Analysis of Ventricular and Clinical Outcome of a Novel Cohort Using a Discriminatory Calculator for Pulmonary Atresia With Intact Ventricular Septum

Abstract

Background: A discriminatory echocardiographic calculator developed using a Western Australian (WA) pulmonary atresia with intact ventricular septum (PAIVS) cohort had a specificity of 93% and sensitivity of 96% in identifying biventricular surgical management, when using a combined right to left ventricular length ratio and tricuspid to mitral valve annulus ratio at birth. We aimed to describe another PAIVS tertiary cohort within Australia, from the Queensland Children’s Hospital (QCH) and evaluate the calculator in identifying surgical management pathway. Methods: Children born within Queensland with a diagnosis of PAIVS between June 2006 and June 2022 were analysed. Demographic, echocardiographic, and clinical data were collated at birth and latest follow up. Results: 26 patients were included for analysis. Total mortality was 34.6% (n=9) with 4 cases of early mortality. 38% of patients had right ventricular dependent coronary circulation (RVDCC; n=10), and 7.6% had absent tricuspid regurgitation (n=2). These patients all proceeded to univentricular palliation. 23.5% of surviving patients achieved biventricular circulation (n=4) and 76.5% required either univentricular or one-and-a-half ventricular palliation (n=13). The calculator had 100% specificity and 50% sensitivity in identifying a biventricular outcome, noting that with only 4 patients achieving biventricular repair, sensitivity was highly susceptible to the low volume of cases. Conclusion: In a cohort with comparable outcomes, absent tricuspid regurgitation and RVDCC remained exclusionary to biventricular repair. The WA cohort-based calculator had specificity of 100% in identifying biventricular management of PAIVS patients. Future collaborative studies are needed to assess its performance in identifying surgical pathways.