Indian J Pediatr (January 2013) 80(1):32–38
DOI 10.1007/s12098-012-0833-6
SPECIAL ARTICLE
Consensus on Timing of Intervention for Common
Congenital Heart Diseases: Part I - Acyanotic Heart Defects
P. Syamasundar Rao
Received: 22 May 2012 / Accepted: 14 June 2012 / Published online: 30 June 2012
# Dr. K C Chaudhuri Foundation 2012
Abstract The purpose of this review/editorial is to discuss
how and when to treat the most common acyanotic congenital
heart defects (CHD); the discussion of cyanotic heart defects
will be presented in a subsequent editorial. By and large, the
indications and timing of intervention are decided by the
severity of the lesion. Balloon pulmonary valvuloplasty is
the treatment of choice for valvar pulmonary stenosis and
the indication for intervention is peak-to-peak systolic pres-
sure gradient >50 mmHg across the pulmonary valve. For
aortic valve stenosis, balloon aortic valvuloplasty appears to
be the first therapeutic procedure of choice; the indications for
balloon dilatation of aortic valve are peak-to-peak systolic
pressure gradient across the aortic valve in excess of 70 mmHg
irrespective of the symptoms or a gradient ≥50 mmHg with
either symptoms or electrocardiographic ST-T wave changes
indicative of myocardial perfusion abnormality. The indica-
tions for intervention in coarctation of the aorta are significant
hypertension and/or congestive heart failure along with a
pressure gradient in excess of 20 mmHg across the coarcta-
tion; the type of intervention varies with age at presentation
and the anatomy of coarctation: surgical intervention for neo-
nates and young infants, balloon angioplasty for discrete
native coarctation in children, and stents in adolescents and
adults. Long segment coarctations or those associated with
hypoplasia of the isthmus or transverse aortic arch require
P. S. Rao
Department of Pediatrics, Division of Pediatric Cardiology,
The University of Texas-Houston Medical School/Children’s
Memorial Hermann Hospital,
Houston, TX, USA
P. S. Rao (*)
The University of Texas-Houston Medical School,
6410 Fannin Street, UTPB Suite # 425,
Houston, TX 77030, USA
e-mail: p.syamasundar.rao@uth.tmc.edu
surgical treatment in younger children and stents in adoles-
cents and adults. For post-surgical aortic recoarctation,
balloon angioplasty in young children and stents in adoles-
cents and adults are treatment options. Transcatheter closure
methods are currently preferred for ostium secundum atrial
septal defects (ASDs); the indications for occlusion are right
ventricular volume overload by echocardiogram. Ostium pri-
mum, sinus venosus and coronary sinus ASDs require surgical
closure. For all ASDs elective closure around age 4 to 5 y is
recommended or as and when detected beyond that age. For
the more common perimembraneous ventricular septal defects
(VSDs) of large size, surgical closure should be performed
prior to 6 to 12 mo of age. Muscular VSDs may be closed with
devices. Patent ductus arteriosus (PDA) may be closed with
Amplatzer Duct Occluder if they are moderate to large and
Gianturco coils if they are small. Surgical and video-
thoracoscopic closure are the available options at some centers.
In the presence of pulmonary hypertension appropriate testing
to determine suitability for closure should be undertaken. The
treatment of acyanotic CHD with currently available medical,
transcatheter and surgical methods is feasible, safe and effec-
tive and should be performed at an appropriate age in order to
prevent damage to cardiovascular structures.
Keywords Congenital heart defects . Pulmonary stenosis .
Aortic stenosis . Coarctation of the aorta . Atrial septal
defect . Ventricular septal defect . Patent ductus arteriosus .
