Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation

Abstract

Objective Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long‐term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure. Design, Settings, and Patients We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there were no absolute contraindications. Intervention and Outcome Measures Demographic and procedural data were correlated with outcome measures. Outcomes analyzed included procedural success, reintervention rates, final circulation type, and functional class. Multivariate analysis and receiver operator curve were used to identify for parameters in predicting biventricular circulation. Results The procedural success rate was 92% (33 out of 36) in this group with moderate right ventricular hypoplasia (tricuspid valve z score −4.2 ± 3.0, 69.4% of patients with z score <−2.5). Early reintervention rate was 39%, mostly being insertion of modified Blalock–Taussig shunt. Overall reintervention‐free survival was 53%, 30%, and 19% at 1, 6, and 12 months postintervention. Despite no significant catch‐up right ventricular growth, majority of survivors (84%) enjoyed a biventricular circulation with good functional status. A tricuspid to mitral valve ratio >0.79 was a good predictor of biventricular outcome. (specificity of 100%, positive predictive value 100%) Conclusion Encouraging long‐term results with biventricular circulation and functional status were demonstrated with transcatheter pulmonary valve perforation in patients even with moderate hypoplastic right ventricle, which is comparable to that with mild right ventricular hypertrophy. The baseline tricuspid to mitral valve ratio was identified as a potentially useful tool in predicting biventricular circulation.

Outcome of Membranous Pulmonary Valve Atresia with Intact Ventricular Septum Treated by Cardiac Catheterization in a Developing Country

“…16 Even with moderate right ventricular hypoplasia, achievement of 2-ventricular outcome is very likely with perforation and dilation of the pulmonary valve, with or without the need for additional pulmonary blood ow. 17,18 In our series 12 out of 14 survivors (85%) achieved 2-ventricular outcome, while one ended up with 1.5 ventricle, and one with uni-ventricular outcome. In the twelve patients with 2-ventricular outcome, we demonstrated signi cant growth of tricuspid valve annulus despite the small sample size with increase of tricuspid valve annulus z-score from − 1.4 (± 0.83) to -0.83 (± 0.80), p = 0.042.…”

Type I and II pulmonary atresia with intact ventricular septum in infants: a 10-year experience in initial surgery at one center

“…Although much more surgical methods of right ventricular decompression are available for PA/IVS [ 4 , 8 – 12 ], surgical failure [ 13 ] and complications [ 14 – 17 ] are also universal. Hypoxemia is one of the most common complications for the following reasons: (1) SpO2 in the early stage is not well maintained because of muscle edema of the right ventricle; (2) Hypoplastic right ventricle is not capable of maintaining the sufficient forward blood flow after the opening of pulmonary valve [ 18 ], and the pulmonary vascular bed pressure that has not been fully decreased in the neonatal period also limits the blood flow.…”

Is there a transcatheter solution for a sick neonate with hypoplastic right heart syndrome?: Pulmonary valve perforation in a neonate with hypoplastic right ventricle with pulmonary atresia, restrictive VSD—a case report

“…In a series of 13 patients who underwent RVOT perforation, there was significant growth of the TV annulus and reduction in TR during at a mean follow-up of 13.2 months [5]. On the contrary there have been studies where there was no significant growth of the right ventricle following perforation [6][7][8]. The surgical aorto pulmonary shunting is a well-established palliative surgery in HRHS, but carries an increased risk in underweight neonates.…”