- dren:
experience in 171 cases and comparison
with conventional strat- egies. J Am Coll
Cardiol. 2001;37:1707-12.
6. Nicholson IA, Bichell DP, Bacha EA, del
Nido PJ. Minimal sternotomy ap- proach
for congenital heart operations. Ann
Thorac Surg. 2001;71:469-72.
7. Seipelt RG, Popov A, Danner B, Paul T,
Tirilomis T, Schoendube FA, et al.
Minimally invasive partial inferior
sternotomy for congenital heart defects
in children. J Cardiovasc Surg. 2010;
51:929-33.
8. Gil-Jaurena JM, Zabala J-I, Conejo L,
Cuenca V, Picazo B, Jiménez C, et al.
Minimally invasive pediatric cardiac
surgery. Atrial septal defect closure
through axillary and submammary
approaches. Rev Esp Cardiol (English
Edition). 2011;64:208-12.
9. Baillot R, Cloutier D, Montalin L, Côté L,
Lellouche F, Houde C, et al. Impact of deep
sternal wound infection management with
vacuum- assisted closure therapy followed
by sternal osteosynthesis: a 15-year
review of 23499 sternotomies. Eur J
Cardiothorac Surg. 2010;37:880-87.
10. Graf K, Ott E, Vonberg RP, Kuehn C,
Haverich A, Chaberny IF. Economic
26
Volume 4, No. 1, January 2019
aspects of deep sternal wound infections.
Eur J Cardiothorac Surg. 2010; 37:893-96.
11. Rao PS, Sideris EB, Chopra PS. Catheter
closure of atrial septal defect- successful
use in a 3.6 kg infant. Am Heart J. 1991;
121:1826-29.
12. Berdat PA, Chatterjee T, Pfammatter J-P,
Windecker S, Meier B, Carrel T. Surgical
management of complications after
transcatheter closure of an atrial septal
defect or patent foramen ovale. J Thorac
Cardiovasc Surg. 2000;120:1034-39.
13. Chessa M, Carminati M, Butera G, Bini
RM, Drago M, Rosti L, et al. Early and late
complications associated with
transcatheter occlusion of secun- dum
atrial septal defect. J Am Coll Cardiol. 2002;
39:1061-65.
14. Sarris GE, Kirvassilis G, Zavaropoulos P,
Belli E, Berggren H, Carrel T, et al. Surgery
for complications of trans-catheter closure
of atrial septal defects: a multi-
institutional study from the European
Congenital Heart Surgeons Association.
Eur J Cardiothorac Surg. 2010; 37: 1285-
90.
15. Bleiziffer S, Schreiber C, Burgkart R,
Regenfelder F, Kostolny M, Libera P, et al.
The inûuence of right anterolateral
thoracotomy in prepubescent female
patients on late breast development and
on the incidence of scoliosis. J Thorac
Cardiovasc Surg. 2004;127:1474-80.
16. Li Q-g, Wang Q, Wang D-j. The right
vertical infra-axillary incision for mitral
valve replacement. J Cardiothorac Surg.
2010;5:104.
17. Refai M, Brunelli A, Salati M, Pompili C,
Xiumè F, Sabbatini A. Efûcacy of anterior
ûssureless technique for right upper
lobectomies: a case- matched analysis. Eur
J Cardiothorac Surg. 2011;39:1043-46.
 Original Article
Various Echocardiographic Presentation of TGA
in Outdoor Settings: A Single Center Experience
Tahmina Karim1, Mohd. Zahid Hussain2, Md. Tariqul Islam3, Kashid Omar4, Diana Islam5

Abstract
Background: Transposition of great arteries (TGA) is the second most common
cyanotic heart disease of paediatric age group. Wide range of cardiac defects
and anomalies are associated with TGA. Echocardiography remains the most
confirmatory tool for understanding and detecting the various presentations of
this cyanotic heart disease.
Objectives: This study was conducted to enlist the various echocardiographic
findings of TGA cases.
Methods: It is a retrospective study. Total 20 patients of TGA who were diagnosed
by echocardiography at outpatient settings of paediatric cardiology department,
BSMMU were enrolled in this study. Their Echo findings were analyzed.
Results: Among 20 patients 18 cases were D-TGA and 2 cases were Cc-TGA.
VSD was the commonest lesion (65%) with muscular (39%) and sub pulmonic
(31%) variety along with ASD (25%), PDA (20%) and coronary artery anomalies
(15%).Aorta was right and anterior of PA in most cases (75%) with directly
anterior (20%) and side by side relationship (5%). Both RVOTO (10%) and LVOTO
(5%) were present. Some rare conditions like DORV (10%), Single ventricle (10%),
Right aortic arch (10%) were also found.
Conclusion: D-TGA is more common than Cc-TGA with strong male
predominance. Ventricular septal defect is the commonest association for TGA
along with ASD, PDA and coronary artery anomalies with muscular and
subpulmonic VSD is the majority subtype. RVOTO and LVOTO both can
complicate TGA. It can occur as a integral part of lesions like DORV and single
ventricle also.
Keywords: Echocardiographic presentation, TGA.
Paed. Heart J of Bangladesh (PHJB) 2019;4(1):27-31

Introduction lesion after tetralogy of Fallot and affects males

Transposition of the great arteries (TGA) is a
conotruncal anomaly where the ventriculo-
arterial connections are discordant; the aorta
aligns with the right ventricle (RV) and the
pulmonary artery aligns with the left ventricle.
TGA is the second most common cyanotic
more than females.1
When there are concordant atrioventricular
connections, TGA results in severe cyanosis
because the circulation is in parallel rather
than in series; the deoxygenated blood that
returns from the systemic veins goes directly

1. Associate Professor, Paediatric Cardiology, BSMMU, Shahbag, Dhaka.

2. Professor of Paediatric Cardiology, BSMMU, Shahbag, Dhaka.
3. Professor, Paediatric Cardiology, BSMMU, Shahbag, Dhaka.
4. Resident Medical Officer, Paediatric Cardiology, BSMMU, Shahbag, Dhaka.
5. Medical Officer, Paediatric Cardiology BSMMU, Shahbag, Dhaka.
Address of correspondence: Dr. Tahmina Karim, Associate Professor, Paediatric Cardiology, BSMMU, Shahbag,
Dhaka. Cell: 01714277272, E-mail: dr.tkarim@yahoo.com
[Received: 02 March 2021 Accepted: 29 April 2020]
Paediatric Heart Journal of Bangladesh (PHJB) Volume 4, No. 1, January 2019

out the aorta and the oxygenated blood that 10 (10%)

returns from the pulmonary veins goes directly
out of the pulmonary artery.2
TAG
Echocardiographic assessment of patients with
transposition of the great arteries (TGA) 90 (90%)
D-TGA
requires a good understanding of the
morphology of defects with their commonly
associated lesions. Echocardiography reveals
origin of great vessels and their relationship, Fig.-1 Pie chart D-TGA vs Cc-TGA
the origin of coronary artery, presence of other
associated lesions like VSD, ASD etc. and also Among total 20 patient only 2 patient (10%)
presence of any obstruction in the form of were diagnosed as congenitally corrected TGA.
LVOTO.1-3 Remaining 18 patients (90%) were diagnosed
as D-TGA (Fig.-1).
Materials and Methods
It was a retrospective study conducted from Relation of Aorta and Pulmonary
January 2018 to June, 2019 total 20 patients,
at paediatric cardiology department of BSMMU.
Patient who has any form of Transposition of
great arteries in their Echocardiography
findings were included in this study and their
Echocardiographic findings were analyzed then
detailed echocardiographic data including
origin of aorta and pulmonary trunk and their
Right anterior Direct anterior Side by side
relationship; presence of VSD, ASD, PDA;
Series 1 75% 20% 5%
anatomy and origin of coronary artery; presence
of any LVOTO were recorded. Continuous Fig.-2 Relation of Aorta and Pulmonary trunk
variables were expressed as mean, median and
standard deviation, while categorical variables Most of the cases (15,75%) showed that aorta
were expressed as numbers and percentages. is right and anterior of PA. Four cases (20%)
The SPSS 22.0 for Windows was used for the resembled directly anterior aorta and only one
statistical computations. case (5%) possesed side by side relation (Fig.-2).

