Version May 2024
| Risk factor | Comment |
| Major risk factors | |
| Family history of HCM-related SCD | SCD due to HCM in a close relative, particularly if <40 years of age, should be considered evidence for increased risk of SCD in other related family members. |
| Syncope | Unexplained syncope that, based on clinical history, appears to be due to arrhythmia (and not neurally mediated) is associated with increased SCD risk, particularly in young patients and when the event occurred close to the time of evaluation (<6 months). |
| Massive LV hypertrophy | An increased risk of SCD in patients with HCM is seen in patients with echocardiographic evidence of ≥30 mm wall thickness anywhere in the LV chamber. If maximal wall thickness is not clearly defined using echocardiography, additional evaluation with CMR to clarify the extent of LV wall thickening may be warranted. |
| LV apical aneurysm | Uncommon subgroup with thin-walled dyskinetic LV apex with regional scarring. LV apical aneurysm is associated with increased risk for sustained monomorphic VT and warrants consideration for ICD. |
| End-stage HCM (LVEF <50 percent) | Higher incidence of life-threatening VT associated with this uncommon phase of HCM. These patients often develop advanced heart failure at a young age and therefore are often considered for ICD as a bridge to definitive therapy with heart transplant. |
| Risk Modifiers | |
| Extensive LGE by contrast-enhanced CMR | Extensive LGE (ie, myocardial fibrosis) occupying ≥15 percent of LV mass is associated with markers of disease severity and adverse outcomes including increased risk for SCD and should be considered an important arbitrator to resolving ICD decision-making when uncertain following assessment with established major risk markers. |
| Age at time of SCD risk assessment | Risk of SCD is greatest in young patients <30 years old and lessens through mid-life. In patients who have achieved advanced age (≥60 years), risk of SCD is low, even in the presence of other risk factors. |
| NSVT on ambulatory monitoring | Defined as ≥3 consecutive ventricular beats at >120 beats per minute, lasting less than 30 seconds. Multiple bursts identified on ambulatory monitoring are associated with increased risk, particularly in younger patients. Although the data relating characteristics of NSVT to SCD risk remain poorly defined, it would be reasonable to give greater weight to increased SCD risk in those patients with HCM with NSVT that is frequent (>1 burst), of long duration (>7 beats), or particularly fast (>200 beats per minute). |
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