Scimitar syndrome and left pulmonary vein stenosis: A serious and rare association

Abstract

Scimitar syndrome is a rare constellation of cardiovascular anomalies consisting of partial right anomalous pulmonary venous drainage to the inferior vena cava, hypoplasia of the right lung, cardiac dextroposition, and in many cases, one or more aortopulmonary collaterals from the descending aorta to the hypoplastic lung. The "infantile form" commonly presents with significant heart failure and pulmonary hypertension. Stenosis of the left pulmonary veins associated with scimitar syndrome is a rare but well-described combination, usually associated with a poor prognosis. We describe two patients with this association in whom the left pulmonary vein stenosis manifested months after the initial diagnosis as a progressive lesion that complicated the course of the disease due to severe pulmonary hypertension. Both patients were successfully treated with a combination of surgical, percutaneous, and hybrid treatment.

Keywords: Pulmonary hypertension; pulmonary vein stenosis; scimitar syndrome.

Figure 1

CT scan at the time of diagnosis showing the anomalous drainage of the right lower pulmonary vein into the IVC (arrows). IVC: Inferior vena cava, RLPV: Right lower pulmonary vein, CT: Computed tomography

Figure 2

(a) Angiography in the right lower pulmonary vein showing its drainage to the IVC and a filiform stenosis (arrow) at the junction between the IVC-right atrium junctions, (b) angiography in the left lower pulmonary vein showing a stenosis (arrow) at its connection to the left atrium. IVC: Inferior vena cava

Figure 3

(a) Hybrid “stop-flow” angiography showing a stenosis at the distal end (black arrow) of the stent previously implanted at the collector of the left pulmonary veins (white arrow), (b) resolution of the stenosis after new stent implantation. The previously implanted stents in the right superior pulmonary vein (white arrow) and in the IVC (black arrow) are also shown. IVC: Inferior vena cava

Figure 4

CT scan at the age of 7 showing patency of the stents implanted in the left pulmonary veins. CT: Computed tomography

Figure 5

CT scan at diagnosis showing the presence of a horseshoe lung (black arrow) in addition to a stenosis of the main right pulmonary bronchus (white arrow). CT: Computed tomography

Figure 6

(a) Angiography in the thoracic descending aorta (white arrow) showing two large collateral arteries (black arrows) to the right lung, (b) both collateral arteries were embolized with multiple coils (black arrows) and two Amplatzer Vascular Plug 4^® (white arrows)