医学
血色病
病因学
心力衰竭
移植
内科学
心脏移植
肝移植
遗传性血色病
心肌病
心脏病学
作者
Monique Robinson,Sadeer Al‐Kindi,Guilherme H. Oliveira
标识
DOI:10.1016/j.ijcard.2017.06.075
摘要
Background Hemochromatosis predisposes to dilated or restrictive cardiomyopathy which can progress to end-stage heart failure, requiring the use of advanced heart therapies including heart (HT) and heart liver (HLT) transplantation. Little is known about the characteristics and outcomes of these patients. Methods and results We queried the United Network for Organ Sharing (UNOS) registry for all patients listed for HT or HLT for a diagnosis of ‘hemochromatosis’ between 1987 and 2014. Waitlist and post-transplantation outcomes were compared between patients with hemochromatosis (HT vs HLT) and other etiologies. Of the 81,356 adults listed for heart transplantation, 23 patients with hemochromatosis were identified (16 listed for HLT; and 7 listed for HT). Compared with other etiologies, HC patients were younger (39 vs 51 years, p < 0.0001), and more likely to need inotropes (56.5% vs 25.6%, p = 0.003) and mechanical ventilation (13% vs 3.4%, p = 0.041). Cumulative hazards of waitlist mortality or delisting were higher in hemochromatosis patients than for other etiologies of heart failure (p < 0.001). There were 4 HT and 4 HLT during the study period. Post-transplantation, patients with HC had a 1- and 2-year cumulative survival of 88% and 75%, respectively. Conclusions Both HT and HLT are viable options for patients with hemochromatosis. Patients with hemochromatosis are younger with increased wait-list mortality compared with other etiologies.
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