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Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome
European Respiratory Journal , 08/11/09

Cottin V et al. – Patients with combined pulmonary fibrosis and emphysema syndrome and pulmonary hypertension confirmed by right heart catherization have a dismal prognosis despite moderately altered lung volumes and flows and moderately severe hemodynamic parameters.

Exclusive Author Commentary
V. Cottin, 08/13/09

The risk of developing precapillary pulmonary hypertension is the main reason why diagnosing the syndrome of combined pulmonary fibrosis and emphysema is so important. In a previous study (Cottin V. et al, Eur Respir J 2005;26:586-593) we demonstrated that about half of the patients with this CT-defined syndrome develop pulmonary hypertension. Here, we report that the one-year overall survival is only 60% in these patients once precapillary pulmonary hypertension has been confirmed by right heart catheterisation. Whether patients may benefit from treatment specific for pulmonary hypertension is currently not known. Patients with the syndrome of combined pulmonary fibrosis and emphysema and pulmonary hypertension should be evaluated for lung transplantation whenever possible.

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