Griffiths Eric R, Kaza Aditya K, Wyler von Ballmoos Moritz C, Loyola Hugo, Valente Anne Marie, Blume Elizabeth D, del Nido Pedro
Department of Cardiac Surgery Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA.
Ann Thorac Surg. 2009 Aug;88(2):558-63; discussion 563-4. doi: 10.1016/j.athoracsur.2009.03.085.
Late complications of the Fontan operation represent a significant management challenge. Failing Fontan patients have two modes of presentation: impaired ventricular function (IVF) and those with preserved ventricular function (PVF) but with failing Fontan physiology (protein-losing enteropathy [PLE] and plastic bronchitis [PB]). This study evaluated whether failing Fontan patients referred for heart transplantation had a different outcome based on the mode of presentation.
The medical records of all Fontan patients evaluated for heart transplantation at a single institution from 1994 to 2008 were retrospectively reviewed. Demographic, hemodynamic, and laboratory data were collected. Patients were stratified into an IVF or PVF group by echocardiographic criteria. Descriptive statistics and Kaplan-Meier analysis were used for hypothesis testing.
Thirty-four Fontan patients were evaluated for heart transplantation. According to echo description of systolic function, 18 were categorized as IVF and 16 as PVF. The IVF group had a significantly lower cardiac index and venous oxygen saturation, and significantly higher systemic vascular resistance vs the PVF group (p < 0.05). PLE or PB was present in 13 PVF patients and none in the IVF group. Twenty patients underwent transplantation, with similar rates amongst the IVF and PVF groups. Within 1 year from evaluation, 2 IVG patients and 7 PVF patients had died (p = 0.052).
Failing Fontan patients with PVF have decreased overall survival independent of whether they underwent transplantation. This trend indicates a need to improve the management and timing for transplantation amongst this population.
Fontan手术的晚期并发症是一个重大的管理挑战。Fontan手术失败的患者有两种表现形式：心室功能受损（IVF）和心室功能保留（PVF）但Fontan生理功能衰竭（蛋白丢失性肠病[PLE]和塑形支气管炎[PB]）。本研究评估了因心脏移植而转诊的Fontan手术失败患者根据表现形式是否有不同的预后。
回顾性分析1994年至2008年在单一机构接受心脏移植评估的所有Fontan患者的病历。收集人口统计学、血流动力学和实验室数据。根据超声心动图标准将患者分为IVF组或PVF组。使用描述性统计和Kaplan-Meier分析进行假设检验。
34例Fontan患者接受了心脏移植评估。根据收缩功能的超声描述，18例被归类为IVF，16例为PVF。与PVF组相比，IVF组的心脏指数和静脉血氧饱和度显著降低，全身血管阻力显著升高（p<0.05）。13例PVF患者存在PLE或PB，IVF组无。20例患者接受了移植，IVF组和PVF组的移植率相似。在评估后的1年内，2例IVG患者和7例PVF患者死亡（p=0.052）。
PVF的Fontan手术失败患者总体生存率降低，无论他们是否接受了移植。这一趋势表明需要改善这一人群的移植管理和时机。
