ABSTRACT
PA-LA communication is a rare congenital heart disease consisting of direct communication between a branch of the PA and LA through an aneurysmal structure. This disease reveals the central cyanosis with clubbed fingers and surgical repair is needed when symptoms are apparent. Computed tomography is highly recommended for definitive diagnosis. Angiographic catheterization is also recommended to support the diagnosis and decide on the treatment. PA-LA communication is categorized into 4 types. Two types do not need cardiopulmonary bypass (CPB) when treated surgically, but the others need CPB. A 16-year-old girl with clubbed fingers was found to have PA-LA communication by 3DCT. She underwent surgery and was discharged in good condition. The surgical procedure was done through median sternotomy without CPB. The anomalous aneurysmal fistula was doubly ligated. No communication was found after ligation by TEE.
ABSTRACT
Background: Chronic hypoxemia is generally attributed to primary cardiac or pulmonary entities. Case characteristics: A 9-year-old boy presenting with cyanosis, clubbing and hypoxemia, without icterus or hepatosplenomegaly. Cardiovascular and respiratory system examinations were normal. Outcome: He was diagnosed as type IB Abernethy malformation, a rare cause of hepatopulmonary syndrome. Message: Pediatricians should consider hepatopulmonary syndrome in the differential diagnosis of chronic hypoxemia, even in the absence of jaundice or hepatosplenomegaly.