When the Heart Runs Out of Heartbeats Treatment Options for Refractory End-Stage Heart Failure

A 50-year-old man was referred to the Advanced Heart Failure clinic at our institution for consideration of additional treatment options. He was diagnosed with heart failure (HF) 4 years earlier. Transthoracic echocardiography revealed a dilated left ventricle (LV) with an LV end-diastolic dimension of 5.5 cm, globally depressed ejection fraction (EF; estimated at 30%), and moderate mitral regurgitation. Complete blood cell count, urinalysis, and liver function tests were normal. Screening for hemochromatosis, human immunodeficiency virus, rheumatologic disease, and amyloidosis was performed and was negative. He did not have a family history of cardiomyopathy or sudden cardiac death. Coronary angiography revealed no evidence of obstructive coronary artery disease. Right heart catheterization demonstrated a right atrial pressure of 15 mm Hg, pulmonary artery pressure of 41/21 mm Hg, pulmonary capillary wedge pressure of 20 mm Hg, and cardiac index of 2.3 L . min(-1) . m(-2). He was started on evidence-based therapies for HF. An implantable cardioverter-defibrillator was placed for primary prevention of sudden cardiac death after no improvement in his EF. He was not considered a candidate for cardiac resynchronization therapy because his QRS duration was 100 milliseconds. He had progressive HF symptoms despite maximal medical therapy and was hospitalized 3 times during the 6 months before referral for acute decompensated HF. The doses of angiotensin-converting enzyme inhibitor and beta-blocker had been recently reduced by his cardiologist secondary to lightheadedness and fatigue accompanied by hypotension. His medical history was remarkable for hypertension and dyslipidemia. He reported adherence to his prescribed medications that included carvedilol 6.25 mg twice daily, lisinopril 5 mg daily, spironolactone 25 mg daily, and furosemide 40 mg daily.