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A 66-year-old woman with a 20-year history of severe primary pulmonary hypertension presented with progressive shortness of breath, weakness, and changes in mental status. There was a palpable systolic impulse in the second left anterior intercostal space. Arterial blood gas values during the administration of oxygen were as follows: pH, 7.14; partial pressure of carbon dioxide, 175 mm Hg; partial pressure of oxygen, 88 mm Hg; and bicarbonate concentration, 60 mmol per liter. Chest radiography performed after intubation (Panel A) revealed 80 percent opacification of the left lung field and an enlarged right hilum. Computed tomographic scanning showed that the opacity in the left lung was a markedly dilated aneurysm in the left pulmonary artery, with calcified walls, that nearly occluded the left main-stem bronchus (Panel B). Bronchoscopy confirmed the presence of a nearly complete and pulsatile extrinsic compression of the left main-stem bronchus. Chronic, severe primary pulmonary hypertension caused a massive pulmonary-artery aneurysm leading to additional obstructive and restrictive defects that contributed to respiratory failure.
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