Sudden death in hypertrophic cardiomyopathy: an integrative review of recent literature
DOI:
https://doi.org/10.55892/jrg.v9i20.3108Keywords:
Sudden death, Hypertrophic cardiomyopathy, Heart diseasesAbstract
The hypertrophic cardiomyopathy is a genetic disease that follows an autosomal dominant inheritance pattern, characterized by a pathological increase in left ventricular mass without the influence of other associated conditions. Given the highly heterogeneous distribution of this genetic disorder, its prevalence ratio can reach up to 1 in 200 individuals, affecting both genders and all ethnicities similarly, and it is strongly associated with sudden death. Furthermore, the lack of information regarding the hereditary nature of the disease and the failure to communicate the diagnosis to close family members are additional factors that directly impact the early detection of other cases and their effective management. OBJECTIVE: To analyze the determinant aspects of hypertrophic cardiomyopathy and its causal relationship with the occurrence of sudden death. METHODOLOGY: This is an integrative review in which the search for research articles occurred in August 2025 across the Science Direct, SciELO, and PubMed databases, resulting in 570 articles; after applying inclusion and exclusion criteria, 21 articles remained for the development of the study. RESULTS: It was found that hypertrophic cardiomyopathy constitutes one of the leading causes of sudden cardiac death in individuals who do not present prior pathologies related to this severe outcome. CONCLUSION: Despite the incidence of sudden cardiac death in patients with hypertrophic cardiomyopathy, advances in technology and medicine have been contributing to the reduction of these episodes, providing a more favorable prognosis.
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References
AMANO, Masashi et al. Validation of Guideline Recommendation on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy. JACC: Heart Failure, v. 13, n. 6, 2025.
BARON, Émilie et al. Management and outcomes of hypertrophic cardiomyopathy in young adults. Archives of Cardiovascular Disease, v.114, p. 465-473, 2021.
BOLETI, Olga D. et al. Sudden cardiac death in childhood RASopathy-associated hypertrophic cardiomyopathy: Validation of the HCM risk-kids model and predictors of events. International Journal of Cardiology, v. 393, Artigo 131405, 2023.
CAMARGO, Luís Marcelo Aranha; SILVA, Romeu Paulo Martins; MENEGUETTI, Dionatas Ulises de Oliveira. Tópicos de metodologia de pesquisa: Estudos de coorte ou cohorte prospectivo e retrospectivo. J. Hum. Growth Dev., São Paulo, v. 29, n. 3, p. 433-436, dez. 2019 .
CHIU, Shuenn-Nan et al. Clinical manifestations, genetic profiles, and sudden cardiac arrest in pediatric hypertrophic cardiomyopathy: Challenges of risk prediction for initial sudden cardiac arrest presentations. Heart Rhythm, v. 6, n. 7, 2025.
DE LEON, J. D. L. P. et al. Hypertrophic Cardiomyopathy in a Latin American Center: A Single Center Observational Study. Journal of Clinical Medicine, v. 12, n. 17, p. 5682–5682, 31 ago. 2023.
EDELSON, Jonathan B. et al. Cardiopulmonary Exercise Testing in Pediatric Patients With Hypertrophic Cardiomyopathy. JACC: Advances, v. 1, n. 4, 2022.
FAHMY, Ahmed S. et al. Radiomics of Late Gadolinium Enhancement Reveals Prognostic Value of Myocardial Scar Heterogeneity in Hypertrophic Cardiomyopathy. JACC Cardiovascular Imaging, v. 17, n. 1, 2024.
HABIB, Manhal et al. Progression of Myocardial Fibrosis in Hypertrophic Cardiomyopathy: A Cardiac Magnetic Resonance Study. JACC Cardiovascular Imaging, v. 14, n. 5, 2021.
HO, C. Y. et al. Valsartan in early-stage hypertrophic cardiomyopathy: a randomized phase 2 trial. Nature Medicine, v. 27, n. 10, p. 1818–1824, 23 set. 2021.
HUTT, E.; DESAI, M. Y. Medical Treatment Strategies for Hypertrophic Cardiomyopathy. The American Journal of Cardiology, v. 212, 1 jan. 2024.
ITO, Yuki et al. Effect of beta-blockers and exercise restriction on the prevention of sudden cardiac death in pediatric hypertrophic cardiomyopathy. Journal of Cardiology, v. 83, n. 4, p. 407-414, 2024.
JACQUEMYN, Xander et al. Myocardial Work in Children With Hypertrophic Cardiomyopathy: Longitudinal Evaluation and Prognostic Implications. JACC: Advances, v. 4, n. 7, 2025.
