Front. Cardiovasc. Med.
Sec. Pediatric Cardiology
doi: 10.3389/fcvm.2022.938118

Congenitally corrected transposition with left ventricular outflow obstruction and cardiac malposition: one-and-a-half ventricular repair vs. Fontan pathway?

 Rui Liu1, Kunjing Pang1, Lu Rui1, Benqing Zhang1, Chao Wang2 and Shoujun Li1*
  • 1Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, China
  • 2Peking University Bejing Jishuitan Hospital, China
Provisionally accepted:
The final, formatted version of the article will be published soon.

Objectives: This study was to assess the mid-term results of the one-and-a-half ventricular repair (hemi-Mustard and bidirectional Glenn procedures combined with the Rastelli procedure) and Fontan pathway for correcting congenitally corrected transposition of great artery (ccTGA) patients with left ventricular outflow tract obstruction (LVOTO) and cardiac malpostion.
Methods: At this retrospective study, 74 consecutive ccTGA with LVOTO and cardiac malpositon underwent the one-and-a-half ventricular repair (group A; 33 cases) and Fontan operation (group B; 41 cases) between October 2011 and March 2018. Median follow-up time are 49 (20-84) and 42 (7-85) months in group A and B. To estimate excise tolerance the 6minute walk test (MWT) was performed.
Results: No in-hospital death. Compared with group A, group B have significant less CPB, mechanical ventilation time and intensive care unit stay, but prolonged pleural effusions developed more frequently in the Group B. The survival probability were 90.2% (95% CI, 80.2%-100%) and 97.2% (95% CI, 92%-100%) at 7 years (p=0.300) in group A and B. The probability of freedom from re-intervention were 80.6% (95% CI, 66.5%-97.6%) and 97.2% (95% CI, 92%-100%) at 7 years (p=0.110). Longitudinal repeated measured echo data at every follow-up time shows that group A have more systemic ventricular EF% (p<0.001) and less moderate systemic ventricular valve regurgitation (p<0.001) compared with group B. Estimated by 6 MWT, group A has better outcomes for six-minute walk distance .
Conclusions: For correction of ccTGA with LVOTO and cardiac malposition, the one-and-a-half ventricular repair had superior midterm heart function and excise tolerance.

Keywords: Congenitally corrected transposition (ccTGA), Left ventricular outflow obstruction (LVOTO), Cardiac malposition, One-and-a-half ventricular repair, Fontan

Received: 07 May 2022; Accepted: 15 Aug 2022.

Copyright: © 2022 Liu, Pang, Rui, Zhang, Wang and Li. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Prof. Shoujun Li, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China