Late Diagnosis of Infantile Scimitar Syndrome with underlying VACTERL Association presenting with Patent Ductus Arteriosus and Severe Pulmonary Hypertension

Scimitar syndrome with VACTERL association is often diagnosed late due to its rarity, resulting in pulmonary hypertension which impairs peri-operative outcomes. In addition, presentation during infancy is associated with high morbidity and mortality. A high index of suspicion is the key to diagnosis, followed by diagnostic cardiac catheterization and/or computed tomography scans. Critical care management of late presenters is difficult due to pulmonary hypertension and abnormal airways.