Backgroud: IAA with an intact ventricular septum is distinctly unusual. Combination with an Aortopulmonary Window (APW), ascending aortic origin of the right pulmonary artery and PDA may be present which is called as Berry syndrome, a rare combination of cardiac anomalies, reported to be 0.046%, lethal combination and die shortly after birth.
Case report: We report a 9 days-old male neonates weighing 3.85 kg was referred by local hospital to our center and was ventilated with history of respiratory distress and severe infection since he was born. Admitted to our PCICU, 2D echo showed an IAA type A associated with a huge APW type II and restrictif PDA. A PGE1 infusion was started, during the following days the baby experienced several epileptic episodes. After improvement of the clinical condition, surgery was performed on the 20th days of life on year 2011. A successful one-stage repair of such anomalies in which cutting of PDA that arised from PA trunk and distally becoming into descending aorta, extended end to end anastomosis to conduct the ascending aortic blood flow into the descending aorta and intra arterial baffle was used. A 4-0 Gore-Tex baffle was used both to close the APW and separated the RPA from aortic origin with a good result, as his recently grown up as a cheerful 9 year old child who is growing actively and has entered elementary school in grade 2.
Conclusion: Berry syndrome is a rare but well-identified and surgically correctable anomaly. Early diagnosis and surgical treatment to avoid irreversible pulmonary hypertension is mandatory.
Keywords: Aortic origin of the right pulmonry artery; Aortopulmonary window; Berry syndrome; Interrupted aortic arch; One-stage repair.
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