Indications for intervention . Transcatheter therapy
Introduction
Congenital heart defect (CHD) is a common congenital
anomaly occurring in 0.6 % to 0.8 % of live births. Approx-
imately 50 % of these defects may be managed by
Indian J Pediatr (January 2013) 80(1):32–38
observation, simple medications, and follow-up without any
major therapeutic intervention [1]. Unfortunately however,
the remaining 50 % require surgical or transcatheter inter-
vention to address these anomalies in an attempt to prevent
mortality, decrease morbidity, and avoid permanent damage
to the heart or pulmonary vasculature [1]. In this review how
and when to treat the most common congenital heart defects,
with emphasis on when, will be addressed. In this article the
comments are limited to acyanotic heart defects and discus-
sion on cyanotic heart defects will be included in a subse-
quent issue of this Journal. The acyanotic defects may be
subdivided into obstructive lesions and left-to-right shunt
lesions [2].
Obstructive Lesions
Three most common obstructive lesions, namely, pulmonary
stenosis, aortic stenosis and coarctation of the aorta will be
included in the discussion.
Pulmonary Stenosis
At the present time balloon pulmonary valvuloplasty is
treatment of choice for valvular pulmonary stenosis. De-
tailed description of the procedure of balloon valvuloplasty
and the results of such a procedure are beyond the scope of
this presentation; the reader is referred elsewhere [3, 4] for
details. Because of the success with balloon pulmonary
valvuloplasty, surgery is largely reserved for cases with
supravalvular pulmonary artery stenosis, severe valve annu-
lar hypoplasia and dysplastic pulmonary valves.
The timing for intervention is dictated by the degree of
obstruction. Indications for intervention are similar to those
prescribed in the past for surgical pulmonary valvotomy,
namely moderate to severe stenosis, defined as catheter-
measured peak-to-peak systolic pressure gradient
>50 mmHg across the pulmonary valve [5] with a normal
cardiac index and normal right ventricular function. Since
cardiac catheterization is not generally performed for diag-
nostic purposes, the peak-to-peak systolic pressure gradient
is estimated by Doppler echocardiography. Peak instanta-
neous gradient appears to overestimate the gradient because
of pressure recovery phenomenon [6]. Mean Doppler gradi-
ent appears to underestimate the catheter gradient. The true
peak-to-peak systolic pressure gradient may be somewhere
in between the peak instantaneous and mean Doppler gra-
dients. When this gradient is reached, elective balloon pul-
monary valvuloplasty may be performed. Many patients
may be asymptomatic and the intervention is indicated when
the above stated gradient has reached even in completely
asymptomatic patients. It should also be noted that in
patients who present late with poor ventricular function,
33
immediate intervention is mandatory. In the neonates and
young infants, the obstruction may become critical, defined
as supra-systemic right ventricular pressure, right to left
inter-atrial shunt and/or ductal dependent pulmonary circu-
lation; [7, 8] in these situations, the procedure should be
performed on an urgent basis.
Aortic Stenosis
Balloon aortic valvuloplasty appears to be the first thera-
peutic procedure of choice for relief of aortic valve obstruc-
tion in children although, at this time, there is no consensus
with regard to the recommended treatment mode. Again,
detailed description of the procedure and results of balloon
aortic valvuloplasty are beyond the scope of this paper; the
interested reader may seek information elsewhere [9–11].
We generally reserve surgery for patients in whom we were
unsuccessful in affecting relief to the obstruction by the
balloon procedure; this is more often seen in patients with
dysplastic and/or unicommisural aortic valve leaflets. When
surgery is undertaken, commissurotomy and/or plastic re-
pair of the aortic valve is performed on cardiopulmonary
bypass via aortotomy.
The timing of intervention is again determined by the
degree of valvar obstruction. The indications, by and large,
for balloon dilatation of aortic valve are similar to those
used for surgical valvotomy: catheter-measured peak-to-
peak systolic pressure gradient across the aortic valve in
excess of 70 mmHg irrespective of the symptoms or a
gradient ≥50 mmHg with either symptoms or electrocardio-
graphic ST-T wave changes indicative of myocardial perfu-
sion abnormality [9, 10]. These gradients are valid
indicators of severity of obstruction in the presence of
normal cardiac index and normal left ventricular function.
Again, since cardiac catheterization is not generally per-
formed for diagnostic purposes, Doppler gradients are used
for estimating the degree of obstruction. Just as described
for pulmonary stenosis, pressure recovery phenomenon
affects our assessment of peak-to-peak gradients and the
“true” catheter gradient is somewhere in between the
Doppler-derived peak instantaneous and mean gradients.