Result 65%
Among total 20 cases mean age was 100.09±
90.8 days and median age was 50 day. Mean
weight for them was 5.1± 2.05 kg. Male patients
were more in number which was 16 (80%) with
a male female ratio 4:1 (Table-I). 25%
20%
15%
10% 10% 10% 10%
Table-I 5%

Demographic data (n=20) VSD ASD PDA CAA DORV SVS PS Rt. Arch LVOTO
Age
Mean age (days) 100.09± 90.8 Fig.-3 Associated anomalies (CAA=Coronary
Median age (day) 50 artery anomalies, SV=Single Ventricle)
Mean Weight (kg) 5.1± 2.05
Regarding other associated anomalies VSD (13
Sex
cases 65%) was the most common defect.
Male 16(80%)
Subsequently ASD (5 cases, 25%), PDA (4 cases,
Female 4(20%) 20%) and Coronary artery anomalies (3 cases,

28
Various echocardiographic presentation of TGA in outdoor settings
15%) were found. Some other less common
anomalies such as DORV (2 cases, 10%), Single
Ventricle (2 cases,10%) and right aortic arch
(2cases, 10%) were also present. Severe
Pulmonary stenosis was a major finding in 2
cases (10%). LVOTO was present in only one
case (5%) (Fig.-3).
15 15
Malaligned
Muscular
Subpulmonic
31
PM
39
Fig.-4: Type of VSD
Among the 13 cases which showed TGA with
VSD most of the cases (5 case, 39%) were
Muscular VSD. Next common type was Sub
pulmonic VSD (4 case, 31%). Other types found
were PM VSD and Malaligned VSD both 2 cases
(15%) (Fig 4).
Discussion
The diagnosis of TGA is established by
demonstrating normal atrial situs,
atrioventricular alignments, and ventricular
looping, in association with ventrículoarterial
discordance. The aorta arises from the right
ventricle, while the pulmonary artery arises
from the left ventricle. In most cases, the aortic
valve is anterior and to the right of the
pulmonary valve, and there is character-
istically fibrous continuity between the
pulmonary and mitral valve. With this
fundamental anatomy established, the
sonographer can then proceed to evaluate
additional key anatomical features such as the
presence or absence of VSD(s), coronary artery
pattern, outflow tract and semilunar valve
anatomy, and aortic arch anatomy.1 Regarding
prevalence of TGA most commonly D-TGA is
found and Cc-TGA is relatively uncommon
condition.2 In this study we found similar result
Tahmina Karim et al
was seen. Among 20 patients D-TGA was
diagnosed in 18 patient which is 90% of total
case and only two patient of Cc-TGA were
diagnosed in the study period.
Pasquini et al 3 stated that Important
morphometric features of TGA include the
relationship of the aorta and the pulmonary
artery. In contrast to the normal heart, the
great arteries in TGA arise in parallel rather
than spiral around each other. Most typically,
the aorta sits anterior and rightward of the
pulmonary artery; however, the great arteries
may be side by side or directly anterior/
posterior to each other.4-6 In this study we also
found similar results with Most of the cases
(15, 75%) showed that aorta is right and
anterior of PA. Four cases (20%) resembled
directly anterior aorta and only one case (5%)
possesed side by side relation.
Many associated cardiac lesions were described
in relation with TGA. Most common defect is
VSD upto 35-40% of cases.2 In this study we
also found that VSD is the most frequent defect
in association with TGA as it occurred in 65%
of our cases (13 cases). Regarding VSD type
Lopez et al6 found muscular, perimembranous
and malaligned VSDs are most common variant.
Also In this study Muscular VSDs are more
commonly detected (5 cases, 39%) along with
Subpulmonic VSD (4 cases, 31%) and two cases
of PM VSD. Malalignment type VSDs are outlet
defects where the conal (outlet) septum is out
of its normal alignment with the muscular
ventricular septum.7 In this study two cases
(15%) of malaligned VSD were associated with
TGA cases.
Other Left to right shunt lesions including ASD
and PDA is also found in association of TGA. In
this study we found 5 cases of ASD (25%) and 4
cases of PDA (20%) in echocardiography. Yacoub
et al5 found coronary artery anomalies in upto
16% of TGA patients. In this study we found
similar results as in 3(15%) among 20 patients
abnormal coronary artery origin were detected
during echocardiography. Han Pil Lee et al8
described a case report showing a patient
having DORV and TGA. We also found two cases
of DORV and TGA in this study. Some other
rare conditions such as Single ventricle (2
29
Paediatric Heart Journal of Bangladesh (PHJB)
cases,10%) and right aortic arch (2 cases,10%)
were also reported.
Rastelli et al and Nikaidoh et al described TGA
cases with severe pulmonary stenosis and
their procedure of correction.9,10 Anterior and/
or rightward malalignment of the conal septum
typically results in RV outflow tract obstruction.
In this study we also found two cases (10%) of
severe PS in TGA cases.
Another important feature is LV outflow tract
obstruction which can occur in 10% of patients
with TGA. There are multiple possible causes
including ventricular septal thickening, fibrous
membranes, aneurysm of the membranous
septum, conal septum malalignment and valvar
pulmonary stenosis.11-14 We found only one case
where LVOTO was present.
Conclusion
D-TGA is more common than Cc-TGA with
strong male predominance. Ventricular septal
defect is the commonest association for TGA
along with ASD, PDA and coronary artery
anomalies with muscular and sub pulmonic
VSD is the majority subtype. RVOTO and LVOTO
both can complicate TGA. It can occur as a
integral part of lesions like DORV and single
ventricle also.
References
1. Cohen MS, Mertens LL. Echo-
cardiographic assessment of transposition
of the great arteries and congenitally
corrected transposition of the great
arteries Echo research and practice.
2019;6:107-19.
2. Mertens LL, Vogt MO, Marek J, Cohen MS.
Transposition of the great arteries.
Echocardiography in Pediatric and
Congenital Heart Disease: From Fetus to
Adult. 2nd ed. 2015; pp 398-416.
3. Pasquini L, Sanders SP, Parness IA, Colan
SD, Van Praagh S, Mayer Jr JE, et al. Conal
anatomy in 119 patients with d-loop
transposition of the great arteries and
ventricular septal defect: an
echocardiographic and pathologic study.
30
Volume 4, No. 1, January 2019
Journal of the American College of
Cardiology. 1993;21:1712-21.
4. Van Praagh R, Van Praagh S. Isolated
ventricular inversion. A consideration of
the morphogenesis, definition and
diagnosis of nontransposed and transposed
great arteries. Am J Cardiol. 1966;17:395-
406.
5. Anderson RH, Weinberg PM. The clinical
anatomy of transposition. Cardiol Young.
2005;15(Suppl 1):76-87.
6. Pasquini L, Sanders SP, Parness IA.
Coronary echocardiography in 406 patients
with d-loop transposition of the great
arteries. J Am Coll Cardiol. 1994;24:
763-68.
7. Yacoub MH, Radley-Smith R. Anatomy of
the coronary arteries in transposition of
the great arteries and methods for their
transfer in anatomical correction. Thorax.
1978; 33:418-24.
8. Lee HP, Bang JH. Aortic Root Translocation
with Arterial Switch for Transposition of
the Great Arteries or Double Outlet Right
Ventricle with Ventricular Septal Defect
and Pulmonary Stenosis. Korean J Thorac
Cardiovasc Surg. 2016; 49:190-94.
9. Rastelli GC, Wallace RB, Ongley PA.
Complete repair of transposition of the
great arteries with pulmonary stenosis.
A review and report of a case corrected by
using a new surgical technique.
Circulation. 1969;39:83-95.
10. Nikaidoh H. Aortic translocation and
biventricular outflow tract reconstruction.
A new surgical repair for transposition
of the great arteries associated with
ventricular septal defect and pulmonary
stenosis. J Thorac Cardiovasc Surg.
1984;88:365-72.
11. Rudolph AM. Transposition of the Great
Arteries (Aortopulmonary Transposition).
Congenital Diseases of the Heart: Clinical-
Physiological Considerations. 2nd ed.
2001; pp 675-736.
Various echocardiographic presentation of TGA in outdoor settings Tahmina Karim et al

12. Kumar B, Jayant A, Munirathinam GK,
Mahajan S. Tricuspid valve straddling: an
uncommon cause of left ventricular
outflow tract obstruction in transposition
of great artery with ventricular septal
14. Helvind MH, McCarthy JF, Imamura M.
defect. Annals of Cardiac Anaesthesia.
2018;21:61-64.
13. Chin AJ, Yeager SB, Sanders SP. Accuracy
of prospective two dimensional echocardio-
graphic evaluation of the left ventricular
outflow tract in complete transposition of
the great arteries. Am J Cardiol. 1985;
55:759-64.
Ventriculo-arterial discordance: switching
the morphologically left ventricle into the
systemic circulation after 3 months of age.
Eur J Cardiothoracic Surg. 1998;14:173-78.

