Long-Term Survivor With Pulmonary Veno-Occlusive Disease

A 51-year-old woman was admitted to our hospital because of shortness of breath. Her mean pulmonary artery pressure was 52 mm Hg, and idiopathic pulmonary artery hypertension was diagnosed. She received beraprost sodium, imidapril, warfarin, and home-based oxygen therapy. At 67 years of age, dyspnea on effort worsened to New York Heart Association functional class III. The plasma level of brain natriuretic peptide was 57 pg/mL. Transthoracic echocardiography showed an enlarged right ventricle, severe tricuspid regurgitation, and a large pericardial effusion. At 68 years of age, she was urgently admitted to our hospital because of severe dyspnea at rest. A chest x-ray showed bilateral pulmonary artery enlargement (Figure 1). Computed tomography of the chest revealed that both main bronchi were compressed by the pulmonary artery aneurysms, with no mural thrombus in the pulmonary arteries (Figure 2). The diameters of the right and left main pulmonary arteries were 69.8 and 55.5 mm, respectively. Bronchoscopy during mechanical ventilation confirmed nearly complete extrinsic compression of the left and right main stem bronchi without positive airway pressure (Figure 3 …