Common pulmonary vein atresia

Thomas Glenn, Jose Honold, Beth F. Printz, Dana Mueller
University of California San Diego and Rady Children’s Hospital San Diego.
United States

Cardiology in the Young
Cardiol Young 2022; 32: 668-670
DOI: 10.1017/S1047951121003565

Abstract
A 4-hour-old infant with profound cyanosis on an alprostadil infusion was urgently transferred to Rady Children’s Hospital with suspected CHD. Upon arrival, urgent echocardiography was performed but could not confirm the presence of discrete pulmonary veins or pulmonary venous drainage. Given the difficulty in delineating the anatomy, a cardiac CT scan was performed and demonstrated a nearly atretic common pulmonary vein with multiple small collaterals that drained to systemic veins. Due to the high risk of mortality associated with operative repair, the decision was made to proceed with compassionate withdrawal of care. The described anatomy of common pulmonary vein atresia remains rare, and to our knowledge, fewer than 40 cases have been reported in the literature. Albeit rare, common pulmonary vein atresia should be considered in the differential diagnosis of a severely cyanotic neonate.

Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing

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: Yes

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