Department of Cardiology, Boston's Children's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA.
Heart. 2013 Apr;99(7):491-6. doi: 10.1136/heartjnl-2012-303347. Epub 2013 Feb 13.
The Fontan operation is a staged palliation for complex congenital heart disease and single ventricle physiology. Perioperative survivors of the Fontan operation experience long-term cardiac complications, including death. Liver and renal dysfunction are reported in these patients and have a direct effect on morbidity and mortality. This study aims to investigate whether the Model for End-stage Liver Disease eXcluding INR score (function of creatinine and total bilirubin, MELD-XI) predicts risk for cardiac mortality or transplantation in patients with Fontan circulation.
Retrospective, single-centre study. Time of first evaluation was the time of the earliest available MELD-XI score measurement, and follow-up was terminated by a cardiac event or by the last clinical evaluation.
Patients surviving after Fontan surgery and evaluated at Boston Children's Hospital between 1993 and 2008.
Composite endpoint of sudden death, death from congestive heart failure or cardiac transplantation.
The MELD-XI score was calculated as MELD-XI=11.76(loge creatinine)+5.112(loge total bilirubin)+9.44. Ninety-six patients were included (52 male, median age 26 years). After a mean follow-up period of 5.7 years, 18 patients (19%) experienced the composite end point. Baseline MELD-XI score was independently and directly related to the incidence of the composite endpoint (HR for high MELD-XI score group of 7.76, 95% CI 2.05 to 29.33, p=0.008).
Fontan patients with a higher MELD-XI score have shorter freedom from sudden cardiac death, death from congestive heart failure and cardiac transplantation.
Fontan 手术是一种针对复杂先天性心脏病和单心室生理的分期姑息疗法。Fontan 手术后的围手术期幸存者会经历长期的心脏并发症，包括死亡。这些患者报告有肝肾功能障碍，对发病率和死亡率有直接影响。本研究旨在探讨排除 INR 的终末期肝病模型评分（肌酐和总胆红素的函数，MELD-XI）是否可预测 Fontan 循环患者的心脏死亡或移植风险。
回顾性、单中心研究。首次评估的时间是最早获得的 MELD-XI 评分测量时间，随访以心脏事件或最后一次临床评估结束。
Fontan 手术后存活并于 1993 年至 2008 年间在波士顿儿童医院接受评估的患者。
猝死、充血性心力衰竭或心脏移植导致的死亡的复合终点。
MELD-XI 评分计算为 MELD-XI=11.76(loge 肌酐）+5.112(loge 总胆红素）+9.44。共纳入 96 例患者（52 例男性，中位年龄 26 岁）。平均随访 5.7 年后，18 例患者（19%）出现了复合终点。基线 MELD-XI 评分与复合终点的发生率独立且直接相关（高 MELD-XI 评分组的 HR 为 7.76，95%CI 2.05 至 29.33，p=0.008）。
MELD-XI 评分较高的 Fontan 患者从心脏性猝死、充血性心力衰竭和心脏移植中获得的无事件生存期较短。
