Pulmonary Hypertension with Atrial Septal Defect in an Infant: A Case Report of Rare Presentation of Scimitar Syndrome?

Abstract

Background: Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung along with pulmonary hypoplasia. Scimitar syndrome is associated with a partial anomalous pulmonary venous connection of the right lung to the inferior vena cava, right lung hypoplasia, heart dextroposition, and anomalous systemic arterial supply to the right lung. Patients are diagnosed either early with severe symptoms (infantile type) including tachypnea, chest infection, heart failure and failure to thrive or late with minimal symptoms (childhood/adult type) as a result of accidental findings. The most common anomalies associated with this syndrome are intracardiac defects with a prevalence of about 40%. Here,Case report: we present a case of a male infant with respiratory distress and manifestation of large ASD2 and pulmonary hypertension, who was diagnosed with scimitar syndrome after more evaluation.Conclusion: This diagnosis should be considered when ASD2 with PH are diagnosed in infantile period.

Keywords

• congenital anomaly
• pulmonary hypertension
• scimitar syndrome