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shared decision making

Secondary prevention

Primary prevention

HCM-risk scores

≥1 clinical risk factorsa

ICD (Class IIb) ICD (Class IIb) ICD (Class IIa)

ICD (Class I)

Low risk 5-year risk <4%

Intermediate risk 5-year risk ≥4 to <6% High risk 5-year risk ≥6%

History 2D and Doppler echocardiogram Ambulatory ECG Symptom limited exercise test

Age Unexplained syncope LV outflow gradient Maximum LV wall thickness

Left atrial diameter NSVT Family history of SCD LV systolic function Extent of myocardial scar

Cardiac arrest due to VT or VF

Spontaneous sustained VT causing syncope or haemodynamic compromise

AND Life expectancy ≥1 year

Figure 16 Flow chart for implantation of an implantable cardioverter defibrillator in patients with hypertrophic cardiomyopathy. 2D, two-dimensional; CMR, cardiac magnetic resonance; ECG, electrocardiogram; HCM, hypertrophic cardiomyopathy; ICD, implantable cardioverter defibrillator; LGE, late gadolinium enhancement; LV, left ventricular; LVEF, left ventricular ejection fraction; NSVT, non-sustained ventricular tachycardia; SCD, sudden cardiac death; VF, ventricular fibrillation; VT, ventricular tachycardia. aClinical risk factors: extensive LGE (>15%) on CMR; LVEF <50%.

Recommendation Table 23 — Additional recommendations for prevention of sudden cardiac death in patients with hypertrophic cardiomyopathy

Recommendations Classa Levelb

Secondary prevention Implantation of an ICD is recommended in patients who have survived a cardiac arrest due to VT or VF, or who have spontaneous sustained VT with haemodynamic compromise.532,534,726,831,832 I B

Continued Primary prevention

The HCM Risk-SCD calculator is recommended as a method of estimating risk of sudden death at 5 years in patients aged ≥16 years for primary prevention.525,821–824 I B Validated paediatric-specific risk prediction models (e.g. HCM Risk-Kids) are recommended as a method of estimating risk of sudden death at 5 years in patients aged <16 years for primary prevention.81,833 I B

Continued

ESC Guidelines                                                                                                                                                                                          3561

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