Risk of death in long-term follow-up of patients with apical hypertrophic cardiomyopathy

Kyle W. Klarich, Christine H. Attenhofer Jost, Josepha Binder, Heidi M. Connolly, Christopher G. Scott, William K. Freeman, Michael John Ackerman, Rick A. Nishimura, A. Jamil Tajik, Steve R. Ommen

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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.

Original languageEnglish (US)
Pages (from-to)1784-1791
Number of pages8
JournalAmerican Journal of Cardiology
Volume111
Issue number12
DOIs
StatePublished - Jun 15 2013

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ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Klarich, K. W., Attenhofer Jost, C. H., Binder, J., Connolly, H. M., Scott, C. G., Freeman, W. K., Ackerman, M. J., Nishimura, R. A., Tajik, A. J., & Ommen, S. R. (2013). Risk of death in long-term follow-up of patients with apical hypertrophic cardiomyopathy. American Journal of Cardiology, 111(12), 1784-1791. https://doi.org/10.1016/j.amjcard.2013.02.040