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Syncope in hypertrophic cardiomyopathy (part I): An updated systematic review and meta-analysis
Giuseppe Mascia1, Lia Crotti2, Antonella Groppelli2
1Department of Cardiology, Cardiovascular Disease Unit, IRCCS Ospedale Policlinico San Martino, Genova, Italy.
Insights
Syncope affects 15.8% of hypertrophic cardiomyopathy (HCM) patients and doubles the risk of life-threatening events. Unexplained syncope in HCM requires clearer diagnostic and management guidelines.
Area of Science:
- Cardiology
- Electrophysiology
- Genetics
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
- Syncope is a common but often unexplained symptom in HCM patients.
- The prognostic significance of syncope in HCM requires further elucidation.
Purpose of the Study:
- To determine the prevalence of syncope in patients with HCM.
- To evaluate syncope as a risk factor for sudden cardiac death and life-threatening arrhythmic events in HCM.
- To highlight the need for improved diagnostic and management strategies for syncope in HCM.
Main Methods:
- Systematic review of original articles published from 1973 to 2021.
- Literature search of PubMed database.
- Meta-analysis of data from 57 articles encompassing 21,791 HCM patients.
Main Results:
- Syncope was reported in 15.8% of HCM patients, with 91% of cases unexplained.
- Patients with syncope had a higher incidence of life-threatening arrhythmic events (7.7% vs 3.6%).
- Syncope was associated with a nearly twofold increased risk (Relative Risk 1.99) of adverse events.
Conclusions:
- Syncope is a significant predictor of adverse outcomes in HCM patients.
- The etiology of syncope remains unexplained in the majority of HCM cases.
- Current clinical practice lacks precise guidelines for syncope evaluation and management in HCM.
Aims:
To describe the proportion of patients with syncope among those affected by hypertrophic cardiomyopathy (HCM) and the relevance of syncope as risk factor for sudden cardiac death and life-threatening arrhythmic events.
Method And Results:
Systematic review of original articles that assessed syncope in HCM patients. Literature search of PubMed including all English publications from 1973 to 2021.We found 57 articles for a total of 21.791 patients; of these, 14 studies reported on arrhythmic events in the follow-up. Syncope was reported in 15.8% (3.452 of 21.791) patients. It was considered unexplained in 91% of cases. Life-threatening arrhythmic events occurred in 3.6% of non-syncopal patients and in 7.7% of syncopal patients during a mean follow-up of 5.6 years. A relative risk of 1.99 (95%CI 1.39 to 2.86) was estimated for syncope patients by the random effect model using Haldane continuity correction for 0 events.
Conclusions:
In the current practice, the cause of syncope remained unexplained in most patients affected by HCM. The management of patients seems mainly driven by risk stratification rather than identification of the aetiology of syncope. There is a need of precise instructions how to apply the recommendations of current guidelines to this disease, which tests are indicated and how to interpret their findings. The protocol was registered in Prospero (ID: 275963).
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