HCM Risk-SCD
Assess risk of sudden cardiac death and need for ICD in hypertrophic cardiomyopathy
Should not be used if age <16 or >80
Transthoracic echocardiographic measurement
Left atrial diameter as determined by M-mode or 2D echocardiography in the parasternal long axis plane at the time of evaluation
Maximum LV outflow gradient determined at rest and with Valsalva provocation (irrespective of concurrent medical treatment) using pulsed and continuous wave Doppler from the apical three and five chamber views. Peak outflow tract gradient should be determined using the modified Bernoulli equation, Gradient = 4V², where V is the peak aortic outflow velocity
The HCM Risk-SCD estimates the risk of sudden cardiac death at 5 years in patients with hypertrophic cardiomyopathy.
The risk of SCD in 5 years for an individual HCM patient can be calculated from the following equation:
Probability of Sudden Cardiac Death at 5 years = 1 - 0.998exp(PrognosticIndex)
where Prognostic Index = 0.15939858*Maximal wall thickness (mm) - 0.00294271*Maximal wall thickness2 (mm2) + 0.0259082*Left atrial diameter (mm) + 0.00446131*Maximal left ventricular outflow tract gradient (mmHg) + 0.4583082*Family history SCD + 0.82639195*NSVT + 0.71650361*Unexplained syncope - 0.01799934*Age at clinical evaluation (years).
It should not be used in:
- Paediatric patients (<16 years)
- Elite/competitive athletes
- HCM associated with metabolic diseases (e.g. Anderson-Fabry disease), and syndromes (e.g. Noonan syndrome).
- Patients with a previous history of aborted SCD or sustained ventricular arrhythmia who should be treated with an ICD for secondary prevention.
Caution should be exercised when assessing the SCD in patients following invasive reduction in left ventricular outflow tract obstruction with myectomy or alcohol septal ablation.
Acccording to ESC Guidelines, in patients with a 5-year risk of SCD <4%, an ICD is generally not indicated, in patients with a risk of 4 to less than 6%, an ICD may be considered and in patients with a 5-year risk ≥6%, an ICD should be considered.
- [1]O'Mahony C, Jichi F, Pavlou M, Monserrat L, Anastasakis A, Rapezzi C, Biagini E, Gimeno JR, Limongelli G, McKenna WJ, Omar RZ, Elliott PM.: A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD).European Heart Journal 2014 August 7, 35 (30): 2010-20
- [2]Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P, Hagege AA, Lafont A, Limongelli G, Mahrholdt H, McKenna WJ, Mogensen J, Nihoyannopoulos P, Nistri S, Pieper PG, Pieske B, Rapezzi C, Rutten FH, Tillmanns C, Watkins H.: 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC).European Heart Journal 2014 October 14, 35 (39): 2733-79
Results
Real-time automated computation
The HCM Risk-SCD estimates the risk of sudden cardiac death at 5 years in patients with hypertrophic cardiomyopathy.
The risk of SCD in 5 years for an individual HCM patient can be calculated from the following equation:
Probability of Sudden Cardiac Death at 5 years = 1 - 0.998exp(PrognosticIndex)
where Prognostic Index = 0.15939858*Maximal wall thickness (mm) - 0.00294271*Maximal wall thickness2 (mm2) + 0.0259082*Left atrial diameter (mm) + 0.00446131*Maximal left ventricular outflow tract gradient (mmHg) + 0.4583082*Family history SCD + 0.82639195*NSVT + 0.71650361*Unexplained syncope - 0.01799934*Age at clinical evaluation (years).
It should not be used in:
- Paediatric patients (<16 years)
- Elite/competitive athletes
- HCM associated with metabolic diseases (e.g. Anderson-Fabry disease), and syndromes (e.g. Noonan syndrome).
- Patients with a previous history of aborted SCD or sustained ventricular arrhythmia who should be treated with an ICD for secondary prevention.
Caution should be exercised when assessing the SCD in patients following invasive reduction in left ventricular outflow tract obstruction with myectomy or alcohol septal ablation.
Acccording to ESC Guidelines, in patients with a 5-year risk of SCD <4%, an ICD is generally not indicated, in patients with a risk of 4 to less than 6%, an ICD may be considered and in patients with a 5-year risk ≥6%, an ICD should be considered.
- [1]O'Mahony C, Jichi F, Pavlou M, Monserrat L, Anastasakis A, Rapezzi C, Biagini E, Gimeno JR, Limongelli G, McKenna WJ, Omar RZ, Elliott PM.: A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD).European Heart Journal 2014 August 7, 35 (30): 2010-20
- [2]Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P, Hagege AA, Lafont A, Limongelli G, Mahrholdt H, McKenna WJ, Mogensen J, Nihoyannopoulos P, Nistri S, Pieper PG, Pieske B, Rapezzi C, Rutten FH, Tillmanns C, Watkins H.: 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC).European Heart Journal 2014 October 14, 35 (39): 2733-79