Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technical University of Munich, School of Medicine, Munich, Germany.
Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany.
Eur J Cardiothorac Surg. 2021 Sep 11;60(3):554-561. doi: 10.1093/ejcts/ezab141.
Heterotaxy is a known risk factor for morbidity and mortality in single ventricle palliation. In this study, we examined our experience with this challenging group of patients.
Records of patients born between 2001 and 2019 with heterotaxy, who needed staged single ventricle palliation were retrospectively analysed.
A total of 53 patients were included in this study. Thirty-five (66%) patients had a right ventricular dominance, common atrioventricular septal defect was present in 37 (70%) patients. Anomalous pulmonary venous drainage was present in 29 (55%) patients. Forty-six (86%) patients underwent first-stage palliation. Forty-one (77.3%) patients received a bidirectional cavopulmonary connection. Thirty-one (58%) patients received total cavopulmonary connection (TCPC). Overall survival rate was 92% at 1 month, 74% at 1 year and 68% at 10 years. Twelve (22.6%) patients died before second palliation stage. Four (10%) patients died before TCPC. No patient died after TCPC. Independent risk factors for mortality in the multivariate COX regression were a presence of restrictive pulmonary blood flow (HR 3.23; 95% CI 1.02-10.2; P = 0.05) and greater than mild atrioventricular valve regurgitation (HR 3.57; 95% CI 1.27-10.0; P = 0.02).
Mortality and morbidity in patients with single ventricle and heterotaxy are high. Restrictive pulmonary blood flow needing early modulation and greater than mild atrioventricular valve regurgitation at presentation are independent risk factors for mortality. Total anomalous pulmonary venous connection was not identified as a risk factor in the current era.
心脏异位是单心室姑息治疗患者发病率和死亡率的已知危险因素。本研究旨在分析该类具有挑战性的患者群体的治疗经验。
回顾性分析了 2001 年至 2019 年间患有心脏异位且需要分期单心室姑息治疗的患者的病历资料。
本研究共纳入 53 例患者。35 例（66%）患者为右心室优势型，37 例（70%）患者存在共同房室间隔缺损，29 例（55%）患者存在肺静脉异常引流。46 例（86%）患者接受了一期姑息治疗。41 例（77.3%）患者接受了双向腔静脉肺动脉连接术。31 例（58%）患者接受了全腔静脉肺动脉连接术。1 个月、1 年和 10 年的总生存率分别为 92%、74%和 68%。12 例（22.6%）患者在接受二期姑息治疗前死亡。4 例（10%）患者在接受全腔静脉肺动脉连接术之前死亡。全腔静脉肺动脉连接术之后无患者死亡。多变量 COX 回归分析的独立死亡危险因素为限制性肺血流量（HR 3.23；95%CI 1.02-10.2；P=0.05）和大于轻度的房室瓣反流（HR 3.57；95%CI 1.27-10.0；P=0.02）。
患有单心室和心脏异位的患者死亡率和发病率较高。需要早期调节的限制性肺血流量和出现时大于轻度的房室瓣反流是死亡的独立危险因素。在当前时代，总肺静脉异常连接并未被确定为危险因素。
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