Pulmonary hypertension associated with parenchymal lung disease

G Chandy. Pulmonary hypertension associated with parenchymal lung disease. Can J Cardiol 2010;26(Suppl B):21B-23B. Pulmonary arterial hypertension (PAH) is a disorder of poor prognosis and significant mortality. Patients with concurrent parenchymal lung disease are classified is group 3 pulmonary hypertension (PH). The evaluation of this particular group of patients is complicated and the therapeutic options are limited. Diagnosis of PH must be confirmed by right heart catheterization, and the extent of lung disease should be investigated by a combination of pulmonary function testing and by thoracic high-resolution computed tomography scanning. Therapy is fraught with potential problems because the use of PAH-specific therapy potentially results in worsening hypoxemia. Currently, there are very limited data demonstrating the efficacy and safety of the use of such vasoactive medications within this population. Further randomized clinical trials (focusing on patients with PH Out of proportion to the underlying parenchymal lung disease) are needed to evaluate the benefits and potential side effects of PAH-specific therapy in patients with parenchymal lung disease.