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Echocardiographic and electrocardiographic identification of those children with hypertrophic cardiomyopathy who should be considered at high-risk of dying suddenly

Published online by Cambridge University Press:  18 November 2005

Ingegerd Östman-Smith ,
Göran Wettrell ,
Barry Keeton ,
Tomas Riesenfeld ,
Daniel Holmgren  and
Ulf Ergander
Ingegerd Östman-Smith
Affiliation:
Division of Paediatric Cardiology, Queen Silvia Children's Hospital, Gothenburg, Sweden Department of Cardiovascular Medicine, John Radcliffe Hospital, Oxford, United Kingdom
Göran Wettrell
Affiliation:
Division of Paediatric Cardiology, University Hospital, Lund, Sweden
Barry Keeton
Affiliation:
Wessex Cardiothoracic Centre, Southampton General Hospital, Southampton, United Kingdom
Tomas Riesenfeld
Affiliation:
Division of Paediatric Cardiology, Academic Hospital, Uppsala, Sweden
Daniel Holmgren
Affiliation:
Division of Paediatric Cardiology, Queen Silvia Children's Hospital, Gothenburg, Sweden
Ulf Ergander
Affiliation:
Division of Paediatric Cardiology, Astrid Lindgren Children's Hospital, Stockholm, Sweden
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Abstract

Background: Hypertrophic cardiomyopathy is a common cause of sudden death in children. In this study, we aimed to identify clinical measures for stratification of this risk in childhood. Patients and methods: By means of a retrospective cohort study from six regional centres of paediatric cardiology, we identified 128 patients with hypertrophic cardiomyopathy presenting below 19 years of age, with a mean follow-up of 10.8 years. Of the patients, 31 had died, 16 suddenly, with a median age at sudden death of 13.3 years. Results: Cox regression shows that electrocardiographic voltages, analysed as the sum of the R and S waves in all six limb leads (p equal to 0.001), and septal thickness expressed as proportion of the 95th centile for age (p equal to 0.036), were independent predictors of sudden death. When the sum of the R and S waves is over 10 millivolts, the odds ratio for sudden death was 8.4, with 95% confidence intervals from 2.2 to 33.7 (p equal to 0.0012), and finding a septal thickness over 190% of 95th centile for age gives an odds ratio of 6.2, with confidence intervals from 1.5 to 25.1 (p equal to 0.011). Noonan's syndrome, with a p value equal to 0.043, and the ratio of the left ventricular wall to its cavity in diastole, with a p value equal to 0.005, were independent predictors of death in cardiac failure, with a ratio of the mural thickness to the dimension of the cavity over 0.30 giving an odds ratio of 36.0, with confidence limits from 4.2 to 311, and a p value equal to 0.00009. At follow-up, patients deemed to be at a high risk of dying suddenly were identified by the combination of the sum of the R and S waves greater than 10 millivolts and septal thickness over 190%, with a sensitivity of 91%, specificity of 78%, positive predictive value of 50%, and a negative predictive value of 97%. Conclusions: Children at high risk of dying suddenly with hypertrophic cardiomyopathy, with a subsequent annual mortality of 6.6%, can be distinguished at the time of diagnosis from those patients having a low risk of sudden death, the latter with an annual mortality of 0.27%.

Keywords

Sudden deathrisk factorsheart failurepropranololverapamil
Type
Original Article
Information
Cardiology in the Young , Volume 15 , Issue 6 , December 2005 , pp. 632 - 642
Copyright
© 2005 Cambridge University Press

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