Proceedings of the Fourth Scientific Meeting of the World Society for Pediatric and Congenital Heart Surgery

World Journal for Pediatric and

Congenital Heart Surgery
Catheter Interventions for Mitral Stenosis 2015, Vol. 6(2) 250-256
ª The Author(s) 2015
Reprints and permission:
in Children: Results and Perspectives sagepub.com/journalsPermissions.nav
DOI: 10.1177/2150135114568785
pch.sagepub.com

Anita Saxena, MD, DM, FACC, FAMS1

Abstract
Stenosis of the mitral valve most often occurs as a result of chronic rheumatic heart disease, causing thickening and fibrosis of the
mitral valve apparatus. Rheumatic heart disease continues to be a major public health problem in several developing countries and
mitral stenosis is also common in these regions. According to the reports from India and Africa, the disease tends to follow a
rapidly progressive course in children. The treatment of choice is balloon dilatation of the mitral valve. Echocardiography is
indispensable for this procedure. Before planning the procedure, it is essential to assess the suitability of balloon dilatation.
Echocardiography performed during the procedure helps to decide whether the size of the balloon needs to be increased in case
of inadequate relief of stenosis. Most published series have reported an immediate success rate of over 90% with balloon dilatation
in children and young adults. With an increase in mitral valve area and improvement in functional class, the left atrial pressure and
the transmitral gradients fall. These gratifying results are also reported from very young children of less than 12 years of age. It is
recommended to start with a smaller balloon size and increase its size in a stepwise fashion to minimize complications. The
complications, seen in about 1% to 2% of cases, include development of significant mitral regurgitation and hemopericardium,
secondary to cardiac chamber perforation. The long-term results indicate slightly higher restenosis rates in children than in adults.
Most children with restenosis can undergo successful repeat dilatation.

Keywords
heart valve, interventional catheterization, mitral valve disease, echocardiography

Submitted August 27, 2014; Accepted December 17, 2014

Presented at the 4th Scientific Meeting of World Society for Pediatric and Congenital Heart Surgery, Sao Paulo, Brazil; July 17-20, 2014.

Introduction in some of the developing countries severe MS at a younger age

Mitral stenosis (MS) refers to restricted opening of the mitral
valve orifice and occurs as a result of abnormally thickened
mitral valve leaflets. In vast majority of instances, the underly-
ing etiology is chronic rheumatic heart disease (RHD). Rarely
MS is secondary to congenital abnormality of the mitral valve.
Generally, the whole of mitral valve apparatus including leaf-
lets, chordate tendinae, and papillary muscles are involved,
irrespective of the underlying etiology. This article will discuss
indications, technique, and results of catheter interventions for
MS, developing secondary to RHD.
Rheumatic heart disease continues to be a major public health
problem in several developing countries, and MS secondary to
RHD is also commonly seen in these regions. Rheumatic fever
results in carditis in more than 60% of cases, affecting the car-
diac valves (mitral valve being most commonly affected), which
over a variable period of time causes stenosis and/or regurgita-
tion of the valve. Mitral stenosis results from leaflet thickening,
commissural fusion, chordal fusion, and/or shortening, all sec-
ondary to chronic rheumatic process. The usual age at presenta-
tion is beyond 20 years or so, that is, in the adult age. However,
is not uncommon. In these regions, MS tends to progress very
rapidly, following a malignant course. Young children and ado-
lescents present with advanced symptoms. Worldwide 75% to
80% of patients with RHD are children and young adults.
Mitral Stenosis in Children
In several developing countries including Africa and India,
severe MS develops at a very young age.1-4 It is generally
believed that 25% of patients with MS are <20 years of age and
10% are <12 years of age in developing countries. Dr S. B. Roy
from India coined the term ‘‘juvenile MS’’ for patients present-
ing with severe MS at an early age of <20 years.5 Juvenile MS
1
Department of Cardiology, All India Institute of Medical Sciences, New Delhi,
India
Corresponding Author:
Anita Saxena, Department of Cardiology, All India Institute of Medical Sciences,
New Delhi 110029, India.
Email: anitasaxena@hotmail.com

