One and One-Half Ventricle Repair: Role for Restricting Antegrade Pulmonary Blood Flow.

Abstract

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. 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). 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. 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.