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Clinical variants and outcomes of progressive chronic heart failure in individuals with hypertrphic cardiomyopathy

Komissarova S. M.1, Zakharova E. Y.1, Iljina E. V.1, Sevruk T. V.1, Kras'ko O. V.2
1 – State Institution Republican Science and Practice Center "Cardiology", R. Luxemburg 110, Minsk 220036, Republic of Belarus
2 – State Science Institution, "United Institute of Informatics Issues of the Belarus National Academy of Sciences", Surganova 6, Minsk 220012, Belarus

Keywords: hypertrophic cardiomyopathy, chronic heart failure, risk factors

DOI: 10.18087/rhfj.2017.2.2326

Background. Determining the types of clinical course and outcomes of hypertrophic cardiomyopathy (HCMP) with symptoms of progressive CHF is still a complicated and not completely solved issue. The article proposes factors identifying patients with a high risk of CHF progression to a pronounced stage. Aim. To determine the types of clinical course and to evaluate survival, outcomes, and effects of factors associated with CHF progression in patients with HCMP. Materials and methods. Clinical and instrumental data were analyzed for 345 patients with HCMP (199 males and 146 females, median age 47, from 17 to 72) who were managed at the Russian Science and Practice Center “Cardiology” from 2006 through 2016. Results. 12 (3.5%) patients with HCMP (mean age, 46±12; males, 58.3%) had symptoms of NYHA FC III-IV CHF associated with pronounced LV myocardial dysfunction. During the observation period (median duration, 6.7 years), unfavorable outcomes were observed for 8 (67%) of 12 patients with symptoms of FC III–IV CHF: sudden cardiac death in one patient; fatal outcomes caused by CHF progression to a “terminal stage” in 6 patients; and acute cerebrovascular disease with a fatal outcome in one patient. Median event-free survival was 4.3 years. During the observation period, symptoms of CHF progressed from NYHA FC II to FC III in 21 (6.1%) patients. According to results of a multifactorial analysis, predictors of the risk for progression of CHF symptoms from NYHA FC II to FC III included reduced LV EF to <56% (HR, 4.8; 95% CI, from 1.6 to 14.5; p<0.001); increased left atrial (LA) size >47 mm (HR, 9.0; 95% CI, from 3.0 to 27.6; p<0.005); and the pseudonormal type of diastolic dysfunction (HR, 9.6; 95% CI, from 2.8 to 33.4; p<0.001). Percent ratio of myocardial fibrosis to total myocardial volume by data of heart MRI identified patients with a risk of unfavorable remodeling at a threshold level of >25%. Conclusion. Isolation of a HCMP patient subgroup with early signs of disease progression is important for timely modification of the therapeutic tactics. Early administration of a standard therapy with β-blockers, ACE inhibitors or angiotensin II receptor antagonists and mineralocorticoid receptor antagonists will allow controlling progression of the disease.
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Komissarova S. M., Zakharova E. Y., Iljina E. V., Sevruk T. V., Kras’ko O. V. Clinical variants and outcomes of progressive chronic heart failure in individuals with hypertrphic cardiomyopathy. Russian Heart Failure Journal. 2017;18 (2):107–114

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