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Saxena
Abbreviations and Acronyms
2D two-dimensional
3D three-dimensional
AHA/ACC American Heart Association/American
College of Cardiology
BMV balloon mitral valvuloplasty
MS Mitral stenosis
MVA mitral valve area
RHD rheumatic heart disease
has a rapidly progressive course of the disease with severe valve
and subvalvular deformity. These patients present in advanced
functional class and have evidence of severe pulmonary hyper-
tension. The majority continue to maintain sinus rhythm, and
atrial fibrillation is uncommon. These patients are also unlikely
to have thrombus in left atrium, which is not an unusual accom-
paniment of MS in adults and middle-aged patients. Throm-
boembolic episodes are uncommon in juvenile MS patients.
Catheter Interventions for Mitral Stenosis in Children
Before the advent of balloon mitral valvuloplasty (BMV),
patients with severe MS were treated with closed or open mitral
commissurotomy through a thoracotomy. The idea of balloon
dilatation of mitral valve for patients with MS was first
described in 1982 by a Japanese cardiac surgeon Dr Kanji
Inoue.6 He demonstrated that a stenosed mitral valve can be
opened by inflating a balloon made of strong but pliant natural
rubber. The inflated balloon will split open fused commissures
of the mitral valve, in a manner similar to the opening of the
mitral valve at the time of closed mitral commissurotomy,
relieving the obstruction. Lock et al first reported the use of a
cylindrical balloon for BMV.7 Later several reports were
published, some of which included children and young adults.
Practitioners in Saudi Arabia introduced the double-balloon
technique using two cylindrical balloons over two guidewires.8
However, this technique was more demanding and required
longer procedure time and was likely to be associated with
higher rates of complications. The most preferred balloon cur-
rently is the Inoue balloon (Toray, Tokyo, Japan), which is
used world over due to the simplicity of the technique and grat-
ifying results. In 1997, a metal commissurotome was intro-
duced for percutaneous relief of MS, claiming that it achieved
better mitral valve area (MVA), but again, it did not become
very popular.9
Mechanism of relief of MS by BMV. Mitral valve commissures,
one or both, split open when an appropriate-sized balloon is
inflated across the valve, resulting in enlargement of the mitral
valve orifice. The degree of increase in MVA and subsequent
restenosis rates are generally better than the results of closed
mitral commissurotomy.10 Rarely, however, the mitral leaflets
or chordate tendinae may rupture or tear during the procedure.
This complication is more likely to occur in those with severe
subvalvular deformity and in those with calcified or heavily
fibrosed commissures. Use of a larger than recommended
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251
balloon size can also produce damage to the valve leaflets or
chordae tendinae, causing acute mitral regurgitation, one of the
most feared complications of this procedure.
Indications for BMV
Indications for BMV in adults were published in 2014 by the
American Heart Association/American College of Cardiology
(AHA/ACC) expert group in the guidelines for management
of valvular heart disease.11 According to these criteria, BMV
is a class I indication for symptomatic MS with MVA of
1.5 cm2 who have a favorable valve morphology, no left
atrial clot, and no or mild mitral regurgitation. Balloon mitral
valvuloplasty is considered a class IIa indication for patients
with very severe MS, even if they are asymptomatic, where
MVA is 1.0 cm2, provided the valve morphology is good. For
asymptomatic severe MS (MVA between 1 and 1.5 cm2), BMV
is a class IIb indication if the valve morphology is favorable.
Therefore, BMV should be performed in all symptomatic
patients with severe or very severe MS, when the valve mor-
phology is favorable, there is no thrombus in left atrium, and
the mitral regurgitation is absent or mild. Currently, closed
mitral commissurotomy has no role in the management of
patients with MS. Even open mitral commissurotomy is hardly
ever indicated. Those with unfavorable valve morphology are
best treated with mitral valve replacement. According to the
AHA/ACC guidelines, if the MVA is >1.5 cm2, the patients are
symptomatic with no other apparent cause, and the pulmonary
capillary wedge pressure is >25 mm Hg on exercise, BMV is a
class IIb indication in such patients.
Unfortunately there are no separate criteria for children, and
the criteria for adults may not be applicable to children who are
likely to have a much smaller body surface area. It is therefore
advisable that MVA in children should be interpreted accord-
ing to the body surface area and indications modified accord-
ingly. Since the majority of children with severe MS present
in advanced functional class with significant dyspnea, BMV
is indicated in all these cases.
Contraindication to BMV include (a) a thrombus in left
atrium or left atrial appendage, (b) mitral regurgitation severity