Balloon aortic valvuloplasty may be performed on an elec-
tive basis, unless ST/T wave changes suggestive of myocar-
dial ischemia are present, when it becomes an urgent
procedure. In patients who present late with poor ventricular
function or those patients who are symptomatic, immediate
intervention is a must. Very severe aortic valve stenosis with
a high gradient, congestive heart failure or ductal dependent
systemic circulation in the neonate and young infant may be
considered as critical obstruction; in these patients relief of
obstruction should be undertaken on an urgent basis. The
author prefers balloon valvuloplasty;[8] this may be per-
formed via retrograde femoral arterial [12], transumbilical
34
arterial [13], anterograde, femoral venous [14, 15] or ante-
rograde, transumbilical venous [16] routes.
Simple balloon valvuloplasty or surgical valvotomy may
not adequately address severe aortic valve disease, especially
when associated with severe aortic regurgitation. Replacement
of the aortic valve with mechanical or bioprosthetic valves or
Ross procedure (autologous pulmonary valve to replace a
diseased aortic valve) [17, 18] may become necessary in such
situations.
Coarctation of the Aorta
Treatment algorithms depend upon the age of the patient as
well as the anatomy of the coarctation segment and surround-
ing region. Surgical intervention for neonatal and young infant
coarctations and balloon angioplasty for discrete native coarc-
tation in children have generally been used. If the coarctation
segment is long or is associated with hypoplasia of the isthmus
or transverse aortic arch, surgical treatment in younger chil-
dren and stents in adolescents and adults are likely to be
beneficial. Surgical options include resection and end-to-end
anastomosis, subclavian flap angioplasty, prosthetic patch
angioplasty, tubular bypass grafts and variations thereof. Type
of surgery used is in large part dependent upon the anatomy
and to some extent by surgeon’s preference. Balloon angio-
plasty is a more recently available option as is stent implanta-
tion [19]. While surgery is generally preferred in the neonate
and young infant, balloon angioplasty may be preferable in
special circumstances such as infants with shock-like syn-
drome [20], severe myocardial dysfunction and hypertensive
cardiomyopathy [21], prior spontaneous cerebral hemorrhage
[22] and biliary atresia awaiting liver transplantation [22, 23].
Significant hypertension and/or congestive heart failure
are indications for intervention. The author believes it is
better to relieve the obstruction promptly rather than
attempting to "treat" hypertension with antihypertensive
drugs. Asymptomatic children should undergo the proce-
dure electively. If neither hypertension nor heart failure is
present, elective relief of the obstruction between the ages of
2 and 5 y is suggested. Waiting beyond 5 y is not advisable
because of evidence for residual hypertension if the aortic
obstruction is not relieved by the age 5 y [24]. More recent
data [25] suggest that intervention prior to first birth day is
likely to prevent hypertension during follow-up. Some
patients are detected late in childhood or adolescence and
intervention should be performed without delay in the pres-
ence of hypertension and an arm-leg peak systolic blood
pressure difference greater than 20 mmHg.
Post-surgical Aortic Recoarctation
Development of recoarctation following surgery is indepen-
dent of the type of surgical repair in that it has been seen
Indian J Pediatr (January 2013) 80(1):32–38
following all types of surgery described above [26, 27]. The
younger the child at surgery, the higher is the chance for
recoarctation [27]. There is consensus among cardiologists
and surgeons that balloon angioplasty is the treatment of
choice for post surgical aortic recoarctations. The technique
of balloon angioplasty [27–29] for the management of post-
surgical recoarctation is similar to that used for native co-
arctation [30, 31]. The immediate and follow-up results of
balloon angioplasty for post surgical recoarctation are es-
sentially similar to those of native coarctation and have been
reviewed in detail elsewhere [11, 19, 27, 29].
The indications for intervention are systemic hyperten-
sion with an arm-leg systolic blood pressure gradient in
excess of 20 mmHg. Once recoarctation is documented by
blood pressure measurements and confirmed by echo-
Doppler studies, balloon angioplasty should be performed.
