Friehs I, Dacar D, Gamillscheg A, Mächler H, Anelli-Monti M, Oberwalder P, Beitzke A, Metzler H, Rigler B
Klinische Abteilung für Herzchirurgie, Karl-Franzens-Universität, Graz.
Wien Klin Wochenschr. 1995;107(23):714-7.
The original Fontan operation was employed for patients with tricuspid atresia, but its application has been extended to a broad spectrum of congenital cardiac defects with a functional or anatomical single ventricle. From 1989 to 1995, 35 patients (23 males, 12 females) underwent a modified Fontan procedure; their age ranged from 14 months to 15 years (mean 5.1 a). Indications for operation were the following: D(S)ILV = 16, TA = 9, MA = 3, TGA with straddling AV-valve = 2, DORV = 4 and criss-cross heart = 1. The same surgical technique was utilized in all patients with redirection of the systemic venous return by means of a bidirectional cavopulmonary anastomosis and an intraatrial baffle, adaptable to all the various forms of underlying anatomy. Associated anomalies such as dextrocardia, coarctation, anomalous systemic or pulmonary venous return, subaortic stenosis and situs anomalies were present in 26%, 9%, 26%, 9%, and 20%, respectively. 12 patients were presumed to be at high risk and underwent a two-stage procedure (n = 7 bidirectional Glenn preceding the definitive repair and n = 5 a fenestration of the intraatrial baffle). Risk factors in our group of patients were: age under 2 years, abnormal systemic venous drainage, stenotic pulmonary arteries, PVR over 2 Wood units, mPAP over 15 mmHg, AV-valve incompetence, subaortic obstruction and ventricular dysfunction. Two patients had failure of the total cavo-pulmonary connection necessitating the take-down to a bidirectional Glenn anastomosis in the early postoperative period, with one death. Hospital mortality was 24% (n = 8). One child did not survive complications following the fenestration closure 4 months postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)
最初的Fontan手术用于三尖瓣闭锁患者，但它的应用已扩展到具有功能性或解剖学单心室的广泛先天性心脏缺陷。1989年至1995年，35例患者（23例男性，12例女性）接受了改良Fontan手术；年龄范围为14个月至15岁（平均5.1岁）。手术适应证如下：双入口左心室（D(S)ILV）=16例，三尖瓣闭锁（TA）=9例，单心房（MA）=3例，伴跨立房室瓣的大动脉转位（TGA）=2例，双心室流出道异常（DORV）=4例，交叉心脏=1例。所有患者均采用相同的手术技术，通过双向腔肺吻合和心房内挡板使体静脉回流改道，该技术适用于所有不同形式的基础解剖结构。合并畸形如右位心、主动脉缩窄、体静脉或肺静脉回流异常、主动脉瓣下狭窄和内脏反位分别占26%、9%、26%、9%和20%。12例患者被认为高危并接受了两阶段手术（7例在最终修复前先行双向Glenn手术，5例进行心房内挡板开窗）。我们这组患者的危险因素包括：2岁以下、体静脉引流异常、肺动脉狭窄、肺血管阻力超过2 Wood单位、平均肺动脉压超过15 mmHg、房室瓣关闭不全、主动脉瓣下梗阻和心室功能障碍。2例患者全腔肺连接失败，术后早期需拆除至双向Glenn吻合，1例死亡。住院死亡率为24%（8例）。1例患儿术后4个月开窗关闭后死于并发症。
