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Heart and heart-liver transplantation in patients with hemochromatosis
Monique R Robinson1, Sadeer G Al-Kindi1, Guilherme H Oliveira1
1Advanced Heart Failure and Transplantation Center, Harrington Heart and Vascular Institute, University Hospitals Cleveland Medical Center, Cleveland, OH, United States.
Insights
Hemochromatosis patients undergoing heart transplantation (HT) or heart-liver transplantation (HLT) are younger but face higher waitlist mortality. Both HT and HLT are viable advanced heart therapies for these individuals.
Area of Science:
- Cardiology
- Transplantation Medicine
- Genetics
Background:
- Hemochromatosis can lead to end-stage heart failure, necessitating advanced therapies like heart transplantation (HT) and heart-liver transplantation (HLT).
- Limited data exists on the characteristics and outcomes of hemochromatosis patients receiving these transplantations.
Purpose of the Study:
- To characterize patients with hemochromatosis undergoing heart transplantation (HT) or heart-liver transplantation (HLT).
- To compare waitlist and post-transplantation outcomes of hemochromatosis patients with those of other etiologies.
Main Methods:
- Utilized the United Network for Organ Sharing (UNOS) registry from 1987 to 2014.
- Identified 23 adult patients with hemochromatosis listed for HT or HLT.
- Compared hemochromatosis patients (n=23) with other heart failure etiologies (n=81,356).
Main Results:
- Hemochromatosis patients were younger (39 vs. 51 years) and required more inotropes (56.5% vs. 25.6%) and mechanical ventilation (13% vs. 3.4%).
- Patients with hemochromatosis had significantly higher cumulative hazards of waitlist mortality or delisting (p<0.001).
- Post-transplantation, 1- and 2-year survival rates for hemochromatosis patients were 88% and 75%, respectively.
Conclusions:
- Heart transplantation (HT) and heart-liver transplantation (HLT) are feasible treatment options for hemochromatosis-induced heart failure.
- Hemochromatosis patients present with unique demographic and clinical profiles, including increased waitlist mortality, compared to other transplant candidates.
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 51years, 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.
