Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand

Kanthalas Lertsakulpiriya,Supaluck Kanjanauthai,Jarupim Soongswang,Paweena Chungsomprasong,Somchai Sriyoschati,Kritvikrom Durongpisitkul,Thaworn Subtaweesin,Prakul Chanthong,Chodchanok Vijarnsorn

doi:10.1038/s41598-020-61879-2

Kanthalas Lertsakulpiriya, Supaluck Kanjanauthai + Show 7 more

Open Access

https://doi.org/10.1038/s41598-020-61879-2

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Journal: Scientific Reports	Publication Date: Mar 20, 2020
Citations: 7	License type: open-access

Affiliation: Siriraj Hospital, Mahidol University

Abstract

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic congenital heart disease with a wide-range of presentations and treatment strategies, depending on the source of pulmonary circulation, anatomy of pulmonary arteries (PAs), and major aortopulmonary collateral arteries (MAPCAs). Data about the outcomes in developing countries is scarce. We therefore conducted a retrospective study to assess survival rates and mortality risks of 90 children with PA/VSD at Siriraj Hospital, Thailand during 2005–2016. Patients with single ventricle were excluded. Survival and mortality risks were analyzed at the end of 2018. The median age of diagnosis was 0.5 (0–13.8) years. The patients’ PAs were categorized into four groups: 1) PA/VSD with confluent PAs (n = 40), 2) PA/VSD with confluent PAs and MAPCAs (n = 21), 3) PA/VSD with non-confluent PAs and MAPCAs (n = 12), and 4) PA/VSD with small native PAs and MAPCAs (n = 17). Of the 88 patients who underwent operations, 32 patients had complete repair at 8.4 ± 4.6 years old. During the follow-up [median time of 5.7 years (7 days-13.6 years)], 17 patients (18.9%) died. The survival rates at 1, 5, and 10 years of age were 95%, 83.7%, and 79.6%, respectively. Significant mortality risks were the presence of associated anomalies and non-confluent PAs.

Highlights

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex congenital cyanotic heart disease that can lead to various clinical manifestations according to the individual central pulmonary artery anatomy and pulmonary distribution[1,2]
The present study evaluates survival rates and mortality risks of children who were diagnosed with PA/VSD at Siriraj Hospital in the last decade
With regards to the patients’ pulmonary arteries, the patients were categorized into four groups: 1) PA/VSD with confluent PAs (n = 40), 2) PA/VSD, confluent PAs, major aortopulmonary collateral arteries (MAPCAs) (n = 21), 3) PA/VSD, non-confluent PAs, MAPCAs (n = 12), and 4) PA/VSD, small native PAs, MAPCAs (n = 17) (Fig. 1)

Summary

Introduction

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex congenital cyanotic heart disease that can lead to various clinical manifestations according to the individual central pulmonary artery anatomy and pulmonary distribution[1,2]. In a more complex subgroup of patients, postulated to have an interruption of blood flow through the ductus arteriosus into the pulmonary artery in utero, leading to inadequate segmental lung perfusion and major aortopulmonary collateral arteries (MAPCAs), form the so called PA/VSD with MAPCAs4,5. The prevailing treatment strategy is primarily to reconnect the pulmonary artery tree (unifocalization) and create an appropriate source of pulmonary blood supply to optimize the segmental lung perfusion and avoid segmental pulmonary hypertension. This is followed by a staged, complete repair comprised of reconnecting the pulmonary artery tree to the right ventricle and closure of VSD. The present study evaluates survival rates and mortality risks of children who were diagnosed with PA/VSD at Siriraj Hospital in the last decade

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