Pulmonary Atresia With Intact Ventricular Septum With Borderline Tricuspid Valve: How Small Is Too Small

PA/IVS is a rare, heterogenous congenital heart defect anatomically defined by complete obstruction to the right ventricular outflow tract with varying degrees of hypoplasia of the right ventricle and tricuspid valve. This lesion can have associated coronary artery anomalies and, in some cases, righ...

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Bibliographic Details
Published in:Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual 2019, Vol.22, p.27-31
Main Authors: LaPar, Damien J., Bacha, Emile
Format: Article
Language:English
Subjects:
Biventricular repair
Clinical Decision-Making
Congenital heart defect
Heart Defects, Congenital - surgery
Humans
Infant, Newborn
Intact ventricular septum
Neonate
Patient Selection
Pulmonary atresia
Pulmonary Atresia - surgery
Tricuspid Valve - abnormalities
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Summary:PA/IVS is a rare, heterogenous congenital heart defect anatomically defined by complete obstruction to the right ventricular outflow tract with varying degrees of hypoplasia of the right ventricle and tricuspid valve. This lesion can have associated coronary artery anomalies and, in some cases, right ventricular-dependent coronary circulation. Due to the wide spectrum of presenting anatomic and clinical features, the treatment options are often dictated by the degree of development of the tricuspid valve and right ventricle. The purpose of this review is to discuss the spectrum of pulmonary atresia with intact ventricular septum morphologies and to evaluate the surgical decision-making process and approaches to surgical repair with respect to the impact of hypoplastic right-sided cardiac features.
ISSN:1092-9126
DOI:10.1053/j.pcsu.2019.02.007