Cemşit KARAKURT, Fazlı Serkan ÇELİK, Ahmet SIĞIRCI

Transcatheter closure of anomalous systemic arterial supply in a child with pulmonary hypertension

Anormal sistemik arter nadir görülen bir durum olup sıklıkla Scimitar sendromlu olgularda görülmektedir. Normal akciğer dokusu ile birlikte görülen anormal sistemik arter bronkopulmoner sekestrasyonun bir varyantı olarak kabul edilir ve normal akciğer parankimi ve bronşiyal bağlantı ile karakterizedir. Bu durum ilk kez Pryce tarafından Pryce tip 1 olarak sınıflandırılmıştır. En sık görülen klinik bulgular göğüs ağrısı, dispne, sık alt solunum yolu enfeksiyonu, hemoptizi ve kalp yetersizliğidir. Bu hastalarda anormal sistemik arter cerrahi veya transkateter yolla tedavi edilebilir. Bu sunumda kliniğimize sık alt solunum yolu enfeksiyonu geçirme şikayeti ile başvuran ve pulmoner hipertansiyon saptanan 6 yaşında Down sendromlu bir olguda sol alt lobu besleyen anormal sistemik arter saptanan ve transkateter yolla başarılı şekilde kapatılan bir olgu sunulmuştur.

Pulmoner hipertansiyonlu bir çocukta transkateter yolla kapatılan anormal sistemik arter

Anomalous systemic arterial supply is a rare condition and it s commonly seen in patients with Scimitar syndrome. A systemic arterial supply to a normal lung without anomaly is a rare variant of bronchopulmonary sequestration and it is characterized by normal pulmonary parenchymal tissue and bronchial connection. The lesion was classified by Pryce as type 1.Common clinical symptoms are chest pain, dyspnea, frequent lower respiratory infection, pulmonary hypertension, hemoptysis, and heart failure. The treatment may be surgical or transcatheter embolization. In this report we described a patient, a 6-year-old boy with Down syndrome and pulmonary hypertension, who had anomalous systemic artery supply to the left lower lob of the lung and its treatment with transcatheter closure by using a vascular plug II.

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