Long-term outcomes of pulmonary atresia with ventricular septal defect by different initial rehabilitative surgical age

Jianrui Ma; Tong Tan; Shuai Zhang; Wen Xie; Yinru He; Miao Tian; Zichao Tujia; Xinming Li; Xiaobing Liu; Jimei Chen; Jian Zhuang; Jianzheng Cen; Shusheng Wen; Haiyun Yuan

doi:10.3389/fcvm.2023.1189954

Long-term outcomes of pulmonary atresia with ventricular septal defect by different initial rehabilitative surgical age

Front Cardiovasc Med. 2023 Oct 16:10:1189954. doi: 10.3389/fcvm.2023.1189954. eCollection 2023.

Authors

Jianrui Ma^#^{1

2

3}, Tong Tan^#⁴, Shuai Zhang^{2

3}, Wen Xie^{2

3}, Yinru He^{2

3}, Miao Tian^{2

3}, Zichao Tujia^{2

3}, Xinming Li^{2

3}, Xiaobing Liu^{2

3}, Jimei Chen^{2

3}, Jian Zhuang^{2

3}, Jianzheng Cen^{1

2

3}, Shusheng Wen^{2

3}, Haiyun Yuan^{1

2

3}

Affiliations

¹ Shantou University Medical College, Shantou, China.
² Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Southern Medical University, Guangzhou, China.
³ Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangzhou, China.
⁴ Department of Cardiovascular Surgery Center, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart, Lung and Blood Vascular Diseases, Beijing, China.

^# Contributed equally.

Abstract

Background: There is a lack of evidence guiding the surgical timing selection in pulmonary atresia with ventricular septal defect. This study aims to compare the long-term outcomes of different initial rehabilitative surgical ages in patients with pulmonary atresia with ventricular septal defect (PAVSD).

Methods: From January 2011 to December 2020, a total of 101 PAVSD patients undergoing the initial rehabilitative surgery at our center were retrospectively reviewed. Receiver-operator characteristics curve analysis was used to identify the cutoff age of 6.4 months and therefore to classify the patients into two groups. Competing risk models were used to identify risk factors associated with complete repair. The probability of survival and complete repair were compared between the two groups using the Kaplan-Meier curve and cumulative incidence curve, respectively.

Results: The median duration of follow-up was 72.76 months. There were similar ΔMcGoon ratio and ΔNakata index between the two groups. Multivariate analysis showed that age ≤6.4 months (hazard ratio (HR) = 2.728; 95% confidence interval (CI):1.122-6.637; p = 0.027) and right ventricle-to-pulmonary artery connection (HR = 4.196; 95% CI = 1.782-9.883; p = 0.001) were associated with increased probability of complete repair. The cumulative incidence curve showed that the estimated complete repair rates were 64% ± 8% after 3 years and 69% ± 8%% after 5 years in the younger group, significantly higher than 28% ± 6% after 3 years and 33% ± 6% after 5 years in the elder group (p < 0.001). There was no significant difference regarding the estimated survival rate between the two groups.

Conclusion: Compared with those undergoing the initial rehabilitative surgery at the age >6.4 months, PAVSD patients at the age ≤6.4 months had an equal pulmonary vasculature development, a similar probability of survival but an improved probability of complete repair.

Keywords: age; complete repair; pulmonary atresia; rehabilitation; timing.

Grants and funding

This study was supported by National Key Research and Development Program of China (No. 2020YFC1107904, No. 2022YFC2407406), National Natural Science Foundation of China (No. 62006050, No. 62276071), Science and Technology Planning Project of Guangdong Province (2019B020230003; 2018B090944002; 2020B1111170011), Science and Technology Fundation for Guangzhou Health (2023A031004), Science and Technology Program of Guangzhou (202206010049), Guangzhou Science and Technology Planning Project (2023B03J0596), Guangdong peak project (DFJH201802; DFJH2020029), and Guangdong Special Support Program-Science and Technology Innovation Talent Project (No. 0620220211).