Common pulmonary vein atresia: report of three cases and review of the literature

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
Absence or Atresia of Normal Pulmonary Venous Connections
Length of Life Associated with Pulmonary Venous Obstruction
Diagnostic Testing. Invasive
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease

Year of Publication: 2016

Age Focus: Pediatric

Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients). Literature Review and Analysis.

Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No