OBJECTIVE: 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. DESIGN AND SETTING: 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: Patients surviving after Fontan surgery and evaluated at Boston Children's Hospital between 1993 and 2008. MAIN OUTCOME MEASURE: Composite endpoint of sudden death, death from congestive heart failure or cardiac transplantation. RESULTS: 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). CONCLUSIONS: Fontan patients with a higher MELD-XI score have shorter freedom from sudden cardiac death, death from congestive heart failure and cardiac transplantation.
OBJECTIVE: 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. DESIGN AND SETTING: 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: Patients surviving after Fontan surgery and evaluated at Boston Children's Hospital between 1993 and 2008. MAIN OUTCOME MEASURE: Composite endpoint of sudden death, death from congestive heart failure or cardiac transplantation. RESULTS: 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). CONCLUSIONS: Fontan patients with a higher MELD-XI score have shorter freedom from sudden cardiac death, death from congestive heart failure and cardiac transplantation.
Authors: Rahul H Rathod; Ashwin Prakash; Yuli Y Kim; Ioannis E Germanakis; Andrew J Powell; Kimberlee Gauvreau; Tal Geva Journal: Circ Cardiovasc Imaging Date: 2014-03-11 Impact factor: 7.792
Authors: William N Evans; Ruben J Acherman; Michael L Ciccolo; Sergio A Carrillo; Alvaro Galindo; Abraham Rothman; Brody J Winn; Noel S Yumiaco; Humberto Restrepo Journal: Pediatr Cardiol Date: 2016-06-14 Impact factor: 1.655
Authors: Robert W Elder; Nancy M McCabe; Emir Veledar; Brian E Kogon; Maan Jokhadar; Fred H Rodriguez; Michael E McConnell; Wendy M Book Journal: Congenit Heart Dis Date: 2014-08-17 Impact factor: 2.007