Background: Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies; the adult form is not usually is associated with pulmonary hypertension.
Case Characteristics: 6-year-old girl with recurrent episodes of cough and breathlessness, along with features of right heart enlargement. Computed tomography angiogram revealed right pulmonary veins draining into inferior vena cava with dextroposition of heart.
Outcome: Successfully managed with surgical correction.
Message: Scimitar syndrome should be considered in any child with unexplained pulmonary hypertension and dextroposed heart.
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