Michael L. O’Byrne, Russell R. Cross, Gerard R. Martin
The Children’s Hospital of Philadelphia,Philadelphia. Children’s National Medical Center and George Washington University School of Health Sciences.
United States
Cardiology in the Young
Cardiol Young 2018; 28: 329-333
DOI: 10.1017/S1047951117001822
Abstract
An asymptomatic 6-year-old boy with a history of right lung hypoplasia was referred for cardiology evaluation. Echocardiography demonstrated right pulmonary artery hypoplasia with flow reversal in that vessel. The right pulmonary veins were not visualised in the echocardiogram. Cardiac catheterisation confirmed the diagnosis of scimitar syndrome with a characteristic large vertical vein; however, the right pulmonary veins were found to be atretic with no connection to the heart with decompression through the azygos vein. In all, four systemic to pulmonary arterial collaterals were identified, supplying the right lung, which were occluded using embolization coils. This case demonstrates the potential for progressive stenosis and atresia of the so-called “scimitar vein” without previous surgical instrumentation, and that this can occur without haemodynamic embarrassment or development of pulmonary vascular disease.
Category
Absence or Atresia of Normal Pulmonary Venous Connections
Diagnostic Testing. Invasive
Year of Publication: 2018
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes