Idiopathic dilatation of the pulmonary artery defines the presence of a benign enlargement of the pulmonary trunk with or without the involvement of the rest of the arterial tree in the absence of a detectable cardiac or arterial systemic disorder. An IDPA patient’s symptoms and physical examination are not significant in diagnosis confirmation. Imaging techniques such as contrast-enhanced computed tomography, right heart catheterization, and angiocardiogram constitute the foundation of IDPA diagnosis. But an extended observation period is also necessary to exclude other possible dilatation explanation. Our patient, a forty-six-year-old female, was admitted to the Pulmonology clinic of “ShefqetNdroqi” University Hospital with dyspnea, dry cough, vertigo, and fatigue for several months before admission. The patient's contrast-enhanced Computed Tomography revealed a dilatation of the pulmonary trunk. The dilatation of the pulmonary trunk was confirmed with the Cardiac CT, and other abnormalities were excluded.

A twenty-three-year-old male truck driver with a 12-pack year smoking history and no previous history of hospitalization was admitted to the “Shefqet Ndroqi” University Hospital pulmonology department after a cardiologist consultation where he was diagnosed with pulmonary hypertension. Two of the patient’s uncles from his mother’s side died during their pediatric age from congenital heart defects. A contrast-enhanced chest computed tomography was performed, and it showed an interruption of the lumen of the proximal part of the left pulmonary artery (atresia), a reduction of the left lung parenchyma volume, and bilateral pulmonary emphysema. The respiratory functional tests detected a moderate obstructive syndrome, and the 6MWT detected a decrease in arterial partial pressure of oxygen and oxygen saturation level. A diagnosis of unilateral pulmonary artery atresia/agenesis was confirmed.