title: [Natural history of hypertrophic obstructive cardiomyopathy in young patients: apropos of 40 cases].
abstract: This retrospective study analysed the outcome of children with hypertrophic obstructive cardiomyopathy. Between 1969 and 1992, 40 patients all under 20 years of age (mean = 10.9 +/- 6.2 years) were followed up for this condition. There was a positive family history of hypertrophic cardiomyopathy and/or sudden death in 21 cases (53%); 21 (53%) were symptomatic: > NYHA Stage II dyspnoea (n = 13); chest pain (n = 8); syncope (n = 7) or palpitations (n = 4). Thirty-two patients were treated by betablockers, 1 by verapamil, 2 by amiodarone (associated with propranolol in 1 case); 7 patients underwent surgery and 6 others, asymptomatic, had no specific treatment. The mean follow-up period was 10.2 +/- 6.2 years with no drop-out: 13 patients died (1 non-cardiac death, 2 of unknown causes and 10 of cardiac causes, including 8 sudden deaths) giving an annual global mortality of 3.2%; the actuarial 5 and 10 year survival rates were 90 and 85% respectively. The 27 survivors were compared with the 13 patients who died: of the 11 clinical and paraclinical criteria examined, only a previous history of syncope correlated with global mortality (p = 0.004) and sudden death (p = 0.0008).
authors.affiliation: Hôpital cardiologique, service de cardiologie C, Lyon.
authors.forename: O
authors.initials: O
authors.lastname: Azzano
authors.forename: A
authors.initials: A
authors.lastname: Bozio
authors.forename: F
authors.initials: F
authors.lastname: Sassolas
authors.forename: S
authors.initials: S
authors.lastname: Di Filippo
authors.forename: C
authors.initials: C
authors.lastname: Agé
authors.forename: M
authors.initials: M
authors.lastname: André
authors.forename: D
authors.initials: D
authors.lastname: Jocteur-Monrozier
authors.forename: J
authors.initials: J
authors.lastname: Normand
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002312:Cardiomyopathy, Hypertrophic / Q000401:mortality / Q000503:physiopathology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D017202:Myocardial Ischemia / Q000503:physiopathology; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D013575:Syncope / Q000503:physiopathology; D017180:Tachycardia, Ventricular / Q000503:physiopathology
vernacular_title: Histoire naturelle de la myocardiopathie hypertrophique obstructive du sujet jeune: à propos de 40 observations.