Cardiac transplantation in a patient with myocardial sarcoidosis: studies on the explanted heart

History and clinical findings: A 40-year-old man with histologically proven sarcoidosis, known for 15 years, which had involved the myocardium was hos pitalized because of intractable heart failure (NYHA class IV). An implanta tion of an intracardiac defibrillator for ventricular arrhythmias (Lown typ e IVa) had preceded. On physical examination severe dyspnoea at rest cough and fever were noted. Investigations:The erythrocyte sedimentation rate (88 /104 mm), C-reactive protein (250 mg/l) and white cell count (20/nl) were m arkedly increased. Serum sodium (113 mmol/l), potassium (3.0 mmol/l) and ch loride (64 mmol/l) were markedly reduced, while creatinine (2.5 mg/dl) and urea (82 mg/dl) were elevated due to renal failure. The chest radiogram dem onstrated central venous congestion, cardiomegaly and right paracardial inf iltration. There were no obvious changes due to sarcoidosis and computed to mography did not indicate pulmonary involvement by sarcoidosis. The echocar diogram revealed severe impairment of left ventricular function with an eje ction fraction of ca. 14%. Diagnosis, treatment and course: Heart failure ( NYHA class IV), caused by a dilated cardiomyopathy of sarcoidosis, was acco mpanied by pneumonia which responded to antibiotics. But the chronic heart failure failed to improve on drug treatment and cardiac transplantation was undertaken. The explanted myocardium was examined histologically, immunolo gically and virologically. Antibodies were demonstrated against vascular en dothelium, sarcolemma and endocardium (IgG and IgA), but not by PCR against cytomegalovirus, enterovirus and adenovirus. The transplantation was witho ut complication and, under azathioprine and methylprednisolone, one rejecti on had occured until now. The patient has been working full-time since 2 ye ars in his preveous occupation of lorry driver. There has been no evidence of renewed sarcoidosis activity. Conclusion: Sarcoidosis may involve the my ocardium in up to 25% of cases. Clinically relevant symptoms are even more rare. Sarcoidosis should be included in the differential diagnosis of unexp lained serious arrhythmias or cardiomyopathy, particularly in young persons . Cardiac transplantation may have to be contemplated if drug or pacemaker treatment fails to control heart failure.