Hostname: page-component-586b7cd67f-t7fkt Total loading time: 0 Render date: 2024-11-23T15:04:07.181Z Has data issue: false hasContentIssue false
  • English
  • Français

Hypertrophic cardiomyopathy: prognostic factors and survival analysis in 128 Egyptian patients

Published online by Cambridge University Press:  29 July 2013

Sonia A. El-Saiedi ,
Zeinab S. Seliem  and
Reem I. Esmail
Sonia A. El-Saiedi
Affiliation:
Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt
Zeinab S. Seliem
Affiliation:
Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt
Reem I. Esmail*
Affiliation:
Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt
*
Correspondence to: R. Ibrahim, 330 Faisal Street, Giza, Egypt. Tel: +2001005459071; Fax: +20235877988; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background: Hypertrophic cardiomyopathy is an important cause of disability and death in patients of all ages. Egyptian children may differ from Western and Asian patients in the pattern of hypertrophy distribution, clinical manifestations, and risk factors. Objectives: The aim of our study was to report the clinical characteristics and outcomes of Egyptian children with hypertrophic cardiomyopathy studied over a 7-year duration and to determine whether the reported adult risk factors for sudden cardiac death are predictive of the outcome in these affected children. Study design and methods: This retrospective study included 128 hypertrophic cardiomyopathy children. The data included personal history, family history, physical examination, baseline laboratory measurements, electrocardiogram, and Holter and echocardiographic results. Logistic regression analysis was used for the detection of risk factors of death. Results: Fifty-one out of 128 patients died during the period of the study. Of the 51 deaths, 36 (70.5%) occurred in patients presenting before 1 year of age. Only eight patients had surgical intervention. Extreme left ventricular hypertrophy, that is, interventricular septal wall thickness or posterior wall thickness Z-score >6, sinus tachycardia, and supraventricular tachycardia were found to be independent risk factors for prediction of death in patients with hypertrophic cardiomyopathy. Conclusions: At our Egyptian tertiary care centre, hypertrophic cardiomyopathy has a relatively worse prognosis when compared with reports from Western and Asian series. Infants have a worse outcome than children presenting after the age of 1 year. A poorer prognosis in childhood hypertrophic cardiomyopathy is predicted by an extreme left ventricular hypertrophy, the presence of sinus tachycardia, and supraventricular tachycardia.

