Pulmonary hypertension in interstitial lung diseases: diagnostic and therapeutic approach in 2011?

Pulmonary hypertension in interstitial lung diseases: diagnostic and therapeutic approach in 2011? Pulmonary hypertension is frequent in late-stage idiopathic pulmonary fibrosis, and is associated with a shorter survival. It should be suspected in case of dyspnea or hypoxemia disproportionate with the degree of parenchymal lung disease. It is particularly frequent in patients with the syndrome of combined pulmonary fibrosis and emphysema, and associated with a short survival (median survival less than 1 year). Pulmonary hypertension associated with chronic infiltrative lung diseases can be detected by echocardiography and must be confirmed by right-sided heart catheterization, especially to rule out post-capillary pulmonary hypertension frequent in this context. Management is mainly palliative and based on supplemental nasal oxygen. Therapy specific for pulmonary arterial hypertension, poorly evaluated in pulmonary hypertension associated with infiltrative lung diseases, is occasionally proposed to patients with disproportionate pulmonary hypertension (mean PAP > 35 mmHg), with often limited efficacy, and requiring careful follow-up (risk of increased hypoxemia) and invasive evaluation. Pulmonary transplantation should be considered in the absence of contra-indication.