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Factors predicting long-term mortality in patients with hypertrophic cardiomyopathy
Cem Bostan1, Umit Yasar Sinan, Polat Canbolat
1Department of Cardiology, Institute of Cardiology, Istanbul University, Haseki, Istanbul/Turkey.
Insights
Long-term mortality in hypertrophic cardiomyopathy (HCM) is predicted by echocardiographic findings like left atrial dimension, not ACE activity. Paroxysmal atrial fibrillation (PAF) also significantly impacts survival in HCM patients.
Area of Science:
- Cardiology
- Genetics
- Medical Diagnostics
Background:
- Hypertrophic cardiomyopathy (HCM) is a complex genetic heart disease.
- Understanding long-term mortality predictors in HCM is crucial for patient management.
- Previous studies have explored various factors, including genetic and echocardiographic markers.
Purpose of the Study:
- To identify factors influencing long-term all-cause mortality in patients with hypertrophic cardiomyopathy (HCM).
- To investigate the role of echocardiographic parameters and angiotensin-converting enzyme (ACE) activity/genetics in HCM mortality.
- To assess the impact of paroxysmal atrial fibrillation (PAF) on mortality risk in HCM.
Main Methods:
- Retrospective analysis of 31 HCM patients diagnosed between 1999 and 2013.
- Echocardiographic examinations were performed, assessing left ventricular (LV) and left atrial (LA) dimensions and LV mass index.
- Plasma ACE activity and gene polymorphism were analyzed, alongside patient history of PAF.
Main Results:
- Left atrial (LA) diameter, LV end-diastolic diameter, LV systolic diameter, and LV mass index were significantly correlated with mortality.
- Patients with a history of paroxysmal atrial fibrillation (PAF) exhibited significantly higher mortality.
- No significant correlation was found between mortality and age, HCM type, ACE gene polymorphism, or plasma ACE levels.
Conclusions:
- Echocardiographic parameters, particularly LA dimension, are significant predictors of long-term mortality in HCM.
- LA diameter (cutoff 4.1 cm) demonstrated high sensitivity and specificity for predicting 13-year mortality.
- PAF is an important factor contributing to long-term mortality in HCM patients, independent of ACE factors.
Objectives:
In this study, we aimed to elucidate the factors affecting long-term all-cause mortality in patients with hypertrophic cardiomyopathy (HCM).
Method:
We retrospectively examined 31 patients (22 males and 9 females) diagnosed with HCM from 1999 to 2013. All subjects had sinus rhythm at the time of evaluation. Four patients had history of paroxysmal atrial fibrillation (PAF). In addition to echocardiographic examination plasma angiotensin-converting enzyme (ACE) activity and gene polymorphism were determined. The variables that were found to be significant in mortality were then included in multivariate analysis.
Results:
At the final follow-up examination, 12 patients had died, including 2 due to congestive heart failure and 10 due to sudden cardiac death. Patients with PAF had significantly higher mortality (P = 0.008). Moreover, left ventricular (LV) end-diastolic diameter (P = 0.04), LV systolic diameter (P = 0.001), LV mass index (P = 0.01), and left atrium diameter (P = 0.003) were found to be significantly correlated with mortality. However, no significant correlation was noted between mortality and age, type of HCM (familial/nonfamilial and obstructive/nonobstructive), ACE gene polymorphism, and plasma ACE level. In the multivariate analysis, left atrial (LA) diameter was still significantly associated with mortality. The LA diameter with a cutoff value of 4.1 cm predicted 13-year mortality with a sensitivity of 82% and specificity of 78%.
Conclusion:
Instead of the ACE genotype and activity, echocardiographic evaluation findings such as LV systolic and diastolic diameters, LV mass index, and particularly LA dimension may predict long-term mortality in patients with HCM. PAF has also significant importance in the long-term mortality in patients with HCM.
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