A 2-month-old infant, suffering from respiratory distress, was suspected to have scimitar syndrome from a chest roentgenogram and was diagnosed by cardiac catheterization. Ligation of the aberrant feeding arteries and right lung resection was performed at three and four months of age, respectively. However, the postoperative course was complicated with right pneumonectomy syndrome requiring continuous artificial ventilation. With supportive management, the patient improved gradually and was eventually weaned off the ventilator at three years of age.
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