more than mild, (c) significant calcification of mitral valve
leaflets or commissures, (d) significantly associated aortic
valve disease, and(e) severe organic tricuspid valve disease.
Role of Echocardiography
Echocardiography is indispensable for patients undergoing
BMV. It is required before, during, and after the procedure.
Before the BMV procedure. Echocardiography is the best tool for
determining the severity of MS and the BMV suitability. Mitral
valve area can be derived by pressure half-time method, plani-
metry, or by continuity equation, using cross-sectional echocar-
diography and Doppler (Figure 1a and b). The anatomy and
morphology of the mitral valve is very meticulously determined
by cross-sectional echocardiography. The degree of leaflet
252
Figure 1. Echo-Doppler for assessment of mitral valve area by pressure half-time (A) and by planimetry (B) in two separate patients.
Figure 2. Echocardiography in parasternal long-axis view showing
thickened mitral valve with doming of the anterior leaflet (arrow).
Note the dilated LA. LA indicates left atrium; LV, left ventricle.
thickening and mobility and the severity of subvalvular defor-
mity, which may determine the safety and feasibility of the BMV
procedure, are best defined by echocardiography (Figure 2). The
anatomy of mitral valve apparatus can be described using the
Wilkins score.12 This scoring system assigns a point value from
1 to 4 for (a) valve mobility, (b) valve thickening, (c) valve cal-
cification, and (d) subvalvular thickening. A score of <8 is con-
sidered suitable for BMV. Those with a score of more than 10
may not be the candidates for BMV and surgery should be con-
sidered, especially if there is associated mitral regurgitation. One
should also carefully assess the state of commissural fusion and
subvalvular apparatus prior to BMV procedure.
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World Journal for Pediatric and Congenital Heart Surgery 6(2)
The other important role of echo is to rule out a thrombus in
the left atrium. The left atrium and left atrial appendage must
be carefully screened for the presence or absence of a throm-
bus, especially in patients who have atrial fibrillation. Patients
with atrial fibrillation or those with poor echo windows may
need a transesophageal echocardiography to rule out left atrial
thrombus, a contraindication to BMV. Fortunately, the major-
ity of children and young adults with MS show sinus rhythm
and the likelihood of left atrial thrombus is low. Balloon mitral
valvuloplasty has been successfully performed in the presence
of a left atrial appendage thrombus, but there is always a risk
of thromboembolism.13 It is advisable to anticoagulate such
patients adequately for three to six months and confirm the
absence of thrombus before proceeding with BMV. If the
thrombus persists, the patient is best referred for surgery, and
the left atrial thrombus can be removed at the time of surgery.
Echo-Doppler can estimate the severity of pulmonary hyper-
tension by measuring the tricuspid regurgitation jet velocity
and using Bernoulli equation.
Rheumatic heart disease may also affect the aortic valve in a
significant number of cases with MS, producing moderate or
severe stenosis or regurgitation of the aortic valve, which may
contraindicate BMV. Echo-Doppler helps to assess aortic valve
lesions with great accuracy.
During the BMV procedure. One prefers to have echocardiogra-
phy equipment available in the catheterization laboratory at the
time of BMV. The MVA can be determined immediately after
the procedure using planimetry. Echocardiography is very use-
ful to detect mitral regurgitation or cardiac temponade, the two
most serious complications of the procedure.
After the BMV procedure. Following BMV, the transmitral gradi-
ents immediately fall and the severity of pulmonary hyperten-
sion reduces, indicating that the procedure is successful. In case
Saxena
the patient develops mitral regurgitation, its mechanism can be
easily identified and the patient can be managed accordingly.
In those with mitral regurgitation due to valve tear or chordal
avulsion, urgent surgery for repair or replacement of the mitral
valve is often required, whereas commissural mitral regurgita-
tion, which is not severe, can be closely followed and conser-
vatively managed.
Echocardiography for long-term follow-up. Balloon mitral valvulo-
plasty is a palliative procedure, and all these children need
follow-up over a long period of time. The MVA, gradients
across the mitral valve, severity of pulmonary hypertension,
and development of restenosis are all very well evaluated by
echocardiography and Doppler. Those who develop restenosis
can be taken up for repeat BMV if the echo-Doppler confirms
favorable valve morphology.
Role of three-dimensional echocardiography in MS. Real-time
three-dimensional (3D) echocardiography provides an en face
view of the complete mitral apparatus, defining the valve mor-
phology in a much better manner. The 3D echo is better than
the two-dimensional (2D) echocardiography because it identi-
