Pulmonary hypertension due to chronic lung diseases

Abstract

Mild-to-moderate pulmonary hypertension is a common complication of chronic lung diseases. Very few patients have severe pulmonary hypertension; if that is the case, it may be due to co-morbidities or correspond to a particular phenotype. Although pulmonary hypertension has little or no involvement in exercise limitation, it is an independent prognostic factor for survival. The first stage of diagnosis of pulmonary hypertension in chronic lung disease is to establish a level of high, intermediate, or low probability using Doppler echocardiography according to the European Society of Cardiology/European Respiratory Society Guidelines. To determine the cause of pulmonary hypertension, it may be necessary to perform a comprehensive search for the most frequent respiratory and cardiovascular diseases. There are few, but important, indications for right heart catheterization in chronic lung diseases. The main indications are candidates for lung transplantation, suspicion of pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension, and when pulmonary haemodynamics are likely to have significant therapeutic implications. The underlying respiratory disease or associations of diseases should be treated according to current guidelines before concluding that a patient has severe pulmonary hypertension. Non-invasive ventilation and long-term oxygen therapy in hypercapnic and hypoxaemic patients, respectively, improve pulmonary haemodynamics. With the exception of cardiovascular co-morbidities, pharmacotherapies for high blood pressure and for left heart diseases are not recommended to treat pulmonary hypertension due to chronic lung diseases. Pulmonary arterial hypertension-approved therapies are not recommended in pulmonary hypertension due to chronic lung diseases.