Laschinger J C, Redmond J M, Cameron D E, Kan J S, Ringel R E
Division of Cardiothoracic Surgery, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.
Ann Thorac Surg. 1996 Nov;62(5):1261-7; discussion 1266-7. doi: 10.1016/0003-4975(96)00747-3.
Fourteen children (ages 2 to 14 years) and 1 adult (32 years) have undergone a modification of the Fontan procedure in which an extracardiac lateral tunnel or conduit is used in combination with staged or simultaneous bidirectional Glenn shunt(s).
Extracardiac lateral tunnels (n = 9) were constructed using a polytetrafluoroethylene patch (n = 7), pericardial patch (n = 1), or in situ pericardial flap (n = 1). Extracardiac lateral conduits (n = 6) were constructed using nonvalved homografts (n = 2) or polytetrafluoroethylene tube grafts (n = 4). Fenestrations were created in 4 patients (2 each in extracardiac lateral tunnel and extracardiac lateral conduit patients). Aortic cross-clamping was completely avoided in 12/15 patients (aortic cross-clamping in 2 patients for atrial septal defect enlargement and 1 for Damus-Kaye-Stansel procedure).
There have been no operative deaths. Prolonged postoperative chest tube drainage (> 2 weeks) has been rare (n = 1). At follow-up (range, 6 to 54 months; mean, 27.5 months), all patients are in New York Heart Association class I or II and remain in normal sinus rhythm. Late protein-losing enteropathy was seen in 1 patient and was successfully treated by percutaneous creation of a stented fenestration from the extracardiac tunnel to the systemic atrium. Late catheterizations reveal unobstructed extracardiac lateral tunnel function and low pulmonary pressures (range, 11 to 13 mm Hg). Advantages of the extracardiac Fontan include (1) avoidance of aortic cross-clamping in most patients, (2) the hemodynamic benefits of total cavopulmonary connection, (3) avoidance of atriotomy and intraatrial suture lines, (4) preservation of sinus rhythm and no arrhythmias at 2 year follow-up, (5) drainage of the coronary sinus to low pressure atrium, (6) allowance for early/late fenestrations, (7) prevention of baffle leaks and intraatrial obstruction, and (8) allowance for growth (tunnel procedures only).
We recommend this extracardiac procedure for all suitable patients undergoing surgical conversion to the Fontan circulation.
14名儿童（年龄2至14岁）和1名成人（32岁）接受了Fontan手术的改良术式，该术式采用心外膜侧隧道或管道与分期或同期双向Glenn分流术联合应用。
使用聚四氟乙烯补片（n = 7）、心包补片（n = 1）或原位心包瓣（n = 1）构建心外膜侧隧道（n = 9）。使用无瓣同种异体移植物（n = 2）或聚四氟乙烯管状移植物（n = 4）构建心外膜侧管道（n = 6）。4例患者（心外膜侧隧道和心外膜侧管道患者各2例）进行了开窗术。12/15例患者完全避免了主动脉阻断（2例患者因房间隔缺损扩大进行主动脉阻断，1例因Damus-Kaye-Stansel手术进行主动脉阻断）。
无手术死亡病例。术后胸管引流时间延长（> 2周）的情况罕见（n = 1）。随访（范围6至54个月；平均27.5个月）时，所有患者纽约心脏协会心功能分级为Ⅰ或Ⅱ级，且维持正常窦性心律。1例患者出现晚期蛋白丢失性肠病，通过经皮在心外膜隧道至体心房创建带支架的开窗术成功治疗。晚期心导管检查显示心外膜侧隧道功能通畅，肺压力低（范围11至13 mmHg）。心外膜Fontan手术的优点包括：（1）大多数患者避免主动脉阻断；（2）全腔肺连接的血流动力学益处；（3）避免心房切开术和心房内缝线；（4）2年随访时维持窦性心律且无心律失常；（5）冠状静脉窦引流至低压心房；（6）允许早期/晚期开窗；（7）预防挡板渗漏和心房内梗阻；（8）允许生长（仅适用于隧道手术）。
对于所有适合接受手术转为Fontan循环的患者，我们推荐这种心外膜手术。
