Right Ventricular Cardiomyopathy Accompanied by Protein-Losing Enteropathy and Chylous Effusion.

Abstract

rrythmogenic right ventricular cardiomyopathy (ARVC) is a cardiac disorder characterized by the fibro-fatty replacement of cardiomyocytes and the main clinical complications are arrhythmia, heart failure, and sudden death. 1 Recently, the genetic locus of Naxos disease, a form of ARVC, has been mapped to chromosome 17q21, in which the gene for plakoglobin is coded. 2 Plakoglobin is a key component of desmosomes and adherens junctions, and is important for the tight adhesion of many cell types, including those in the heart. The finding of a plakoglobin abnormality in ARVC suggests that the proteins involved in cell -cell adhesion play an important role in maintaining myocyte integrity, and when junctions are disrupted, cell death and fibro-fatty replacement occur. The relation between ARVC and fat replacement of the right ventricle (FaRV) has also been discussed recently. 3 ARVC is characterized by fibro-fatty replacement of myocytes with scattered foci of inflammation, but fat infiltration per se is considered a different process and FaRV may be a distinct clinicopathological entity.The new WHO classification includes the term 'arrhythmogenic' in ARVC, 4 but some patients do not suffer from ventricular arrhythmias. FaRV is reported to be less arrythmogenic than typical ARVC 3 and we describe a patient who developed right ventricular dilatation accompanied by chylous pleural effusion and ascites, but without life-threatening ventricular arrythmias. Case ReportA female patient had been prescribed diuretics at the age of 37 years for general fatigue accompanied by facial and leg edema. When she was 45 years old, she began to suffer from pleural effusion, watery diarrhea and hypoalbuminemia. Because her symptoms became resistant to medical therapy, she was referred to hospital at age 47. Her past history included left femoral thrombophlebitis at the age of 45, and her family history included dilated cardiomyopathy (DCM) in a younger sister who had died at the age of 40 after an operation for tricuspid regurgitation.On examination, she was 139 cm tall and weighted 29 kg. There was pretibial and facial edema as well as dilated neck veins. Her blood pressure was 90/44 mmHg with an irregular pulse of 76 beats/min and her ECG showed atrial fibrillation. The chest radiograph showed massive right pleural effusion and transthoracic echocardiography revealed a markedly dilated hypokinetic right ventricle, tricuspid Jpn Circ J 2001; 65: 912 -914 (Received January 30, 2001; revised manuscript received March 8, 2001; accepted March 19, 2001