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Arrhythmogenic right ventricular dysplasia: a United States experience
- Source :
- Circulation. 112(25)
- Publication Year :
- 2005
-
Abstract
- Background— Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy characterized by right ventricular dysfunction and ventricular arrhythmias. The purpose of our study was to describe the presentation, clinical features, survival, and natural history of ARVD in a large cohort of patients from the United States. Methods and Results— The patient population included 100 ARVD patients (51 male; median age at presentation, 26 [interquartile range {IQR}, 18 to 38; range, 2 to 70] years). A familial pattern was observed in 32 patients. The most common presenting symptoms were palpitations, syncope, and sudden cardiac death (SCD) in 27%, 26%, and 23% of patients, respectively. Among those who were diagnosed while living (n=69), the median time between first presentation and diagnosis was 1 (range, 0 to 37) year. During a median follow-up of 6 (IQR, 2 to 13; range, 0 to 37) years, implantable cardioverter/defibrillators (ICD) were implanted in 47 patients, 29 of whom received an appropriate ICD discharge, including 3 patients who received the ICD for primary prevention. At follow-up, 66 patients were alive, of whom 44 had an ICD in place, 5 developed signs of heart failure, 2 had a heart transplant, and 18 were on drug therapy. Thirty-four patients died either at presentation (n=23: 21 SCD, 2 noncardiac deaths) or during follow-up (n=11: 10 SCD, 1 of biventricular heart failure), of whom only 3 were diagnosed while living and 1 had an ICD implanted. On Kaplan-Meier analysis, the median survival in the entire population was 60 years. Conclusions— ARVD patients present between the second and fifth decades of life either with symptoms of palpitations and syncope associated with ventricular tachycardia or with SCD. Diagnosis is often delayed. Once diagnosed and treated with an ICD, mortality is low. There is a wide variation in presentation and course of ARVD patients, which can likely be explained by the genetic heterogeneity of the disease.
- Subjects :
- Adult
Male
medicine.medical_specialty
Adolescent
Cardiomyopathy
Ventricular tachycardia
Sudden death
Syncope
Sudden cardiac death
Interquartile range
Physiology (medical)
Internal medicine
Palpitations
medicine
Humans
Child
Arrhythmogenic Right Ventricular Dysplasia
Aged
Family Health
business.industry
Arrhythmias, Cardiac
Middle Aged
medicine.disease
United States
Arrhythmogenic right ventricular dysplasia
Defibrillators, Implantable
Survival Rate
Death, Sudden, Cardiac
Heart failure
Child, Preschool
Cardiology
Tachycardia, Ventricular
Female
medicine.symptom
Cardiology and Cardiovascular Medicine
business
Subjects
Details
- ISSN :
- 15244539
- Volume :
- 112
- Issue :
- 25
- Database :
- OpenAIRE
- Journal :
- Circulation
- Accession number :
- edsair.doi.dedup.....76fd64244210dd0ab95d8e4db92f3611