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

**Authors:** Shuai Zhang, Jianrui Ma, Xiang Liu, Tong Tan, Wen Xie, Haozhong Liu, Huimin Wang, Hailong Qiu, Shusheng Wen, Jimei Chen, Jian Zhuang, Haiyun Yuan, Jianzheng Cen

PMC · DOI: 10.31083/j.rcm2503084 · 2024-03-04

## 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.

## Key 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.

## Linked entities

- **Diseases:** pulmonary atresia with ventricular septal defect (MONDO:0008343), PA/VSD (MONDO:0020437)

## Full-text entities

- **Diseases:** VSD (MESH:D004310), Ventricular Septal Defect (MESH:D006345), Pulmonary Atresia (MESH:D018633), PA (MESH:C535387), ventricle septal defect (MESH:D006343)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11263827/full.md

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Source: https://tomesphere.com/paper/PMC11263827