Right ventricular dysplasia in an asymptomatic young man: An uncommon casewith biventricular involvement and no known family history

Citation
Vv. Patel et al., Right ventricular dysplasia in an asymptomatic young man: An uncommon casewith biventricular involvement and no known family history, J AM S ECHO, 14(4), 2001, pp. 317-320
Citations number
6
Categorie Soggetti
Cardiovascular & Respiratory Systems
Journal title
JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY
ISSN journal
08947317 → ACNP
Volume
14
Issue
4
Year of publication
2001
Pages
317 - 320
Database
ISI
SICI code
0894-7317(200104)14:4<317:RVDIAA>2.0.ZU;2-E
Abstract
A 33-year-old man had cardiomegaly on a routine x-ray examination. He was a symptomatic with no history of infarction, syncope, or palpitations. There was no family history of congenital heart disease or sudden death. Two-dime nsional transthoracic echocardiography demonstrated marked enlargement of t he right atrium and ventricle with severely depressed right and left ventri cular function that was consistent with right ventricular dysplasia. The pa tient was treated with an angiotensin-converting enzyme inhibitor and did w ell for 6 months, but then developed symptomatic left-sided congestive hear t failure. Short-term improvement was obtained with intravenous inotropic t herapy, but he continued to have progressive symptoms of heart failure. App roximately 7 months after his initial presentation, the patient underwent o rthotopic heart transplantation for intractable congestive heart failure. P athologic examination of the explanted heart established the diagnosis of r ight ventricular dysplasia with left ventricular involvement. This is an un common presentation of right ventricular dysplasia with biventricular invol vement and no known family history.