Cardiology in the Young Transcatheter pulmonary balloon valvuloplasty
cambridge.org/cty
Brief Report
Cite this article: Siagian SN, Haas NA, and
Prakoso R (2021) Transcatheter pulmonary
balloon valvuloplasty of severe valvar
pulmonary stenosis and atrial septal defect in
patient with severe cyanosis and very low
ventricle ejection fractions: a bailout
procedure. Cardiology in the Young 31:
2028–2030. doi: 10.1017/S1047951121001967
Received: 10 November 2020
Revised: 10 March 2021
Accepted: 27 April 2021
First published online: 21 May 2021
Keywords:
Pulmonary stenosis; atrial septal defect;
pulmonary balloon valvuloplasty; low ejection
fraction; cyanosis; bailout
Author for correspondence:
Sisca N. Siagian, Department of Cardiology and
Vascular Medicine, Faculty of Medicine
Universitas Indonesia, National Cardiovascular
Center Harapan Kita, Jakarta, Indonesia.
E-mail: sisca.ped.car@gmail.com
© The Author(s), 2021. Published by Cambridge
University Press.
https://doi.org/10.1017/S1047951121001967 Published online by Cambridge University Press
of severe valvar pulmonary stenosis and atrial
septal defect in patient with severe cyanosis and
very low ventricle ejection fractions: a bailout
procedure
Sisca N. Siagian1 , Nikolaus A. Haas2 and Radityo Prakoso1
1
Department of Cardiology and Vascular Medicine, Faculty of Medicine Universitas Indonesia, National
Cardiovascular Center Harapan Kita, Jakarta, Indonesia and 2Department Pediatric Cardiology and Pediatric
Intensive Care, Ludwig Maximilians University, University Hospital, Muenchen, Germany
Abstract
Most cases of severe or critical pulmonary stenosis are detected early and interventional man-
agement is routine within the first days of life. We present a case of a thirteen-year-old boy
diagnosed with pulmonary stenosis and atrial septal defect with low ventricle ejection fraction.
The patient underwent staged pulmonary balloon valvuloplasty and interventional atrial septal
defect closure with good results.
In cases with severe or even subatretic valvar pulmonary stenosis and substantial right ventricu-
lar hypertrophy, diastolic dysfunction develops and right ventricle filling is impaired.1 Left
alone, severe right ventricle outflow tract obstruction with severe cyanosis may cause biventric-
ular dysfunction.2 We present a case of a teenager with combined pulmonary stenosis and an
atrial septal defect with severe cyanosis and secondary biventricular heart failure.
Case report
A 13-year-old boy came to the emergency room with severe breathlessness that worsened one
day before admission. The blood pressure was 123/81 mmHg, and heart rate was 100 bpm with
severe cyanotic (oxygen saturation 40–50%). Echocardiography examination revealed severe
valvar pulmonary stenosis with minimal forward flow to the pulmonary artery and a pressure
gradient of 110 mmHg, moderate tricuspid regurgitation, right to left shunt secundum atrial
septal defect, and biventricular dysfunction (ejection fraction of 20% and TAPSE 1.0 cm).
The patient was originally discussed to undergo surgical valvotomy, but was rejected due to
the poor general condition and the estimated high perioperative risk. Therefore, pulmonary bal-
loon valvuloplasty was deemed the only favourable approach.
Right ventricular and pulmonary artery pressures were measured, then right ventriculogra-
phy was performed. The pulmonary valve was found to be subatretic (Fig 1a) and could only be
crossed with straight 0.035”RADIOFOCUSR guidewire (Terumo, Tokyo, Japan). As reperfusion
oedema was deemed possible, a staged approach was chosen, and only incomplete balloon dila-
tation was performed, using a TyshakR balloon catheter 12 × 30 cm (NuMed, Cornwall, Canada)
(Fig 1b, c, d). The pressure gradient only decreased marginally, and peak-to-peak transvalvular
pressure gradient decreased from 88 mmHg to 67 mmHg. The oxygen saturation, however,
improved immediately, and therefore, the initial target of the procedure was achieved despite
the significant resting gradient.
Following the initial valve dilation, normal saturation, and oxygen delivery to the body,
marked improvement in peripheral oedema, regression of hepatomegaly, and onset of massive
diuresis were observed. His haemodynamic was improving, and he was discharged on the
seventh day. After 6 months, we performed the scheduled re-dilation of the pulmonary valve.
Peak-to-peak transvalvular pressure gradient decreased, and the right ventricular end diastolic
pressure decreased to 12 mmHg compared to 23 mmHg at the initial intervention (Fig 2).
Evaluation in the following year showed left to right atrial septal defect; therefore, it was closed
percutaneously.
Discussion
In severe cases of pulmonary valve stenosis, severe right ventricular hypertrophy will cause dia-
stolic dysfunction.3 This patient’s right ventricle was exposed to high pressures from the
 Cardiology in the Young
Figure 1. Pulmonary Balloon Valvuloplasty Procedure: A. Right
ventriculography in lateral projection showed sub-atretic pulmo-
nary valve with pulmonary valve annulus size of 25 mm. B, C and
D showed balloon (TyshakR balloon catheter 12 × 30 cm, NuMed,
Cornwall,Canada) dilated progressively to maximum until bal-
loon silhouette disappear, as indicated by the arrow.
Figure 2. End-diastolic pressures of the RV at initial intervention and after the second procedure. RVEDp decreased from 23 mmHg (a) to 10 mmHg (b).
obstructed outflow tract and volume overload due to the atrial sep-
tal defect. The coronary blood flow may be impaired, causing
ischaemia and cardiomyocyte apoptosis and ultimately leads to
dilatation and stiffening of right ventricle, further impairing the
stroke volume. Finally, the profound cyanosis will aggravate the
deterioration of biventricular myocardial impairment.4
This case presented a clinical challenge due to the presence of
biventricular failure. A study by Pieri et al mentioned the higher
risk of complications in patient with low left ventricle ejection frac-
tion.5 Thus, staged valvuloplasty is the preferred treatment of
choice.6 Following percutaneous balloon valvuloplasty, there was
a high incidence of pulmonary oedema, and pulmonary haemor-
rhage in severe cases. Relieving the right ventricular obstruction at
https://doi.org/10.1017/S1047951121001967 Published online by Cambridge University Press
2029
once may result in a high preload to the left heart for which the left
ventricle may not be prepared, also the reperfusion damage may
occur within the pulmonary vascular bed due to a great haemody-
namic change. Many literatures said none of the patients who
underwent graded dilation developed evidence of reperfusion
injury/pulmonary oedema.6 The recovery of left and right ven-
tricles function in this patient proved that the dramatic increase
in saturation was the key factor in the improvement of myocardial
function and subsequent cardiac output, organ perfusion, and
recovery. This could be achieved by submaximal dilatation of
the pulmonary valve alone. In addition, the improvement of right
ventricle function reversibly increased the performance of left ven-
tricular function.1,6,7
 2030
Supplementary material. To view supplementary material for this article,
please visit https://doi.org/10.1017/S1047951121001967
Acknowledgements. We would like to thank Oktavia Lilyasari, MD, and
Bayushi Eka Putra, MD for their valuable guidance and support.
Financial support. The author(s) received no financial support for the
research, authorship, and/or publication of this article.
Conflicts of interest. None.
Ethical standards. No human and/or animal experimentation was conducted
in this study.
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