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European Heart Journal 1991 12(11):1240-1243;
Copyright © 1991 by the European Society of Cardiology.
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© 1991 The European Society of Cardiology

Right ventricular dilated cardiomyopathy associated with primary biliary cirrhosis

F. ALFONSO*, A. L. P. CAFOALO{dagger}, J. DEL TORO*, E. TORRECILLA*, M. REY* and P. DE RABAGO*

*Cardiac Department, Fundacion Jimenez Diaz University of Madrid Spain
{dagger}Deparment of Cardiological Sciences, St George's Hospital Medical School, University of London U.K.

Received 10 November 1990; revised 26 July 1990; .

Correspondence: Alida L. P. Caforio, MD, Research Fellow, Department of Cardiological Sciences, St George's Hospital Medical School, Cranmer Terrace, London SWI 7 ORE, U.K.

Abstract

A case of right ventricular dilated cardiomyopathy associated with primary biliary cirrhosis is described. The patient was a middle aged woman, who initially complained of fatigue and itching. The diagnosis of primary biliary cirrhosis was made based on clinical, biochemical and histological evidence of the disease. Seven years later severe right-sided heart failure developed. The diagnosis of right ventricular dilated cardiomyopathy was made based on echocardiographic and angio graphic evidence of a globally dilated and poorly contracting right ventricle. Left ventricular function was within normal limits. Autoimmune serology screening at this time revealed the presence of organ-spec cardiac antibody (titre 1/20) and of antinuclear antibody (titre 1/80) by indirect immunofluorescence. There were no findings of mitochondrial antibody or other non-organ specific or organ-specific antibodies. Overall, this assessment demonstrates autoimmunity in both hepatic and heart muscle disease in a patient with primary biliary cirrhosis and right ventricular dilated cardiomyopathy.

Key Words: Right ventricular dilated cardiomyopathy • primary biliary cirrhosis • autoimmunity


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