Left-to-Right Shunt Lesions
In this section, three most common left-to-right shunt lesions,
namely, atrial septal defect (ASD), ventricular septal defect
(VSD) and patent ductus arteriosus (PDA) will be discussed.
Atrial Septal Defect
There are several types of defects in the atrial septum
namely, ostium secundum, ostium primum, sinus venosus
and coronary sinus ASDs and patent foramen ovale (PFO).
Ostium Secundum ASD in Children
The conventional treatment of choice for ostium secundum
ASD in the past was surgical correction; however, transcath-
eter methods are currently preferred for closure of ostium
secundum ASDs. Two devices namely, Amplatzer Septal
Occluder and HELEX device are currently approved by US
FDA for ASD closure; the Amplatzer Septal Occluder is
useful in most defects and HELEX device may be used for
small to medium sized ASDs. At the present time, surgical
repair is mostly used for defects with deficient septal rims in
which the interventionalist deems that defect could not be
closed with trans-catheter methodology or was unsuccessful
in occluding the ASD. In addition, if intra-cardiac repair of
other defects is contemplated, surgical closure of ASD could
be performed at the same sitting.
The indications for intervention are right ventricular vol-
ume overload by echocardiogram and a pulmonary to sys-
temic blood flow ratio (Qp:Qs) >1.5 (if the child had cardiac
catheterization). Elective closure around age 4 to 5 y is
recommended. Closure during infancy is not necessary un-
less the infant is symptomatic. Even though the patients do
not have symptoms at presentation, closure of the ASD is
Indian J Pediatr (January 2013) 80(1):32–38
performed in order to a) prevent development of pulmonary
vascular obstructive disease in adulthood, b) reduce proba-
bility for supra-ventricular arrhythmias at a later life and c)
prevent development of symptoms during adolescence and
adulthood.
Ostium Secundum ASD in Adults
In the past it was generally thought that closure ASDs in adult
subjects is not necessary unless they are symptomatic. More
recent analysis however, suggests that the ASDs should be
closed as and when they are identified in adult subjects [32].
However, when dealing with elderly subjects, precautions
should be taken (prior to ASD closure) in patients with re-
duced diastolic elasticity of the left ventricle [33] and pulmo-
nary hypertension [34], sometimes necessitating implantation
of fenestrated device to decompress the left ventricle [33] or
reduce the left to right shunt [34] respectively.
Ostium Primum, Sinus Venosus and Coronary Sinus ASD
These defects are not amenable to transcatheter closure and
surgical correction is the treatment of choice. In the ostium
primum defects, apart from closing the ASD, the mitral valve
cleft should be closed and the valve repaired in order to
preserve its function. In the sinus venosus defects, diversion
of the anomalously connected pulmonary veins into the left
atrium along with the closure of the ASD should be undertak-
en. In the coronary sinus defect, surgical correction with patch
closure of the defect, leaving the entry of coronary sinus in the
left atrium is the conventional method of approach. These
defects are not usually amenable to transcatheter closure.
However, some, particularly small, defects may be amenable
to transcatheter occlusion [35].
Although surgical correction can be performed at any age,
surgery in asymptomatic patients is usually recommended at
the age of 3 to 4 y. In the ostium primum defect, if symptoms
are present or if there is associated severe mitral insufficiency,
surgical repair may be performed at presentation, after medi-
cally controlling the heart failure.
Patent Foramen Ovale
Persistent patency of the foramen ovale in nearly one third of
normal population [36] makes the PFO a normal variant. Right
to left shunt through PFO can also occur in patients who had
prior correction of complex congenital cardiac anomalies; clo-
sure of these defects [37] is indicated to prevent hypoxemia and
paradoxical embolism. Some the PFOs in otherwise normal
hearts are considered to be the seat of right to left shunt causing
paradoxical embolism and cerebrovascular accidents [38–40]
and hypoxemia as seen in platypnea-orthodeoxia syndrome
[41, 42]. Decompression (Caisson’s) illness [43, 44] and
35
migrane [44] have also been attributed to right to left shunt
across PFO. There are varying degrees of evidence regarding
the benefits of transcatheter occlusion of PFOs in above de-
scribed conditions.
