Syncope and risk of sudden death in hypertrophic cardiomyopathy

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

doi:10.1161/circulationaha.108.798314

Cited by 365Cardiomyopathy and Myosin StudiesCardiovascular Syncope and Autonomic DisordersCardiac electrophysiology and arrhythmias

Syncope and risk of sudden death in hypertrophic cardiomyopathy

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Spirito, Paolo, Autore, Camillo, Rapezzi, Claudio, Bernabò, Paola, Badagliacca, Roberto, Maron, Martin S., Bongioanni, Sergio, Coccolo, Fabio +7 more·2009·Circulation

DOI 10.1161/circulationaha.108.798314

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Abstract

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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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, but this varies between specific conditions.
“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]
The Abstract reports a relative risk of sudden death of 1.78 for unexplained syncope in patients with hypertrophic cardiomyopathy, but the confidence interval includes no increase; recent unexplained syncope carried a higher risk, while remote syncope in older patients did not. This supports variation in risk within hypertrophic cardiomyopathy, but the paper does not establish a general two- to four-fold increase across structural heart diseases or inheritable arrhythmia syndromes, or compare risk between those conditions. (LLM, conf=0.87)
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Self-citation
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Part 12: Education, implementation, and teams
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Syncope and Risk of Sudden Death in Hypertrophic
(2016)
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Comment: Syncope and Sudden Death in Hypertrophic Cardiomyopathy
Sheldon, R. S., & Editorial Board (1998) · Clinical Science · doi:10.1042/cs0940335

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: Spirito, Paolo
Autore, Camillo
Rapezzi, Claudio
Badagliacca, Roberto
Maron, Martin S.
Estes, N.A. Mark
Biagini, Elena
Bruzzi, Paolo
Maron, Barry J.
Venue: Circulation
Publisher: Ovid Technologies (Wolters Kluwer Health)
Published: 2009-04-07
Volume: 119
Issue: 13
Pages: 1703-1710
ISSN: 0009-7322 (ISSN-L)
1524-4539
DOI: 10.1161/circulationaha.108.798314
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 › Surgery › 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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Syncope and risk of sudden death in hypertrophic cardiomyopathy

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