SCİMİTAR SENDROMU

Öz

Scimitar sendromu sağ pulmoner venin, vena cava inferior veya sağ atriuma açılmasıyla karakterize nadir görülen bir pulmoner venöz dönüş anomalisidir. Sağ akciğer hipoplazisi, pulmoner sekestrasyon, atriyal septal defekt (ASD) ve kalbin dextropozisyonu eşlik eden diğer anomalilerdir. Olgu: 36 yasında bayan hasta halsizlik ve nefes darlığında 2 aydır artma şikayetiyle göğüs hastalıkları polikliniğimize başvurdu. Hastanın çocukluk çağından beri birkaç kez öksürük ve nefes darlığı ile hastaneye basvurusu olduğu öğrenildi. Hastanın başvurusunda yapılan fizik muayenesinde akciğer ve kalp sesleri normal olarak değerlendirildi. Çekilen akciğer filminde sağ akciğer parakardiyak alanda opasite artışı görüldü. Bilgisayarlı tomografik anjiyografide (BT anjografi) sağ alt ve üst pulmoner venlerin doğrudan hepatik ven- inferior vena cava düzeyine açıldığı görüldü. Olgu radyolojik olarak Scimitar Sendromu olarak tanı aldı. Nadir görülen bir pulmoner venöz dönüş anomalisi olması nedeniyle literatür eşliğinde sunuldu.

Anahtar Kelimeler

• Dekstrokardi,konjenital anomali, akciğer hipoplazisi

SCİMİTAR SYNDROME

Abstract

Scimitar Syndrome is a rare congenital disorder that is characterised by an anomalous connection of the pulmonary vein with the inferior vena cava. The other components of the syndrome include hypoplasia of the right lung, hypoplasia of the right pulmonary artery, pulmonary sequestration, dextrocardia, atrial septal defect (ASD). A 36 years old woman suffering from weakness and shortness of breath was admitted in our clinic. It has been learned that the patient has been referred to the hospital with coughing and shortness of breath several times since childhood. Physical examination was normal. The chest radiograh demonstrated increased opacity along the right paracardiac area. Computed tomography angiography (CT angiography) was evaluated. It was detected that the right pulmonary veins drained in to the inferior vena cava named as Scimitar Syndrome. This case was presented with the review of literature.

Keywords

• Dextrocadia, congenital anomaly, lung hypoplasia

Kaynakça

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