31
 Review Article
A Compendium Review on Paediatric Cyanotic
Nephropathy
Azmeri Sultana1, Syed Saimul Hoque2, Jubaida Rumana3, Shahbuddin Mahmud4,
Laila Sharmin5, Md. Abdul Qader6, Ranjit Ranjan Roy7, Mohammed Hanif8

Abstract
Children with congenital heart disease may cause potential development of
glomerulopathy and tubulopathy due to pathophysiological changes related to
a structurally abnormal heart and circulation. Nephrotic range of proteinuria is
a rare but important complication. Even children may suffer from chronic kidney
disease, which has an adverse impact on health outcomes. Unfortunately, this
issue is addressed by very few studies. Therefore, it is crucial to consider that
patients with congenital heart disease represent renal involvement, take an
account, and prevention strategies to reduce negative outcomes. This review
aims to discuss the prevalence of cyanotic nephropathy, it’s diagnosis, and
treatment in children with cyanotic congenital heart disease.
Keywords: Cyanotic nephropathy, congenital cyanotic heart disease.
Paed. Heart J of Bangladesh (PHJB) 2019;4(1):32-38

Introduction proteinuria is a serious complication of

Congenital heart disease (CHD) is the
commonest type of congenital anomaly among
all birth defects, affecting approximately 1% of
live births. One out of four children with
congenital heart disease requires cardiac
surgery.1 Since 1960, it has been known that
between 30 and 50% of cyanotic heart diseases
can set off secondary glomerulopathy known
as cyanotic nephropathy. 2,3 It is also
established that the preliminary damage is in
the tubule, especially in the proximal tubule,
as reflected by an elevated urinary N-acetyl
glucosamine and a1-microglobulin that
typically occurs in the first decade of life and
continue in the next decade with a glomerular
injury (albuminuria, proteinuria, and decrease
in glomerular filtration).4,5 Cyanotic CHD can
be presented with protein leakage resulting
from glomerular injury especially in older
children and adults. Nephrotic range of
cyanotic CHD.6,7 Understanding this, the goal
of the present review is to determine the
prevalence, pathogenesis, and treatment
outcome of cyanotic nephropathy in children.
Protienuria and Glomerulopathy
Among all CHD, cyanotic heart disease can be
presented with protein leak resulting from
glomerular injury especially in older children
and adults. It is termed cyanotic nephropathy
(CN) almost seen in 30-50% of patients with
congenital cyanotic heart disease (CCHD),
which affects both tubular and glomerular
function, resulting in proteinuria and
azotemia.8 Nephrotic range of proteinuria is a
significant complication of cyanotic congenital
heart disease.9,10 Histopathologic findings
revealed glomerular enlargement, capillary
dilatation, capillary thickening, focal or diffuse
mesangial proliferation, and segmental or global

