Management der pulmonalarteriellen Hypertonie
摘要
Pulmonary arterial hypertension (PAH) is a rare and progressive disease associated with high morbidity and mortality. Modern PAH therapy aims to achieve a low-risk profile and currently includes combination regimens with agents targeting distinct pathophysiological signaling pathways. Recently, new therapies, such as sotatercept have expanded the therapeutic landscape. This article provides an overview of the current treatment algorithm, emphasizing the importance of structured risk stratification, early treatment re-evaluation and timely escalation. It also addresses the management of specific clinical situations, including pregnancy, perioperative care, comorbidities, medication adherence and palliative support. Close follow-up in specialized centers is essential to ensure the success of treatment.