KANNEGAANTI, Vineetha et al. Implantable cardioverter defibrillators for primary prevention in cardiomyopathies. Indian Heart Journal, v. 76, p. 118-122, 2024.
LEE, Hyun-Jung et al. Performance of 2020 AHA/ACC HCM Guidelines and Incremental Value of Myocardial Strain for Predicting SCD. JACC: Asia, v. 4, n. 1, 2024.
LÓPEZ BLÁZQUEZ, María et al. Implicación pronóstica de factores no clásicos en el riesgo de muerte súbita en miocardiopatía hipertrófica. Anales de Pediatría, v. 102, Artigo 503814, 2025.
MAHMOD, Masliza et al. Right ventricular function declines prior to left ventricular ejection fraction in hypertrophic cardiomyopathy. Journal of Cardiovascular Magnetic Resonance, v. 24, n. 36, 2022.
MARON, Barry J. et al. Diagnosis and Evaluation of Hypertrophic Cardiomyopathy: JACC State-of-the-Art Review. Journal of the American College of Cardiology, [s. l.], v. 79, n. 4, p. 372-389, 1 fev. 2022. DOI: 10.1016/j.jacc.2021.12.002.
MASRI, Ahmad et al. Efficacy and Safety of Aficamten in Symptomatic Nonobstructive Hypertrophic Cardiomyopathy: Results From the REDWOOD-HCM Trial, Cohort 4. Journal of Cardiac Failure, v. 30, p. 1439-1448, 2024.
MAURIZI, Niccolò et al. Lifetime Clinical Course of Hypertrophic Cardiomyopathy: Outcome of the Historical Florence Cohort Over 5 Decades. JACC: Advances, v. 2, n. 4, 2023.
NAKASUKA, Kosuke et al. Future bradyarrhythmia in patients with hypertrophic cardiomyopathy. IJC Heart & Vasculature, v. 33, Artigo 100735, 2021.
OLIVEIRA, J. L. M. et al. Ecocardiografia sob estresse físico na cardiomiopatia hipertrófica: como, quando e por quê? Arquivos Brasileiros de Cardiologia: Imagem Cardiovascular, v. 3, eabc130, mar. 2021.
PETEIRO, Jesus et al. Value of a comprehensive exercise echocardiography assessment for patients with hypertrophic cardiomyopathy. Journal of Cardiology, v. 77, p. 525-531, 2021.
SALEH, Danish; ESKANDARI, Maeve; CHOUDHURY, Lubna. Aficamten in the treatment of obstructive hypertrophic cardiomyopathy. Future Cardiology, [s. l.], v. 21, n. 9, p.685-691, 2025. DOI: 10.1080/14796678.2025.2521183.
SANTOS-VELOSO, Marcelo Antônio Oliveira et al. ICD indication in hypertrophic cardiomyopathy: which algorithm to use? Revista da Associação Médica Brasileira, São Paulo, v. 68, n. 8, p. 1097-1104, ago. 2022.
STACHERA, M. et al. Comparison of Nonclassic and Classic Phenotype of Hypertrophic Cardiomyopathy Focused on Prognostic Cardiac Magnetic Resonance Parameters: A Single-Center Observational Study. Diagnostics, v. 12, n. 5, p. 1104–1104, 28 abr. 2022.
TSUDA, Etsuko et al. Thirty-year outcome in children with hypertrophic cardiomyopathy based on the type. Journal of Cardiology, v. 80, p. 557-562, 2022.
VEHMEIJER, J. T. et al. Identification of patients at risk of sudden cardiac death in congenital heart disease: The prospective study on implantable cardioverter defibrillator therapy and sudden cardiac death in adults with congenital heart disease (PREVENTION-ACHD). Heart Rhythm, v. 18, n. 5, p. 785–792, 1 maio 2021.
VELICKI, L. et al. Sacubitril/valsartan for the treatment of non‐obstructive hypertrophic cardiomyopathy: An open label randomized controlled trial (SILICOFCM). European Journal of Heart Failure, v. 26, n. 6, p. 1361–1368, 27 maio 2024.
VULLAGANTI, Sirish et al. Fibrosis in Hypertrophic Cardiomyopathy Patients With and Without Sarcomere Gene Mutations. Heart, Lung and Circulation, v. 30, p. 1496-1501, 2021.
ZEGKOS, Thomas et al. Validation of the new American College of Cardiology/American Heart Association Guidelines for the risk stratification of sudden cardiac death in a large Mediterranean cohort with Hypertrophic Cardiomyopathy. Hellenic Journal of Cardiology, v. 63, p. 15-21, 2022.
