Hypertrophic Cardiomyopathy
Conditions
Keywords
sudden cardiac death, risk assesment, HCM Risk-SCD Calculator
Brief summary
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.
Detailed description
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.
Interventions
DEVICECardioverter-defibrillator
We analyzed how many HCM patients reached an end-point of sudden cardiac death defined as adequate intervention of cardioverter-defibrillator or sudden cardiac arrest.
Sponsors
Poznan University of Medical Sciences
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Sudden cardiac death (SCD) in risk groups | 2005-2018 | Number of SCD cases in each of risk groups according to HCM Risk-SCD Calculator |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| New risk factors of sudden cardiac death (SCD) | 2005-2018 | New echocardiographic features, such as glibal longitudinal strain, average strain, left atrial volume index, which allow to better estimate the risk of SCD |
Countries
Poland
Outcome results
None listed