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(Circulation. 2009;119:1703-1710.)
© 2009 American Heart Association, Inc.

Arrhythmia/Electrophysiology

Syncope and Risk of Sudden Death in Hypertrophic Cardiomyopathy

Paolo Spirito, MD; Camillo Autore, MD; Claudio Rapezzi, MD; Paola Bernabò, MD; Roberto Badagliacca, MD; Martin S. Maron, MD; Sergio Bongioanni, MD; Fabio Coccolo, MD; N.A. Mark Estes, MD; Caterina S. Barillà, MD; Elena Biagini, MD; Giovanni Quarta, MD; Maria Rosa Conte, MD; Paolo Bruzzi, MD, PhD; Barry J. Maron, MD

From the Ente Ospedaliero Ospedali Galliera (P.S., P. Bernabò), Genoa, Italy; Università La Sapienza (C.A., R.B., C.S.B., G.Q.), Roma, Italy; Universitá di Bologna (C.R., F.C., E.B.), Bologna, Italy; Tufts-New England Medical Center (M.S.M., N.A.M.E.), Boston, Mass; Ospedale di Rivoli (S.B., M.R.C.), Torino, Italy; Istituto Nazionale per la Ricerca sul Cancro (P. Bruzzi), Genoa, Italy; and Hypertrophic Cardiomyopathy Center of the Minneapolis Heart Institute Foundation (B.J.M.), Minneapolis, Minn.

Correspondence to Paolo Spirito, MD, Divisione di Cardiologia, Ente Ospedaliero Ospedali Galliera, Via Volta 8, Genova 16128, Italy. E-mail paolo.spirito{at}galliera.it

Received June 11, 2008; accepted January 14, 2009.

Background— The prognostic significance of syncope has not been investigated systematically in hypertrophic cardiomyopathy, and treatment strategies have been based largely on intuition and experience.

Methods and Results— We assessed the relationship between syncope and sudden death in 1511 consecutive patients with hypertrophic cardiomyopathy. Unexplained (n=153) or neurally mediated (n=52) syncope occurred in 205 patients (14%). Over a 5.6±5.2-year follow-up, 74 patients died suddenly. Relative risk of sudden death was 1.78 (95% confidence interval 0.88 to 3.51, P=0.08) in patients with unexplained syncope and 0.91 (95% confidence interval 0.00 to 3.83, P=1.0) in those with neurally mediated syncope compared with patients without syncope. In multivariable analysis, the temporal proximity of unexplained syncope to initial patient evaluation was independently associated with risk of sudden death (P=0.006). Patients with unexplained syncope within 6 months before the initial evaluation showed a 5-fold increase in risk compared with patients without syncope (adjusted hazard ratio 4.89, 95% confidence interval 2.19 to 10.94), a relationship that was maintained throughout all age groups (<18, 18 to 39, and 40 years). Older patients (40 years of age) with remote episodes of syncope (>5 years before initial evaluation) did not show an increased risk of sudden death (adjusted hazard ratio 0.38, 95% confidence interval 0.05 to 2.74).

Conclusions— In the present large cohort of patients with hypertrophic cardiomyopathy, unexplained syncope was a risk factor for sudden death. Patients with syncopal events that occurred in close temporal proximity to the initial evaluation showed a substantially higher risk of sudden death than patients without syncope. Older patients with remote syncopal events did not show an increased risk.

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				Developed in collaboration with, European Heart Rhythm Association (EHRA), Heart Failure Association (HFA), and Heart Rhythm Society (HRS), Endorsed by the following societies, European Society of Emergency Medicine (EuSEM), European Federation of Internal Medicine (EFIM), European Union Geriatric Medicine Society (EUGMS), American Geriatrics Society (AGS), European Neurological Society (ENS), et al. Guidelines for the diagnosis and management of syncope (version 2009): The Task Force for the Diagnosis and Management of Syncope of the European Society of Cardiology (ESC) Eur. Heart J., November 1, 2009; 30(21): 2631 - 2671. [Full Text] [PDF]

				P. Elliott and W. McKenna The Science of Uncertainty and the Art of Probability: Syncope and Its Consequences in Hypertrophic Cardiomyopathy Circulation, April 7, 2009; 119(13): 1697 - 1699. [Full Text] [PDF]