Long-term survivor diagnosed with arrhythmogenic right ventricular cardiomyopathy/dysplasia

The subject was a 70 year-old man who survived for 31 years after being diagnosed with right ventricular cardiomyopathy, having undergone right ventricular (RV) aneurysmectomy at the age of 39. His arrhythmia and syncopal attacks were effectively abolished after the original aneurysmectomy. Although he frequently suffered from right heart failure, hemodialysis improved his status. However, the patient died due to worsening anasarca caused by RV low output syndrome. Autopsy results indicated extensive replacement of the RV myocardium with fibrous and fatty tissues. This case suggests that patients with arrhythmogenic RV cardiomyopathy, but without left ventricular abnormalities and rapid ventricular arrhythmia, have a relatively favorable prognosis, although RV abnormalities may be progressive.