fies the ideal plane where the planimetry should be done for
MVA calculation. The likelihood of obtaining good images is
greater with 3D than with 2D echocardiography. It has been
observed that the MVA measured by 3D is less than that mea-
sured by 2D echocardiography, but 3D is more likely to be cor-
rect for estimating mitral orifice area for the reasons mentioned
previously. The 3D echocardiography also allows excellent
assessment of commissures, both before and after BMV.14,15
In those who develop acute mitral regurgitation, the 3D echo-
cardiography may be superior to the 2D echocardiography in
defining the regurgitation mechanism.
Technique of the BMV Procedure
The procedure is performed via a transfemoral vein approach,
internal jugular vein has been rarely used,16 especially in those
where the femoral route is not available. Light sedation is
required in children. The right heart catheterization is done.
A small-sized sheath is placed in the femoral artery. An arterial
catheter such as a pigtail catheter is placed in the aortic root
through this sheath. Next an interatrial septal puncture is done
using the Brockenbrough needle introduced from the femoral
vein, within the sheath. The puncture is attempted in the fossa
ovalis region. Needle entry into the left atrium is confirmed by
high pressure in the chamber and by withdrawing a sample of
blood that is fully oxygenated. One can also inject 2 to 3 mL of
the contrast agent gently to confirm the left atrial entry. This is
an important step in the procedure, since the perforation of
atrium or injury to the aorta can occur at this stage, leading
to cardiac temponade. Once successful septal puncture is done,
unfractionated heparin is given intravenously, usually in a dose
of 100 u/kg. The needle is withdrawn after pushing the Brock-
enbrough sheath into the left atrium and a coiled wire is then
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253
placed in the left atrium through the sheath over which the
dilating balloon is passed.
Selection of balloon size. In most instances the Inoue balloon is
used. The sizing is calculated by the simple formula height in
centimeter/10 þ 10.17 However, the relationship of one’s height
with mitral valve orifice diameter is not linear and this formula is
not validated in children. When this formula is used, it is better
to start with an undersized balloon, 2 to 4 mm less than the cal-
culated balloon size, in order to avoid injury to mitral valve
apparatus.18 This is especially true for very young children aged
less than 15 years. Another method for balloon sizing is to mea-
sure the actual mitral annular diameter on echocardiography in
the apical four-chamber or two-chamber view. The annular dia-
meter is measured during mid to end systole. Since different val-
ues are likely to be obtained at different views, it is better to start
with the smallest balloon size. Irrespective of the method used, it
is better to start with a smaller balloon size and the balloon dia-
meter can be gradually increased in a stepwise manner to avoid
unnecessary injury to mitral valve leaflets.
The Inoue catheter is then advanced from left atrium to left
ventricle across the mitral valve. Slight inflation of balloon
using 1 to 2 mL of dilute contrast helps to make it float from
left atrium to left ventricle. After this, the distal part of the bal-
loon is inflated with dilute contrast agent using a special grad-
uated syringe. The balloon catheter is then pulled back and its
middle part or waist is placed across the mitral valve, and it is
fully inflated with a preset amount of dilute contrast agent.
When the waist in the middle of balloon disappears, it is rapidly
deflated (Figure 3). The pressure in left atrium is measured and
if the transmitral gradients are still high, the procedure is
repeated with a balloon of bigger size by filling it with more
dilute contrast solution. The volume of the contrast solution
in the balloon should be increased gradually so as to increase
the balloon diameter in 1-mm increments until the pressure
in the left atrium falls and the transmitral gradients are abol-
ished or are negligible. It is very important to measure the left
atrial pressure after each increment in balloon size, and the
appearance of tall v waves may indicate the development of
mitral regurgitation. Echocardiography should be done after
each dilatation. One can assess MVA (by planimetry and not
by pressure half-time method), splitting of commissures, and
presence or absence of mitral regurgitation.
Results of BMV
Success of the BMV procedure is defined as (a) >50% increase
in the MVA, (b) final MVA of >1.5 cm2 or 1 cm2/m2, (c)
absence of significant mitral regurgitation, and (d) absence of
any complication. All these criteria must be satisfied.
Immediate Results
Immediate success is obtained in more than 90% of cases,
seen as immediate fall in left atrial pressure, reduction in
transmitral gradients, fall in pulmonary pressures, increase in
MVA, and improvement in functional class. Children who are
254 World Journal for Pediatric and Congenital Heart Surgery 6(2)