1. Associate Professor of Paediatric Nephrology, Dr. MR Khan Shishu Hospital & Institute of Child Health, Dhaka.
2. Associate Professor of Paediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka.
3. Associate Consultant, Asgor Ali Hospital, Dhaka.
4. Assistant Professor of Paediatrics, Shaheed Suhrawardy Medical College, Dhaka.
5. Assistant Professor of Paediatrics, Rajshahi Medical College, Rajshahi.
6. Associate Consultant, Paediatric Nephrology, Square Hospital Limited, Dhaka.
7. Professor of Paediatric Nephrology, Banagabandhu Sheikh Mujib Medical Unuversity, Dhaka.
8. Professor of Paediatric Nephrology, Dhaka Shishu (Children) Hospital, Dhaka.
Address of correspondence: Dr. Azmeri Sultana, Associate Professor of Paediatric Nephrology, Dr. MR Khan
Shishu Hospital & Institute of Child Health, Dhaka. Cell: +8801972817777, E-mail: jhilni_me@yahoo.com
[Received: 04 Fabruary 2020 Accepted: 28 April 2021]
A compendium review on pediatric cyanotic nephropathy
glomerulosclerosis.11 Although proteinuria is
the foremost urinary abnormality in patients
with congenital cyanotic heart disease,
nephrotic syndrome is a rare complication and
renal biopsy has been seldom performed.12 The
risk of developing renal impairment is high in
cyanotic patients particularly in patients with
long-standing cyanotic congenital heart
disease.9,10
Prevalence
Hongsawong et al13 showed in their prospective
study that prevalence of cyanotic nephropathy
(CN) in CCHD is 92.55% according to
proteinuria. High hematocrit (>40%) and
platelet count <2,90,000/mm 3 are early
predictors of developing microalbuminuria
whereas early corrective surgery decreased
the risk of developing CN.
Pathogenesis
The occurrence of proteinuria related to
cyanotic CHD though remains indistinct, a
large number of reports have described potential
Cyanotic heart disease
Angiogenesis Polycythemia
Endothelial proliferation Hyperviscosity
Podocyte hypertrophy Decrease renal BF & FF
Decrease tubular
Podocyte Injury
capillary Flow
Minimal Change GN Glomerular hyperfiltration
FSGS Glomerulosclerosis
Fig-1 Pathophysiology of cyanotic nephropathy
FSGS: focal segmental glomerulosclerosis; BF: blood flow; FF: Filtration fraction; PCT: Proximal convoluted
tubule; GN: glomerulonephritis; GLM: Glomerulous; NO: nitric oxide; VD: vasodialation; GHF: glomerular
hyperfiltration.
Azmeri Sultana et al
mechanisms. Patients with cyanotic CHD are
exposed to chronic hypoxic glomerular injury.
The risk of developing glomerular lesions rose
sharply during the second decade of life if the
cyanosis remains unchanged for more than ten
years.14,15 Polycythemia set off hyperviscosity
which induces an angiogenic increase in the
glomerular capillary beds, in turn leading to
glomerulomegaly. Glomerulomegaly is a
consequence of the hyperperfusion of Glomeruli
associated with chronic hypoxia and the
increased hydrostatic pressure in the capillary
wall. This situation act as a causative factor
for the deterioration and the decline of renal
function, in the condition of polycythemia.
Furthermore, the failure of a compensatory
mechanism to respond to reduced renal blood
flow by hyperfiltration may be accompanied by
the development and progression of
microalbuminuria and proteinuria (Fig-1).16,17
Hypoxia, raised hematocrit, and polycythemia
may be contributory factors for glomerulopathy
in congenital cyanotic heart disease, but it is
Lesion in PCT NO release
Afferent arteriolar VD
Interstisial fibrosis
GLM increase
capillary pressure
Renal insufficiency
GHF
33
Paediatric Heart Journal of Bangladesh (PHJB)
not always necessary conditions. Some studies
linked cardiac defect with steroid-resistant
nephrotic syndrome by mutation of NPHS2 gene
encoding podocin.18,19
Investigation
Renal function may be impaired in cyanotic
nephropathy. There are many cases reported
renal impairment in children with cyanotic
heart disease. Vrushank et al reported a case
from India where they found blood urea nitrogen
18.92 mmol/L, serum creatinine 300.56
micromole/L, and eGFR 16.28ml/min/m2.20
Another two studies reported the same
findings.21,22 In contrast to this Azmeri et al
reported two cases where they found normal
renal function in cyanotic heart disease.23
Proteinuria is a solemn consequence of long-
standing cyanosis. Few case reports showed
mild to nephrotic range proteinuria in children
with cyanotic heart disease. They also found
normal cholesterol and normal S.albumin to
mild hypoalbuminemia with urine routine
microscopic examination revealed Albumin+
to +++. Urinary spot protein creatinine ratio
ranges from mild to nephrotic range of
proteinuria.21-23
Renal biopsy findings in congenital cyanotic
heart disease with proteinuria showed light
microscopic findings were glomerulomegaly,
with 12 glomeruli 3 had global sclerosis and 3
had focal segmental glomerular sclerosis.
Electron microscopy showed segmental
hyalinosis of glomeruli and some podocyte
hypertrophy. Immunofluorescence imaging
revealed focal segmental deposition of IgM and
C3 in the mesangium.24,25 Another particular
concern of using contrast during cardiac
catheterization and renal evaluation. A study
showed the use of a non-ionic contrast medium
does not deteriorate cyanotic glomerulopathy.
This may had happened as use of non ionic
contrast medium reduces blood viscosity.
However careful renal monitoring is advised
after cardiac catheterization.
Treatment
The treatment consists of early surgical
correction of heart disease, periodic
phlebotomies, and inhibition of the renin–
34
Volume 4, No. 1, January 2019
angiotensin-aldosterone axis with ACE
inhibitors.26 ACE inhibitor (Enalapril) which
seemed to be effective for reducing proteinuria
may be glomerular hyperfiltration was probably
the most crucial contributory factor for
proteinuria. In a retrospective analysis
between 1973 and 1989, Flanagan et al25 did
not report any nephrotic syndrome cases in 83
congenital cyanotic heart disease patients, with
or without urinary abnormalities, which were
treated with ACEI (captopril). Another two
studies reported remission of nephrotic
syndrome with ACE inhibitor in a patient who
has cyanotic heart disease with focal
segmental glomerulosclerosis (FSGS). 19,24
There few other studies where they reported,
treatment with ACE inhibitor in a cyanotic
patient with proteinuria revealed successful.
21-23 Role of steroids to reduce proteinuria still
debatable due to lack of research. Moreover,
the pathogenesis of proteinuria in cyanotic
nephropathy is totally different from the
pathogenesis of Nephrotic syndrome. Therefore
further study should be needed for evaluating
steroids in CN especially in FSGS.
Restoration of renal function occurs after
corrective cardiac surgery. Improved hypoxia
and polycythemia after surgical correction may
results in good renal perfusion thus restore
renal function towards normal.23 Few other
studies support the same findings.19,24,25
Although phlebotomy is not routinely practiced
in the treatment of cyanotic nephropathy in
children but it has been shown in one study
that an adult patient with adverse effects
(hyperkalemia) of ACE inhibitor led to
improvement and stabilization of proteinuria.
Phlebotomy was done due to severe
polycythemia and which additionally effect
improvement of renal function and
proteinuria.27 Renal replacement therapy in
patients with cyanotic nephropathy needs
vigilant selection because of hemodynamic
problems. Only few adult cases were reported
regarding renal replacement therapy in
cyanotic heart disease. Hemodialysis was used
in these patients due to fluid overload, uremia,
and acute tubular necrosis.28-31 Till date there
is no obvious evidence on the best blood
purification technique for renal replacement
A compendium review on pediatric cyanotic nephropathy
therapy in cyanotic heart disease namely
tetralogy of Fallot. The dilemma with
hemodialysis in cyanotic nephropathy is that
temporary or permanent catheterization is
probably to cause complications such as
pulmonary embolism, sepsis, infective
endocarditis, and paradoxical embolism.32-34
Moreover in cyanotic patients have complex
hemodynamics so creation of peripheral
vascular access may precipitate heart
failure.35
Acute uremia can be treated with continuous
renal replacement therapy (CRRT) or
Sustained low efficiency dialysis (SLED) due to
hemodynamic instability. CRRT allows for both
slow and controlled subtraction of fluid, avoiding
significant changes in the patient’s fluid
balance. Though there is few data on renal
replacement therapy in children with cyanotic
nephropathy it is more like to be assumed that
cyanotic nephropathy children are at risk of
hemodynamic instability before or after cardiac
surgery and they need continuous renal
replacement (CRRT) as they are critically ill.36
However, in resource- limiting country
peritoneal dialysis is the better option due to
low cost availability, ease of venous access and
placement of the catheter and the lack of need
for anticoagulation therapy.37
There may be considerable variation in the
mode and intensity of managements provided
to end-stage kidney disease (ESRD) patients
with congenital heart disease, stemming from
the uncertainty of the advantages and
disadvantages of kidney care in this population.
A lack of prospective data suggests that
abundant therapeutic decisions among such
subjects are potentially empirical. Indeed, only
a few subjective reports describing patients
with TOF who began dialysis treatments during
the observation periods for the disease are
available.38.39
End stage renal disease (ESRD) requiring
dialysis, continuous ambulatory peritoneal
dialysis (CAPD), or continuous cyclic peritoneal
dialysis (CCPD) are preferred method due to
complication of hemodialysis. Abe T et al.
reported one patient with tetralogy of Fallot with
ESRD was managed well with CCPD.40 PD does
Azmeri Sultana et al
not appear to be an extra peril of infectious
endocarditis among patients with ESRD.41 On
the other hand , the therapeutic nature of PD,
including minimal disparity in the
intravascular volume status, reduction in
cardiovascular stress, avoidance of peaks and
troughs in uremic toxins, arrhythmia
prevention, and the better preservation of
residual renal function, also encouraged us to
promote the procedure as a good modality of
renal replacement therapy.42,43
Patients with end-stage kidney disease, a renal
transplant is the treatment of choice as it
replicates the standard renal physiology much
more closely than dialysis treatments and
offers an better quality of life as well as survival
outcome. Despite the high risk of cardiac death
before and after renal transplantation.44-46
Conclusion
Pediatric cyanotic nephropathy is not so
uncommon. Polycythemia, hyperviscosity, and
hypoxia play a major role to develop
glomerulopathy. Protein leak and renal
impairment can be found in cyanotic
nephropathy. ACE inhibitor seems to be
effective to reduce proteinuria. Surgical
correction of primary heart lesion leads to
improve renal function as well. Peritoneal
dialysis is the preferred method both in AKI
and ESRD patients with cyanotic nephropathy.
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3. Dimopoulos K, Diller GP, Koltsida E.
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4. Billett J, Cowie MR, Gatzoulis MA, Vonder
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Dähnert I, Lange PE. Renal impairment
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15. Krull F, Ehrich JH, Wurster U, Toel U,
Rothgänger S, Luhmer I. Renal
involvement in patients with congenital
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Scand. 1991;80:1214-19.
16. Inatomi J, Matsuoka K, Fujimaru R,
Nakagawa A, Iijima K. Mechanisms of
development and progression of cyanotic
nephropathy. Pediatric Nephrology.
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17. Yaacov F, Sofia F, Choni R, Rachel B
Cohen, Israela L, Annick Raas-Rothschild.
The Heart of Children with Steroid-
Resistant Nephrotic Syndrome: Is It All
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18. Rosenthal G. Prevalence of congenital
heart disease. In: Garson A, Bricker JT,
Fisher DJ, Neish SR, editors. The Science
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pp1089-1093.
19. Hida K, Wada J, Yamasaki H, Nagake Y,
Zhang H, Sugiyama H. Cyanotic congenital
heart disease associated with
glomerulomegaly and focal segmental
glomerulosclerosis: remission of nephrotic
syndrome with angiotensin converting
enzyme inhibitor. Nephrology Dialysis
Transplantation. 2002;17:144-47.
20. Naik VS, Phadke VD, Kanhere SV.
Nephropathy in a child suffering from
Tetralofy of Fallot. Indian J Case Reports.
2020;6:312-13.
21. Akita H, Matsuoka S, Kuroda Y.
Nephropathy in patient with cyanotic
heart disease. Tokushima J Exp Med.
1993;40:47-53.
22. Dittrich S, Haas NA, Bührer C, Müller C,
Dähnert I, Lange PE. Renal impairment
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in patient with long standing congenital
cyanotic heart disease. Acta paediatrica.
1998;87:949-54.
23. Azmeri S, Naharuma Aive HC, Jesmine
H, Shahreen K, Islam SM. Nephrotic range
of protienuria in congenital cyanotic heart
disease: A rare complication. Bangladesh
J child health. 2020;44:178-80.
24. Fujimoto Y, Matsushima M, Tsuzuki K.
Nephropathy of cyanotic congenital heart
disease: clinical characteristics and
effectiveness of an angiotensin-converting
enzyme inhibitor. Clinical Nephrology.
2002;58:95-102.
25. Flanagan MF, Hourihan M, Keane JF.
Incidence of renal dysfunction in adults
with cyanotic congenital heart disease.
Am J Cardiol. 1991;68:403-406.
26. Mayra OD, Puerta Carretero M, Corchete
E, Juan A, Navarro M, Jaldo T, Albalate M,
et al. A case report of cyanotic nephropathy.
Nefrologia. 2019;39:84-109.
27. Omonuwa OK, Talwar A, Dedopoulos S,
Mailloux L. Repeated phlebotomies improve
and stabilise renal function in cyanotic
nephropathy. BMJ case reports. 2009;
2009: bcr1020081084.
28. Dittrich S, Kurschat K, Dähnert I, Vogel
M, Müller C, Lange PE. Cyanotic
nephropathy and use of non-ionic contrast
agents during cardiac catherization in
patients with cyanotic congenital heart
disease. Cardiol Young. 2000;10:8-14.
29. Ohara K, Akimoto T, Miki T. Therapeutic
challenges to end-stage kidney disease in
a patient with tetralogy of Fallot. Clin Med
Insights Case Rep. 2015;8:97-100.
30. Shimizu A, Takei T, Moriyama T. A case
study of initiating hemodialysis in a
patient with tetralogy of Fallot (TOF). J Jpn
Soc Dial Ther. 2009;42:159-64.
31. Yan X, Freeman LJ, Ross C. Unoperated
tetralogy of Fallot: case report of a natural
survivor who died in his 73rd year; is it
ever too late to operate? Postgrad Med J.
2005;81:133-34.
Azmeri Sultana et al
32. Manian FA. Vascular and cardiac
infections in end-stage renal disease. Am
J Med Sci. 2003;325:243-50.
33. Sadjadi SA, Sharif-Hassanabadi M. Fatal
pulmonary embolism after hemodialysis
vascular access declotting. Am J Case
Rep. 2014;15:172-75.
34. Pinard EA, Fazal S, Schussler JM.
Catastrophic paradoxical embolus after
hemodialysis access thrombectomy in a
patient with a patent foramen ovale. Int
Urol Nephrol. 2013;45:1215-17.
35. Tanaka H, Kakuta T, Fukagawa M. Overt
intracardiac shunt flow after arteriovenous
graft placement. Clin Exp Nephrol. 2013;
17:592-93.
36. Thomas MC, Harris DCH. Problems and
advantages of continuous renal
replacement therapy. Nephrology. 2002;
7:110-114.
37. Toda Y, Sugimoto K. AKI after pediatric
cardiac surgery for congenital heart
diseases-recent developments in
diagnostic criteria and early diagnosis by
biomarkers. J Intensive Care. 2017;5:49.
38. Kobayashi M, Magara T, Machida K.
Chronic renal failure caused by long-term
cyanosis in a patient with tetralogy of
Fallot. Tokyo Jikeikai Med J.
1994;109:1055-61.
39. Shimizu A, Takei T, Moriyama T. A case
study of initiating hemodialysis in a
patient with tetralogy of Fallot (TOF) J Jpn
Soc Dial Ther. 2009;42:159-64.
40. be T, Aoyama T, Sano K. Initiation of
peritoneal dialysis in a patient with
chronic renal failure associated with
tetralogy of Fallot: a case report. BMC
Nephrol. 2020;21:277 .
41. Fernández-Cean J, Alvarez A, Burguez S,
Baldovinos G, Larre-Borges P, Cha M.
Infective endocarditis in chronic
haemodialysis: two treatment strategies.
Nephrol Dial Transplant. 2002;17:2226-30.
37
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42. Thodis E, Passadakis P, Vargemezis V, 44. Goodlad C, Brown E. The role of peritoneal
Oreopoulos DG. Peritoneal dialysis: better dialysis in modern renal replacement
than, equal to, or worse than therapy. Postgrad Med J. 2013;89:584-90.
hemodialysis? Data worth knowing before
45. Devine PA, Aisling EC. Renal replacement
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43. Shahab I, Khanna R, Nolph KD. Peritoneal
46. Le A, Wilson R, Douek K, et al. Prospective
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risk stratification in renal transplant
the nephrologist. Adv Perit Dial. 2006;
candidates for cardiac death. Am J Kidney.
22:180-85.
Dis. 1994;24:65-71.

