Nalan KARABIYIK, Sultan KAVUNCUOĞLU, Resmiye BEŞİKÇİ, Kazım ÖZTARHAN, Emel K. ALTUNCU, Zeynel ALBAYRAK, Sibel ÖZBEK

Yaşamın ilk haftasında konjenital kalp hastalığı sıklığı

Amaç: Yaşamın ilk haftasında saptanabilen konjenital kalp anomalisi sıklığını araştırmak. Yöntem: l Ocak 2000-1 Ocak 2001 arasında SSK Bakırköy Doğumevi Kadın ve Çocuk Hastalıkları Eğitim Hastanesi'nde doğan, yaşamın ilk haftası içinde rutin muayeneeri sırasında üfürüm, siyanoz, taşipne ya da ritm bozukluğu gibi semptom ve/veya bulguları olan ya da prenatal dönemde konjenital kalp hastalığı tanısı konan veya Down sendromu tanısı alan tüm yenidoğanlara telegrafi ve EKG çekildikten sonra ekokardiyografi yapıldı. Bulgular: 19,222 canlı bebeğin toplam 111'i çalışmaya alındı. Hastaların 60'ı kız (% 54.9), 5i'i erkek (% 46.1) olup, 92'sinde (% 82.9) üfürüm, 13'ünde (% 11.7) siyanoz, 7'sinde (% 6.3) taşipne, birinde (% 0.9) ritm bozukluğu mevcuttu. Telegrafilerin 18'inde pulmoner vaskülarizasyonda artma, 21'inde pulmoner damarlanmada belirgin azalma ve 26'sında kardiyomegali saptandı. EKG'de 16 vakada sağ ventrikül hipertrofisi, 25 vakada sol ventrikül hipertrofisi bulguları, 32 vakada ise aks değişiklikleri gözlendi. Ekokardiyografilerin l5'inde (% 13.5) patoloji saptanmazken, geriye kalan 96 (% 86.5) vakada tek ya da birden fazla kardiyak anomali mevcuttu. En sık görülen anomaliler VSD (% 30.2), PDA (% 27.1), ASD (% 14.6), pulmoner stenoz (% 13.5) ve F allot tetralojisi (%8.3) idi. Prenatal dönemde hipoplastik sol kalp sendromu tanıtı hastanın postnatal ilk günde yapılan ekokardiyografi ile tanısı doğrulandı. Down sendromlu 9 hastanın 7'sinde AV kanal defekti saptanırken, 2'sinde patoloji saptanmadı. Ekokardiyografisonrası hastaların 29 (% 30.2)'u interne edildi. Bu vakaların lO'u açık ya da kapalı cerrahi girişim yapılması için sevkedilirken, 4 (% 4.2) vaka yatışı sırasında eksitus oldu. Sonuç: Yaşamın ilk haftasında konjenital kalp hastalığı olan yenidoğanların yaklaşık yansına tanı konmaktadır. Çalışmamızda yaşamın ilk haftasında 1000 canlı bebeğin 5'inde konjenital kalp hastalığı olasılığı ortaya konmuştur.

Anahtar Kelimeler:

Kalp hastalıkları, Kalp üfürümleri, Kalp kusuru, doğuştan, Süt çocuğu, yenidoğan, Doğumsal, kalıtsal ve yenidoğan hastalıkları ve anormallikleri

The incidence of congenital heart disease within first week of life

Objective: To evaluate the incidence of congenital cardiac anomalies that can be diagnosed within the firs t week of life. Methods: All newborns born between January 1, 2000 -January 1, 2001 at the SSK Bakırköy Maternity and Children Hospital who had symptoms and/or findings such as murmur, cyanosis, tachypnea or rhythm disorders within the first week of life or had a prenatal diagnosis of congenital heart disease or Down syndrome were evaluated with chest radiographs, ECG s and echocardiograms. Results: A total of 111 live infants out of 19,222 were included in the study. 60 were females (54.9 %) 51 were males (46.1 %); murmur was found in 92 of the infants (82.9 %) while 13 experienced cyanosis (11.7 %), 7 tachypnea (6.3 %) and 1 rhythm disorders (0.9 %). In chest radiographs there were increased pulmonary vascularization in 18 patients, a significant decrease in pulmonary vascularization in 21 cases and cardiomegaly in 26. ECGs, in 16 cases indicated right ventricle hypertrophy, in 25 cases left ventricle hypertrophy and axial variations in 32 cases. 15 of the echocardiograms (13.5 %) displayed no pathological status whereas there were single or multiple cardiac anomalies in the remaining 96 cases (86.5 %). The most frequently encountered anomalies were VSD (30.2 %), PDA (27.1 %), ASD (14.6 %), pulmonary stenosis (13.5 %) and Fallot tetralogy (8.3 %). The prenatal diagnosis of a hypoplastic left cardiac syndrome was confirmed by an echocardiogram at the first postnatal day. An AV canal defect was determined in 7 of 9 Down syndrome patients, while no other pathologies were found in the other 2 patients. After echocardiograms were obtained, 29 patients (30.2 %) were admitted to Neonatology Unit. 10 of these cases were referred to other centers in order to undergo an open or closed surgical intervention; 4 patients (4.2 %) died during hospitalization. Conclusion: Nearly half of the newborns whom have a congenital heart disease are diagnosed during the first week of their life. In our study in the first week of life the incidence of congenital heart disease was found as 5 in 1000 live birth.

