Clinical InvestigationRisk of Sudden Death in End-Stage Hypertrophic Cardiomyopathy
Section snippets
Study Population and Diagnostic Criteria
A total of 490 consecutive patients with HCM (334 with nonobstructive HCM and 156 with obstructive HCM; the mean age at diagnosis: 46 ± 15 years) were diagnosed and followed-up at Tokyo Women’s Medical University Hospital (Tokyo, Japan), which is a referral center, between 1980 and 2005.13 We retrospectively analyzed the incidence and clinical characteristics of end-stage HCM during follow-up after the initial diagnosis of HCM. We also analyzed cardiovascular mortality in patients with
Incidence and Baseline Characteristics
Among 490 patients with HCM, 43 (8.8%) patients showed progression to end-stage disease during a mean follow-up period of 12 ± 7 years after the initial diagnosis of HCM. The annual incidence of end-stage HCM was 0.73%. Among these 43 patients, 28 patients (65%) had an LVEF <50% and left ventricular diastolic dimension (LVDD) ≥55 mm, whereas the remaining 15 patients (35%) only had an LVEF <50%. The patients with end-stage HCM were aged 44 ± 15 years (range, 6 to 68 years) at the initial
Discussion
This study demonstrated that 43 (8.8%) of 490 HCM patients progressed to end-stage disease during a mean follow-up period of 12 ± 7 years after the initial diagnosis of HCM, with the annual incidence of progression being 0.73%. The prevalence of cardiovascular mortality, especially sudden death, was also very high during a mean follow-up of 5 ± 3 years after the diagnosis of end-stage HCM. Furthermore, the presence of syncope at the time of diagnosing end-stage HCM diagnosis was an independent
Acknowledgments
We thank Naomi Kawashiro, MD, Bun Yashiro, MD, and Yutaka Terajima, MD, for their excellent clinical support, and also express our appreciation to Katsunori Shimada, PhD (STATZ Institute, Inc, Tokyo, Japan), for his expert statistical assistance.
Disclosures
None.
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Cited by (28)
Validation of the Risk Stratification for Sudden Cardiac Death in Chinese Patients With Hypertrophic Cardiomyopathy
2023, Current Problems in CardiologyImportance of newer cardiac magnetic resonance–based risk markers for sudden death prevention in hypertrophic cardiomyopathy: An international multicenter study
2022, Heart RhythmCitation Excerpt :Beginning in 2003 and most recently in 2020, North American expert consensus guidelines recommended a strategy for primary prevention ICD decisions based on ≥1 risk markers regarded as major within the clinical profile of HCM patients.2,4–6 A strength of this approach is its inherent flexibility, providing the opportunity to evolve by incorporating emerging risk markers, evident most recently by the inclusion into the 2020 AHA/ACC HCM guidelines of a number of newer CMR-based SD risk markers, including LV apical aneurysm, extensive LGE, and/or end-stage progression with systolic dysfunction (EF <50%).11–26 In contrast to the AHA/ACC, the ESC promoted a predictive approach to risk stratification with risk scores based on a formula comprising selected clinical variables.3,27
Cardiovascular magnetic resonance predictors of heart failure in hypertrophic cardiomyopathy: the role of myocardial replacement fibrosis and the microcirculation
2021, Journal of Cardiovascular Magnetic ResonanceOutcomes in Patients With Hypertrophic Cardiomyopathy and Left Ventricular Systolic Dysfunction
2020, Journal of the American College of CardiologyCitation Excerpt :HCM patients in the ES phase are characterized by systolic dysfunction, often associated with adverse LV remodeling due to diffuse myocardial replacement scarring (17,18,30–32). This HCM subgroup represents a small but highly visible and malignant expression of a diverse disease who are at high risk for heart failure–related morbidity and mortality and sudden death (8–13). To date, most ES reports are comprised of small numbers of patients reflecting early experiences with ES treatment, and therefore, some uncertainty remains regarding the long-term natural history of ES with the application of more contemporary treatments and interventions.
Recent progress in end-stage hypertrophic cardiomyopathy
2015, American Journal of the Medical SciencesCitation Excerpt :Over 11 ± 9 years of follow-up, Biagini et al2 identified LV wall thickness and left ventricular end-diastolic diameter as independent predictors of cardiovascular death after a mean 5-year progression to ES-HCM. Kawarai et al7 reported that sudden death was the main cause of cardiovascular death in incident ES-HCM over a mean of 5 ± 3 years of follow-up, with syncope, an independent factor for sudden death. However, the population numbers in these reports are low.
Conflict of interest: None.
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