Factors Predicting Long-Term Mortality in Patients with Hypertrophic Cardiomyopathy

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.