Journal of Kerman University of Medical Sciences

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

Document Type : Case Report

Authors

Yazdan Ghandi 1
Alireza Farsi 2

1 Amirkabir Hospital, School of Medicine, Department of Pediatrics, Pediatric Cardiology, Arak University of Medical Sciences, Arak, Iran

2 Amirkabir Hospital, School of Medicine, Arak University of Medical Sciences, Arak, Iran

https://doi.org/10.22062/jkmu.2021.91836
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
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Journal of Kerman University of Medical Sciences
Volume 28, Issue 6
November and December 2021
Pages 621-625

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