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
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.