Figure 3. Fluoroscopy images during mitral valve balloon dilatation with the Inoue balloon showing initial inflation of distal balloon (A), partial
inflation across mitral valve (B), and fully inflated balloon across mitral valve (C).

Table 1. Published Series on Mitral Valve Balloon Dilatation in Patients Younger Than 20 Years of Age.

Age in years MVA Pre-BMV, MVA Post-BMV, Success,

Author, year Country No. (mean) cm2 cm2 % Complications

Joseph et al, 1997 India 107 10-18 (14.5 þ 2.3) 0.73 + 0.18 1.7 + 0.53 98 MVR-1, temponade-1,
Mod MR-5
Zaki et al, 1999 Egypt 46 7-19 (15.5 þ 3.2) 0.65 + 0.14 1.54 + 0.23 98 MVR-1, CVA-1
(indexed) (indexed)
Mattos et al, 1999 Brazil 40 10-18 (15.5 +2.2) 0.86 + 0.21 2.03 + 0.50 91.7 MVR-3, temponade-1
Yonga and Nairobi 45 9-20 (14 þ 2.6) 0.6 + 0.2 1.9 þ 0.2 100 MR-0
Bonhoeffer, 2003
Gamra et al, 2003 Tunisia 110 <20 (16 + 2.8) 0.9 +0.2 2.2 + 0.4 100 MR-0
0.63 +0.1 1.57 +0.2
(indexed) (indexed)
Fawzy et al, 2005 Saudi Arabia 84 10-20 (16.7 + 3.3) 0.84 + 0.2 2.0 + 0.59 98 Temponade-1, MR-0
Harikrishnan et al, India 33 18.1 + 2.1 0.89 + 0.2 1.79 + 0.29 93 MVR-1, MR-2,
2006 temponade-1
Fawzy et al, 2008 Saudi Arabia 57 10-18 (15.3 + 2.4) 0.85 + 0.19 1.99 þ 0.57 98.3 Temponade-1

Abbreviations: BMV, balloon mitral valvuloplasty; CVA, cerebrovascular accident; MVA, mitral valve area; MR, mitral regurgitation.

Table 2. Published Series on Mitral Valve Balloon Dilatation in Very Young Patients.

Age in years MVA pre MVA post

Author, year Country No. (mean) BMV, cm2 BMV, cm2 Success, % Complications

Krishnamoorthy and India 13 <12 <1 >1.5 93% Mod MR-2, MVR-0, temponade-0
Tharakan, 2003
Kothari et al, 2005 India 100 7-12 (11 + 1.2) 0.73 + 0.14 1.7 + 0.4 94% MVR-2, MR-2, suboptimal result-2
Shrestha et al, 2013 Nepal 100 7-15 (13 + 1.6) 0.7 + 0.15 1.5 + 0.32 94% Nil
Abbreviations: BMV, balloon mitral valvuloplasty; MR, mitral regurgitation; MVA, mitral valve area; MVR, mitral valve replacement.

in functional class III or IV show a very dramatic improvement
in dyspnea, which is obvious immediately after the mitral valve
has been dilated. Some of the published series on the results of
BMV in young adults are shown in Table 1.18-25 Table 2
describes three series of BMV in very young children.26-28
A case report from India describes successful BMV in a
four-year-old child with MS secondary to RHD.29
Complications. Most often complications occur either during the
interatrial septal puncture or during balloon dilatation of the
valve. Mortality is rare and in most recent series it is nil. One
of the serious complications is cardiac temponade, occurring
secondary to perforation of one of the chambers of heart or
aorta leading to homopericardiam. Its incidence varies from
0% to 2% in various series. Immediate treatment includes

