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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Low risk | Low risk of sudden cardiac death according to HCM Risk-SCD Calculator |
| |
| Intermediate risk | Intermediate risk of sudden cardiac death according to HCM Risk-SCD Calculator |
| |
| High risk | High risk of sudden cardiac death according to HCM Risk-SCD Calculator |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Cardioverter-defibrillator | Device | 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. |
| Measure | Description | Time Frame |
|---|---|---|
| Sudden cardiac death (SCD) in risk groups | Number of SCD cases in each of risk groups according to HCM Risk-SCD Calculator | 2005-2018 |
| Measure | Description | Time Frame |
|---|---|---|
| New risk factors of sudden cardiac death (SCD) | New echocardiographic features, such as glibal longitudinal strain, average strain, left atrial volume index, which allow to better estimate the risk of SCD | 2005-2018 |
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Inclusion Criteria:
Exclusion Criteria:
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The study group consisted of 252 patients with hypertrophic cardiomyopathy aged 20-88 (mean 53,8 ± 15,1, median 54); 49,6% were men.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Poznan Univeristy of Medical Sciences | Poznan | Wielkopolska | 61-848 | Poland |
Not possible due to Polish law regulations.
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| ID | Term |
|---|---|
| D002312 | Cardiomyopathy, Hypertrophic |
| D016757 | Death, Sudden, Cardiac |
| ID | Term |
|---|---|
| D009202 | Cardiomyopathies |
| D006331 | Heart Diseases |
| D002318 | Cardiovascular Diseases |
| D001020 | Aortic Stenosis, Subvalvular |
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| D001024 |
| Aortic Valve Stenosis |
| D000082862 | Aortic Valve Disease |
| D006349 | Heart Valve Diseases |
| D006323 | Heart Arrest |
| D003645 | Death, Sudden |
| D003643 | Death |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |