Outcomes of Rehabilitation Strategies for Pulmonary Atresia with Ventricular Septal Defect: A Single Center’s Experience

TL;DR

This study compares two surgical techniques for treating a heart defect and finds one leads to better long-term repair rates.

Contribution

The study provides new evidence that RV-PA connection is superior to systemic-to-pulmonary shunt for initial treatment of PA/VSD.

Findings

RV-PA connection resulted in higher PaO2 and lower RBC, hemoglobin, and hematocrit levels.

RV-PA connection had a significantly higher 5-year complete repair rate (56%) compared to systemic-to-pulmonary shunt (36%).

Survival rates were similar between the two groups, but RV-PA connection was an independent predictor for complete repair.

Abstract

Both systemic-to-pulmonary shunt and right
ventricle-pulmonary artery (RV-PA) connection are extensively applied to
initially rehabilitate the pulmonary artery in pulmonary atresia with the
ventricle septal defect (PA/VSD). However, which of these options is the most
ideal for promoting pulmonary artery development and improving outcomes remains
controversial.

A total of 109 PA/VSD patients undergoing
initial rehabilitative surgery at Guangdong Provincial People’s Hospital from
2010 to 2020 were enrolled in this study. A series of clinical data were
collected to compare the perioperative and postoperative outcomes between
systemic-to-pulmonary and RV-PA connection.

The mean duration
of follow-up was 61.1 months in the systemic-to-pulmonary shunt group and 70.3
months in the RV-PA connection group (p
> 0.05). The RV-PA connection
technique resulted in a significantly higher PaO2, lower red blood cells
(RBC), lower hemoglobin, and lower hematocrit (Hct) (p
< 0.05). The
cumulative incidence curve estimated a cumulative complete repair rate of 56
± 7% after 5 years in the RV-PA connection group, significantly higher
than 36 ± 7% after 5 years in the systemic-to-pulmonary shunt group
(p
< 0.05). The Kaplan-Meier curve revealed a similar estimated
survival rate between the two groups (p = 0.73). The RV-PA connection
was identified as an independent predictor for complete repair in the
multivariable analysis (HR = 2.348, 95% CI = 1.131–4.873).

The RV-PA connection is a more ideal initial rehabilitative
technique than systemic-to-pulmonary shunt in treating PA/VSD as a consequence of
comparable probability of survival but improved definitive complete repair rate.

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