38
 Review Article
Tachycardia-induced Cardiomyopathy (TIC) in
Children: A Review
Rezoana Rima

Abstract
Tachycardia-induced cardiomyopathy is a reversible form of heart failure
characterized by left ventricular dilatation and dysfunction that is usually
reversible once the tachyarrhythmia is controlled. Its development is related to
both atrial and ventricular arrhythmias. Paediatrician should be aware that
children with unexplained systolic dysfunction may have tachycardia-induced
cardiomyopathy. This review describes the pathophysiology, proposed
mechanisms, clinical features and management in various arrhythmic conditions
in children.
Paed. Heart J of Bangladesh (PHJB) 2019;4(1):39-48

Introduction the most common arrhythmias were ectopic

Tachycardia-induced cardiomyopathy (TIC), is
a reversible condition of left ventricular
dysfunction and symptomatic heart failure
caused by supraventricular or ventricular
tachyarrhythmias or frequent ventricular
ectopy.1,2 Left ventricular systolic function
improves or normalizes and symptoms resolve
if rhythm is corrected or rate controlled is
achieved. Different studies suggested that
despite apparent resolution of the problem,
tachycardia recurrence causes a precipitous
decline in LVEF with concomitant symptoms
and that there is an association with sudden
cardiac death. 1 TIC be classified into 2
categories: one in which arrhythmia is the sole
reason for ventricular dysfunction (arrhythmia
induced) and another in which the arrhythmia
exacerbates ventricular dysfunction and/or
worsens HF in a patient with concomitant heart
disease (arrhythmia mediated).3
Epidemiology
The incidence and prevalence of TIC in children
are uncertain, because it remains under
recognized. In a large paediatric series of TIC
Address of correspondence: Dr. Rezoana Rima,
Associate Professor and Interventional Cardiologist,
Department of Paediatric Cardiology, Bangladesh
Institute of Child Health and Dhaka Shishu (Children)
Hospital. Cell: 01713257521, E-mail: drrezoana@
gmail.com
[Received: 04 February 2021 Accepted: 28 April 2021]
atrial tachycardia (59%), permanent junctional
reciprocating tachycardia (23%), and
ventricular tachycardia (7%).4
Pathophysiology
From animal models
Tachycardia-induced cardiomyopathy in
animal model was first described by Whipple et
al5 in 1962. In response to rapid pacing in
animals, left ventricular (LV) remodeling and
heart failure occur in a time-dependent and
highly predictable manner, similar to the
phenotype of Tachycardia-induced cardio-
myopathy.6-12 Several cellular and molecular
events in response to the initial tachy-
arrhythmia stimulus take place over a period
of approximately 3 to 4 weeks and involve both
extracellular matrix (ECM) and myocyte
remodeling. Specifically, there is loss of normal
extracellular matrix and architecture. This,
coupled with alterations in cellular growth and
viability, defects in Ca2+ handling, and
neurohormonal activation, results in LV
dilation and severe contractile dysfunction. The
natural history of rapid pacing-induced
cardiomyopathy progresses from a
compensatory phase (approximately >7 days) to
an LV dysfunction phase (approximately 1 to 3
weeks) to an LV failure phase (approximately
>3 weeks), characterized by progressive LV
dilation and dysfunction and increasing
neurohormonal activation.
Paediatric Heart Journal of Bangladesh (PHJB)
Cellular & molecular events
Initial tachycardia stimulus
Extracellular matrix remodelling
Cellular remodelling & contractile
dysfunction, vibility
Defect in Ca handling & severe
contractile dysfunction
Fig.-1 Natural history and contributing cellular and molecular events in rapid pacing-induced
cardiomyopathy and HF.
Implications of animal studies
Chronic rapid pacing in animals causes dilated
cardiomyopathy resembling clinical TIC. With
rapid atrial pacing in pigs, an atrioventricular
(AV) conduction pattern is maintained and
ventricular dyssynchrony as the stimulus for
adverse remodeling and HF is unlikely.6,7,13
However, rapid ventricular pacing, where
myocardial dyssynchrony is inevitable, results
in a more rapid and precipitous fall in LV
systolic performance.8-11 Thus, the tachycardia
stimulus is sufficient to drive the
cardiomyopathy process, whereby electrical
dyssynchrony would constitute an additive
factor accelerating the process.
Phases and structural milestones that occur
with chronic arrhythmias in animal models
translate to clinical forms of TIC. Specifically,
there is an adaptive/ compensatory phase
whereby LV dilation, extracellular matrix
remodeling, and neurohormonal system
activation occurs but severe LV dysfunction
40
Volume 4, No. 1, January 2019
Natural history Time
Compensatory phase
LV pump function normal 7 days
Sympathetic system activation
LV dysfunction phase
LV pump dysfunction & dilatation 1-3 weeks
LV myocardial contractile dysfunction
Neurohumoral activation (RAAS activation)
LV failure phase
LV pump failure & severe dilatation
Systemic hemodynamic compromise 3 weeks
Significant Neurohumoral activation
RAAS, Vasoactive peptides
Pulmonary / systemic oedema
does not. This phase could be identified in
patients by LV imaging and biomarker profiling
and is followed by a phase in which LV
dysfunction becomes manifest and associated
with defects in excitation-contraction coupling
and LV myocyte remodeling and dysfunction.
This phase likely represents a critical
transition when this process may not
completely reverse, leading to neurohormonal
activation accompanied by the full patho-
physiological spectrum of HF.
Several challenges and limitations exist in
translating animal data to humans. The time
course for development of TIC and HF in
patients with otherwise structurally normal
hearts is usually months to years, which is
quite different from the acute changes seen
in pacing models. Some arrhythmias (e.g., AF
and PVCs) may have mechanisms leading to
TIC that are not explained fully by rapid-pacing
animal models.14-16 Although ultrastructural
changes may explain the initial development
Tachycardia-induced Cardiomyopathy (TIC) in Children: A Review Rezoana Rima

Persistent Tachyarrhythmia or frequent ventricular ectopy (PVC)

No known structural
uctural heart disease
diseas Underlying
ying structural heart
hear disease

