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Broniolit Semptomlar ile Bavuran Sa Pulmoner Arter Agenezisi ve Sa Pulmoner Hipoplazi [Respir Case Rep]
Respir Case Rep. 2019; 8(2): 74-77 | DOI: 10.5505/respircase.2019.49403

Broniolit Semptomlar ile Bavuran Sa Pulmoner Arter Agenezisi ve Sa Pulmoner Hipoplazi

Tayfun Kermenli1, Asl Serter2
1Medicalpark Elaz Hastanesi Gs Cerrahisi Klinii, Elaz
2Medicalpark Elaz Hastanesi Radyoloji Birimi, Elaz

Unilateral pulmoner arter agenezisi (UPAA) tek tarafl pulmoner arterin yokluu ile karakterize nadir grlen bir anomalidir. Fallot tetralojisi, aort koarktasyonu, atriyal septal defekt, turunkus arteriozus gibi kalp defektleri ile birlikte olabilir. Etyolojisi tam olarak aydnlatlamamtr. ocukluk anda pulmoner hipertansiyon ile bulgu verebilecei gibi hastalar erikin yaa kadar da bulgu vermeden gelebilirler. Broniolit bulgular ile bavuran 33 yandaki kadn hasta erikin yata tan almas nedeniyle sunuldu. Tan annda PA akcier grafisi ve Toraks BTsinde sa hipoplazik akcier mevcuttu ve kardiyak ultrasonografide pulmoner hipertansiyon ile minimal trikspit yetmezlik tespit edildi. UPAA tansnda hastann yks, fizik muayene bulgular ve grntleme yntemlerinden yararlanlr. Altn standart teknik pulmoner anjiografidir. Klinik semptomlar egzersiz intolerans, hemoptizi ve tekrarlayan alt solunum yolu enfeksiyonlarn ierir.

Anahtar Kelimeler: pulmoner arter, agenezi, hipoplazik akcier, doumsal akcier anomalileri

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

Tayfun Kermenli1, Asl Serter2
1Medicalpark Elaz Hospital Thoracic Surgery Clinic, Elaz, Turkey
2Medicalpark Elaz Hospital Radiology Department, Elaz, 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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Tayfun Kermenli, Asl Serter. Right Pulmonary Artery Agenesis and Right Pulmonary Hypoplasia, presenting with Bronchiolitis Symptoms. Respir Case Rep. 2019; 8(2): 74-77

Sorumlu Yazar: Tayfun Kermenli, Trkiye
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