Ventricular Septal Defect
The treatment strategies depend, to a large degree, on the size
and type of the VSD [45]. Patch closure by open heart surgery
under cardiopulmonary bypass is the treatment of choice for
most VSDs. With regard to transcatheter techniques, currently
FDA has only approved Amplatzer muscular VSD occluder
for transcatheter closure of muscular VSDs. Results of clinical
trials with the device appear encouraging [46]. Some mem-
branous defects may have sufficient septal rim in the subaortic
region so that the device can be implanted without interfering
with aortic valve function. While the muscular VSDs can be
closed by transcatheter methodology, some large muscular
VSDs in small babies may be closed by hybrid procedures
via sternotomy and a purse-string suture in the right ventricle
under transesophageal echo guidance [47]. Specially designed
Amplatzer perimembranous VSD occluders have been used in
clinical trials [48, 49]. However, there was significant inci-
dence of heart block during follow-up [50]. No devices to
transcatheter occlude perimembranous VSD are currently ap-
proved. Endocardial cushion, outlet (supracrystal) and mala-
ligned VSDs require surgical closure, along with addressing
the associated cardiac anomalies.
In small VSDs, reassurance of the parents, subacute bacte-
rial endocarditis prophylaxis and periodic clinical follow-up
are all that are necessary. If however, prolapse of the aortic
valve into the defect causing aortic insufficiency is present,
surgical closure of the defect along with resuspension of the
aortic valve leaflets is in order. In moderate-sized defects,
treatment of heart failure, if present, should be undertaken.
Failure to thrive and markedly enlarged left atrium and left
ventricle are indications for surgical closure. In large defects
the heart failure should be treated aggressively. If congestive
heart failure is difficult to control with the usual anti-
congestive measures or if failure to thrive is present, surgical
closure should be undertaken.
In large defects with near systemic pressures in the right
ventricle and pulmonary artery, surgical closure should be
performed prior to 6 to 12 mo of age even if heart failure
control and adequate weight gain are present; this is to prevent
irreversible pulmonary vascular obstructive disease. This time
horizon may be shorter (before 6 mo of age) in patients with
Down syndrome. Total surgical correction is currently recom-
mended. The previously used approach of initial pulmonary
artery banding in small and young babies followed by surgical
closure of the VSD later is no longer recommended. However,
in muscular, Swiss-cheese variety of defects, initial pulmonary
artery banding may be appropriate.
36
When the pulmonary vascular resistance (PVR) is elevat-
ed, a comprehensive assessment, as reviewed elsewhere [51]
followed by testing of PVR response to oxygen and nitric
oxide (NO), pulmonary arterial wedge angiography and
sometimes, even lung biopsy may be necessary to determine
the suitability for surgical closure. Positive response of
pulmonary vascular reactivity has been variously defined
as decrease in mean pulmonary pressure (mPAP) by 20 %, a
decrease of more than 20 % in both mPAP and PVR index, a
fall in total pulmonary resistance of more than 30 %, a
decrease in mPAP of 10 mmHg or more along with reaching
a mPAP of 40 mmHg, all without a change in cardiac index,
a ratio of pulmonary to systemic vascular resistance (Rp:Rs)
<0.33 and a 20 % decrease in Rp:Rs and others. During four
decades of experience at several institutions of higher learn-
ing providing state of the art pediatric cardiac care, the
author has advocated surgery for patients with calculated
pulmonary vascular resistance less than 6 to 8 Wood units
with a Qp:Qs >1.5. Some authors state that it is difficult to
define a definite cut-off value, but PVR index less than 6
Wood units and/or a Rp:Rs less than 0.35 may permit safe
surgical repair [52]. In cases with higher pulmonary vascular
resistance, if the resistance drops to levels below 6 to 8 units
after administering oxygen and NO [52, 53], the patient
becomes a candidate for surgery. Large VSDs with severe
elevation of pulmonary resistance (irreversible pulmonary
vascular obstructive disease) are not candidates for surgery.
