Sa� Pulmoner Arter Agenezisinin E�lik Etti�i Scimitar (Pala) Sendromu

Ta�, Dilaver; Okutan, O�uzhan; �zk�sa, Tuncer; Ayten, �mer; Demirer, Ersin; �ztutgan, Turgut

Cilt : 13 Say� : 3 Y�l : 2024

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Sa� Pulmoner Arter Agenezisinin E�lik Etti�i Scimitar (Pala) Sendromu [Respir Case Rep]

Respir Case Rep. 2013; 2(1): 44-47 | DOI: 10.5505/respircase.2013.96268

Sa� Pulmoner Arter Agenezisinin E�lik Etti�i Scimitar (Pala) Sendromu

Dilaver Ta�, O�uzhan Okutan, Tuncer �zk�sa, �mer Ayten, Ersin Demirer, Turgut �ztutgan
GATA Haydarpa�a E�itim Hastanesi

Scimitar sendromu, nadir bir hastal�kt�r ve hipogenetik akci�er sendromu olarak da bilinir. Sendrom sa� pulmoner venin, vena kava inferiora drenaj� ile karakterizedir. Burada sa� pulmoner arter agenezisinin e�lik etti�i Scimitar sendromu sunuldu. Yirmi bir ya��nda erkek hasta nefes darl��, �ks�r�k, balgam ��karma �ikayetleri ile ba�vurdu. Akci�er grafisinde sa� akci�erde vol�m kayb�, kalp ve mediastende sa�a do�ru yer de�i�tirme izlendi. Bronkoskopik incelemede ve toraks BT�de orta lob bron�u izlenmedi. Toraks BT anjiografi g�r�nt�lerinde sa� akci�er alt loba ait venin sa� hepatik vene drene oldu�u ve sa� pulmoner arter �ap�nda azalma izlendi. Akci�er perf�zyon sintigrafisinde sa� akci�erde total perf�zyon kayb� izlendi. Scimitar sendromlu hastalarda akci�er grafisinde scimitar (pala) i�aretinin olmas� karakteristiktir. Ancak bu i�aret her zaman g�r�lmeyebilir. Akci�er grafisinde vol�m kayb� ve mediastinal �ifti olan gen� hastalarda di�er hastal�klar�n yan�nda Scimitar Sendromu da akla gelmelidir.

Anahtar Kelimeler: Scimitar sendromu, pulmoner arter agenezisi, BT anjiografi.

Scimitar Syndrome Associated with Agenesis of Right Pulmonary Artery

Dilaver Ta�, O�uzhan Okutan, Tuncer �zk�sa, �mer Ayten, Ersin Demirer, Turgut �ztutgan
GATA Haydarpa�a Training Hospital

Scimitar syndrome is a very rare disorder and also known as hypogenetic lung syndrome. The syndrome is characterized with anomalous connection of the pulmonary vein with the vena cava inferior. In this text, scimitar syndrome associated with pulmonary artery hypoplasia is presented. A 21-year-old male patient with complaints of cough, shortness of breath and sputum was admitted. Chest X-ray revealed volume loss of right lung and shift to the right of heart and mediastinum. The middle lobe wasn�t observed in bronchoscopy and thorax CT. Thoracic CT angi-ography images revealed that vein of right lung lower lobe drains right hepatic vein and right pulmonary artery diameter is reduced. Lung perfusion scintigraphy revealed total loss of perfusion of right lung. Scimitar sign on chest X ray is characteristics for scimitar syndrome. However, this sign cannot be seen in some patients. If there is volume loss of right lung and shift to the right of heart and mediastinum, scimitar syndrome should be considered in addition to other diseases.

Keywords: Scimitar syndrome, agenesis of pulmonary artery, CT angiography.

Sorumlu Yazar: Dilaver Ta�, T�rkiye
Makale Dili: T�rk�e

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