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One and One-Half Ventricle Repair: Role for Restricting Antegrade Pulmonary Blood Flow
Anagha Prasanna1, Corinne W Tan2, Alexandra Anastasopulos3
1Harvard Medical School, Boston, Massachusetts.
Insights
The one and one-half ventricle (1.5V) repair offers good survival for hypoplastic subpulmonary ventricles but carries risks of superior vena cava (SVC) hypertension. Pulmonary artery septation during 1.5V repair may lower this risk.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiovascular Physiology
Background:
- The one and one-half ventricle (1.5V) repair is an alternative surgical approach for patients with hypoplastic subpulmonary ventricles, offering an alternative to the Fontan procedure.
- Pulsatile pulmonary blood flow in 1.5V repair patients can lead to complications such as superior vena cava (SVC) hypertension or right atrial hypertension.
Purpose of the Study:
- To evaluate patient outcomes following 1.5V repair.
- To investigate if pulmonary artery septation during 1.5V repair reduces the incidence of SVC or right atrial hypertension.
Main Methods:
- Retrospective review of 74 patients who underwent 1.5V repair between 1989 and 2020.
- Primary outcome: transplant-free survival. Secondary outcomes: postoperative SVC hypertension and right atrial hypertension, with specific diagnostic criteria defined.
- Comparison of outcomes between patients with and without pulmonary artery septation during repair.
Main Results:
- Ten-year transplant-free survival was 92.4%.
- Long-term SVC hypertension occurred in 39% of survivors, and right atrial hypertension in 12%.
- Patients with unseptated pulmonary arteries had a significantly higher risk of SVC hypertension (44% vs. 10%, P = .04) compared to those with septated arteries. No significant difference in right atrial hypertension was observed.
Conclusions:
- The 1.5V repair provides favorable survival rates and avoids Fontan-associated complications.
- SVC hypertension is a notable long-term complication of 1.5V repair.
- Pulmonary artery septation during 1.5V repair may mitigate the risk of developing SVC hypertension.
Background:
In patients with hypoplastic subpulmonary ventricles, the one and one-half ventricle (1.5V) repair is an alternative to the Fontan procedure. However, in 1.5V-treated patients with pulsatile pulmonary blood flow, superior vena cava (SVC) hypertension or right atrial hypertension may develop. This study aimed to (1) describe patient outcomes after 1.5V repair and (2) determine whether pulmonary artery septation at 1.5V repair confers a lower risk of SVC or right atrial hypertension.
Methods:
This study retrospectively reviewed patients who underwent a 1.5V repair between 1989 and 2020. The primary outcome was transplant-free survival. Secondary outcomes were postoperative SVC hypertension (defined by mean Glenn pressures greater than 17 mm Hg, SVC flow reversal or pulsatility, venovenous collateral vessels, or SVC syndrome) and right atrial hypertension (defined as mean right atrial pressures greater than 10 mm Hg with inferior vena cava and hepatic vein dilation or flow reversal).
Results:
A total of 74 patients underwent 1.5V repair at a median age of 29.6 months (interquartile range, 8.9 to 45.5 months). Median follow-up time was 39.9 months (interquartile range, 11.4 to 178.1 months). Transplant-free survival at 10 years was 92.4%. Among survivors, 12% (8 of 69) had right atrial hypertension and 39% (27 of 69) had SVC hypertension on follow-up. Survivors with unseptated pulmonary arteries had a greater risk of SVC hypertension compared with patients with septated pulmonary arteries (44% vs 10%; P = .04). No difference was found in right atrial hypertension between the 2 groups.
Conclusions:
Patients with 1.5V repair avoid Fontan-associated complications with favorable transplant-free survival. However, SVC hypertension remains a significant long-term complication. Pulmonary artery septation at 1.5V repair may reduce the risk of SVC hypertension.
