Abstract
Background: Infantile Scimitar syndrome (SS) is a rare, severe congenital anomaly involving anomalous right pulmonary venous drainage into the inferior vena cava (IVC). It poses significant diagnostic and management challenges in lower middle-income countries (LMICs) due to symptomatic overlap with endemic diseases.
Case Series: This series details three distinct presentations of infantile SS in Pakistan: Case 1: A 7-month-old female, initially misdiagnosed and treated empirically for pulmonary tuberculosis, was diagnosed with SS and an atrial septal defect (ASD). She underwent a successful intra-atrial pericardial patch baffle (Roger Mee procedure) at age two, demonstrating favorable long-term survival (>10 years). Case 2: A male neonate presented with severe persistent pulmonary hypertension (PPHN) and was diagnosed with SS associated with a rare horseshoe lung anomaly. Due to delayed treatment and parental refusal of timely admission, the patient clinically deteriorated and died at seven months. Case 3: A preterm male neonate presenting with dextroposition, severe right lung hypoplasia, and pulmonary sequestration underwent a successful life-saving right pneumonectomy on the 22nd day of life and was stabilized. Conclusion: Infantile SS exhibits high clinical variability and carries a poor prognosis if treatment is delayed. Advanced multimodality imaging is vital to avoid critical misdiagnoses, and early, tailored surgical interventions ranging from baffle repair to pneumonectomy are essential for survival.