Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
Eur J Cardiothorac Surg. 2023 Dec 1;64(6). doi: 10.1093/ejcts/ezad311.
The goal of this study was to determine the long-term surgical outcomes of patients with functional single ventricles associated with heterotaxy syndrome, risk factors for mortality and factors associated with Fontan stage completion.
Overall, 279 patients with a functional single ventricle associated with heterotaxy syndrome who underwent an initial surgical procedure at our institute between 1978 and 2021 were grouped into 4 "eras" based on the surgical year during which the initial procedure was performed: era 1 (1978-1989, n = 71), era 2 (1990-1999, n = 98), era 3 (2000-2009, n = 64) and era 4 (2010-2021, n = 46). Neonatal surgery was more frequent in eras 3 and 4 than in eras 1 and 2.
Overall, 228 patients had right atrial isomerism; 120 patients (43.0%) had a total anomalous pulmonary venous connection; and 58 patients (20.8%) underwent an initial procedure as neonates. Overall survival rates at 10, 20 and 30 years after the initial procedure were 47.1%, 40.6% and 36.1%, respectively. Neonatal surgery (P < 0.001), total anomalous pulmonary venous connection repair at the initial procedure (P < 0.001) and early era (P < 0.001) were identified as risk factors for mortality, with the last 2 variables being negatively associated with Fontan stage completion (P < 0.001 for both).
Although era had a favourable effect on survival, total anomalous pulmonary venous connection with intrinsic pulmonary vein obstruction was associated with both mortality and Fontan stage completion.
R19092.
本研究旨在确定患有功能性单心室伴异构综合征患者的长期手术结果，包括死亡率的风险因素和完成 Fontan 阶段的相关因素。
共有 279 例功能性单心室伴异构综合征患者于 1978 年至 2021 年在我院接受了初始手术治疗，根据初始手术实施年份将患者分为 4 个“时代”：时代 1（1978-1989 年，n=71）、时代 2（1990-1999 年，n=98）、时代 3（2000-2009 年，n=64）和时代 4（2010-2021 年，n=46）。新生儿手术在时代 3 和时代 4 比在时代 1 和时代 2 更常见。
共有 228 例患者为右房异构；120 例患者（43.0%）存在完全性肺静脉异位连接；58 例患者（20.8%）作为新生儿接受了初始手术。初始手术后 10、20 和 30 年的总体生存率分别为 47.1%、40.6%和 36.1%。新生儿手术（P<0.001）、初始手术时的完全性肺静脉异位连接修复术（P<0.001）和早期时代（P<0.001）被确定为死亡的风险因素，后两个变量与 Fontan 阶段完成呈负相关（均 P<0.001）。
尽管时代对生存率有积极影响，但伴有固有肺静脉阻塞的完全性肺静脉异位连接与死亡率和 Fontan 阶段完成均相关。
R19092。
