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 doubleinlet left ventricle and portal hypertension secondary to cardiac cirrhosis. We document complete resolution of these varices after heart and liver transplantation.

Bronchial varices in a child with tricuspid atresia six years post Fontan correction

“…Venous collaterals, on the other hand, are commonly seen post bidirectional Glenn and Fontan due to the pressure gradient created between the higher pressure superior caval system and lower pressure veins supplying the receiving atrium [1,15]. Development of haemoptysis because of these collaterals is also uncommon [4].…”

“…Bronchial varices are abnormally dilated, tortuous blood vessels in the pulmonary system and are a rare cause of haemoptysis in children. They can occur due to prolonged increased pulmonary vascular resistance, including portal and pulmonary hypertension [4]. Pulmonary or portal vein stenosis [5,6], pulmonary vein atresia [7], chronic liver congestion [8], cirrhosis [9,10], and portal venous thrombosis [11] have all been reported to cause tracheal‐bronchial varices.…”

Fontan-Associated Liver Disease: Pathophysiology, Staging, and Management

“…60 In the Fontan circulation, the increased systemic venous pressure is directly transmitted through the azygos system to the esophageal venous plexus, contributing to variceal development in the upper ("downhill varices") and lower esophagus. 61 Unlike classic portal hypertension secondary to cirrhosis, the portal hypertension model in FALD is characteristically hypodynamic due to low cardiac output. 1 Consequently, the esophageal varices are usually small with low risk of bleeding and the use of primary prophylaxis is thus debatable.…”

Large Tracheal Varices in a Patient With Superior Vena Cava Compression

“…1 It also recommends that all patients being considered for radical treatment undergo a PET-CT scan to exclude metastatic …”