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Published on: June 14, 2016
Survival and prognostic factors in hypertrophic cardiomyopathy: a meta-analysis
Qun Liu1, Diandian Li1,2, Alan E Berger1
1Division of Allergy & Clinical Immunology, Johns Hopkins University School of Medicine, Baltimore, MD, 21224, USA.
Insights
This meta-analysis reveals survival rates for hypertrophic cardiomyopathy (HCM) patients are high, with key factors like non-sustained ventricular tachycardia (nsVT) and obstruction predicting cardiovascular death. Early identification of these risk factors is crucial for timely interventions.
Area of Science:
- Cardiology
- Genetics
- Clinical Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is a complex genetic disorder with variable clinical presentations.
- Existing data on survival rates and prognostic factors in HCM patients are conflicting and lack comprehensive review.
Purpose of the Study:
- To perform a meta-analysis of cohort studies to determine pooled survival rates in HCM patients.
- To identify significant prognostic factors associated with survival in individuals diagnosed with HCM.
Main Methods:
- A meta-analysis was conducted, synthesizing data from nineteen cohort studies.
- The analysis included a total of 12,146 patients diagnosed with hypertrophic cardiomyopathy.
Main Results:
- Pooled survival rates at 1, 3, 5, and 10 years were 98.0%, 94.3%, 82.2%, and 75.0%, respectively.
- Significant prognostic factors for cardiovascular death included age, NYHA class, family history of sudden death (FHSD), syncope, atrial fibrillation, non-sustained ventricular tachycardia (nsVT), maximum left ventricular wall thickness, and obstruction.
- Non-sustained ventricular tachycardia (nsVT) and left ventricular outflow tract obstruction/mid-ventricular obstruction (LVO/MVO) were identified as the strongest predictors for cardiovascular and all-cause/sudden cardiac death, respectively.
Conclusions:
- Survival rates in HCM patients are generally high, but specific risk factors significantly impact outcomes.
- Identifying patients at higher risk through factors like nsVT and obstruction is critical for targeted therapeutic interventions.
- These findings can guide early diagnosis and management strategies to improve long-term survival in hypertrophic cardiomyopathy.
Abstract:
Hypertrophic cardiomyopathy (HCM) is a clinically and genetically heterogeneous disorder but data on survival rates are still conflicting and have not so far been quantitatively reviewed. The aim of this study is to conduct a meta-analysis of cohort studies to assess pooled survival rates and prognostic factors for survival in patients with HCM. Nineteen studies were included representing 12,146 HCM patients. The pooled 1-, 3-, 5- and 10-year survival rates were 98.0%, 94.3%, 82.2% and 75.0%, respectively. Among patients with HCM, age, NYHA functional class, family history of sudden death (FHSD), syncope, atrial fibrillation, non-sustained ventricular tachycardia (nsVT), maximum left ventricular wall thickness and obstruction were significant prognostic factors for cardiovascular death. For sudden cardiac death, FHSD, nsVT, and obstruction showed significant predictive values. Moreover, estimation of population attributable risk (PAR) suggested that nsVT was the strongest predictor for cardiovascular death (13.02%, 95% CI 3.60-25.91%), while left ventricular outflow tract obstruction/mid-ventricular obstruction (LVO/MVO) was the strongest predictor for all-cause death and sudden cardiac death (10.09%, 95% CI 4.72-20.42% and 16.44%, 95% CI 7.45-31.55%, respectively). These risk factors may thus be useful for identifying HCM patients who might benefit from early diagnosis and therapeutic interventions.
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