ABSTRACT
Pulmonary arteriovenous fistula (PAVF) is a rare pulmonary vascular lesion, more than 80% of which is caused by congenital abnormal development of pulmonary capillaries. The incidence of PAVF ranges from 2/100,000 to 3/100,000, with no difference in the male and female ratio. Congenital PAVF is often associated with hereditary hemorrhagic telangiectasia (HHT). In this article, we report a patient with only congenital PAVF that was successfully treated by bilateral lung transplantation (BLT) with intraoperative venovenous extracorporeal membrane oxygenation (ECMO) support because both lungs have been affected by PAVF and secondary pulmonary hypertension. To the best of our knowledge, this is the first report of BLT for PAVF in China and the second report that explains the clinical course of a patient to receive BLT for congenital PAVF without HHT. Some investigators have proposed lung transplantation as a definitive treatment, but the results are controversial. On the basis of the current condition of this patient, we believe lung transplantation is a viable option for certain patients, but the long-term effect remains to be studied.
ABSTRACT
Congenital pulmonary arteriovenous fistula is a clinical rare abnormality of pulmonary vessels.The main reason is pulmonary capillary network hypoplasia.The clinical disease progression is occult.Exertional dyspnea,cyanosis and acropachy are the classical phenotypes.The key diagnosis methods include angiogram and multi-slice spiral CT angiography.The research progress of this disease was reviewed in this paper.
