OYA DEMİRCİ, Taner YAVUZ, Emre ERDOĞDU, Resul ARISOY, PINAR KUMRU, Oya PEKİN, Bülent TANDOĞAN

Pulmoner kapak yokluğu sendromu: olgu serisi

Giriş: Pulmoner Kapak Yokluğu Sendromu (APVS), Fallot tetralojisinin özelliklerini içeren, pulmoner kapağın fonksiyonel yokluğunun eşlik ettiği, pulmoner çıkış yolunda darlık ve yetersizlik sonucu ana pulmoner arter ve dallarında anevrizmal dilatasyonun izlendiği bir kardiyak anomalidir.Olgular: 2013- 2014 yılları arasında prenatal dönemde APVS tanısı konulan dört vakanın incelenmesi yapılmıştır.Sonuç: APVS’u nadir görülen bir kardiyak anomali olmasına rağmen tipik ultrason bulguları ile tanısı kolaydır. Dilate pulmoner arter ve dallarının akciğer dokusuna basısı sonucu gelişen bronkomalazi nedeniyle belirgin perinatal mortalite ve morbidite sahiptir.

Anahtar Kelimeler:

pulmoner kapak yokluğu sendromu, Fallot tetralojisi, kromozomal anomali, 22q11 mikrodelesyon, prenatal tanı, fetal sonuçlar

Absent pulmonary valve syndrome: case series

Introduction: Absent pulmonary valve syndrome (APVS) is a variant of Tetralogy of Fallot (TOF). It shares the features of the classical form of TOF with the addition of incompetent or absent pulmonary valve. It is associated with severe dilatation of the main and branch pulmonary artreies because of concurrent severe pulmonary stenosis and regurtation.Cases: Four cases with a fetal diagnosis of APVS between 2013 and 2014 were analyzed.Conclusion: APVS is easy to recognise with typical ultrasound images although it is a rare cardiac abnormality. It is associated with significant perinatal mortality and morbidity because of the bronchomalacia secondary to bronchial compression of the dilated pulmonary artery.

Keywords:

absent pulmonary valve syndrome, tetralogy of Fallot, chromosome abnormalitly, 22q 11 microdeletion, prenatal diagnosis, fetal outcomes,

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