Hypertrophic cardiomyopathy risk factors for sudden death

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Patients with hypertrophic cardiomyopathy are increased risk of sudden cardiac death. Identification of those patients at an increased risk can facilitate early treatment with an automatic implantable cardiac defibrillator.

Risk Factors for Sudden Death in Patients with Hypertrophic Cardiomyopathy

Risk factors for sudden death in individuals with HCM include:

• A young age at first diagnosis (age < 30 years)
• An episode of aborted sudden death
• A family history of HCM with sudden death of relatives
• Specific mutations in the genes encoding for troponin T and myosin
• Sustained supraventricular or ventricular tachycardia
• Ventricular septal wall thickness over 3 cm
• A hypotensive response to exercise
• Recurrent syncope (especially in children)
• Bradyarrhythmias (slow rhythms of the heart).

2011 ACCF/AHA Guideline Recommendations: SCD Risk Stratification (DO NOT EDIT)

Class I 1. All patients with HCM should undergo comprehensive SCD risk stratification at initial evaluation to determine the presence of the following: (Level of Evidence: B)

a. A personal history for ventricular fibrillation, sustained VT, or SCD events, including appropriate ICD therapy for ventricular tachyarrhythmias.

b. A family history for SCD events, including appropriate ICD therapy for ventricular tachyarrhythmias.

c. Unexplained syncope.

d. Documented NSVT defined as 3 or more beats at greater than or equal to 120 bpm on ambulatory (Holter) ECG.

e. Maximal LV wall thickness greater than or equal to 30 mm.

Class III

1. Invasive electrophysiologic testing as routine SCD risk stratification for patients with HCM should not be performed.(Level of Evidence: C)

Class IIa

1. It is reasonable to assess blood pressure response during exercise as part of SCD risk stratification in patients with HCM. (Level of Evidence: B)

2. SCD risk stratification is reasonable on a periodic basis (every 12 to 24 months) for patients with HCM who have not undergone ICD implantation but would otherwise be eligible in the event that risk factors are identified (12 to 24 months). (Level of Evidence: C)

Class IIb

1. The usefulness of the following potential SCD risk modifiers is unclear but might be considered in selected patients with HCM for whom risk remains borderline after documentation of conventional risk factors:

a. CMR imaging with LGE. (Level of Evidence: C)

b. Double and compound mutations (ie, >1). (Level of Evidence: C)

c. Marked LVOT obstruction. (Level of Evidence: B)

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