title: Risk of death in long-term follow-up of patients with apical hypertrophic cardiomyopathy.
abstract: Apical hypertrophic cardiomyopathy (HC) has been considered a "benign" form of HC, with limited data on long-term outcome. We compared apical HC patients with a non-HC, age- and gender-matched Minnesota white population to identify outcomes and prognostic factors. Between 1976 and 2006, 193 patients (62% men) with apical HC were seen at our clinic. Their most recent echocardiographic examinations were reviewed. Mean ± SD age at first presentation was 58 ± 17 years. A family history of HC or sudden cardiac death (SCD) was reported by 43 patients (22%); coronary artery disease was known in 22 (11%). An apical pouch was present in 29 patients, including an apical aneurysm in 6 and apical dilatation with hypokinesis in 23. Median follow-up (187 patients [97%]) was 78 months (range, 1-350). Death from all causes occurred in 55 patients (29%; 33 women) at a mean age of 72 years (range, 20-92). During follow-up, more women had heart failure (p = 0.001), atrial fibrillation (p = 0.009), or died (p <0.001) than men. Survival was worse than expected (p = 0.001); the observed versus expected 20-year survival was 47% versus 60%. SCD, resuscitated cardiac arrest, and/or defibrillator discharge was observed in 11 patients (6%) during follow-up. Multivariate predictors of decreased survival were higher age at baseline (p <0.001), female gender (p <0.001), and atrial fibrillation at baseline (p = 0.06). In conclusion, apical HC in this population was associated with increased mortality, especially in women. Because apical HC is less benign than previously suspected, careful longitudinal care is warranted.
authors.affiliation: Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA. klarich.kyle@mayo.edu
authors.forename: Kyle W
authors.initials: KW
authors.lastname: Klarich
authors.forename: Christine H
authors.initials: CH
authors.lastname: Attenhofer Jost
authors.forename: Josepha
authors.initials: J
authors.lastname: Binder
authors.forename: Heidi M
authors.initials: HM
authors.lastname: Connolly
authors.forename: Christopher G
authors.initials: CG
authors.lastname: Scott
authors.forename: William K
authors.initials: WK
authors.lastname: Freeman
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Ackerman
authors.forename: Rick A
authors.initials: RA
authors.lastname: Nishimura
authors.forename: A Jamil
authors.initials: AJ
authors.lastname: Tajik
authors.forename: Steve R
authors.initials: SR
authors.lastname: Ommen
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001281:Atrial Fibrillation / Q000209:etiology; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000000981:diagnostic imaging / Q000235:genetics / Q000401:mortality* / Q000628:therapy; D024741:Cardiomyopathy, Hypertrophic, Familial / Q000401:mortality; D016022:Case-Control Studies; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D018570:Risk Assessment; D012307:Risk Factors; D017678:Sex Distribution; D016019:Survival Analysis; D013997:Time Factors; D014463:Ultrasonography