McElhinney D B, Reddy V M, Moore P, Hanley F L
Division of Cardiothoracic Surgery, University of California, San Francisco, USA.
Ann Thorac Surg. 1996 Nov;62(5):1276-82; discussion 1283. doi: 10.1016/0003-4975(96)00567-X.
In patients who have received an atriopulmonary Fontan connection, complications such as right pulmonary vein obstruction, atrial arrhythmias, and thromboembolism are often secondary to right atrial enlargement. When such complications develop despite good ventricular function, there are few management options available. Extracardiac or intraatrial conduit cavopulmonary anastomosis, which improves central systemic venous flow patterns, avoids atrial distention, and does not involve the extensive atrial suturing required by other forms of cavopulmonary anastomosis, may provide relief for this group of patients.
Between October 1992 and October 1995, 7 patients presented 8 to 20 years after atriopulmonary connection with severe right atrial dilatation (7), Fontan pathway obstruction (4), progressive congestive heart failure (4), atrial tachydysrhythmias (3), right atrial thrombus (1), obstruction of right pulmonary veins by an enlarged right atrium (1), and subaortic stenosis (1). After evaluation of the options, they underwent revision of the atriopulmonary connection to extracardiac (5) or intraatrial (2) conduit cavopulmonary anastomosis.
One patient with severe cachexia, in whom transplantation was contraindicated for social reasons, died in the early postoperative period of massive effusions. Two patients eventually required permanent pacing for atrial dysrhythmias (1) or complete heart block secondary to subaortic fibromuscular resection (1), and 2 demonstrated marked improvement in unstable preoperative rhythm disturbances. At a median follow-up of 17 months, 4 of the 6 survivors were functioning at higher New York Heart Association levels than preoperatively, and 1 had recently undergone heart transplantation.
In properly selected patients with atrial complications, revision of a prior Fontan connection to extracardiac or intraatrial conduit cavopulmonary anastomosis appears to be a viable option.
在接受心房-肺循环Fontan连接的患者中，诸如右肺静脉梗阻、房性心律失常和血栓栓塞等并发症通常继发于右心房扩大。当尽管心室功能良好但仍出现此类并发症时，可供选择的治疗方法很少。心外或心房内管道腔肺吻合术可改善中心体循环静脉血流模式，避免心房扩张，且无需其他形式腔肺吻合术所需的广泛心房缝合，可能为这类患者提供缓解。
1992年10月至1995年10月期间，7例患者在心房-肺循环连接术后8至20年出现严重右心房扩张（7例）、Fontan通路梗阻（4例）、进行性充血性心力衰竭（4例）、房性快速心律失常（3例）、右心房血栓（1例）、扩大的右心房导致右肺静脉梗阻（1例）以及主动脉瓣下狭窄（1例）。在评估各种选择后，他们接受了将心房-肺循环连接改为心外（5例）或心房内（2例）管道腔肺吻合术。
1例因严重恶病质且因社会原因禁忌移植的患者在术后早期因大量积液死亡。2例患者最终因房性心律失常（1例）或主动脉瓣下纤维肌性切除术后的完全性心脏传导阻滞（1例）需要永久性起搏，2例术前不稳定的节律紊乱有明显改善。在中位随访17个月时，6例幸存者中有4例纽约心脏协会心功能分级高于术前，1例最近接受了心脏移植。
对于经过适当选择的有心房并发症的患者，将先前的Fontan连接改为心外或心房内管道腔肺吻合术似乎是一种可行的选择。
