Am J Cardiol. 2014 Feb 12. pii: S0002-9149(14)00637-7. doi: 10.1016/j.amjcard.2014.01.435. [Epub ahead of print]
Risk of Sudden Death and Outcome in Patients With Hypertrophic Cardiomyopathy With Benign Presentation and Without Risk Factors.
Spirito P1, Autore C2, Formisano F3, Assenza GE2, Biagini E4, Haas TS5, Bongioanni S6, Semsarian C7, Devoto E3, Musumeci B2, Lai F4, Yeates L7, Conte MR6, Rapezzi C4, Boni L8, Maron BJ5.
Patients with hypertrophic cardiomyopathy (HC) are reported to have a mortality rate of about 1.0% per year, and those patients without sudden death risk factors and with no or mild symptoms are generally considered to have a benign clinical presentation. However, the risk of sudden death and the outcome in this latter subgroup have not been investigated systematically and remain unresolved. We assessed the risk of sudden death and outcome in 653 consecutive patients with HC without risk factors and with no or mild symptoms. Over a median follow-up of 5.3 years, 35 patients (5.4%) died of HC-related causes. Mean age at death was 46 ± 20 years in patients who died suddenly and 66 ± 15 and 72 ± 9 years, respectively, in patients who died of heart failure or stroke. Event rate was 0.6% per year for sudden death, 0.2% per year for heart failure death, and 0.1% per year for stroke-related death. Sudden death risk was independently and inversely related to age, and risk of heart failure or stroke death was directly related to age (p = 0.020). At 10 years after the initial evaluation, sudden death risk was 5.9%, with sudden death rate being the lowest (0.3% per year) in patients with normal left atrial dimension (≤40 mm). In conclusion, in patients with HC without conventional risk factors and with no or mild symptoms, the risk of sudden death was not negligible, with an event rate of 0.6% per year. Heart failure and stroke-related death were less common and largely confined to older patients. These results underscore the need for a more accurate assessment of the sudden death risk in patients with HC.
Copyright © 2014 Elsevier Inc. All rights reserved.
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