Adverse cellular, cytoskeletal, neurohumoral, proapoptotic changes,

Adve
abnormal Calcium handling

Development of left ventricular dysfunction, dilated cardiomyopathy,

heart failure
Tachycardia induced cardiomyopathy (TIC)

Suppression/elimination of arrhythmia by rate and/or rhythm control

Heart failure therapy

Heart failure resolution LV function recovery No significant improvement of

--- confirm diagnosis of TIC LV function - No TIC

No evidence of persistent
ultrastructural changes by - Good Prognosis
Follow Up Echo/MRI
-Maintain sinus rhythm/Strict rate control
-Close surveillance to avoid
recurrence Persistent ultrastructural changes :
-Continue betablocker/ACE inhibitor -Increase LV dimension
-Close surveillance
-F/U imaging-Echo/MRI -Evidence for fibrotic
-Continue betablocker/ACE
changes in MRI
inhibitor
-Development of LV
hypertrophy

Fig.-2 Mechanisms to management and prognosis

of TIC, the relationship between identifiable can be difficult to determine because an

ultrastructural changes and the mechanisms
by which cardiomyopathy develops are less
clear. An overview of the current understanding
of the relationship between arrhythmias and
cardiomyopathy, from mechanisms to
management and prognosis, is shown in Fig.-2.
Clinical Features and Diagnosis
The key diagnostic feature of TIC is the
presence of persistent arrhythmia or frequent
ventricular ectopy (PVC) in the presence of an
otherwise unexplained cardiomyopathy. The
relationship of arrhythmia to cardiomyopathy
arrhythmia could exist for years before its
recognition and before cardiomyopathy
develops. Although a high index of clinical
suspicion may point to subtle diagnostic clues,
the condition may go unrecognized for years.
The presentation can be late, only after
manifest systolic HF develops. Similarly, if the
arrhythmia is detected early but a
nonaggressive approach is taken, progressive
worsening of symptoms and insidious
development of cardiomyopathy ensue. It can
be difficult to determine whether an

41
Paediatric Heart Journal of Bangladesh (PHJB)
arrhythmia is the initiator or consequence of
cardiomyopathy in a patient with tachycardia
and HF.
History, physical examination, and clinical
investigations should focus on determining the
etiology of cardiomyopathy. Specific patient
characteristics may suggest a higher
likelihood of TIC. Patients with TIC have a
smaller LV end-diastolic diameter and mass
index versus those with pre-existing dilated
cardiomyopathy and concomitant
tachyarrhythmia. 17,18 Cardiac magnetic
resonance imaging (CMR) may help
differentiate AIC from dilated cardiomyopathy.
Serial assessment of the N-terminal pro-BNP
(NT-proBNP) ratio (NT-proBNP at baseline/NT-
proBNP during follow-up) can differentiate TIC
from irreversible dilated cardiomyopathy. Saiki
et al19 showed 40 patients with AF or atrial
flutter with ventricular rates >100 beats/min
and LVEF <40% were cardioverted, and NT-
proBNP was measured on day 1 and weekly for
4 weeks. An NT-proBNP ratio cutoff >2.3 at 1
week, suggesting rapid decline in NT-proBNP
levels, was associated with reversible
cardiomyopathy, with an accuracy of 90%.19
When TIC cannot be easily differentiated from
dilated cardiomyopathy with consequent
tachycardia, treatment for both problems
becomes necessary.20
Principles of management
TIC management should focus on concerted
attempts to eliminate or control the
arrhythmia, with the goal of improving
symptoms, reversing LV dysfunction, and
preventing arrhythmia recurrence.1,21-24 A
favorable response to arrhythmia elimination/
control establishes the diagnosis of TIC. Along
with arrhythmia control, use of neurohormonal
antagonists can result in favorable ventricular
remodeling. Controversy remains about the
need to continue these medications if there is
complete recovery of LVEF with arrhythmia
control.
Tachyarrhythmias are a reversible cause of
cardiomyopathy from fetal life onward.25-28
Children often present late because they fail
to recognize palpitations or are unable to
42
Volume 4, No. 1, January 2019
verbalize symptoms and come to medical
attention only after the development of HF.
Compared with adults, different arrhythmia
mechanisms are represented in paediatric AIC.
Supraventricular arrhythmias are more
common than ventricular arrhythmias in
children and therefore are more frequently
associated with TIC. In the newborn, a single,
sustained episode of typical supraventricular
tachycardia may be unrecognized until HF
symptoms emerge; thus, neonates may present
with decreased LV function, or even shock. In
this group, prognosis and recovery of cardiac
function are excellent after control of
supraventricular tachycardia. This is in
contrast to the more incessant tachycardias,
for which control is more challenging and
recovery less rapid.
Etiologically, sustained rapid rates, QRS
duration, AV dyssynchrony, and heart rate
irregularity all could contribute to TIC, yet not
all children with incessant tachycardia develop
TIC. In children, the tachycardias most often
associated with TIC have a narrow QRS complex
and 1:1 AV conduction. Heart rate irregularity
occurs in pediatric AIC as salvos of tachycardia
interspersed with periods of sinus rhythm,
rather than as the persistent heart rate
irregularity seen in AF.
As evident in many cardiac conditions, genetic
factors may underlie the development of AIC.
Mutations in the cardiac Na+ channel and in
the ryanodine receptor, long known to be
involved in arrhythmogenesis, can now be
linked to abnormalities of contractile
function.29
Pediatric arrhythmias associated with TIC
Atrial ectopic tachycardia (AET)
AET is the most common arrhythmia
associated with TIC in children.30 Although P-
wave morphology and axis usually differ from
sinus rhythm, AET foci near the sinus node
are hard to differentiate from sinus tachycardia,
especially in patients with HF, when
tachycardia is expected. Increased
automaticity is the most likely mechanism;
others include triggered activity and micro-
reentry. 31-34 AET usually occurs without
Tachycardia-induced Cardiomyopathy (TIC) in Children: A Review
structural heart disease but has been
described after congenital heart disease
surgery 34 and in the setting of channel-
opathies.35
Kany et al36 in a multicenter study of 249
children with AET, found in TIC 28%. The
cardiomyopathy varied from asymptomatic mild
LV dysfunction to the need for HF medications
in 52%; 3 required extracorporeal membrane
oxygenation. 36 Multiple antiarrhythmic
medications or combinations of medications
were used, with beta-blockers being the most
common first-line therapy. Class Ic
antiarrhythmic medications was most
commonly useful for FAT management
compared with other medications such as
amiodarone that were less useful.
No trends emerged to define the most
successful medication. Catheter ablation was
effective in 81% of patients in whom it was
used. The use of electroanatomic mapping for
ablation improved success37 and decreased
recurrence.38
Spontaneous resolution of AET can occur,
especially in those presenting within the first
year of life, where 74% had resolution. 36
Ablation proved safe and effective, but the
investigators suggested a trial of medical
therapy in the youngest patients, in whom
ablation may have more risk39,40 and when
spontaneous resolution is more likely.
Permanent junctional reciprocating
tachycardia (PJRT)
PJRT is an accessory pathway-mediated
tachycardia with a long RP interval that occurs
predominantly in infants and children. The
pathway can be located anywhere in the AV
junction but is usually posteroseptal. 41
Pathways are tortuous and slow conducting;
thus, tachycardia is often incessant.
In a recent review, 27% of patients with PJRT
presented in fetal life, 7% with hydrops, a fetal
manifestation of TIC.42 Isolation of the AV
junction is a continuing process that may not
be complete at birth, and the presence of
accessory connections crossing the annulus
fibrosis could result in persistent perinatal
supraventricular tachycardia. Although most
Rezoana Rima
children with PJRT present with palpitations
or are noted to have rapid heart rates, 18%
presented with TIC in a series of 194 children.42
Cardiomyopathy is more likely to occur in
children with longer RP interval/cycle length
ratios, consistent with an accessory pathway
with slow retrograde conduction and a wide,
excitable gap43 PJRT is often incessant, and
those with incessant PJRT had longer RP
intervals, were younger at diagnosis, and more
often had TIC.42 The clinical course of PJRT is
not benign, and spontaneous resolution is
unlikely.42
A number of antiarrhythmic medications are
used, but a single most effective agent has not
emerged.42 Beta-blockers are the common first
choice, likely reflecting physician comfort,
rather than proven efficacy. Complete
tachycardia suppression with medications
varies from 25% in the recent series42 to >80%
in a study using regimens that included
initially with amiodarone or verapamil in
combination with digoxin.44 Medical therapy is
commonly used in neonates and infants,
whereas older children undergo ablation.
Catheter ablation is the primary treatment for
PJRT, with reported success rates of 90% (42).
Thus, the role for medical therapy is limited to
the neonate and small infant as a temporary
measure to allow for the rare patient with
spontaneous resolution, to suppress the
tachycardia, and to prevent TIC and allow time
and growth before undertaking catheter
ablation.
Junctional ectopic tachycardia (JET)
JET, most commonly seen in small children
following congenital heart surgery45, is due to
abnormal automaticity in the region of the AV
junction. JET unassociated with cardiac
surgery can present at any age, and congenital
JET, presenting in infancy, is associated with
high morbidity and mortality.45-47 In an early
study, mortality was 34%, with sudden death
occurring in infants.48 In a multicenter study
of non-operative JET, overall mortality was low,
with all deaths occurring in children <6 months
of age.49 Although only 16% presented with HF,
the tachycardia was incessant in >40%. JET
can be incessant or paroxysmal, although
43
Paediatric Heart Journal of Bangladesh (PHJB)
infants <6 months of age are more likely to
have incessant tachycardia.
Medical management is commonly undertaken
(89%) as the first step but was variably effective,
with a variety of drugs used. Amiodarone is the
initial treatment of choice and is used most
frequently, as a first-line agent and has been
used either alone or in combination with
propranolol or flecainide in infants. Ivabradine,
which works by selective inhibition of
hyperpolarization-activated cyclic nucleotide-
gated channels, has been shown to be
effective.50-52 It has been shown that selective
blockade of the rapidly activating delayed
rectifier K+ channel (I(Kr)) by pure class III
antiarrhythmic drugs like nifekalant has been
useful in CJET refractory to amiodarone and
other drugs.53
Catheter ablation for JET can be accomplished
with the preservation of AV nodal conduction,
and cryoablation has been associated with
success rates similar to those of radiofrequency
ablation but without AV block.54-57
Ventricular tachycardia and PVCs
Although rare in children, ventricular
tachycardia can result in TIC. Incessant
ventricular tachycardia of infancy occurs in
association with ventricular Purkinje cell
tumors or histiocytoid or lymphocytoid
tumors.58-61 Both left and right ventricular
tachycardias can result in paediatric TIC , and
there are reports of PVC- induced TIC in
children.62,63 The burden of ectopy needed, or
other features associated with TIC risk, have
not been defined. Idiopathic fascicular VT
represented an extraordinary situation because
it is infrequent in childhood and when present
generally has a favorable prognosis.64,65 Of the
37 cases published by Ohe et al., 25% were
younger than 15 years of age and notably, only
one case had a severe clinical course refractory
to drugs. There are only three reports in the
international literature of unique cases with
TIC induced by idiopathic fascicular VT, a
clinical course with normalization of the left
ventricular function after successful treatment
with catheter ablation.66-69
44
Volume 4, No. 1, January 2019
Conclusion
TIC has been reported in any pediatric age
group-from fetus to adolescence. Tachy-
arrhythmia’s resulting in TIC in children differ
from those in the adult. There is a predictable
pattern of resolution with treatment. Recovery
seems independent of treatment strategy
(ablation vs. medical therapy). TIC can be one
of the most common and unrecognized cause
of potentially curable CHF and needs to be
taken into consideration in the differential
diagnosis of idiopathic DCM.
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 Case Report
Surgical Management of Ascending Aortic
Pseudoaneurysm in A 12 Years Old Girl:
A Case Report
Mohammad Rokonujjaman1, Shaheedul Islam2, Nusrat Ghafoor3, Nowshin Siraj4,
Syed Tanvir Ahmad5, Ibrahim Khalilullah6, Abdullah Al Shoyeb7, Atiqur Rahman8,
ZA Faruquee9, Sirajul Islam10

