A case of tracheal varices in an adolescent patient with cyanotic heart disease

Abstract

Tracheal varices and bronchial varices are infrequently reported in adults as a complication of an underlying vascular obstruction, including portal hypertension, pulmonary arterial hypertension, or pulmonary venous hypertension. Tracheal varices and bronchial varices have been reported in adults with failing Fontan physiology, but this occurrence is rare in children. We report the unusual presentation of tracheal-bronchial varices due to veno-venous collaterals in an adolescent patient with Glenn physiology for double-inlet left ventricle and portal hypertension secondary to cardiac cirrhosis. We document complete resolution of these varices after heart and liver transplantation.

Figure 1.

Our patient’s cardiac anatomy. The arrow demonstrates anastomosis of the superior vena cava (SVC) to the right pulmonary artery (RPA) in a bi-directional Glenn. Elevated mean pressures are demonstrated in the pulmonary arteries, right atrium (RA), and right ventricle (RV). LA = left atrium; LV = left ventricle; RPV = right pulmonary vein; LPV = left pulmonary vein.

Figure 2.

(A) Varices extending from the distal trachea, carina (black arrow) and into both main stem bronchi. Left mainstem bronchus (L). (B) Left main stem bronchus with varices extending into the segmental bronchi. LUL = left upper lobe; LLL = left lower lobe.

Figure 3.

(A) Varicosity (arrow 2) filling with contrast from the veno-venous collateral arising from the base of the innominate vein (arrow 1). The innominate vein drains into the superior vena cava that drains into the right atrium. (B) Amplatzer vascular plugs (arrow 3) inserted with effective occlusion of the vascular channel.