Pulmonary hypertension with lung and left-sided heart diseases

Increasing awareness of pulmonary hypertension (PH) leads to an increased detection rate even in elderly patients with a higher rate of comorbidities. In patients with PH and left-sided heart or pulmonary diseases it is necessary to assess if these cardiac or pulmonary conditions are the cause of PH or only independent comorbidities. Additionally, it has to be evaluated if a chronic left-sided ventricular disease with pulmonary congestion has induced a pulmonary vasculopathy with severe PH. Sleep apnea syndrome can underlie PH and severe PH can lead to central sleep apnea. These aspects require experience and a careful and extensive diagnostic evaluation, including complete hemo-dynamic assessment, imaging techniques, lung function and cardiopulmonary exercise testing. Experienced PH centers should be involved in this diagnostic process at an early stage. Although PH leads to a worsening of the prognosis of pulmonary fibrosis and chronic obstructive pulmonary dis- ease (COPD) as well as of diastolic and systolic heart failure, published data do not support targeted PH therapies under these conditions due to a lack of evidence. Therefore, the treatment of the underlying disease is the primary goal. Mitral valve repair and non-invasive pressure ventilation in patients with alveolar hypoventilation lead to hemodynamic and functional improvement. Patients with PH and left heart disease show improvement following normalization of volume load. So far there are no approved medical therapies for PH due to left-sided heart disease and PH due to lung diseases. Patients with PH and left-sided heart diseases and lung diseases should be introduced in specific clinical studies.