Congenital anomalous/aberrant systemic artery to pulmonary venous fistula: Closure with vascular plugs & coil embolization

Abstract

A 7-month-old girl with failure to thrive, who, on clinical and diagnostic evaluation [echocardiography & CT angiography] to rule out congenital heart disease, revealed a rare vascular anomaly called systemic artery to pulmonary venous fistula. In our case, there was dual abnormal supply to the entire left lung as(1) anomalous supply by normal systemic artery [internal mammary artery](2) and an aberrant feeder vessel from the abdominal aorta. Left Lung had normal bronchial connections and normal pulmonary vasculature. The fistula drained through the pulmonary veins to the left atrium leading to 'left-left shunt'. Percutaneous intervention in two stages was performed using Amplatzer vascular plugs and coil embolization to close them successfully. The patient gained significant weight in follow up with other normal developmental and mental milestones.

Arteriovenous fistula between descending aorta and left inferior pulmonary vein: closure with vascular plugs

“…Congenital AVF arising from thoracic aorta and draining into the left inferior pulmonary vein without lung sequestration is an extremely rare malformation. [1,6,7] Pernot et al reported a case in which left lobectomy showed a fistula arising from the aorta and a pulmonary vein, associated with absence of a branch of the pulmonary artery. [8] Dahiya et al reported a fistula between the abdominal aorta and right inferior pulmonary vein in an asymptomatic 25-year-old man; surgical ligation performed.…”

“…In cases in which AVF is clinically and echocardiographically suspected, multislice CTA is an appropriate imaging method to confirm diagnosis and select therapy modality. [4,7] In addition, cardiac catheterization and angiography is necessary for both diagnosis and intervention procedures. [3] Brühlmann et al described the first transarterial therapeutic embolization of systemic arterialization of the lung without sequestration.…”

“…[11] Coils and vascular plugs were utilized for successful occlusion during endovascular intervention in the selected cases. [2,5,7] Amplatzer vascular plugs were used for transcatheter embolization in the peripheral vasculature and for occlusion of abnormal vessel communications with reported technical success rate of 100%. [7,12] The patient in the present report had clinically congestive heart failure, cardiomegaly demonstrated by X-ray, and increased left ventricular end-diastolic diameter and moderate mitral regurgitation demonstrated by echocardiography.…”

“…[2,5,7] Amplatzer vascular plugs were used for transcatheter embolization in the peripheral vasculature and for occlusion of abnormal vessel communications with reported technical success rate of 100%. [7,12] The patient in the present report had clinically congestive heart failure, cardiomegaly demonstrated by X-ray, and increased left ventricular end-diastolic diameter and moderate mitral regurgitation demonstrated by echocardiography. Therefore, the fistulous communication between the thoracic aorta and left inferior pulmonary vein was occluded with Amplatzer vascular plugs.…”

A rare case of congenital anomalous/aberrant systemic artery to pulmonary venous fistula in a 4-month-old child with co-existent mitral valve prolapse and mitral regurgitation

“…Cardiac CT is helpful when echocardiography fails to identify the cause of abnormal pressure variation in cardiac chambers. Cardiac CT can also diagnose the presence of lung sequestration,1 which decides subsequent disease management 2. Systemic branches from the aorta supply the primitive lung and are replaced by the pulmonary arteries (from the 6th aortic arch) around the fifth week, failure of which (due to pulmonary oligaemia) is proposed as the theory for pulmonary arterialisation (pseudosequestration) 3…”