Abstract
Aortic pseudoaneurysms are rare but life-threatening complication after cardiac
surgery. The causes could be multifactorial, as a sequel of cardiac surgery,
trauma or infection. The development and expansion of aortic false aneurysms
is often silent. Their evolution is unpredictable, and rupture can be fatal. Despite
advances in endovascular techniques, the treatment is chiefly surgical. In recent
years, improved results in surgical management have been reported; however,
treatment is still burdened by a high morbidity rate. We report the surgical
management of a postoperative pseudoaneurysm of the ascending aorta 2
months after repair of Tetralogy of Fallot in a Bangladeshi girl. She was operated
on for resection of the aneurysm and repair. A computed tomography scan at 2
weeks following surgery showed no aneurysm and our patient was discharged
with uneventful recovery. Aortic false aneurysm can develop silently. Surgical
procedures should be proposed even to asymptomatic patients because of the
unpredictable evolution of the condition. Early diagnosis and appropriate
treatment of such rare complication can be lifesaving.
Keywords: Ascending aortic pseudo-aneurysm, congenital heart surgery, TOF
repair.
Paed. Heart J of Bangladesh (PHJB) 2019;4(1):49-45

Introduction in children. Several cases have been reported,

Aortic pseudoaneurysms are rare, life- most of which were secondary to bacterial
threatening sequelae of cardiac surgery.1 They endocarditis. 6-8 Various management
can also occur as a result of infections, genetic strategies have been suggested, including
disorders, or trauma.2-5 They are uncommon surgical and catheter-based interventions.
1. Associate Professor & Consultant, Department of Cardiac surgery, Ibrahim Cardiac Hospital & Research Institute.
2. Associate Professor & Consultant, Department of Cardiology, Ibrahim Cardiac Hospital & Research Institute.
3. Associate Professor & Consultant, Department of Radiology, Ibrahim Cardiac Hospital & Research Institute.
4. Associate Professor & Consultant, Department of Radiology, Ibrahim Cardiac Hospital & Research Institute.
5. Assistant Professor & Associate Consultant, Department of Cardiac surgery, Ibrahim Cardiac Hospital &
Research Institute.
6. Assistant Professor & Associate Consultant, Department of Cardiac Anesthesiology, Ibrahim Cardiac Hospital
& Research Institute.
7. Registrar & Specialist, Department of Cardiac surgery, Ibrahim Cardiac Hospital & Research Institute
8. Registrar & Specialist, Department of Cardiac surgery, Ibrahim Cardiac Hospital & Research Institute
9. Associate Professor & Consultant, Department of Cardiac Anesthesiology, Ibrahim Cardiac Hospital & Research
Institute.
10. Professor & Senior Consultant, Department of Cardiac Anesthesiology, Ibrahim Cardiac Hospital & Research
Institute.
Address of correspondence: Dr. Mohammad Rokonujjaman, Associate Professor & Consultant, Department of
Cardiac surgery, Ibrahim Cardiac Hospital & Research Institute. Cell: 01711311200, E-mail: drselim74@gmail.com
[Received: 22 December 2020 Accepted: 28 March 2021]
Paediatric Heart Journal of Bangladesh (PHJB)
Because of the age and size of our patient, and
the nature of the lesion, we opted for surgical
intervention. Timely diagnosis with effective
surgical treatment in combination with
antibiotic coverage can result in good survival
without complications in such life threatening
situations.
Case presentation
Our patient is a 12-year-old Bangladeshi girl,
weighing 38 kg, with large anterior malaligned
VSD, 50% overriding of aorta, Right ventricular
Hypertrophy and combined infundibular and
pulmonary valvular stenosis of RVOT. She was
the product of a consanguineous marriage and
there is no history of congenital heart disease
in the family. She underwent VSD closure with
Fig.-1 Initial postoperative CT scan reveals a moderate sized pseudoaneurysm arising from aorta with
a narrow neck.
Fig.-2 This picture also shows the same pseudoaneurysm measuring 36mm X 36mm.
50
Volume 4, No. 1, January 2019
PTFE patch, resection of hypertrophied
infundibular muscle bands. Pulmonary valve
was resected and PV annulus opened. 0.1 mm
PTFE patch was used for modified single patch
reconstruction of pulmonary valve.
Transannular patch was given for RVOT
reconstruction using autologus gluteraldehyde
treated pericardium. 5 mm small ASD was kept
open. She was extubated after 12 hours of
ventilation with minimum inotropic support.
Adrenalin and Milrinone was used in ICU. She
had an uneventful recovery and discharged
from hospital on 10th POD. Per operative
epicardial echocardiography showed no
residual shunt, RVOT gradient was 15 mm Hg.
No pulmonary regurgitation, no LVOTO.
Patient was on aspirin 75 mg once daily.
Surgical Management of Ascending Aortic Pseudoaneurysm in A 12 Years
After 2 months postoperatively, patients
presented with hemoptysis of several episodes
containing fresh unclotted blood in the sputum
and dull aching chest pain which she could not
localize. After hemoptysis pain relieved. She
also had a tense, non pulsatile, tender swelling
in the upper part of sternal wound along with
raised temperature. Transthoracic echo-
cardiogram revealed a small pseudo-aneurysm
in front of lower part of ascending aorta.
“Smoky” blood flow into the aneurysm cavity
was seen, but no thrombi or vegetation was
detected. These findings were confirmed on
computed tomography (CT) angiogram, which
showed a pseudoaneurysm arising from the
anterior wall of the Ascending Aorta just above
the sinotubular junction. The lesion was 36mm
X 36mm in size and had a 6mm neck (Fig.-
1&2). As the patients party denied surgery of
the pseudoaneurysm, subcutaneous swelling
incised and altered venous blood evaquated.
There was no feature of sternal wound infection
evidenced by culture report. After few days of
surgical toileting secondary suturing of sternal
wound performed.
After two weeks patient again presented with
frequent hemoptysis and dull aching chest pain
but no sternal complication. Echocardiogram
Fig.-3 Here CT image reveals that the aorta is
compressed and huge extension of pseudo-
aneurysm. It measured about 62mmX81mm.
Mohammad Rokonujjaman et al
revealed expanded previous Pseudoaneurysm
and CT scan revealed huge expansion of
previous pseudoaneurysm with subcutaneous
extrension. Neck of the lesion increased from
6 mm to 10 mm (Fig.-3, 4, 5, 6).
It extended up to the sub cutaneous tissue
through the sternum (Fig.-4). There was
fracture of two sternal wires. After evaluation
and preparation she was sent to the operating
theater. Surface cooling was started early while
exposing his right femoral vessels. After
heparin was given, a 12 Fr arterial cannula
was inserted into the right femoral artery, and
was fixed. Right femoral vein cannulated and
femoro-femoral bypass initiated. When his core
temperature reached 28 °C, the lower part of
her sternum was opened and on the half way
to the dissection upto the middle part of
sternum, pseudoaneurysm ruptured during
dissection of sub cutaneous tissue. Cooling was
continued on bypass until a core temperature
of 26 °C was reached. Her head was additionally
cooled with ice packs.
Very low flow was used to improve exposure.
Bleeding site was controlled using finger
pressure and a 10 mm hole at the site of the
previous cardioplegia needle insertion site was
Fig.-4 This CT image demonstates that the
Pseudoaneurysm has crossed through the sternal
gap up to the sub cutaneous plane and impending
to rupture.
51
Paediatric Heart Journal of Bangladesh (PHJB) Volume 4, No. 1, January 2019

