title: Hypertrophic cardiomyopathy with longevity to 90 years or older.
abstract: Hypertrophic cardiomyopathy (HC) is the most common cause of sudden death in the young, but survival to particularly advanced age is less well appreciated. The investigators report the prevalence, clinical features, and demographics of patients with HC surviving to ≥90 years of age. Of 1,297 patients with HC in the Hypertrophic Cardiomyopathy Center database (Minneapolis Heart Institute Foundation), 26 (2.0%) were identified who had achieved the age of ≥90 years; 18 (69%) were women. HC diagnosis came late in life, at 61 to 92 years (mean 80 ± 8; ≥75 years in 21 patients), recognized fortuitously by the detection of a heart murmur or during family screening (n = 6) or after onset of new symptoms (n = 20). At most recent evaluation (or death) patients were aged 90 to 96.7 years (mean 92.2 ± 2), with 6 presently alive at 91 to 96 years of age; HC did not appear to be the primary cause of death in any patient. Left ventricular wall thicknesses were 15 to 31 mm (mean 20 ± 3); 8 patients (31%) had obstruction to left ventricular outflow at rest (peak instantaneous gradients, 38 to 135 mm Hg). Significant HC-related complications occurred in 13 patients (50%), including progressive heart failure symptoms, atrial fibrillation, and nonfatal embolic stroke. Although no patient died suddenly, 13 (50%) nevertheless carried conventional HC risk markers. A greater proportion of cohort patients reached ≥90 years of age (2.0%) than expected in the general population (0.8%) (p <0.001). In conclusion, HC may be unrecognized until late in life and is consistent with survival to particularly advanced age into the 10th decade of life without the need for major HC-related treatment interventions, and with demise ultimately largely unrelated to this disease. This principle regarding the natural history of HC can afford a measure of reassurance to many patients.
authors.affiliation: Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minnesota, USA. hcm.maron@mhif.org
authors.forename: Barry J
authors.initials: BJ
authors.lastname: Maron
authors.forename: Susan A
authors.initials: SA
authors.lastname: Casey
authors.forename: Tammy S
authors.initials: TS
authors.lastname: Haas
authors.forename: Carrie L
authors.initials: CL
authors.lastname: Kitner
authors.forename: Ross F
authors.initials: RF
authors.lastname: Garberich
authors.forename: John R
authors.initials: JR
authors.lastname: Lesser
mesh_terms: D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000401:mortality*; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000209:etiology*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D008910:Minnesota / Q000453:epidemiology; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D013997:Time Factors