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Syncope and Risk of Sudden Death in Hypertrophic Cardiomyopathy
Open accessAbstract
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 > or =40 years). Older patients (> or =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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“In general, a history of syncope in patients with structural heart disease or inheritable arrhythmia syndromes is associated with a two- to four-fold increased risk of death, 348,350-353 but varies between specific conditions. 354-356”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [354]
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“Personal history of non-vagal syncope is also predictive, particularly if recent.”
From Current management of hypertrophic cardiomyopathy (2025) · cites this paper as [104]
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Citations
Cites
Hypertrophic Cardiomyopathy: A Systematic Review
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Maron, B. J., Spirito, P., Shen, W.-K., Haas, T. S., Formisano, F., Link, M. S., Epstein, A. E., Almquist, A. K., Daubert, J. P., Lawrenz, T., et al. (2007) · JAMA · doi:10.1001/jama.298.4.405 · Cited by 802
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Metadata
- Authors
- Spirito, P., Autore, C., Rapezzi, C., Bernabò, P., Badagliacca, R., Maron, M. S., Bongioanni, S., Coccolo, F., Estes, N. A. M., Barillà, C. S., Biagini, E., Quarta, G., Conte, M. R., Bruzzi, P., & Maron, B. J.
- Affiliations
- Tufts Medical Center (US)
- 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.
- ORCID
- Venue
- Circulation
- Published
- 2009-04-07
- Volume
- 119
- Issue
- 13
- Pages
- 1703-1710
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2103423712
- Cited by
- 365
- References
- 30
- Citations by year
- 2026: 8 · 2025: 24 · 2024: 16 · 2023: 18 · 2022: 12 · 2021: 18 · 2020: 23 · 2019: 12 · 2018: 26 · 2017: 34 · 2016: 21 · 2015: 25 · 2014: 21 · 2013: 19 · 2012: 30
- FWCI
- 18.84
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- Archivio istituzionale della ricerca (Alma Mater Studiorum Università di Bologna) (submittedVersion)
- IRIS Research product catalog (Sapienza University of Rome) (submittedVersion)
- Institutional Research Information System University of Ferrara (University of Ferrara) (submittedVersion, cc-by-nc-nd)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiomyopathy and Myosin Studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Syncope and Autonomic Disorders
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Keywords
- hypertrophic cardiomyopathy, syncope, sudden death, unexplained syncope, neurally mediated syncope, risk factors, prognosis, age-related risk
- MeSH terms
- Adolescent, Adult, Aged, Cardiomyopathy, Hypertrophic, Female, Follow-Up Studies, Humans, Male, Middle Aged, Prognosis, Risk Factors, Syncope, Multivariate Analysis, Proportional Hazards Models, Death, Sudden, Cardiac, Defibrillators, Implantable, Age Distribution, Kaplan-Meier Estimate, Young Adult
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