Successful occlusion of a large pulmonary arterio-venous fistula with Amplatzer septal occluder in a 16-year-old cyanotic boy

Abstract

Pulmonary arterio-venous fistula is an uncommon cause of cyanosis and should be suspected when normal cardiac examination is associated without evidence of intra-cardiac shunt. Diagnosis of extra-cardiac shunt can be suspected by contrast echocardiography using agitated saline and confirmation of pulmonary arterio-venous fistula can be made by computed tomography pulmonary angiography with information regarding the size feeding vessels necessary for the planning of intervention. With the advancement of trans-catheter devices, fistula can be occluded successfully by embolotherapy. Coils, duct occluders, and vascular plugs are some of the commonly used trans-catheter devices among the armamentarium. Each device has its own inherent advantages and limitations. However, operators’ familiarity and expertise is an important parameter to choose the device to be employed in closure of fistula. The experience of Amplatzer family of devices in closure of pulmonary arterio-venous fistula is limited in the literature. We report a case of large pulmonary arterio-venous fistula successfully closed with a 20 mm Amplatzer septal occluder device in a 16-year-old cyanotic boy. Post-procedure contrast echocardiography confirmed absence of right to left shunt and computed tomography pulmonary angiography confirmed the device in situ closing the feeding vessel. Over a follow-up of six months reversal of clubbing and cyanosis was noted.<Learning objective: Patients with cyanosis with normal cardiac examination without evident intra-cardiac shunt in echocardiography should be evaluated for pulmonary arterio-venous fistula. Computed tomography Pulmonary angiography is gold standard but contrast echocardiography can be valuable. Percutaneous trans-catheter closure using coils, duct occluders, or vascular plugs can be an alternative to surgery. Choice of device depends on size and tortuosity of the feeding vessel as well as operator's familiarity with the device.>