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

Damien J LaPar; Emile Bacha

doi:10.1053/j.pcsu.2019.02.007

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

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2019:22:27-31. doi: 10.1053/j.pcsu.2019.02.007.

Authors

Damien J LaPar¹, Emile Bacha²

Affiliations

¹ Congenital and Pediatric Cardiac Surgery, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University, New York, New York. Electronic address: djl2180@cumc.columbia.edu.
² Congenital and Pediatric Cardiac Surgery, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University, New York, New York.

PMID: 31027561
DOI: 10.1053/j.pcsu.2019.02.007

Abstract

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.

Keywords: Biventricular repair; Congenital heart defect; Intact ventricular septum; Neonate; Pulmonary atresia.

Publication types

Review

MeSH terms

Clinical Decision-Making*
Heart Defects, Congenital / surgery*
Humans
Infant, Newborn
Patient Selection*
Pulmonary Atresia / surgery*
Tricuspid Valve / abnormalities*

Supplementary concepts

Pulmonary Atresia with Intact Ventricular Septum