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Saxena
reversing heparin effect by protamine sulfate and inserting a
pigtail catheter in the pericardium for drainage of blood. If
the bleeding does not stop, one has to resort to surgical explo-
ration. Since this complication is most likely to occur at the
time of interatrial septal puncture, most centers give heparin
only after the septal puncture has been done successfully so
as to minimize accumulation of blood in pericardium, should
perforation occur.
Appearance of, or an increase in mitral regurgitation, is the
most common complication of BMV, seen in up to 20% of
cases. However, in most cases, the degree of regurgitation is
mild or low moderate, secondary to excessive commissure
opening, and does not require surgical intervention. Severe
degree of mitral regurgitation is seen in 1% to 2% of cases
and often requires early or urgent surgical intervention. The
mechanism of severe mitral regurgitation is most often due to
mitral leaflet rupture (in 70%). Posterior leaflet tear is rela-
tively uncommon (10%). Other causes include commissural
tear with annular involvement.30 Rare complications include
cerebral stroke secondary to thromboembolism (less than
1%) and residual small shunts at atrial septal level (10%), the
shunt being <1.5:1 in majority. Local vascular complications
are more likely in children (1%-3%) as large-sized sheath (9F
or 10F) is placed in the femoral vein.
Long-term results. Children and young adults with favorable
valve morphology are likely to have best immediate and
long-term results. Over 90% of patients with successful BMV
are alive and free of intervention for more than five to seven
years after the initial procedure. The report from India of 100
children younger than 12 years of age reported an event-free
survival of 75.4% + 8.7% (95% confidence interval 53.3%-
88.1%) at 100 months.27 In another study from India of 38
children younger than 12 years of age, event-free survival at
one, three, and five years was 97.1%, 91.4%, and 88.57%,
respectively.31 In this series, 78.2% + 7.5% of children were
in functional New York Heart Association class I or II and free
of any intervention at five years. In the study from Tunisia, data
of 110 patients with juvenile MS (age  20 years, mean 16 +
2.8years) were compared with 554 adults with MS (age 37 +
11.9 years).22 At follow-up, MVA, indexed for body surface
area, was higher in younger patients. At 10 years, event-free
survival was 74% in patients with juvenile MS when compared
to 69% in older patients (P ¼ .15). In the study from Saudi
Arabia of 57 children aged 18 years, event-free survival
rates at 10, 15, and 18 years were 87% + 6%, 62% + 1%, and
20% + 2%, respectively.25 These results were not different
from that reported in adults (474 patients) in the same study.
Restenosis in the long term. In a study from India,31 restenosis by
echocardiography occurred in 5 (14.3%) of 33 cases at 24
months, 4 of those had successful repeat BMV. Similarly, in the
other report from India, 14 (16%) of 94 patients had echocar-
diographic restenosis over a mean follow-up of 34.4 + 25.9
months. Of these 14 patients, 7 underwent a repeat BMV. In the
study from Tunisia, freedom from restenosis was 61% in the
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255
group with juvenile MS.22 A relatively high restenosis rate is
reported from Saudi Arabia.25 Restenosis developed in 26%
of 57 children younger than 18 years, at a follow-up of 1.5 to
18 years (mean 8.5 + 4.8 years). A Wilkins score of more than
8 on echocardiography was predictive of restenosis. Actuarial
freedom from restenosis at 10, 15, and 18 years was 78% +
7%, 64% + 9%, and 18% + 14%, respectively. These results
were not different from the results obtained in 474 adult
patients in this study. In an earlier data from Brazil, restenosis
developed in 5 of 32 patients aged 18 years following BMV
at a follow-up of 29.7 + 11.9 months.20 All the five patients
were successfully redilated.
Conclusion
Mitral stenosis is still a common disease in regions where RHD
is prevalent. In children, it tends to follow a relatively rapid,
progressive course. Balloon mitral valve dilatation is the treat-
ment of choice for these children, and most centers use the
Inoue balloon for this purpose. It is advisable to start with a rel-
atively smaller balloon size in children to avoid complications.
The immediate and long-term results and the complication
rates in children are comparable to the results in adults. Reste-
nosis on long-term follow-up may be slightly more when com-
pared to adults, especially in those younger than 12 years.
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to
the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research,
authorship, and/or publication of this article.
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