[Pathophysiology, diagnosis, prognosis and treatment of pulmonary hypertension associated with chronic lung disease]. / Pulmonale Hypertonie bei Lungenerkrankungen und/oder Hypoxie.

ABSTRACT

The pathophysiology of pulmonary hypertension associated with chronic lung disease (PH-CLD) is complex, multifactorial, and not consistent among pulmonary diseases. However, pulmonary vasculopathy triggered by various factors, such as chronic alveolar hypoxia or cigarette smoking, seems to play a central role in the pathogenesis of PH-CLD. While the initial workup of PH-CLD is usually complicated by an overlap of symptoms of PH and the underlying lung disease, PH-CLD should be considered when there is a discrepancy between symptoms (especially exertional dyspnea) and pulmonary function tests. Clinical suspicion of PH-CLD can be strengthened by noninvasive diagnostic tools such as transthoracic echocardiography (TTE) or N-terminal pro-B-type natriuretic peptide (NT-pro-BNP). However, a right heart catheterization should only be performed in specialized centers to establish the diagnosis if therapeutic consequences for the patient were expected.The basic treatment of PH-CLD is optimal management of the underlying lung disease. Among the existing interventional and registry-based studies, only a small number of data suggests favorable outcomes when treating PH-CLD patients with PAH-specific medications. Some publications even suggest negative effects. Nevertheless, recent data on inhaled vasoactive therapy in PH-CLD showed positive results for inhaled Treprostinil, although long-term data for this therapeutic approach are still lacking. Treatment of PH-CLD patients with PAH-specific drugs should only be performed in specialized centers and preferably in the context of clinical trials.