Deferoxamine as a Treatment for Cardiac Siderosis Induced Cardiomyopathy Post Liver Transplantation

Abstract

Introduction Iron overload cardiomyopathy (CM) occurring after liver transplantation in the absence of preexisting myocardial dysfunction or severe hepatic iron overload (ie hemochromatosis) has been described post-mortem. Cardiac siderosis without primary iron overload syndromes was found to be positively correlated to both higher pretransplant transferrin saturation (TSAT) and transfusion of red blood cells. We report an unusual case of a patient with alcohol-induced cirrhosis and heterozygous C282Y mutation who developed a dilated CM post-liver transplant with confirmed cardiac siderosis treated with IV deferoxamine leading to improvement in cardiac function. Case Report A 47-year-old woman with alcohol-induced cirrhosis underwent liver transplantation with a normal pre-operative cardiac evaluation including a transthoracic echocardiogram (TTE) with left ventricular ejection fraction (LVEF) 55-60%. Pre-operative iron studies showed an iron saturation of 91% with ferritin of 156. Her post-op course was uncomplicated, receiving minimal blood products (4 units of packed red blood cells). Two months later she was hospitalized for dyspnea and large pleural effusions. TTE showed depressed LVEF 17%. A cardiac MRI demonstrated a severely dilated LV indexed to BSA (1.5) with severe myocardial siderosis indicated by decreased T2* of 9-14 ms as well as significant mid-wall myocardial fibrosis. Right heart catheterization demonstrated a normal right atrial pressure, wedge pressure 18 mmHg, mean pulmonary pressure 26 mmHg and Fick cardiac index of 2.02 L/min/m2 for which milrinone was initiated. Endomyocardial biopsy confirmed intramyocardial iron deposition and native liver biopsy had grade 2-3 iron deposition. Etiology of her CM post-transplant was attributed to HFE gene heterozygosity in the setting of cirrhosis with post-transplant iron sensitivity (hepcidin depletion). She was initiated on IV chelation therapy with deferoxamine with follow up CMRI showing improved cardiac siderosis indicated by T2* of 13-17 ms with RV function 41% from 20% and LV function 25% from 17%. She was weaned off milrinone and continued on weekly deferoxamine chelation sessions. Summary This case highlights an unusual cause of cardiac siderosis related cardiomyopathy post-liver transplantation treated with IV iron chelation therapy leading to partial LV/RV recovery.