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Outcomes after implantable cardioverter-defibrillator treatment in children with hypertrophic cardiomyopathy
  1. Juan Pablo Kaski1,
  2. María Teresa Tomé Esteban1,
  3. Martin Lowe1,
  4. Simon Sporton2,
  5. Philip Rees1,
  6. John E Deanfield1,
  7. William J McKenna1,
  8. Perry M Elliott1
  1. 1Inherited Cardiovascular Diseases Unit, Great Ormond Street Hospital, London, UK
  2. 2St Bartholomew’s Hospital, London, UK
  1. Correspondence to:
    Dr P M Elliott
    The Heart Hospital, 16-18 Westmoreland Street, London W1G 8PH, UK; perry.elliott{at}

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Implantable cardioverter-defibrillators (ICDs) have been shown to successfully treat life-threatening arrhythmias in high-risk adults with hypertrophic cardiomyopathy (HCM).1 Children represent <1% of individuals with ICDs, and paediatric studies include few children with HCM. This study reports the experience with ICDs in children with HCM in a single referral centre.


Between 1993 and February 2006, 160 consecutively referred patients with HCM (age ⩽16 years) underwent clinical evaluation using 12-lead ECG, two-dimensional, M-mode and Doppler echocardiography, Holter monitoring and cardiopulmonary exercise testing using previously described methods.2 Risk markers for sudden cardiac death (SCD) were: (1) family history of SCD; (2) unexplained syncope; (3) abnormal blood pressure response during upright exercise; (4) non-sustained ventricular tachycardia; and (5) severe left ventricular hypertrophy (⩾30 mm).2 Patients with previous cardiac arrest or with ⩾2 risk factors were considered for ICD implantation.2 All patients who underwent ICD implantation during this period were included in this study.

Informed consent for ICD implantation was obtained from a parent in all cases. Devices were implanted under general anaesthesia into subpectoral pockets, using transvenous lead systems. Sixteen patients received dual chamber devices and six received single-chamber devices. Stored ICD data were obtained every 6 months or within 24 h of treatment. Discharges were judged appropriate when triggered by ventricular tachycardia or ventricular fibrillation, or inappropriate if triggered by …

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  • Competing interests: None.