Carey J A, Hamilton J R, Hilton C J, Dark J H, Forty J, Parry G, Hasan A
Department of Cardiothoracic Surgery, Freeman Hospital, High Heaton, Newcastle-upon-Tyne, UK.
Eur J Cardiothorac Surg. 1998 Jul;14(1):7-13; discussion 13-4. doi: 10.1016/s1010-7940(98)00130-4.
Modified Fontan procedures are now employed in several conditions unsuitable for bi-ventricular repair. Selection criteria have been relaxed. The procedure is palliative. Longterm outlook is unknown. This study evaluated factors associated with the development of a failing Fontan circulation and transplantation results.
Retrospective review of patients referred to a single centre for cardiac transplant assessment.
Between 1985 and 1996, 46 of 448 cardiac transplants were performed for congenital heart disease. Nine of these were performed in patients with a failing Fontan circulation (four adults, five children). In six cases, the dominant ventricle had left ventricular (LV) morphology. Congenital anomalies included double outlet right ventricle (three cases), double inlet left ventricle (two cases), tricuspid atresia (two cases), and pulmonary atresia with intact ventricular septum (one case). Fontan procedures were performed in absence of sinus rhythm (four cases), atrio-ventricular (AV) valve regurgitation (two cases), aortic regurgitation and systolic LV dysfunction (one case), elevated mean pulmonary artery pressure (one case), and older age (>7 years, eight cases). Three patients required early re-operation and two needed permanent pacing. Subsequent deterioration associated with loss of sinus rhythm (four cases) and progressive AV valve regurgitation (seven cases) led to transplant assessment (at < 1 year, five cases; at 2-12 years, four cases). All patients were listed for transplantation. Three patients required intravenous inotropic support and three patients with lymphocytotoxic antibodies needed prospective crossmatching. Donor cardiectomy was modified to facilitate implantation. The recipient operation involved pulmonary artery reconstruction (using pericardium), modified atrial and direct caval anastomoses. Three patients died within 24 h of surgery (two graft failures, one haemorrhage). In operative survivors (n = 6), intensive care stay was 3-16 days, and hospital stay ranged from 14 to 32 days. There have been no subsequent deaths (follow up, 0.5-4.7 years).
In high-risk Fontan candidates, transplantation may be preferable at the outset. Previous surgery, lymphocytotoxic antibodies, indeterminate pulmonary vascular resistance, emergency status, sub-optimal donor selection, and perioperative bleeding contribute to peri-operative mortality. In survivors, the outcome remains very encouraging.
改良Fontan手术目前应用于几种不适合双心室修复的情况。选择标准已经放宽。该手术是姑息性的。长期前景尚不清楚。本研究评估了与Fontan循环衰竭发展相关的因素以及移植结果。
对转诊至单一中心进行心脏移植评估的患者进行回顾性研究。
1985年至1996年间，448例心脏移植中有46例是针对先天性心脏病进行的。其中9例是在Fontan循环衰竭的患者中进行的（4例成人，5例儿童）。6例中，优势心室具有左心室（LV）形态。先天性异常包括右心室双出口（3例）、左心室双入口（2例）、三尖瓣闭锁（2例）和室间隔完整的肺动脉闭锁（1例）。Fontan手术在无窦性心律（4例）、房室（AV）瓣反流（2例）、主动脉反流和左心室收缩功能障碍（1例）、平均肺动脉压升高（1例）以及年龄较大（>7岁，8例）的情况下进行。3例患者需要早期再次手术，2例需要永久起搏。随后与窦性心律丧失（4例）和进行性房室瓣反流（7例）相关的病情恶化导致了移植评估（<1年，5例；2 - 12年，4例）。所有患者均被列入移植名单。3例患者需要静脉注射正性肌力药物支持，3例有淋巴细胞毒性抗体的患者需要进行前瞻性交叉配型。供体心脏切除进行了改良以利于植入。受体手术包括肺动脉重建（使用心包）、改良心房和直接腔静脉吻合。3例患者在术后24小时内死亡（2例移植失败，1例出血）。手术存活者（n = 6），重症监护停留时间为3 - 16天，住院时间为14至32天。后续无死亡病例（随访0.5 - 4.7年）。
在高危Fontan候选患者中，一开始进行移植可能更可取。既往手术、淋巴细胞毒性抗体、不确定的肺血管阻力、紧急状态、供体选择不佳以及围手术期出血导致围手术期死亡率增加。在存活者中，结果仍然非常令人鼓舞。
