Harvard Medical School, Boston, Massachusetts.
Department of Cardiovascular Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Ann Thorac Surg. 2022 Jul;114(1):176-183. doi: 10.1016/j.athoracsur.2021.04.058. Epub 2021 May 5.
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.
在患有肺血减少型左心发育不良的患者中，一体半心室修复术（1.5V）是法洛四联症根治术的替代方案。然而，在接受 1.5V 治疗且存在肺搏动性血流的患者中，可能会发展出上腔静脉（SVC）高血压或右心房高血压。本研究旨在：（1）描述 1.5V 修复术后患者的结局；（2）确定 1.5V 修复时肺动脉分隔是否会降低 SVC 或右心房高血压的风险。
本研究回顾性分析了 1989 年至 2020 年期间接受 1.5V 修复术的患者。主要结局为免于移植的生存。次要结局为术后 SVC 高血压（定义为平均 Glenn 压力大于 17mmHg，SVC 血流逆转或搏动性，静脉-静脉侧支血管，或 SVC 综合征）和右心房高血压（定义为平均右心房压力大于 10mmHg，同时伴有下腔静脉和肝静脉扩张或血流逆转）。
共有 74 例患者在中位年龄为 29.6 个月（四分位距 8.9 至 45.5 个月）时接受了 1.5V 修复术。中位随访时间为 39.9 个月（四分位距 11.4 至 178.1 个月）。10 年免于移植的生存率为 92.4%。在幸存者中，12%（8/69）患有右心房高血压，39%（27/69）在随访时有 SVC 高血压。与肺动脉分隔的患者相比，未分隔肺动脉的幸存者发生 SVC 高血压的风险更高（44%比 10%；P=0.04）。两组之间在右心房高血压方面无差异。
接受 1.5V 修复术的患者避免了法洛四联症根治术相关并发症，且具有良好的免于移植的生存率。然而，SVC 高血压仍然是一个重要的长期并发症。1.5V 修复时的肺动脉分隔可能会降低 SVC 高血压的风险。
