Giannico Salvatore, Hammad Fatma, Amodeo Antonio, Michielon Guido, Drago Fabrizio, Turchetta Attilio, Di Donato Roberto, Sanders Stephen P
Department of Pediatric Cardiology and Cardiac Surgery, Rome, Italy.
J Am Coll Cardiol. 2006 May 16;47(10):2065-73. doi: 10.1016/j.jacc.2005.12.065. Epub 2006 Apr 27.
We sought to evaluate the mid-term outcome of hospital survivors with extracardiac Fontan circulation.
Few data exist about the mid-term and long-term results of the extracardiac Fontan operation.
From November 1988 to November 2003, 221 patients underwent an extracardiac Fontan procedure as primary (9 patients) or secondary (212 patients) palliation, at a mean age of 72.2 months (range 13.1 to 131.3 months). A total of 165 of 193 early survivors underwent programmed noninvasive follow-up evaluations and at least one cardiac catheterization.
The overall survival, including operative deaths, was 85% at 15 years. Freedom from late failure among hospital survivors is 92% at 15 years. A total of 127 of 165 survivors (77%) were in New York Heart Association functional class I. The incidence of late major problems was 24% (42 major problems in 36 of 165 patients): 19 patients had arrhythmias (11%), 5 patients had obstruction of the extracardiac conduit (3%) and 6 of the left pulmonary artery (3.5%), and 5 patients experienced ventricular failure (3%), leading to heart transplantation in 3 patients. Protein-losing enteropathy was found in two patients (1%). The incidence of late re-interventions was 12.7% (21 of 165 patients, including 15 epicardial pacemaker implantations). Four patients died (2.3%), two after heart transplantation.
After 15 years of follow-up, the overall survival, the functional status, and the cardiopulmonary performance of survivors of the extracardiac Fontan procedure compare favorably with other series of patients who underwent the lateral tunnel approach. The incidence of late deaths, obstructions of the cavopulmonary pathway, re-interventions, and arrhythmias is lower than that reported late after other Fontan-type operations.
我们试图评估心脏外Fontan循环的医院幸存者的中期结局。
关于心脏外Fontan手术的中期和长期结果的数据很少。
从1988年11月至2003年11月，221例患者接受了心脏外Fontan手术作为一期（9例）或二期（212例）姑息治疗，平均年龄72.2个月（范围13.1至131.3个月）。193例早期幸存者中的165例接受了计划性非侵入性随访评估和至少一次心导管检查。
包括手术死亡在内的总生存率在15年时为85%。医院幸存者中15年时无晚期衰竭的比例为92%。165例幸存者中有127例（77%）纽约心脏协会心功能分级为I级。晚期主要问题的发生率为24%（165例患者中的36例出现42个主要问题）：19例患者发生心律失常（11%），5例患者心脏外管道阻塞（3%），6例左肺动脉阻塞（3.5%），5例患者发生心室衰竭（3%），其中3例患者因此接受心脏移植。2例患者（1%）出现蛋白丢失性肠病。晚期再次干预的发生率为12.7%（165例患者中的21例，包括15例植入心外膜起搏器）。4例患者死亡（2.3%），2例在心脏移植后死亡。
经过15年的随访，心脏外Fontan手术幸存者的总生存率、功能状态和心肺功能与接受侧隧道法的其他系列患者相比具有优势。晚期死亡、腔肺途径阻塞、再次干预和心律失常的发生率低于其他Fontan型手术后期报道的发生率。
