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

  • Ingegerd Östman-Smith (a1) (a2), Göran Wettrell (a3), Barry Keeton (a4), Tomas Riesenfeld (a5), Daniel Holmgren (a1) and Ulf Ergander (a6)...

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%.

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Corresponding author

Correspondence to: Ingegerd Östman-Smith, Division of Paediatric Cardiology, Queen Silvia Children's Hospital, SE-416 85, Gothenburg, Sweden. Tel: +46 31 3434512; Fax: +46 31 3435947; E-mail: ingegerd.ostman-smith@pediat.gu.se

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References

Sugishita Y, Matsuda M, Iida K, Koshinaga J, Ueno M. Sudden cardiac death at exertion. Jpn Circ J 1983; 47: 562572.
Maron B, Roberts W, McAllister H, Rosing DR, Epstein SE. Sudden death in young athletes. Circulation 1980; 62: 218229.
Fananapazir L, Epstein ND. Prevalence of hypertrophic cardiomyopathy and limitations of screening methods. Circulation 1995; 92: 700704.
Arola A, Jokinen E, Ruuskanen O, et al. Epidemiology of idiopathic cardiomyopathies in children and adolescents: a nationwide study in Finland. Am J Epidemiol 1997; 146: 385393.
Lipshultz SE, Sleeper LA, Towbin JA, et al. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med 2003; 348: 16471655.
McKenna W, Deanfield J, Faruqui A, England D, Oakley C, Goodwin J. Prognosis in hypertrophic cardiomyopathy: role of age and clinical, electrocardiographic and hemodynamic features. Am J Cardiol 1981; 47: 532538.
Maron BJ, Mathenge R, Casey SA, Poliac LC, Longe TF. Clinical profile of hypertrophic cardiomyopathy identified de novo in rural communities. J Am Coll Cardiol 1999; 33: 15901595.
Östman-Smith I, Wettrell G, Riesenfeld T. A cohort study of childhood hypertrophic cardiomyopathy: improved survival following high-dose beta-adrenoceptor antagonist treatment. J Am Coll Cardiol 1999; 34: 18131822.
McKenna WJ, England D, Doi YL, Deanfield JE, Oakley C, Goodwin JF. Arrhythmia in hypertrophic cardiomyopathy. I: Influence on prognosis. Br Heart J 1981; 46: 168172.
Monserrat L, Elliott PM, Gimeno JR, Sharma S, Penas-Lado M, McKenna WJ. Non-sustained ventricular tachycardia in hypertrophic cardiomyopathy. an independent marker of sudden death risk in young patients. J Am Coll Cardiol 2003; 42: 873879.
Spirito P, Bellone P, Harris KM, Bernabo P, Bruzzi P, Maron BJ. Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy. N Engl J Med 2000; 342: 17781785.
Maron B, Lipson L, Roberts W, Savage DD, Epstein SE. “Malignant” hypertrophic cardiomyopathy: identification of a subgroup of families with unusually frequent premature death. Am J Cardiol 1978; 41: 11331140.
Sadoul N, Prasad K, Elliott PM, Bannerjee S, Frenneaux MP, McKenna WJ. Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy. Circulation 1997; 96: 29872991.
Maron MS, Olivotto I, Betocchi S, et al. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med 2003; 348: 295303.
Cecchi F, Olivotto I, Montereggi A, Squillatini G, Dolara A, Maron BJ. Prognostic value of non-sustained ventricular tachycardia and the potential role of amiodarone treatment in hypertrophic cardiomyopathy: assessment in an unselected non-referral based patient population. Heart 1998; 79: 331336.
Olivotto I, Maron BJ, Montereggi A, Mazzuoli F, Dolara A, Cecchi F. Prognostic value of systemic blood pressure response during exercise in a community-based patient population with hypertrophic cardiomyopathy. J Am Coll Cardiol 1999; 33: 20442051.
Elliott PM, Gimeno Blanes JR, Mahon NG, Poloniecki JD, McKenna WJ. Relation between severity of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy. Lancet 2001; 357: 420424.
Sokolow M, Lyon TP. The ventricular complex in left ventricular hypertrophy as obtained by unipolar precordial and limb leads. Am Heart J 1949; 37: 161186.
Sahn D, DeMaria A, Kisslo J, Weyman A. Recommendations regarding quantitaion in M-mode echocardiography: results of a survey of echocardiographic measurements. Circulation 1978; 58: 10721083.
Östman-Smith I, Devlin AM. A simple method for assessing the regression or progression of ventricular hypertrophy in the growing child and adult: the value of left ventricular wall-to-cavity ratios. Eur J Echocardiogr 2001; 2: 2230.
Maron BJ, Henry NL, Clark CE, Redwood DR, Roberts WC, Epstein SE. Asymmetric septal hypertrophy in childhood. Circulation 1976; 53: 919.
McKenna WJ, Deanfield JE. Hypertrophic cardiomyopathy: an important cause of sudden death. Arch Dis Child 1984; 59: 971975.
Yetman AT, Hamilton RM, Benson LN, McCrindle BW. Long-term outcome and prognostic determinants in children with hypertrophic cardiomyopathy. J Am Coll Cardiol 1998; 32: 19431950.
Ackerman M, VanDriest S, Ommen S, et al. Prevalence and age-dependence of malignant mutations in the beta-myosin heavy chain and Troponin T genes in hypertrophic cardiomyopathy: a comprehensive outpatient perspective. J Am Coll Cardiol 2002; 39: 20422048.
Mayosi BM, Keavney B, Kardos A, et al. Electrocardiographic measures of left ventricular hypertrophy show greater heritability than echocardiographic left ventricular mass. Eur Heart J 2002; 23: 19631971.
Havndrup O, Bundgaard H, Andersen PS, et al. Outcome of clinical versus genetic family screening in hypertrophic cardiomyopathy with focus on cardiac beta-myosin gene mutations. Cardiovasc Res 2002; 57: 347357.
Blair E, Redwood C, Ashrafian H, et al. Mutations in the gamma(2) subunit of AMP-activated protein kinase cause familial hypertrophic cardiomyopathy: evidence for the central role of energy compromise in disease pathogenesis. Hum Mol Genet 2001; 10: 12151220.
Elliott PM, Poloniecki J, Dickie S, et al. Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol 2000; 36: 22122218.
Devlin AM, Ostman-Smith I. Diagnosis of hypertrophic cardiomyopathy and screening for the phenotype suggestive of gene carriage in familial disease: a simple echocardiographic procedure. J Med Screen 2000; 7: 8290.
Hjalmarson A. Cardioprotection with beta-adrenoceptor blockers. Does lipophilicity matter? Basic Res Cardiol 2000; 95 (Suppl 1): I141I145.

Keywords

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

  • Ingegerd Östman-Smith (a1) (a2), Göran Wettrell (a3), Barry Keeton (a4), Tomas Riesenfeld (a5), Daniel Holmgren (a1) and Ulf Ergander (a6)...

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