PULMONARY ARTERIOVENOUS-FISTULA - A RARE CAUSE OF PROGRESSIVE ASYMPTOMATIC CYANOSIS IN BABIES

A six month old girl with no significant medical history was admitted to hospital for progressive cyanosis of recent onset refractory to oxy gen therapy. There were no detectable cardiac murmurs. Chest X-ray sho wed an irregular left posterior parahilar infiltration. Echocardiograp hy showed dilatation of the left atrium and ventricle but no cardiac m alformation. The diagnosis of pulmonary arteriovenous fistula was susp ected. Chest CT scan, magnetic resonance imaging and pulmonary angiogr aphy demonstrated the arteriovenous fistula in the lower lobe of the l eft lung. It was much bigger than the appearances of the chest X-ray s uggested. After the left lower lobectomy, the cyanosis completely disa ppeared. Progressive cyanosis refractory to oxygen therapy without any apparent cardiac or pulmonary disease is strongly suggestive of pulmo nary arteriovenous fistula. Surgical treatment is curative whereas the spontaneous outcome of this condition may be lethal.