Abstract
Two children (both females) aged 15 months and 4 years are described as very rare cases of central cyanosis in childhood being caused by a congenital pulmonary arteriovenous fistula. The initial diagnosis was made based on cyanosis and chest radiographs, with normal physical, ECG, and radiological findings of the heart. They had no family history of the Rendu-Weber-Osler syndrome. The patients underwent cardiac catheterization and pulmonary angiography, where the diagnosis was confirmed. After the surgery, both were symptom-free, and had no evidence of the disease.
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