Cenk Eray YILDIZ, Kadir BABAOĞLU, Gürkan ÇETİN, Mustafa GÜDEN, Aşkın Ali KORKMAZ, Burak ONAN

Anormal pulmoner venöz dönüşün karmaşık formu; scimitar sendromunda yeni nesil görüntüleme ve cerrahi yaklaşım

“Scimitar” sendromu nadir görülen anormal pulmoner venlerin vena kava inferiyora açıldığı ciddi bir doğuştan kalp anomalisidir. Patojenezi belli olmamakla birlikte, akciğer tomurcuğunun erken embriyolojik döneminde gelişen bir bozukluktan kaynaklanır. Göğüs radyografisinde sağ akciğer alt bölgesi ile kalp arasında Türk palası (Turkish sword) veya “scimitar” adı verilen orjinal bir görüntü oluşturur. Diğer anomalilerle birliktelik gösterir; bunlar en sık sağ akciğer hipoplazisi, kardiyak dekstropozisyon, sağ akciğerin anormal sistemik arteriyal dolaşımı, pulmoner sekestrasyon ve atriyal septal defekttir. Bu yazıda; opere ettiğimiz vena kava inferiyora açılan parsiyel pulmoner venöz dönüş anomalisi, atriyal septal defekt, dekstropozisyon ve sağ akciğer hipoplazisi olan sekiz yaşında “scimitar” sendromlu bir olgu sunuldu. Yeni nesil görüntüleme yöntemlerinin tanıdaki önemi ve cerrahi yöntem irdelendi.

The complex form of anomalous pulmonary venous return; new generation imaging and surgical approach of scimitar syndrome

Scimitar syndrome is a rare congenital heart disease where anamolous pulmonary veins drain into inferior vena cava. Its pathogenesis is unknown and it is caused by abnormal development of the pulmonary bud during early embryogenesis. In the chest radiography, the shadow of anomalous veins resembles a Turkish sword (better known as scimitar) located between the lower right lung and the heart. It is associated with other anomalies such as right pulmonary hypoplasia, cardiac dextroposition, anomalous systemic arterial supply to the right lung, pulmonary sequestration and atrial septal defect. In this article, we present an 8-year-old boy operated for scimitar syndrome with partial anomalous pulmonary venous return draining into the inferior vena cava, atrial septal defect, dextropositon of the heart and hypoplastic right lung. The diagnostic significance of new generation imaging methods and surgical strategy have been scrutinized.

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