Tülay DEMİRCAN, Özgür KIZILCA, Cüneyt ZİHNİ, Bahattin ÖNCÜ, MUSTAFA KIR, Nurettin ÜNAL

Halsizlik ve iştahsızlık yakınması ile gelen restriktif kardiyomiyopatili bir olgu

Restriktif kardiyomiyopati (RKMP) çocukluk çağının nadir görülen kardiyomiyopatilerindendir. Çocukluk çağı RKMP’lerinin çoğunda altta yatan patoloji bulunamaz ve mortalitesi yüksektir. Karakteristik morfolojik özelliği ventrikül boyutları normal, ventriküler duvar kalınlığı normal veya artmış ve biatriyal büyümedir. Konstriktif perikarditle ayırıcı tanısı önem taşır. Bu yazıda, 2,5 yaşında RKMP’li bir olgu literatür eşliğinde tartışılmıştır.

Anahtar Kelimeler:

Restriktif kardiyomiyopati, Konstriktif perikardit, çocuk

A CASE OF RESTRICTIVE CARDIOMYOPATHY IN CHILDHOOD PRESENTING WITH WEAKNESS AND ANOREXIA

Restrictive cardiyomyopathy (RCM) is a very rarely diagnosed cardiyomyopathy in childhood. Most RCM in childhood is idiopathic with a high mortality. Biatrial enlargement and normal sized ventricles with normal or increased ventricular wall thickness are the characteristic morphological features of RCM. The differential diagnosis of constrictive pericarditis is important. In this article a patient at 2.5 years of age with RCM is discussed regarding the literature.

Keywords:

Restrictive cardiomyopathy, Constrictive pericarditis, child,

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Report of the WHO/ISFC Task Force on the Definition and Classificatio of Cardiomyopathies. Br Heart J. 1980;44:672– 673.
Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Contemporary Definitions and Classification of the Cardiomyopathies: An American Heart Association Scientific Statement From the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation 2006;113:1807-1816
Rachel J. Weller, Robert Weintraub, Linda J. Addonizio et all. Outcome of Idiopathic Restrictive Cardiomyopathy in Children. Am J Cardiol 2002;90:501–506
Ammash N. M., Seward J. B., Bailey K. R., Edwards W. D., Tajik A. J. Clinical profile and outcome of idiopathic restrictive cardiomyopathy. Circulation. 2000;101(21):2490–2496. doi: 10.1161/01.CIR.101.21.2490.
Denfield SW. Restrictive Cardiomypathy In:Allen H.D.,Driscoll D.J.,Shaddy R.E.,Feltes T. Moss and Adams' heart disease in infants, children, and adolescents : including the fetus and young adult. 8 th ed. Philadelphia: Lippincott Williams & Wilkins; 2013 p.1267-1276
Çil E., Özkutlu S, Saraçlar M. Ve ark. Çocuklarda restriktif kardiyomiyopati: 12 va¬kanın klinikopatolojik incelemesi. Türk Kardiol Der Arş 1995;23:123-7.
Walsh M.A., Grenier M.A. et all. Conduction abnormalities in pediatric patients with restrictive cardiomyopathy. Circ Heart Fail. 2012;5:267-73.
Özkutlu S, Karagöz T. Restriktif kardiyomiyopati. Katkı Pediatri Dergisi 2001;22:51-63
Zwas DR, Gotsman I, Admon D, Keren A. Advances in the differentiation of constrictive pericarditis and restrictive cardiomyopathy. Herz 2012;37:664-74.
Sorajja P. Invasive hemodynamics of constrictive pericarditis, restrictive cardiomyopathy, and cardiac tamponade. Cardiology Clinics. 2011;29:191–199. doi: 10.1016/j.ccl.2011.01.003.
L M Russo and S A Webber Idiopathic restrictive cardiomyopathy in children Heart. 2005; 91: 1199–1202.
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(2):270–276. doi: 10.1093/eurheartj/ehm342.
Cooper L. T., Baughman K. L., Feldman A. M., et al. The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology. Endorsed by the Heart Failure Society of America and the Heart Failure Association of the European Society of Cardiology. J Am Coll Cardiol. 2007;50:1914–1931
Lewis AB. Clinical profile and outcome of restrictive cardiomyopathy in children. Am Heart J 1992;123:1589–1593.
Eren E, Güler M, Ömeroğlu SN. ve ark. Ailevi Restriktif Kar¬diyomiyopatili Hastada Ortotopik Kalp Transplantasyonu: Olgu Sunumu. Turkish J Thorac Cardiovasc Surg 2004;12:188-90