Background: Communication between the right pulmonary artery (RPA) and left atrium (LA) is a rare cause of central cyanosis in pediatric patients. Case presentation: We describe a 3-year-old female patient with an oxygen saturation of 70% at admission. The echocardiogram indicated an abnormal color flow Doppler in the LA and she underwent standard cardiac catheterization. The angiography of pulmonary artery revealed a 7.4 mm × 7.6 mm fistula between the RPA and LA and achieved successful closure using ventricular septal defect occlusion.
Conclusion:The fistula between pulmonary artery and left atrium is an extremely rare but treatable congenital defect. It should be considered in differential diagnosis of cyanosis in children.
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