Respir Case Rep. 2019; 8(2): 74-77 | DOI: 10.5505/respircase.2019.49403

Right Pulmonary Artery Agenesis and Right Pulmonary Hypoplasia, presenting with Bronchiolitis Symptoms

Tayfun Kermenli¹, Aslý Serter^2
1Medicalpark Elazýð Hospital Thoracic Surgery Clinic, Elazýð, Turkey
²Medicalpark Elazýð Hospital Radiology Department, Elazýð, Turkey

Unilateral pulmonary artery agenesis (UPAA) is a rare ailment, characterized by the absence of a unilateral pulmonary artery. UPAA may be associated with heart defects such as Tetralogy of Fallot, coarctation of the aorta, atrial septal defects and truncus arteriosus. The etiology has not been fully elucidated to date. Patients may present with signs of pulmonary hypertension during childhood, and symptoms of UPAA may not manifest until adulthood. A 33-year-old woman diagnosed at adulthood presented with symptoms of bronchiolitis. At the time of diagnosis, PA chest X-ray and Thorax CT images showed a right hypoplasic lung. Pulmonary hypertension and minimal tricuspid regurgitation were detected in a cardiac ultrasonography. For a diagnosis of UPAA, the patient's story, physical examination findings and imaging are used. The optimum imaging technique is pulmonary angiography. Clinical symptoms include exercise intolerance, hemoptysis and recurrent lower respiratory tract infections.

Keywords: pulmonary artery, agenesis, hypoplasic lung, congenital lung anomalies

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Bronþiolit Semptomlarý ile Baþvuran Sað Pulmoner Arter Agenezisi ve Sað Pulmoner Hipoplazi

Tayfun Kermenli¹, Aslý Serter^2
1Medicalpark Elazýð Hastanesi Göðüs Cerrahisi Kliniði, Elazýð
²Medicalpark Elazýð Hastanesi Radyoloji Birimi, Elazýð

Unilateral pulmoner arter agenezisi (UPAA) tek taraflý pulmoner arterin yokluðu ile karakterize nadir görülen bir anomalidir. Fallot tetralojisi, aort koarktasyonu, atriyal septal defekt, turunkus arteriozus gibi kalp defektleri ile birlikte olabilir. Etyolojisi tam olarak aydýnlatýlamamýþtýr. Çocukluk çaðýnda pulmoner hipertansiyon ile bulgu verebileceði gibi hastalar eriþkin yaþa kadar da bulgu vermeden gelebilirler. Bronþiolit bulgularý ile baþvuran 33 yaþýndaki kadýn hasta eriþkin yaþta taný almasý nedeniyle sunuldu. Taný anýnda PA akciðer grafisi ve Toraks BT’sinde sað hipoplazik akciðer mevcuttu ve kardiyak ultrasonografide pulmoner hipertansiyon ile minimal triküspit yetmezlik tespit edildi. UPAA tanýsýnda hastanýn öyküsü, fizik muayene bulgularý ve görüntüleme yöntemlerinden yararlanýlýr. Altýn standart teknik pulmoner anjiografidir. Klinik semptomlar egzersiz intoleransý, hemoptizi ve tekrarlayan alt solunum yolu enfeksiyonlarýný içerir.

Anahtar Kelimeler: pulmoner arter, agenezi, hipoplazik akciðer, doðumsal akciðer anomalileri

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Image Slices of the Case

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