Keywords:

Heart Diseases Heart Murmurs, Heart Defects, Congenital, Infant, Newborn, Congenital, Hereditary, and Neonatal Diseases and Abnormalities,

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1. Park MK. Pediatric cardiology for practitioners. 3rd ed. St. Louis: Mosby-Year Book, Inc, 1996: 34-51.
2. Flanagan MF, Yeager SB, Weindling SN. Cardiac disease. In: Avery GB, Fletcher MA, MacDonald MG, editors. Neonatology Pathophysiology&Management of the Newborn, 5th ed. Philadelphia: Lippincott Williams&Wilkins, 1999: 577-96.
3. Paul KE. Recognition, stabilization, and early management of infants with critical congenital heart disease presenting in the first days of life. Neonatal Netw 1995; 14:13-20.
4. Ainsworth S, Wyllie JP, Wren C. Prevalence and clinical significance of cardiac murmurs in neonates. Arch Dis Child Fetal Neonatal Ed 1999; 80:43-5.
5. Richmond S, Wren C. Early diagnosis of congenital heart disease. Semin Neonatol 2001; 6:27-35.
6. Wren C, Richmond S, Donaldson L. Presentation of congenital heart disease in infancy: implications for routine examination. Arch Dis Child Fetal Neonatal Ed 1999; 80:49-53.
7. Wilkinson JL, Cooke RWI. Cardiovascular disorders. In: Roberton NRC, editor. Textbook of Neonatology, 2nd ed. United Kingdom: Churchill Livingstone, 1999: 559.
8. Rein AJ, Omokhodion SI, Nir A. Significance of cardiac murmur as the sole clinical sign in the newborn. Clin Pediatr (Phila) 2000; 39:5111-20.
9. Du ZD, Roguin N, Barak M. Clinical and echocardiographic evaluation of neonates with heart murmurs. Acta Pediatr 1997; 86:752-6.
10. Shima Y, Takechi N, Ogawa S, et al. Clinical characteristics of congenital heart disease diagnosed during neonatal period. J Nippon Med Sch 2001; 68:510-5.
11. Danford DA, McNamara DG. Infants with congenital heart disease in the first year of life. In: Garson A, editor. The Science and Practice of Pediatric Cardiology. Lea&Febiger Comp. 1990: 1959-63.
12. Park MK. Pediatric cardiology for practitioners, 3th ed. St. Louis: Mosby-Year Book, Inc 1996: 67-77.
13. Amaral FT, Franzotti JA, Nunes MA. Management of children wih heart murmurs. Diagnostic importance of noninvasive complementary tests. Arq Bras Cardiol 1995; 64:195-9.
14. Bofferding L, Hascoet JM. Early management of neonates with suspected congenital heart disease. Arch Pediatr 2001; 8:116-20.
15. Samanek M, Voriskova ME. Congenital heart disease among 815,569 children born between 1980 and 1990 and 15- year survival: a prospective Bohemia survival study. Pediatr Cardiol 1999; 20:411-7.
16. Meberg A, Otterstad JE, Froland G, Hals J, Sorland SJ. Early clinical screening of neonates for congenital heart defects: the cases we miss. Cardiol Young 1999; 9:169-74.
17. Say B, Tunçbilek E, Balcı S, et al. Incidence of congenital malformations in a sample of the Turkish population. Hum Hered 1993; 23:434-41.
18. Murat Z. Yenidoğanlarda konjenital kalp hastalıklarının sıklığı, tanı dağılımı, kısa süreli prognoz ve risk faktörleri (uzmanlık tezi) İstanbul, 1993.
19. Yeşilipek MA, Melikoğlu M, Anlar B, Balcı S. Samsun yöresinde konjenital anomali sıklığı: 25,657 yenidoğan bebeğin değerlendirilmesi. Çocuk Sağ ve Hast Dergisi 1988; 31:253-63.
20. Manetti A, Pollini I, Cecchi F, et al. The epidemiology of cardiovascular malformations. HI: The prevalence and follow-up of 46,895 live births at the Careggi Maternty Hospital, Florance, in 1975-1984. G Ital Cardiol 1993; 23:145-52.
21. Shima Y, Takechi N, Ogawa S, et al. Clinical characteristics of congenital heart disease diagnosed during neonatal period. J Nippon Med Sch 2001; 68:510-5.