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Strategy for Prevention of Sudden Death in Hypertrophic Cardiomyopathy
Study Questions:
How accurately can patients with hypertrophic cardiomyopathy (HCM) who are at risk of sudden cardiac death (SCD) be identified?
Methods:
This was a single-center, retrospective cohort study of 2,094 patients (mean age 62.7 years) with HCM. Based on enhanced American College of Cardiology/American Heart Association (ACC/AHA) guidelines, a primary prevention implantable cardioverter-defibrillator (ICD) was recommended based on the presence of ≥1 of the following risk factors: family history of SCD due to HCM, left ventricular wall thickness ≥30 mm, unexplained syncope, nonsustained ventricular tachycardia, extensive fibrosis on cardiac magnetic resonance imaging, ejection fraction <50%, and left ventricular apical aneurysm. The primary endpoint was an SCD event (SCD or an appropriate ICD therapy) during a mean follow-up of approximately 5 years.
Results:
An ICD was implanted based on prospective risk factor analysis in 527 patients and 15.6% of these patients received an appropriate ICD therapy during follow-up. SCD occurred in 0.3% of the 1,567 patients who did not receive an ICD. The risk factors used in this study predicted an SCD event with a sensitivity of 95%. Specificity for predicting patients without an SCD event was 78%. The European Society of Cardiology risk score was applied retrospectively and found to have a much lower sensitivity for identifying high-risk patients.
Conclusions:
Risk factor analysis based on enhanced ACC/AHA guidelines identifies almost all HCM patients who are at risk of SCD and who are appropriate candidates for an ICD.
Perspective:
The 2011 ACC/AHA guidelines for assessing risk factors were enhanced by the addition of extensive fibrosis, ejection fraction <50%, and left ventricular apical aneurysm to the original five risk factors. This study reassuringly demonstrates that HCM patients without these risk factors are at very low risk of SCD, probably no higher than the risk of SCD in the general population.
Clinical Topics: Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Prevention, Vascular Medicine, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Acute Heart Failure, Magnetic Resonance Imaging
Keywords: Aneurysm, Arrhythmias, Cardiac, Cardiomyopathy, Hypertrophic, Death, Sudden, Cardiac, Defibrillators, Implantable, Heart Failure, Magnetic Resonance Imaging, Primary Prevention, Risk Factors, Stroke Volume, Syncope, Tachycardia, Ventricular
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