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Sava DEDEOLU
 


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AN EXTREMELY RARE SYNDROME OF A NEWBORN: BERRY SYNDROME
 
A newborn in the first day of life weighing 2.8 kg presented for a cyanosis and murmur. On physical examination, tachypnea, blood saturation of 92% was noted as well as a cardiac murmur. On chest X-ray, the cardiac silhouette was slightly enlarged, and the pulmonary vasculature was engorged. An electrocardiogram demonstrated normal sinus rhythm with right ventricular hypertrophy and right-axis deviation. Echocardiography revealed a type A interrupted aortic arch, an AP window, and a PDA. It also identified the RPA arising from the right side of the ascending aorta. A 1-stage surgical correction was performed through a median sternotomy. Under deep hypothermic circulatory arrest, the ascending aorta was transversely incised at the level of the AP widow, and the pulmonary arteries were disconnected from the aorta. The posterior wall of the ascending aorta was retained in order to form the confluence between the RPA and the main pulmonary artery (MPA). The anterior half of the RPA was reconstructed with a patch of autologous pericardium. Then, after removal of ductal tissue, the descending aorta was anastomosed to the aortic arch. The ascending aorta, which was divided in order to separate the pulmonary artery, was repaired with an end-to end anastomosis. After re-warming, the patient was easily weaned from cardiopulmonary bypass. The subsequent hospitalization was uneventful, and the patient was discharged in good clinical condition on postoperative day 19. One year later, echocardiography and cardiac catheterization showed a satisfactory arch reconstruction. However, it also revealed stenosis of the RPA junction, with a pressure gradient of 20 mmHg. This was successfully relieved with percutaneous balloon angioplasty, As of the 6-month follow-up, the patient was growing satisfactory and has remained asymptomatic.

Anahtar Kelimeler: berry aneurysm,newborn,surgery