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Congenitally corrected transposition with left ventricular outflow obstruction and cardiac malposition:
Rui Liu1, Kunjing Pang2, Lu Rui1
1Division of Pediatric Cardiac Surgical Centre, Chinese Academy of Medical Sciences and Peking Union Medical College Fuwai Hospital, Beijing, China.
Insights
The one-and-a-half ventricular repair offers better mid-term heart function and exercise tolerance for complex congenital heart disease compared to the Fontan pathway. This surgical approach is crucial for patients with congenitally corrected transposition of great arteries (ccTGA).
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Congenital Heart Disease
Background:
- Congenitally corrected transposition of great arteries (ccTGA) with left ventricular outflow tract obstruction (LVOTO) and cardiac malposition presents complex surgical challenges.
- Surgical repair strategies aim to improve long-term outcomes and quality of life for affected patients.
Purpose of the Study:
- To evaluate and compare the mid-term results of the one-and-a-half ventricular repair (hemi-Mustard, bidirectional Glenn, and Rastelli procedures) versus the Fontan pathway in ccTGA patients with LVOTO and cardiac malposition.
Main Methods:
- A retrospective study of 74 consecutive ccTGA patients with LVOTO and cardiac malposition, divided into two groups: one-and-a-half ventricular repair (Group A, 33 cases) and Fontan operation (Group B, 41 cases).
- Median follow-up was 49 months for Group A and 42 months for Group B.
- Exercise tolerance was assessed using the 6-minute walk test (MWT).
Main Results:
- No in-hospital deaths were reported.
- Group A demonstrated significantly shorter cardiopulmonary bypass, mechanical ventilation, and ICU stay compared to Group B, though Group B had more frequent prolonged pleural effusions.
- At 7 years, survival probabilities were 90.2% for Group A and 97.2% for Group B (p=0.300). Freedom from re-intervention was 80.6% for Group A and 97.2% for Group B (p=0.110).
- Group A showed superior systemic ventricular ejection fraction (EF%) and less moderate systemic ventricular valve regurgitation (p < 0.001) compared to Group B.
- The 6-minute walk distance was greater in Group A, indicating better exercise tolerance.
Conclusions:
- The one-and-a-half ventricular repair provides superior mid-term cardiac function and exercise tolerance for ccTGA patients with LVOTO and cardiac malposition.
- This surgical approach is a favorable option for managing this complex congenital heart defect.
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 malposition.
Methods:
In this retrospective study, 74 consecutive ccTGA with LVOTO and cardiac malposition 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. The Median follow-up time was 49 (20-84) and 42 (7-85) months in groups A and B, respectively. To estimate excise tolerance the 6-min walk test (MWT) was performed.
Results:
No in-hospital death. Compared with group A, group B have significantly less CPB, mechanical ventilation time, and intensive care unit stay, but prolonged pleural effusions developed more frequently in Group B. The survival probability was 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 has 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 6-min 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.
