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SİYANOTİK DOĞUMSAL KALP HASTALIKLARINDA HEMATOLOJİK BULGU VE KOMPLİKASYONLAR

THE HEMATOLOGICAL SIGN AND COMPLICATIONS OF CYANOTIC CONGENITAL HEART DISEASES

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Abstract (2. Language): 
Aim: This review sought to determine the relationship between cyanosis and hematological signs and findings. Main finding: Patients with cyanotic congenital heart disease are succeptible to deve-lope hematological side effects. Several findings, including polycytemia, thrombocytopenia, factor deficiencies, iron-deficiency anemia and disseminated intravasculary coagulation have been report¬ed. Decreased arterial oxygen saturation in cyanotic congenital heart disease causes compensatory rise in hemoglobin and haematocrit levels. Erythrocytosis is an adaptive response to improve oxy¬gen transport in cyanotic congenital heart disease. However, at highly increased haematocrit levels patients may experience hyperviscosity symptoms. Iron-deficiency in cyanotic congenital heart dis-ease patients is often overlooked due to elevated hemoglobin concentrations. The detection of a hemostatic abnormality and its correction by appropriate therapy are likely to minimize the side effects. Results: Children with cyanotic congenital heart disease are prone to both thrombosis and hemorrhage. Hematological management of cyanotic congenital heart disease has received little attention. The lack of practical therapeutic guidelines forced us to consolidate our observations on patients with cyanotic congenital heart disease.
Abstract (Original Language): 
Amaç: Siyanotik doğumsal kalp hastalıklarında hematolojik belirti ve bulgular derlenmiştir. Ana bul¬gular: Siyanotik doğumsal kalp hastalıklı hastalarda hematolojik yan etkilerin görülme ihtimali yük¬sektir. Polisitemi, trombositopeni, faktör eksiklikleri, demir eksikliği anemisi ve yaygın damariçi pıhtılaşması gibi birçok bulgu ve belirtiler bildirilmiştir. Siyanotik doğumsal kalp hastalarında azalmış arteriyel oksijen saturasyonu hemoglobin ve hematokrit değerlerinde kompanzatuar artışlara yol açar. Eritrositoz siyanotik doğumsal kalp hastalarında yeterli oksjenizasyonu sağlamak için ortaya çıkan bir cevaptır. Ancak hematokrit değerleri çok artmış hastalarda hiperviskozite bulguları ortaya çıkabilir. Siyanotik doğumsal kalp hastalıklı çocuklarda demir eksikliği hemoglobin değerleri yüksek olduğu için gözden kaçabilir. Hemostatik anormalliklerin zamanında tespiti ve uygun tedavi yaklaşımları ile yan etkiler azaltılabilir. Sonuç: Siyanotik doğumsal kalp hastalığı olan çocuklar hem tromboz hem de kanamaya eğilimlidir. Bu çocukların hematolojik açıdan takipleri az ilgi çekmekte¬dir. Bu hastalara yönelik olarak pratik tedavi yaklaşımları geliştirilmediği için siyanotik doğumsal kalp hastalıklı çocuklara yönelik çalışmalar yapılmalıdır.
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