Long-term development of pulmonary hypertension in cryptogenic fibrosing alveolitis

Values characterizing the central haemodynamics in patients with cryptogenic fibrosing alveolitis tend to vary considerably in the individual subjects over the years, but the long-term average indicates a trend towards a gradual deterioration. In most patients, the development of pulmonary hypertension is rather slow, amounting to 0.712 kPa (1.3 mmHg) per year on the average. This development is due both to the increase of the transpulmonary driving pressure gradient and to an increase in the pulmonary artery wedge pressure.