Therapie der pulmonal arteriellen Hypertonie

Zusammenfassung

Die Betreuung von Patienten mit pulmonal arterieller Hypertonie (PAH) basiert auf einer komplexen Strategie, welche neben dem Einsatz gezielter PAH-Medikamente auch Allgemeinmaßnahmen und supportive Therapien einschließt. Gezielte PAH-Therapien umfassen Endothelin-Rezeptor-Antagonisten, Phosphodiesterase 5-Inhibitoren, Stimulatoren der löslichen Guanylatzyklase, Prostazyklin-Analoga und Prostazyklin-Rezeptor-Agonisten, die als Mono- oder Kombinationstherapien eingesetzt werden. Vor Einleitung einer gezielten PAH-Therapie sollten die Patienten zur Diagnosesicherung und Klassifikation in einem Expertenzentrum vorgestellt werden. Therapieentscheidungen basieren auf der initialen Ermittlung der Krankheitsschwere und einer individuellen Risikostratifizierung, dem Vorhandensein von Komorbiditäten und Risikofaktoren, dem Therapieansprechen und dem Krankheitsverlauf. Die aktuellen ESC/ERS-Leitlinien und die Empfehlungen der Kölner Konsensus-Konferenz betonen den Stellenwert von Kombinationstherapien, wobei je nach Vorhandensein kardialer und/oder pulmonaler Begleiterkrankungen zwischen „typischer" und „atypischer" PAH unterschieden werden sollte. Allgemeinmaßnahmen und supportive Therapien haben zum Teil erheblichen Einfluss auf die Morbidität und Mortalität und sind daher integraler Bestandteil der Behandlung und Versorgung von PAH-Patienten.

• Literatur

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