Michael Perez, T. K. Susheel Kumar, Mario Briceno-Medina, Mohammed Alsheikh-Ali, Shyam Sathanandam, Christopher J. Knott-Craig
University of Tennessee Health Science Center and Le Bonheur Children’s Hospital.
United States
Cardiology in the Young
Cardiol Young 2016; 26: 629-635
DOI: 10.1017/S1047951115002334
Abstract
Common pulmonary vein atresia is a rare and usually fatal congenital anomaly, in which the pulmonary veins come together to form a confluence that does not connect to the left atrium. We report our experience with three cases of common pulmonary vein atresia and review the literature on this anomaly. The diagnosis of common pulmonary vein atresia must be entertained in any newborn that presents with cyanosis, refractory acidosis, and decreased systemic perfusion within the first 48 hours of life. Echocardiography is a useful screening tool, but cardiac catheterisation is the preferred diagnostic tool. Common pulmonary vein atresia can be fatal without surgical intervention, but survival after surgery continues to be poor.
Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No