An infant with congenital midaortic syndrome

Öz Midaortic syndrome is an uncommon disease characterized by segmental or diffuse narrowing of abdominal or distal descending thorasic aorta. Majority of the cases are idiopathic but some are secondary to fibromuscular dysplasia, giant cell arteritis, Moyamoya disease or genetic diseases such as neurofibromatosis, Williams’ syndrome and Alagille syndrome. It is very rare in newborn period. Here we present a 40 days old infant who admitted to hospital for respiratory distress, hypertension, heart failure and was diagnosed as midaortic syndrome. She had medical treatment but unfortunately died due to heart failure when she was two months old age. Midaortik sendrom nadir bir hastalık olup abdominal veya distal inen aortanın segmental veya difüz darlığı ile karakterizedir. Olguların çoğu idiyopatik olmakla birlikte bir kısmı da fibromüsküler displazi, dev hücreli aterit, Moyamoya hastalığı veya nörofibramatozis, Williams’ sendromu ve Alagille sendromu gibi genetik hastalıklara ikincildir. Burada 40 günlük iken hastaneye solunum sıkıntısı, kalp yetmezliği tablosunda başvuran ve midaortik sendrom tanısı alan bir olgu sunulmuştur. Olguda medikal tedavi tercih edilmiş ancak bebek iki aylık iken eksitus olmuştur.