Patent Ductus Arteriosus
Until recently, surgical closure [54] of patent ductus arteriosus
(PDA) was the treatment of choice. Over last three decades
several, less invasive, transcatheter closure techniques were
developed [55, 56]. These transcatheter methods are increas-
ingly being used in closing PDAs. Currently, three devices are
approved and available for general clinical use; these are
Gianturco coil, Gianturco-Grifka vascular occlusion device
(GGVOD) and Amplatzer Duct Occluder. Amplatzer Duct
Occluder II may be available outside US, but is not approved
for general use. Because of the bulkiness of the device and
potential complications, GGVOD G [57] is not generally
used. The other two devices namely, Gianturco coil [58] and
Amplatzer Duct Occluder [59] are widely used for ductal
closure. Gianturco coil occlusion of the PDA can be per-
formed with small caliber catheters (#4 French) and is the
currently preferred method of occlusion for small sized ducti
[57, 58, 60]. For moderate to large-sized PDA, surgical [55,
61], video-thoracoscopic [62, 63] and transcatheter device
closure [56, 59, 64] are the available options, but most car-
diologists prefer transcatheter occlusion. Amplatzer duct
occluder is preferred for moderate to large PDA. Some types
of PDAs, particularly tubular ducts may require Amplatzer
vascular plugs or Amplatzer duct occluder II to close the PDA
Indian J Pediatr (January 2013) 80(1):32–38
[65, 66]. Issues related to ductal closure in premature babies
with respiratory distress syndrome are beyond the scope of
this presentation and will not be described here.
Children with clinical and echo-Doppler evidence of PDA
are candidates for ductal closure. The procedure is indicated
only in patients with continuous murmur suggestive of PDA
with echo-Doppler confirmation. Closure is not generally rec-
ommended in the so called "silent ductus" detected incidentally
without typical auscultatory features [67]. Very small and small
PDAs without hemodynamic overload are candidates for clo-
sure because of the risk of subacute bacterial endocarditis.
Medium- and large-sized ductus should be closed to prevent
further volume overloading of the left heart structures, treat
congestive heart failure and to prevent pulmonary vascular
obstructive disease apart from eliminating the endocarditis risk.
PDA closure can be performed at anytime, especially if asso-
ciated with heart failure or pulmonary compromise. If the
patient is asymptomatic, waiting until 6 to 12 mo of age is
generally recommended. Issues related to elevated pulmonary
vascular resistance and pulmonary vascular obstructive disease
should be addressed in a manner described in the VSD section.
Patients with ductal dependent congenital cardiac anomalies
and those associated with pulmonary vascular obstructive
disease should not undergo closure.
Conclusions
Methods of management of six most common acyanotic CHD
are briefly described in this, first part of this editorial. This is
followed by discussion of indications and timing of interven-
tion which are by and large determined by severity of the
lesion. Conceptually, these recommendations are not signifi-
cantly different from those made by the Working Group on
Management of Congenital Heart Diseases in India under the
auspicious of Cardiological Society of India, published not too
long ago [68, 69]. It is acknowledged that late presentation
and presence of co-morbidities in the Indian population may
pose a higher risk than seen in western counter parts. Based on
this review, it appears that the treatment of acyanotic CHD
with currently available medical, transcatheter and surgical
methods is feasible, safe and effective. Management options
and timing of intervention for common cyanotic congenital
heart defects will be described in Part II, in a future issue of
this Journal.
Acknowledgements The author of this editorial and the editors of
this Journal thank Dr. Bharat Dalvi of Glenmark Cardiac Centre,
Matunga (E), Mumbai, India; Dr. Vikas Kohli of Indraprastha Apollo
Hospital and Delhi Child Heart Center, New Delhi, India; Dr. R.
Krishna Kumar of Amrita Institute of Medical Sciences and Research
Center, Kochi, Kerala, India and Dr. I.B. Vijayalakshmi of Sri Jayadeva
Institute of Cardiovascular Sciences and Research, Bangalore, India for
their review and constructive criticism of this paper.
Indian J Pediatr (January 2013) 80(1):32–38
Conflict of Interest None.
Role of Funding Source None.
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