Advanced Dilated Cardiomyopathy in a Patient With Hutterite Limb-Girdle Muscular Dystrophy
Use of a Left Ventricular Assist Device
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A 39-year-old male was diagnosed with Hutterite hereditary limb-girdle muscular dystrophy subtype 2I (LGMD2I), complicated by a secondary cardiomyopathy, ventricular arrhythmias, and heart failure. He had mild muscle weakness and independent mobility. Medical therapy included diuretic, amiodarone, angiotensin-converting enzyme inhibitor, β-blocker, and mineralocorticoid receptor antagonist and had an implanted cardiac device inserted for secondary prophylaxis. Twelve-lead ECG showed premature ventricular complexes, first-degree atrioventricular block, and a nonspecific intraventricular conduction delay (Figure [A]). Transthoracic echocardiogram showed a severely dilated left ventricle (LV), with an LV end-diastolic internal diameter of 8.6 cm, eccentric left ventricular hypertrophy with markedly reduced ejection fraction (26%), and severe mitral regurgitation. Heart catheterization showed a wedge pressure of 30 mm Hg, mean pulmonary arterial pressure of 51 mm Hg, and a transpulmonary gradient of 21 mm Hg indicative of left-sided heart failure and pulmonary hypertension. Coronary angiogram revealed normal origin, course, and lumen of the coronary arteries. Given his advanced heart failure, he was admitted and given intravenous inotropic support. He required an LV assist device (LVAD) as a bridge-to-transplant which was successfully inserted using a minimally invasive technique (Figure [A]). The patient was discharged in stable condition on appropriate …