Fig.-5 This 3D reconstructed Image reveals proper Fig.-6 This sagittal image reveals the very large
orientation and relation of Pseudoaneurysm with pseudoaneurysm.
other structures.

found. The edges of the hole and the

surrounding tissue appeared very much soft
and friable. Cultures were taken. No cross
clamp applied. A bovine pericardial patch was
used to repair the ascending aortic wall.
Because of tissue friability, the patch was
sutured away from the edges using 7/0
polypropylene mattress continuous stitches.
This was further reinforced by an outer 6/0
polypropylene stitch. After rewarming, the
cardiopulmonary bypass was weaned off easily.
The femoral arterial line was removed and the
femoral arterial cannulation site was repaired
with bovine pericardial patch using 7/0 prolene.
Our patient had a smooth postoperative course
with no signs of infection and no neurological
or ischemic complications except pain in left
lower limb which persisted for repeated a
cardiac CT scan (Fig. 8) at 1 months following
surgery which showed no aneurysm except
small leaking of dye near the repair area . At 6
month outpatient follow up she had an
Fig.-7 Contrast CT showing Neck of the Pseudo
unremarkable clinical examination and an
aneurysm increased to 10 mm.
echocardiogram showed no signs of aneurysm

52
Surgical Management of Ascending Aortic Pseudoaneurysm in A 12 Years
Fig.-8 Post repair CT scan of the patient reveals
closed pseudoaneurysm.
except trivial leaking of dye in the repair area
and it was not extended in comparison to the
previous CT scan. She gained weight to 43 kg.
She is under follow up.
Discussion
Though the incidence of aortic pseudo
aneurysm is rare after cardiac surgery, its
inhospital mortality rates and midterm
outcomes are satisfactory after operation.1,4-
6,8-12 Patients with aortic false aneurysm can
present with chest pain, dyspnea, and fever,
and less frequently with mass effect symptoms
or erosion of surrounding structures. 1,5,9
Asymptomatic patients can be diagnosed with
false aneurysm during routine monitoring and
several years after the prior operation.4,5,7-9
Acquired aneurysms of the ascending aorta,
although rarely seen, are most commonly found
at cannulation sites and suture lines. As
reported these aneurysms are diagnosed with
a high index of suspicion as symptoms are
usually absent or nonspecific. They carry a
high risk of mortality if diagnosed late and
surgery is performed after the rupture. In the
past, infected aneurysms were seen mostly as
a result of infected endocarditis and repaired
valvular diseases. These aneurysms can occur
as direct invasion of aortic intima by circulating
bacteria or through lymphatics in the presence
of infection and mediastinitis. An added risk
Mohammad Rokonujjaman et al
is therapeutic intervention like aortic
cannulation. It is extremely important to
promptly diagnose these cases with a high
index of suspicion especially after open heart
repair or history of aortic cannulation like in
our case. An echocardiogram is a noninvasive,
immediately available screening tool; cardiac
CT angiogram is more sensitive. In our patient,
with a high index of suspicion as there was
sero sanguinous discharge from wound, an
echocardiogram was done immediately and,
later, cardiac CT confirmed the diagnosis.
These findings justify long term monitoring
after aortic surgery, especially in the presence
of recognized risk factors for aortic false-
aneurysm development: previous aortic
dissection, inherited connective tissue
disorders, or postoperative graft infection,
sepsis, or mediastinitis.1-4,7-9,13 The use of
biological glues at the suture lines is reportedly
a chief cause of aortic wall disruption.10,14
However, the cautious use of albumin
glutaraldehyde glue has been shown to be safe
and advisable.15
Infection is the most threatening condition,
because of systemic involvement and the
fragility of tissues. Previously, infection was
reported to be the main cause of aortic false
aneurysm in a widely variable number of
patients, from 10% to 75%. 1,4,5,7,8,16
Furthermore, positive blood cultures, tissue
cultures, or both were described in half or fewer
of these patients (30%-50%). 1,5,6,8 We have
described one case who presented with aortic
false aneurysm.
Direct closure is not the optimal solution in
uninfected patients because of the fragility of
the suture line tissue. However, at reoperation,
patients’ conditions need to be evaluated
carefully, and closure of the aneurysm might
be the only appropriate life saving procedure
in severely ill patients.
The opportunity to avoid open surgery is
appealing, especially in patients who present
in critical condition preoperatively.
Percutaneous stent-graft placement, device
occlude implantation, and coil embolization
have been proposed for the treatment of false
aneurysm of the thoracic aorta. 17-21
Endovascular techniques have some
53
Paediatric Heart Journal of Bangladesh (PHJB)
limitations, depending on the location of the
false aneurysm and the size of the
communication in the tear. Stent grafts
require adequate landing zones and might not
be a safe option in proximity to the coronary
ostia and supra aortic vessels. Coil embolization
could be effective in small aortic false
aneurysms with narrow necks; however,
reports of these procedures are anecdotal.22
Occluding devices could be used more
extensively, but the absence of firm tissue,
especially in infective lesions, increases the
risk of device migration and embolization and
the possibility of residual leak. The results
reported in case reports and small case series
are inconsistent with a cumulative
intraprocedural success rate of about 86%.20
The effectiveness and safety of transcatheter
procedures are still not optimal, so surgical
repair has to be considered the procedure of
choice for treating false aneurysms in patients
who present in acceptable general condition.
Infective false aneurysms suggest higher risk
conditions, especially in association with
sepsis. Less invasive treatment might be
advisable for these patients; however, the
necessity of complete tissue debridement and
extensive repair are the cornerstone in
controlling infective lesions and avoiding
recurrence.
We do not know the actual cause of leaking
from antegrade cardioplegia insertion site as
we could not establish any infectious source.
The reason of hemoptysis was not understood
as well as there was no bronchial
communication in CT scan. Our approach
included surface cooling, femorofemoral bypass,
and use of hypothermia. We found this method
to be a safe and reproducible. One case was
reported by Atiyah et al23 and their case was a
two years old boy who underwent arch
augmentation with VSD closure and developed
an ascending aortic aneurysm due to MRSA
infection.
Conclusions
In conclusion, aortic pseudoaneurysms are
rare but recognized complications of cardiac
surgery that can be life-threatening. Prompt
diagnosis and management, as in our case, can
result in successful outcomes.
54
Volume 4, No. 1, January 2019
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