End-Stage Cardiomyopathy Because of Hereditary Hemochromatosis Successfully Treated With Erythrocytapheresis in Combination With Left Ventricular Assist Device Support
2014
A 51-year-old man with a 2-year history of type II diabetes mellitus, erectile dysfunction, and 3-month history of fatigue was referred in March 2011 to the cardiology department of a local hospital because of 1 week of progressive dyspnea, orthopnea, leg edema, and increasing abdominal girth. His only medication was tolbutamide and metformin. He drank alcohol sporadically with maximum of 1 drink/d and had a history of shoulder arthralgias. On physical examination, the patient had brownish-gray skin pigmentation. He was tachypneic with blood pressure of 105/80 mm Hg and regular heart rate of 118 beats per minute. He had jugular venous distension, bilateral pulmonary crackles, II/VI systolic murmur heard at the apex, ascites, and pitting edema in both lower legs. The ECG showed sinus tachycardia with left atrial and ventricular hypertrophy and repolarization abnormalities. A chest radiograph (Figure 1) revealed cardiomegaly and bilateral pleural effusion. The echocardiogram showed a dilated left and right ventricle with severely compromised systolic function (left ventricular ejection fraction, 20%) and no ventricular hypertrophy. There was moderate mitral and tricuspid insufficiency with elevated right sided pressures (40–45 mm Hg). Coronary angiography was notable for no significant coronary artery disease, and the diagnosis of a nonischemic dilated cardiomyopathy (DCM) was made. Laboratory findings included increased levels of urea 9.3 (2.5–6.4) mmol/L, …
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