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

Öz

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 Turk Arch Ped 2013; 48: 173 5

Anahtar Kelimeler

• Computerized tomography, congenital heart disease, “scimitar” syndrome

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

Öz

“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 ldquo;scimitar rdquo; 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 ldquo;scimitar rdquo; sendromlu bir olgu sunuldu Yeni nesil görüntüleme yöntemlerinin tanıdaki önemi ve cerrahi yöntem irdelendi.

Anahtar Kelimeler

• Bilgisayarlı tomografi
• doğuştan kalp hastalığı
• “scimitar” sendromu

Kaynakça

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