Iqra Javed, Musawer Khan, Haya, Sana Imtiaz, Muhammad Shoaib, MBBS, Yusra Iqbal, Khwaja Waleed Maqbool, Kamil Ahmad Kamil
Combined Military Hospital Quetta. SMBZAN Institute of Cardiology. Quetta Institute of Medical Sciences. Mirwais Regional Hospital.
Pakistan and Afghanistan
Radiology Case Reports
Radiol Case Rep 2026; 21: 2982-2987
DOI: 10.1016/j.radcr.2026.03.021
Abstract
Scimitar syndrome is a rare congenital cardiopulmonary anomaly characterized by partial anomalous pulmonary venous return (PAPVR) from the right lung to the inferior vena cava, often associated with right-lung hypoplasia and dextroposition of the heart. The infantile form usually presents early with respiratory distress and pulmonary hypertension, posing diagnostic and management challenges. We report a term male neonate born at 38 weeks of gestation, who developed severe respiratory distress soon after birth and was initially managed for meconium aspiration syndrome. Echocardiography revealed dextroposition of the heart, right atrial and ventricular dilatation, moderate pulmonary hypertension, and a small secundum-type atrial septal defect. Despite supportive care, oxygen dependency persisted, prompting CT angiography, which demonstrated an anomalous right pulmonary venous drainage into the infra-diaphragmatic inferior vena cava confirming scimitar syndrome. The patient was managed conservatively with continuous positive airway pressure (CPAP), diuretics, and pulmonary vasodilators, leading to gradual clinical improvement and successful weaning off oxygen by day 40 of life. Surgical repair was deferred until pulmonary function optimization. Scimitar syndrome should be suspected in neonates with unexplained respiratory distress and right-sided cardiac silhouette on chest radiograph. Early multidisciplinary evaluation and timely management can significantly improve clinical outcomes.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes