Efficacy of Risk Assessment for Sudden Cardiac Death in Patients With Hypertrophic Cardiomyopathy

Efficacy of Risk Assessment for Sudden Cardiac Death in Patients With Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium and is most often caused by mutations in sarcomere genes. The structural and functional abnormalities cannot be explained by flow-limiting coronary artery disease or loading conditions. The disease affects at least 0,2% of the population worldwide and is the most common cause of sudden cardiac death (SCD) in young people and competitive athletes due to fatal ventricular arrhythmia, but in most patients, however, HCM has a benign course. Therefore, it is of utmost importance to properly evaluate patients and identify those who would benefit from a cardioverter-defibrillator (ICD) implantation.

Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium and is most often caused by mutations in sarcomere genes. The structural and functional abnormalities cannot be explained by flow-limiting coronary artery disease or loading conditions. The disease affects at least 0,2% of the population worldwide and is the most common cause of sudden cardiac death (SCD) in young people and competitive athletes due to fatal ventricular arrhythmia, but in most patients, however, HCM has a benign course. Therefore, it is of utmost importance to properly evaluate patients and identify those who would benefit from a cardioverter-defibrillator (ICD) implantation.

This study assesses the accuracy of the HCM SCD-Risk Calculator, recommended by European Society of Cardiology guidelines, in patients treated in the Ist Department of Cardiology of Poznan University of Medical Sciences from 2005 to 2018.

The study group consisted of 252 patients aged 20-88 (mean 53,8 ± 15,1, median 54); 49,6% were men. The protocol consisted of medical history collection (including a questionnaire), physical examination and additional tests such as echocardiography with the assessment of global longitudinal strain and average strain, cardiac magnetic resonance, ambulatory ECG monitoring, control of implantable devices and exercise testing.

Inclusion Criteria:

1. Hypertrophic cardiomyopathy,
2. Minimum 18 years,
3. Questionnaire,
4. Assessment of HCM SCD-Risk Calculator at the beginning of the observation,
5. Regular visits in Cardiology Outpatient Clinic,
6. Agreement to participate in the study

Exclusion Criteria:

1. Poorly controlled hypertension (systolic pressure on the next two visits in the Cardiology Outpatient Clinic >180 mmHg),
2. Haemodynamically significant valvular heart disease or valve replacement condition,
3. Past myocardial infarction (haemodynamically significant changes found in coronarography),
4. Heart transplant,
5. Insufficient amount of data from the subject and additional tests allowing for further analysis,
6. Lack of consent to participate in the study.