Gaebert Paula, Schaeffer Thibault, Palm Jonas, Di Padua Chiara, Niedermaier Carolin, Piber Nicole, Hager Alfred, Ewert Peter, Hörer Jürgen, Ono Masamichi
Department of Congenital and Pediatric Heart Surgery, Technische Universität München, German Heart Center Munich, Munich, Germany.
Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany.
Cardiol Young. 2024 Nov;34(11):2406-2413. doi: 10.1017/S1047951124025782. Epub 2024 Oct 3.
Failing Fontan poses a significant clinical challenge. This study aims to improve patients' outcomes by comprehensively understanding the incidence, pathophysiology, risk factors, and treatment of failing Fontan after total cavopulmonary connection.
We performed a retrospective analysis of patients who underwent total cavopulmonary connection at the German Heart Center Munich between 1994 and 2022. The onset of failing Fontan was defined as: protein-losing enteropathy, plastic bronchitis, NYHA class IV, NYHA class III for > one year, unscheduled hospital admissions for heart failure symptoms, and evaluation for heart transplantation.
Among 634 patients, 76 patients presented with failing Fontan, and the incidence was 1.48 per 100 patient-years. Manifestations included protein-losing enteropathy (n = 34), hospital readmission (n = 28), NYHA III (n = 18), plastic bronchitis (n = 16), evaluation for heart transplantation (n = 14), and NYHA IV (n = 4). Risk factors for the onset of failing Fontan were dominant right ventricle (p = 0.010) and higher pulmonary artery pressure before total cavopulmonary connection (p = 0.004). A total of 72 interventions were performed in 59 patients, including balloon dilatation/stent implantation in the total cavopulmonary connection pathway (n = 49) and embolization of collaterals (n = 24). Heart transplantation was performed in four patients. The survival after the onset of Fontan failure was 77% at 10 years. Patients with failing Fontan revealed significantly higher zlog-NT-proBNP levels after onset compared to those without (p = 0.021).
The incidence of Fontan failure was 1.5 per 100 patient years. Dominant right ventricle and higher pulmonary artery pressure before total cavopulmonary connection were significant risks for the onset of failing Fontan. Zlog-NT-proBNP is only a late marker of Fontan failure.
功能性单心室Fontan循环衰竭是一项重大的临床挑战。本研究旨在通过全面了解全腔静脉肺动脉连接术后功能性单心室Fontan循环衰竭的发生率、病理生理学、危险因素及治疗方法来改善患者的预后。
我们对1994年至2022年期间在德国慕尼黑心脏中心接受全腔静脉肺动脉连接术的患者进行了回顾性分析。功能性单心室Fontan循环衰竭的发病定义为：蛋白丢失性肠病、塑形性支气管炎、纽约心脏协会（NYHA）心功能IV级、NYHA心功能III级持续超过1年、因心力衰竭症状非计划入院以及接受心脏移植评估。
在634例患者中，76例出现功能性单心室Fontan循环衰竭，发生率为每100患者年1.48例。表现包括蛋白丢失性肠病（n = 34）、再次入院（n = 28）、NYHA III级（n = 18）、塑形性支气管炎（n = 16）、接受心脏移植评估（n = 14）以及NYHA IV级（n = 4）。功能性单心室Fontan循环衰竭发病的危险因素为右心室优势（p = 0.010）和全腔静脉肺动脉连接术前较高的肺动脉压力（p = 0.004）。59例患者共进行了72次干预，包括在全腔静脉肺动脉连接路径中行球囊扩张/支架植入术（n = 49）和侧支血管栓塞术（n = 24）。4例患者接受了心脏移植。Fontan循环衰竭发病后的10年生存率为77%。与未发生功能性单心室Fontan循环衰竭的患者相比，发生功能性单心室Fontan循环衰竭的患者发病后zlog-NT-proBNP水平显著更高（p = 0.021）。
Fontan循环衰竭的发生率为每100患者年1.5例。右心室优势和全腔静脉肺动脉连接术前较高的肺动脉压力是功能性单心室Fontan循环衰竭发病的重要危险因素。Zlog-NT-proBNP只是Fontan循环衰竭的一个晚期标志物。
