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.

Keywords: Congenital AV fistula; Sequestration of lung.

Fig. 1

a: Subcostal echocardiography to assess Situs Solitus revealed an aberrant vessel with mosaic color pattern with continuous flow pattern (Doppler) originating from the upper abdominal aorta which could trace up to the diaphragm. b: Four chamber view showing dilated left ventricle and left atrium without any evidence of shunt. c: Suprasternal view demonstrated dilated left subclavian artery with turbulent flow. d: Pulse wave interrogation of abdominal aorta which showed diastolic runoff suggestive of hyper dynamic circulation. e: Continuous wave interrogation of the aberrant vessel showed continuous flow with wide pulse pressure. f: Pulse wave interrogation of left subclavian artery showed a continuous flow pattern with diastolic runoff suggestive of hyper dynamic circulation.

Fig. 2

a–c: CT angiography with 3D volume rendering showing aberrant vessels originating from the left subclavian artery and abdominal aorta to the entire left lung which drained into the left atrium via normal pulmonary veins.

Fig. 3

a–c: Arch and thoracic aortic angiography using a pigtail catheter showed large aberrant feeder vessels originating from the left subclavian artery and supplying the left lung. Post arterial phase contrast created vascular sponge followed by venous phase showing dilated pulmonary veins draining into the left atrium. d–f: Abdominal angiography showing the aberrant feeder vessel origin from the upper abdominal aorta piercing left hemi-diaphragm and supplying left lower lobe of lung creating similar post arterial vascular sponge and draining into left lower pulmonary vein to the left atrium.

Fig. 4

a: 1st intervention showing Judkin's right guiding catheter across left subclavian artery and the aberrant systemic artery and angiography check showing pacified medial feeder vessel. b: Deployment of Amplatzer vascular plug into the aberrant feeder systemic artery and guiding catheter engaged into middle aberrant feeder and angiography check showed supplying to left middle lobe of the lung. c: Deployment of Amplatzer vascular plug into middle feeder. d: Guiding catheter now into a lateral feeder vessel opacifying lateral portion of lung adjacent to the chest wall. e: Largest Amplatzer vascular plug was deployed into the lateral feeder vessel. f: Final angiogram using diagnostic Judkin's right catheter which revealed complete closure of left subclavian aberrant feeder systemic arterial supply. It also showed aberrant supply to the left lung from the axillary artery across the chest wall.

Fig. 5

a and b: 2nd stage intervention showing angiography using renal guiding catheter of abdominal aberrant feeder systemic artery opacifying left lower lobe of lung. c: Deployment of Amplatzer vascular plug into the abdominal aberrant feeder vessel and angiography check demonstrated near closure with faint streaks of contrast across the device. d and e: Closure of lest feeder vessel originating from the axillary artery using detachable coil which was deployed using deep engagement diagnostic Judkin's’ right catheter. f: Final angiography showing complete closure of all aberrant systemic supply to the left lung with previously deployed Amplatzer vascular plugs and a detachable coil with patent left subclavian and axillary artery.

Fig. 6

a and b: CT angiography with 3D volume rendering in front and back views showing complete closure of all aberrant feeders from left subclavian, axillary arteries and from abdominal aorta with Amplatzer vascular plugs and a detachable coil in situ. It also showed normal vasculature to the left lung and patent left subclavian and axillary artery.