Infantile Scimitar Syndrome With Contralateral Pulmonary Vein Stenosis and Refractory Pulmonary Hypertension

Janelle Buysse, Ramya Deepthi Billa, Daniel McLennan, Ravi Ashwath, Aditya Badheka, Madhuradhar Chegondi
University of Iowa Stead Family Children’s Hospital.
United States

Cureus
Cureus 2021; 13:
DOI: 10.7759/cureus.17203

Abstract
Infantile scimitar syndrome is associated with pulmonary hypertension which can be difficult to manage. We present a three-month-old infant with scimitar syndrome, who eventually developed refractory pulmonary hypertension, posing a significant management challenge. Further workup demonstrated contralateral pulmonary vein stenosis, which is rarely described in scimitar syndrome. Our index case highlights the importance of follow-up cardiac catheterizations in these patients with severe pulmonary hypertension.

Category
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis

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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