Matsubara Muneaki, Dahmen Vincent, Gaebert Paula, Palm Jonas, Niedermaier Carolin, Osawa Takuya, Schaeffer Thibault, Heinisch Paul Philipp, Röhlig Christoph, Piber Nicole, Hager Alfred, Ewert Peter, Hörer Jürgen, Ono Masamichi
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany.
Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität München, Munich, Germany.
Eur J Cardiothorac Surg. 2025 Feb 4;67(2). doi: 10.1093/ejcts/ezaf005.
This study investigated the longitudinal changes in ventricular function and atrioventricular valve function to clarify the timing and the mechanisms of failing Fontan.
Patients who underwent total cavopulmonary connection between 1994 and 2023 were reviewed, and longitudinal echocardiographic examinations of ventricular function and atrioventricular valve regurgitation were analysed.
A total of 650 patients were included. The most frequent primary diagnosis was hypoplastic left heart syndrome in 175 patients. Dominant right ventricle was observed in 337 patients (51.8%). The median age at total cavopulmonary connection was 2.3 (1.8-3.3) years. Failing Fontan was observed in 78 patients (12%) during the median follow-up of 6.2 years. Among them, dominant right ventricle was observed in 51 patients (65.4%). Patients with protein-losing enteropathy or plastic bronchitis (n = 37) developed Fontan failure early (median 2.6 years post-total cavopulmonary connection). Still, patients maintained ventricular function (93.1% normal at 5 years) and atrioventricular valve competence (no case of moderate/severe regurgitation at 5 years) over time. Patients who developed failing Fontan associated with progression of heart failure (n = 41) had later onset (median 8.3 years post-total cavopulmonary connection) but indicated progressive ventricular dysfunction (68.3% normal at 5 years, 53.8% normal at 10 years) and atrioventricular valve regurgitation (12.3% moderate/severe at 5 years, 15.3% moderate/severe at 10 years).
Patients with failing Fontan indicated different serial ventricular and atrioventricular valve function profiles. Ventricular function was preserved in failing Fontan patients with protein-losing enteropathy or plastic bronchitis, whereas progressive ventricular dysfunction was observed in failing Fontan patients with heart failure.
本研究调查心室功能和房室瓣功能的纵向变化，以阐明功能性单心室（Fontan）衰竭的时间和机制。
回顾了1994年至2023年间接受全腔静脉肺动脉连接术的患者，并分析了心室功能和房室瓣反流的纵向超声心动图检查结果。
共纳入650例患者。最常见的主要诊断是175例患者的左心发育不全综合征。337例患者（51.8%）观察到右心室优势。全腔静脉肺动脉连接术时的中位年龄为2.3（1.8 - 3.3）岁。在中位随访6.2年期间，78例患者（12%）出现功能性单心室衰竭。其中，51例患者（65.4%）观察到右心室优势。患有蛋白丢失性肠病或塑形支气管炎的患者（n = 37）早期出现功能性单心室衰竭（全腔静脉肺动脉连接术后中位2.6年）。不过，随着时间推移，患者仍维持心室功能（5年时93.1%正常）和房室瓣功能（5年时无中度/重度反流病例）。出现与心力衰竭进展相关的功能性单心室衰竭的患者（n = 41）发病较晚（全腔静脉肺动脉连接术后中位8.3年），但显示出进行性心室功能障碍（5年时68.3%正常，10年时53.8%正常）和房室瓣反流（5年时12.3%为中度/重度，10年时15.3%为中度/重度）。
功能性单心室衰竭患者表现出不同的连续心室和房室瓣功能特征。患有蛋白丢失性肠病或塑形支气管炎的功能性单心室衰竭患者心室功能得以保留，而患有心力衰竭的功能性单心室衰竭患者则观察到进行性心室功能障碍。
