Right ventricular dysplasia: the Mayo Clinic experience.
OBJECTIVE
To determine the initial clinical manifestations and echocardiographic features of right ventricular dysplasia as encountered in a major cardiovascular referral center in the United States.


METHODS
We conducted a retrospective study of cases of right ventricular dysplasia diagnosed at the Mayo Clinic between January 1978 and January 1993.


METHODS
In an institutional data-base search, we identified 20 patients with right ventricular dysplasia. Echocardiographic, electrophysiologic, Holter monitoring, cardiac catheterization, and endomyocardial biopsy results were analyzed. The mean duration of follow-up was 7 years.


RESULTS
In the 12 female and 8 male patients (mean age, 30 years; range, 3 to 60), the initial manifestations of right ventricular dysplasia included ventricular arrhythmia (45%), congestive heart failure (25%), heart murmur (10%), asymptomatic (10%), complete heart block (5%), and sudden death (5%). First-order relatives were affected in 30% of the patients. Ventricular tachycardia with morphologic features of left bundle branch block was inducible in seven of nine patients. On Holter monitoring, all but 2 of 15 patients studied had frequent ventricular ectopic activity (Lown grade 2 or more). Characteristic fatty infiltration of the myocardium was present in 7 of 13 right ventricular biopsy specimens. Inordinate right ventricular enlargement was present in 60% of the patients at first echocardiographic assessment and in two other patients on follow-up assessment. Variable left ventricular involvement was noted in 50% of the cases. During the follow-up period, four patients died: two died suddenly, one died of congestive heart failure, and one died of respiratory failure after a coronary artery bypass operation. Of the 16 living patients, 8 are doing well, 3 have an implanted cardiac defibrillator, 3 are receiving antiarrhythmic agents, and 2 have undergone cardiac transplantation because of progressive biventricular failure.


CONCLUSIONS
Patients with right ventricular dysplasia have varied initial manifestations and a high frequency of serious cardiovascular symptoms and complications.
Kullo
I J
IJ
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic Rochester, Minnesota 55905, USA.
Edwards
W D
WD
Seward
J B
JB
D000293:Adolescent; D000328:Adult; D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000209:etiology; D004452:Echocardiography; D015716:Electrocardiography, Ambulatory; D005260:Female; D005500:Follow-Up Studies; D006327:Heart Block / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D006333:Heart Failure / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D006337:Heart Murmurs / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D006352:Heart Ventricles / Q000473:pathology*; D006801:Humans; D017380:Hypertrophy, Right Ventricular / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D018497:Ventricular Dysfunction, Right / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D016278:Ventricular Function, Right