Keywords

Hypertrophic cardiomyopathyechocardiographychildren
Type
Original Articles
Information
Cardiology in the Young , Volume 24 , Issue 4 , August 2014 , pp. 702 - 708
Copyright
Copyright © Cambridge University Press 2013 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Elliott, P, Andersson, B, Arbustini, E, et al. Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2008; 29: 270–276.CrossRefGoogle Scholar
2. Maron, BJ. Hypertrophic cardiomyopathy: a systematic review. JAMA 2002; 287: 1308–1320.Google Scholar
3. Maron, BJ, Towbin, JA, Thiene, G, et al. American Heart Association; Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; Council on Epidemiology and Prevention. Contemporary definitions and classification of the cardiomyopathies. Circulation 2006; 113: 1807–1816.Google Scholar
4. Melacini, P, Basso, C, Angelini, A, et al. Clinicopathological profiles of progressive heart failure in hypertrophic cardiomyopathy. Eur Heart J 2010; 31: 2011–2123.CrossRefGoogle ScholarPubMed
5. 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: 1778–1785.CrossRefGoogle ScholarPubMed
6. Ostman-Smith, I, Wettrell, G, Riesenfeld, TA. Cohort study of childhood hypertrophic cardiomyopathy: improved survival following highdose beta-adrenoceptor antagonist treatment. J Am Coll Cardiol 1999; 34: 113–122.Google Scholar
7. Nugent, AW, Daubeney, PE, Chondros, P, et al. Clinical features and outcomes of childhood hypertrophic cardiomyopathy: results from a national population-based study. Circulation 2005; 112: 1332–1338.Google Scholar
8. Colan, SD, Lipshultz, SE, Lowe, AM, et al. Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the Pediatric Cardiomyopathy Registry. Circulation 2007; 115: 773–781.CrossRefGoogle ScholarPubMed
9. Maron, BJ, McKenna, WJ, Danielson, GK, et al. ACC/ESC clinical expert consensus document on hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines (Committee to Develop an Expert Consensus Document on Hypertrophic Cardiomyopathy). Eur Heart J 2003; 24: 1965–1991.Google Scholar
10. Nasermoaddeli, A, Miura, K, Matsumori, A, et al. Prognosis and prognostic factors in patients with hypertrophic cardiomyopathy in Japan: results from a nationwide study. Heart 2007; 93: 711–715.CrossRefGoogle ScholarPubMed
11. 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: 873–879.Google Scholar
12. Ostman-Smith, I, Wettrell, G, Keeton, B, Riesenfeld, T, Holmgren, D, Ergander, U. Echocardiographic and electrocardiographic identification of those children with hypertrophic cardiomyopathy who should be considered at high-risk of dying suddenly. Cardiol Young 2005; 15: 632–642.Google Scholar
13. Harris, KM, Spirito, P, Maron, MS, et al. Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy. Circulation 2006; 114: 216–225.Google Scholar
14. Grenier, MA, Osganian, SK, Cox, GF, et al. Design and implementation of the North American Pediatric Cardiomyopathy Registry. Am Heart J 2000; 139: S86–S95.Google Scholar
15. Sluysmans, T, Colan, SD. Theoretical and empirical derivation of cardiovascular allometric relationships in children. J Appl Physiol 2005; 99: 445–457.Google Scholar
16. Maron, BJ, Tajik, AJ, Ruttenberg, HD, et al. Hypertrophic cardiomyopathy infants: clinical features and natural history. Circulation 1982; 65: 7–17.Google Scholar
17. Skinner, JR, Manzoor, A, Hayes, AM, Joffe, HS, Martin, RP. A regional study of presentation and outcome of hypertrophic cardiomyopathy in infants. Heart 1997; 77: 229–233.Google Scholar
18. Decker, JA, Rossano, JW, Smith, EO, et al. Risk factors and mode of death in isolated hypertrophic cardiomyopathy in children. J Am Coll Cardiol 2009; 54: 250–254.Google Scholar
19. Maron, BJ. Hypertrophic cardiomyopathy in childhood. Pediatr Clin N Am 2004; 51: 1305–1346.CrossRefGoogle ScholarPubMed
20. Maron, BJ, Spirito, P. Impact of patient selection biases on the perception of hypertrophic cardiomyopathy and its natural history. Am J Cardiol 1993; 72: 970–972.Google Scholar
21. Lewin, MB, Towbin, JA, Thapar, MK, Dreyer, WJ, Feltes, TF. The rare association of tetralogy of Fallot with hypertrophic cardiomyopathy. Report of 2 neonatal patients. Tex Heart Inst J 1997; 24: 215–217.Google Scholar
22. Carvalho, AM, Diógenes, TC, Jucá, ER, Carvalho, AF, Carvalho, CF, Paes Júnior, JN. Tetralogy of Fallot and hypertrophic cardiomyopathy: a rare association. Arq Bras Cardiol 2003; 80: 217–219; 214–216.Google Scholar
23. Will, PM, Serrian, JL, Dawson, JT. An unusual case of cyanotic heart disease in a patient with patent foramen ovale and right ventricular hypertrophy. Clin Cardiol 1996; 19: 429–432.Google Scholar
24. Nishimura, RA, Holmes, DR. Hypertrophic obstructive cardiomyopathy. N Engl J Med 2004; 350: 1320–1327.Google Scholar
25. McKenna, WJ. The natural history of hypertrophic cardiomyopathy. Cardiovasc Clin 1988; 19: 135–148.Google ScholarPubMed
26. 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: 1943–1950.Google Scholar
27. Moran, AM, Colan, SD. Verapamil therapy in infants with hypertrophic cardiomyopathy. Cardiol Young 1998; 8: 310–319.Google Scholar
28. McMahon, CJ, Nagueh, SF, Pignatelli, RH, et al. Characterization of left ventricular diastolic function by tissue Doppler imaging and clinical status in children with hypertrophic cardiomyopathy. Circulation 2004; 109: 1756–1762.Google Scholar
29. Olivotto, I, Gistri, R, Petrone, P, et al. Maximum left ventricular thickness and risk of sudden death in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 2003; 41: 315–321.Google Scholar
30. Adabag, AS, Casey, SA, Kuskowski, MA, Zenovich, AG, Maron, BJ. Spectrum and prognostic significance of arrhythmias on ambulatory Holter electrocardiogram in hypertrophic cardiomyopathy. J Am Coll Cardiol 2005; 45: 697.Google Scholar
31. Cha, Y-M, Gersh, BJ, Maron, BJ, et al. Electrophysiologic manifestations of ventricular tachyarrhythmias provoking appropriate defibrillator interventions in high-risk patients with hypertrophic cardiomyopathy. J Cardiovasc Electrophysiol. 2007; 18: 1–5.Google Scholar