Acquired pulmonary vein occlusion is a rare complication of surgical correction of complex congenital heart diseases. Associated vascular remodeling of arterial and venous vessels includes medial thickening, intimal fibrosis, and progressive development of aorto-pulmonary collaterals. The consequent pulmonary perfusion changes impact more importantly on a low pressure circulation such as the Fontan circuit. The management of these patients is still controversial. We describe this condition in two patients with single ventricle who underwent staged cavopulmonary connection. In both cases, we found acquired occlusion of one pulmonary vein, poor antegrade flow in the involved hypoplastic pulmonary arteries due to a widespread thin collateral circulation producing a backward washout of unopacified blood flow with competitive mechanism. Percutaneous embolization of a segmental pulmonary artery was performed with restoration of a more homogeneous perfusion of both main pulmonary arteries. Extracardiac total cavopulmonary connection was successfully performed a few months later in both patients.
- pediatric interventions
- pulmonary angiography
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
- Radiology Nuclear Medicine and imaging