Vol.22 No.1

Case Report

Systemic sclerosis complicated by arrhythmogenic right ventricular dysplasia that was misinterpreted as pulmonary arterial hypertension

Authors

Satoko Arai¹ , Kazuhiro Kurasawa¹ , Reika Maezawa¹ , Ryosuke Hanaoka¹ , Takeshi Fukuda¹

Received:

12 April 2011

Accepted:

30 May 2011

Published online:

25 June 2011

Full Text

PDF　(member's only)

Abstract

A 58-year old Japanese woman who had been diagnosed with and managed for systemic sclerosis (SSc) with pulmonary arterial hypertension died suddenly. However, the autopsy revealed marked right ventricular dilatation, and the myocardium had been replaced by fatty tissue. These findings were consistent with arrhythmogenic right ventricular dysplasia (ARVD). A literature search identified nine cases of SSc with ARVD in Japan, including this case; this number is significantly higher than the value estimated from the prevalences of ARVD and SSc in Japan, suggesting an association between these two rare diseases.

Key words

Arrhythmogenic right ventricular dysplasia - Pulmonary hypertension - Systemic sclerosis