Carnitine Levels in Infants with Congestive Heart Failure

Abstract

Aim: In this study, we aimed to determine the carnitine levels in infants with cardiac failure due to congenital heart defects. Materials and methods: Forty-two patients with isolated ventricular septal defect (21 with cardiac failureGroup 3 and 21 without it-Group 2) and also 22 healthy infants (Group 1) were included. Serume free and total carnitine levels were studied by Cederblad and Harper method. Results: Age, sex and height among the groups were similar. Weight was lower in Group 3 than the others. Acyl-carnitine level and free/total carnitine ratio were similar among the groups. However free carnitine level in Group 3 was lower than the remaining two groups (p< 0.05). Total carnitine level in Group 3 was lower than the level in Group 1(p< 0.009). Although this level was lower than the one in Group 1, the difference was not significant. The duration was nort correlated with carnitine levels. Conclusion: Serum free carnitine levels in the patients cronic congestive heart failure were low

Keywords

• Congestive heart failure,carnitine,ventricular septal defect,infant

Konjestif Kalp Yetersizliği olan Süt çocuklarında Serum Karnitin Düzeyleri

Abstract

Amaç: Süt çocukluğu döneminde doğuştan kalp anomalisine bağlı kalp yetersizliğinde serum karnitin düzeylerini araştırmaktı. Yöntem ve Gereçler: Çalışmamızda izole ventriküler septal defekt tanısı ile izlenen 42 hasta (21 kalp yetersizliği olan-Grup 3, 21 kalp yetersizliği olmayan-Grup 2) ve 22 sağlıklı sütçocuğu (Grup 1) üzerinde yapıldı. Serum örneklerinde serbest ve total karnitin düzeyleri Cederblad ve Harper tarafından tarif edilen yöntem ile çalışıldı. Bulgular: Gruplar arasında yaş, cinsiyet ve boy bakımından anlamlı fark yoktu. Kalp yetersizliği olan grupta ağırlık diğer iki gruba göre anlamlı derecede düşüktü. Açil karnitin ile serbest / total karnitin oranları açısından gruplar arasında fark saptanmadı. Serbest karnitin ise kalp yetersizliği olan grupta, hem sağlıklı süt çocuğuna hem de hastalıklı kontrol grubuna göre anlamlı derecede düşük bulundu (p< 0.05). Total karnitin düzeyleri kalp yetersizliği olan hastalarda, sağlıklı kontrol grubuna göre anlamlı olarak düşük (p< 0.009), kalp yetersizliği olmayan gruba göre düşük ancak istatistiksel olarak anlamlı değildi. Kalp yetersizliği süresi ile karnitin düzeyleri arasında bir korelasyon saptanmadı. Sonuç: Serum serbest karnitin düzeyi volüm yüküne bağlı kronik konjestif kalp yetersizliği olan hastalarda düşük bulundu

Keywords

• Konjestif kalp yetersizliği,karnitin,vantrikuler septal defekt,süt çocuğu

References

1. Drayer WJ, Fisher DJ. Clinical recognation and management of chronic ongestive cardiac failure. In Garson A Jr, Bricker JT, Fisher DJ, et al: The Science and Practice of Pediatric Cardiolaogy, Baltimore, Williams &Wilkins, 2309-2325
2. Tein I, Di Mauro S. Primary systemic carnitine deficiency manifested by carnitine- responsive cardiyomyopathy. In Ferrari R (ed), L-Carnitine and its Role in Medicine. Academic Press, San Diego, 155-183, 1992.
3. Visioli O, Pasini E, De Giuli F and Ferrari R. Moleculer nechanism of action of L-carnitine in treatment of myocardial dısorders et the experimental level. In Ferrari R (ed), L-carnitine and Its Role in Medicine: Academic Press, San Diego, 238-259, 1992.
4. Cederblad G, Harper P, Lindgren K. Spitrophotometry of carnitine in biological fluids and tissue with a Cobas biocentrifugal analyzer. Clin Chem 32:342-346, 1986.
5. Stanley CA. Carnitine Disorders. Advences in Pediatrics. 42: 209-241, 1995.
6. Pepine CJ. The therapeutic potential of carnitine in cardiovasculer disorders. Clinical Therapeu- tics, 13:2-21, 1991.
7. Regitz V and Fleck E. Role of carnitine in heart failure. In Ferrari R (ed), L-carnitine and Its Role in Medicine: Academic Press, San Diego, 322, 1992.
8. Schwarz SM, Gewitz MH, See CC, Berezin S, Glassman MS, Medow CM, Fish BC, Newman LJ. Enteral nutrition in infants with conenital heart failure and growt failure. Pediatrics 86:368- , 1990.

9. Tripp ME, Shug AL. Plasma carnitine consentrations in cardiyomyopathy patients. Biochem Med 32:199-206, 1984.
10. Waber LJ, Valle D, Neill C, DiMauro S, Shug A. Carnitine deficiency presenting as familial cardiomyopathy: A treatable defect in carnitine transport. J Pediatrics. 101:700-705. Ino T, Sherwood WG, Benson LN, Wilson GJ, Freedom RM, Rowe RD. Cardiac manifestations in disorders of fat and carnitine metabolism in infancy. J Am Coll Cardiol. :1301-1308, 1988.
11. Ergür T, Tanzer F, Çetinkaya Ö. Serum free- carnitine levels in children with heart failure . J Trop Pediatrics. 45:168-169, 1999.
12. Whitmer JT. Energy metabolism and mecha- nical function in perfused hearts of Syrian hamsters with dilated or hypertrophic cardiomyopathy. J Mol Cell Cardiol. 18:307- 1986.
13. Pierpont ME, Judd D, Goldenberg IF, Ring WS, Olivari MT, Pierpont GL. Myocardial carnitine in end-stage congestive heart failure. Am J Cardiol. 64:56-60, 1989.