Pulmonary veno-occlusive disease: a case report

The authors present the case of a 22-year-old female patient, white, referred to the cardiovascular outpatient clinic in November 2006 for pulmonary arterial hypertension. Complementary diagnostic exams revealed elevated pulmonary arterial pressure, normal capillary wedge pressure and a reduced cardiac index on invasive hemodynamic study. A high-resolution pulmonary CT scan identified a diffuse ground-glass opacity with a centrilobular pattern, and a marked decrease in CO diffusion on respiratory function assessment. An open lung biopsy was accordingly performed in January 2007, which was compatible with pulmonary arterial hypertension with associated venous lesions: pulmonary veno-occlusive disease. Therapy was begun with oxygen support, warfarin and bosentan (62.5 mg twice a day) Monthly follow-up was maintained, but her clinical and functional status progressively worsened and one year after the diagnosis the patient was admitted to our heart failure clinic for acute right heart failure. She was stabilized with inotropic therapy and diuretics and was subsequently referred to an international pulmonary transplantation center. The authors highlight the diagnostic challenge of this entity and its poor response to medical therapy and dismal prognosis.