Pediatrik dilate kardiyomiyopatide medikal tedavinin prognoza etkisi

Öz

The purpose of this study was to review our experience with conventional treated pediatric dilated caridomyopathy patients in our region and attempt to discover prognostic factors. We analyzed retrospectively the outcome of 41 patients during last six years. The patients were 23 girls, and 18 boys, ejection fraction 31.6±7.8%, follow-up period 14.07Ü4.2 months. Eight patients (19.5%) had died, 17 (41.4%) had recovery of systolic function. When analyzed from regained normal systolic function, first ejection fraction was not statistically significant (p=0.743). When separated from patient presentation age, 58.5% were smaller than two years. Sixth patients (25%) were died in smaller and two (11.7%) in bigger. Comparison between groups were not statistically differences (p=0.632). We concluded that age; degrees of systolic functions were not differentiated favorable outcomes.

Anahtar Kelimeler

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Prognostic effect of conventional treatment in pediatric dilated cardiomyopathy

Öz

Pediatrik dilate kardiyomiyopatili hastalarımızda konvensiyonel tedavide prognoza etki eden faktörleri araştırmayı planladık. Son altı yıl içinde hastanemizde tedavi edilen 41 dilate kardiyomiyopatili çocuk hastanın retrospektif değerlendirmesi yapıldı. 23 kız, 18 erkek, başlangıç ejeksiyon fraksiyonu ortalama %31.6±7.8, izlem süresi ise 14.0±14.2 ay idi. İzlemde 8 hasta (%19.5) öldü, 17 hastanın (%41.4) sistolik fonksiyonu zamanla düzeldi. Sistolik fonksiyonu düzelen hastaların başlangıç ejeksiyon fraksiyon değerleri düzelmeyenlere göre istatistiksel olarak anlamlı değildi (p=0.743). Başvuru yaşlarına göre hastalar iki gruba ayrıldığında %58.5 hasta iki yaştan küçüktü. İki yaştan küçük hastalardan altısı (%25), iki yaştan büyüklerden ise ikisi (%11.7) öldü. Grupların karşılaştırılması istatistiksel olarak anlamlı bir fark vermedi (p=0.632). Hastalığın başlangıç yaşı ve sistolik fonksiyonun başlangıç değerinin hastalık prognozunu göstermede etkin olmadığını düşünmekteyiz.

Anahtar Kelimeler

• Dilate kardiyomiyopati, Prognoz, Çocukluk çaðý

Kaynakça

1. l.Grenier MA, Osganian SK, Cox GF, Towbin JA, Colan SD, Lurie PR, et al. Design and implementation of the North American Pediatric Cardiomyopathy Registry. Am Heart J-2000; 139:86-95
2. 2.Towbin JA. Pediatric myocardial disease. Pediatr Clin North Am. 1999;46:289-312
3. 3.Schwartz ML, Cox GF, Un AE, Korson MS, Perez-Atayde A, Lacro RV, et al. Clinical approach to genetic cardiomyopathy in children. Circulation. 1996;94:2021-2038
4. 4.Felker GM, Hu W, Hare JM, Hruban RH, Baughman KL, Kasper EK. The spectrum of dilated cardiomyopathy. The Johns Hopkins experience with 1,278 patients. Medicine (Baltimore), 1999;78:270-283
5. 5.Arola A, Tuominen J, Ruuskanen O, Jokinen E. Idiopathic dilated cardiomyopathy in children: prognostic indicators and outcome. Pediatrics. 1998;101:369-376
6. 6.Burch M, Siddiqi SA, Celermajer DS, Scott C, Bull C, Deanfield JE. Dilated cardiomyopathy in children: determinants of outcome. Br Heart J. 1994;72:246-250
7. 7.Nugent AW, Daubeney PE, Chondros P, Carlin JB, Cheung M, Wilkinson LC, et al. National Australian Childhood Cardiomyopathy Study. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med. 2003;348:1639-1646
8. 8.Lipshultz SE, Sleeper LA, Towbin JA, Lowe AM, Orav EJ, Cox GF, et al. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med. 2003;348:1647-1655

9. 9.Venugopalan P, Agarwal AK, Akinbami FO, El Nour IB, Subramanyan R. Improved prognosis of heart failure due to idiopathic dilated cardiomyopathy in children. Int J Cardiol. 1998;65:125-128
10. lO.Redfield MM, Gersh BJ, Bailey KR, Ballard DJ, Rodeheffer RJ. Natural history of idiopathic dilated cardiomyopathy: effect of referral bias and secular trend. J Am Coll Cardiol. 1993;22:1921-1926
11. ll.Hofmann T, Meinertz T, Kasper W, Geibel A, Zehender M, Hohnloser S, et al. Mode of death in idiopathic dilated cardiomyopathy: a multivariate analysis of prognostic determinants. Am Heart J. 1988;116:1455-1463
12. 12.Lewis AB. Prognostic value of echocardiography in children with idiopathic dilated cardiomyopathy. Am Heart J. 1994; 128:133-136. 13.McMahon CJ, Nagueh SF, Eapen RS, Drewer WJ, Finkelshtyn I, Cao X, et al. Echocardiographic predictors of adverse clinical events in children with dilated cardiomyopathy: a prospective clinical study. Heart. 2004;90:908-915
13. 14.Matitiau A, Perez-Atayde A, Sanders SP, Sluymans S, Parness IA, Spevak PJ, et al. Infantile dilated cardiomyopathy. Relation of outcome to left ventricular mechanics, hemodynamics, and histology at the time of presentation. Circulation. 1994;90:1310-1318
14. 15.Lewis AB, Chabot M. Outcome of infants and children with dilated cardiomyopathy. Am J Cardiol. 1991;68:365-369
15. 16.Tsirka AE, Trinkaus K, Chen SC, Lipshultz SE, Towbin JA, Colan SD, et al. Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation. J Am Coll Cardiol. 2004;44:391-397
16. 17.Cicoira M, Zanolla L, Latina L, Rossi A, Golia G, Briqhetti G, et al. Frequency, prognosis and predictors of improvement of systolic left ventricular function in patients with 'classical' clinical diagnosis of idiopathic dilated cardiomyopathy. Eur J Heart Fail. 2001;3:323-330
17. 18.Strauss A, Lock JE. Pediatric cardiomyopathy-a long way to go. N Engl J Med. 2003;348:1703-1705
18. 19.Poss KD, Wilson LG, Keating MT. Heart regeneration in zebrafish. Science. 2002;298:2188-2190