Scimitar syndrome: A rare cause of respiratory distress in infants – Case report

Scimitar syndrome is a rare combination of congenital cardiopulmonary anomalies that includes a partially anomalous right pulmonary vein (PARPV) from the right lung. Here we report an infant who presented with recurrent respiratory tract infections, respiratory distress and persistent pulmonary hypertension who was diagnosed to have scimitar syndrome. The diversity of the clinical presentation and its rarity itself make it difficult to diagnose. Here we intend to highlight the importance of key diagnostic investigations such as chest X-rays, echocardiograms and computerized tomography in arriving at the diagnosis. A high degree of suspicion is required to arrange crucial investigations without delay.

Authors

Publication Details

Journal
Ethiopian Journal of Pediatrics and Child Health
Published
2025-05-22
DOI
https://doi.org/10.4314/ejpch.v20i1.7
Primary Topic
Congenital Diaphragmatic Hernia Studies
Type
article
Field-Weighted Citation Impact
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article

Scimitar syndrome: A rare cause of respiratory distress in infants – Case report

Hashan Pathiraja, Rasika Gunapala, Chanika Lokuhewage, Shehan Perera
Ethiopian Journal of Pediatrics and Child Health
Congenital Diaphragmatic Hernia Studies
article

Scimitar syndrome: A rare cause of respiratory distress in infants – Case report

Hashan Pathiraja, Rasika Gunapala, Chanika Lokuhewage, Shehan Perera
article en

Abstract

Scimitar syndrome is a rare combination of congenital cardiopulmonary anomalies that includes a partially anomalous right pulmonary vein (PARPV) from the right lung. Here we report an infant who presented with recurrent respiratory tract infections, respiratory distress and persistent pulmonary hypertension who was diagnosed to have scimitar syndrome. The diversity of the clinical presentation and its rarity itself make it difficult to diagnose. Here we intend to highlight the importance of key diagnostic investigations such as chest X-rays, echocardiograms and computerized tomography in arriving at the diagnosis. A high degree of suspicion is required to arrange crucial investigations without delay.

Ethiopian Journal of Pediatrics and Child HealthVol. 20(1)
Openalex Percentile: Top 61%
Congenital Diaphragmatic Hernia Studies
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