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Major risk factors for sudden cardiac death in pediatric hypertrophic cardiomyopathy

Major risk factors for sudden cardiac death in pediatric hypertrophic cardiomyopathy
Risk factor Comment
Massive LVH Massive LVH is determined by echocardiography and can be defined with an absolute cut-off or a z-score:
  • Absolute cut-off: Septal thickness ≥30 mm.
  • Z-score cut-off: Various z-score cut-offs are used. We generally use a z-score of >10 when LVH is the sole criterion prompting ICD; other experts use z-scores of >8 or >6 as the cut-off for consideration of ICD placement.
Unexplained syncope Unexplained syncope that, based on clinical history, is consistent with an arrhythmic event (ie, not vasovagal or neurally mediated).
NSVT on ambulatory monitoring

Defined as ≥3 consecutive ventricular beats at >120 beats per minute, lasting <30 seconds.

Although the data relating specific characteristics of NSVT to SCD risk are poorly defined, it is reasonable to place greater weight on this finding if NSVT is frequent (multiple bursts), of long duration (>7 beats), or particularly fast (>200 beats per minute).
Family history of HCM-related SCD SCD due to HCM in a first-degree relative, particularly if <40 years of age, should be considered evidence for increased risk of SCD in other related family members.
HCM: hypertrophic cardiomyopathy; LVH: left ventricular hypertrophy; ICD: implantable cardioverter defibrillator; SCD: sudden cardiac death; NSVT: nonsustained ventricular tachycardia.
Graphic 142801 Version 1.0

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