Giuseppe Mascia1, Lia Crotti2, Antonella Groppelli2, Marco Canepa3, Andrea Carlo Merlo3, Stefano Benenati3, Paolo Di Donna1, Roberta Della Bona1, Davide Soranna2, Antonella Zambon2, Italo Porto3, Iacopo Olivotto4, Gianfranco Parati2, Michele Brignole2, Franco Cecchi5. 1. Department of Cardiology, Cardiovascular Disease Unit, IRCCS Ospedale Policlinico San Martino, Genova, Italy. 2. Department of Cardiology, IRCCS Istituto Auxologico Italiano, Department of Cardiovascular, Neural and Metabolic Sciences, Ospedale San Luca, Milan, Italy. 3. Department of Cardiology, Cardiovascular Disease Unit, IRCCS Ospedale Policlinico San Martino, Genova, Italy; Department of Cardiology, IRCCS Istituto Auxologico Italiano, Department of Cardiovascular, Neural and Metabolic Sciences, Ospedale San Luca, Milan, Italy; Department of Cardiology, Department of Internal Medicine, University of Genoa, Italy. 4. Department of Cardiology, Cardiomyopathy Unit, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. 5. Department of Cardiology, IRCCS Istituto Auxologico Italiano, Department of Cardiovascular, Neural and Metabolic Sciences, Ospedale San Luca, Milan, Italy. Electronic address: francocecchi337@gmail.com.
Abstract
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).
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).