Hypertrophic cardiomyopathy: outcomes of the disease and their prediction
Abstract
Hypertrophic cardiomyopathy (HCM)-related cardiovascular death occurred suddenly, espe- cially in young patients, or as a result of progressive heart failure (HF) or stroke in midlife and beyond.
The aim – to examine the outcomes of HCM in prospective observation of HCM patients during 17 years.
Material and methods. 313 patients with HCM [166 (53%) were men] aged from 16 to 87 years (58.7±15.7 years) were examined. The diagnosis of HCM and functional status of patients was established according to ACCF/AHA recommendations (2011) using ECG and echocardiography (n=313), MRI with gadolinium (n=44), genetic study (n=13), 24-h ECG monitoring, exercise tests. The period of patient’s observation was 1–17 years (6.3±4.9 years). At the end of the study 91 HCM patients remained in observation and 206 HCM patients were dropped out with unknown outcomes.
Results. 9 patients died due to congestive HF progression and thromboembolic complications, 1 patient died due to hemorrhagic stroke. SCD had occurred in 8 cases, and 2 patients were resuscitated. European HCM-Risk-SCD calculator showed a false-negative result (low risk score) in 3 (37.5%) patients with SCD, and ACCF/AHA recommendations – in 2(25%) patients. With the use of logistic regression analysis, we identified additional risk factors for SCD in HCM patients: low exercise tolerance during bicycle ergometer test (<6 min, AUC – 0.92) and high minimum heart rate (>53 per min, OR 11.6, p=0.04) during 24-h ECG monitoring. A prognostic model for SCD risk estimation was proposed in addition to the generally accepted algorithms.
Conclusion. Sudden cardiac death occurred in 7.3% of cases (2 patients were resuscitated) in the observed cohort of HCM patients. Congestive HF progression and thromboembolic complications were the causes of death in 9.2% of cases. The use of the ACCF/AHA algorithm and the European HCM Risk-SCD calculator does not allow to reveal all high risk patients. Additional risk factors were evaluated and a prognostic model was proposed to clarify the risk of SCD in patients with HCM.
Keywords:hypertrophic cardiomyopathy, sudden cardiac death, risk stratification
Clin Experiment Surg. Petrovsky J. 2018; 6 (3): 25–33.
doi: 10.24411/2308-1198-2018-13003. Received: 03.06.2018. Accepted: 10.08.2018.
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