Therapie der pulmonal arteriellen Hypertonie

Zusammenfassung

Die pulmonale Hypertonie (PH) gliedert sich nach der 5. Weltkonferenz in Nizza in fünf Gruppen. Die pulmonal arterielle Hypertonie (PAH; Gruppe 1) umfasst neben der idiopathischen PAH genetisch und medikamentös bedingte Formen sowie die assoziierte PAH. Bewiesen oder ausgeschlossen werden kann die Diagnose einer PH nur durch die Rechtsherzkatheteruntersuchung. Ohne Diagnosestellung mittels Rechtsherzkatheters sollte keine gezielte Therapie begonnen werden. Neben allgemeinen und supportiven Maßnahmen existieren aktuell vier Gruppen von Medikamenten, die für die gezielte Behandlung der PAH zugelassen sind. Kombinationstherapien, entweder sequenziell oder initial („up-front“), gewinnen an Bedeutung in der Behandlung der PAH. Therapieziele zur Steuerung der Behandlung und Abschätzung des individuellen Patientenrisikos wurden definiert. Trotz aller Innovationen ist ein kurativer Therapieansatz bei der Behandlung der PAH noch in weiter Ferne. Daher stellt nach Ausschöpfung der derzeit verfügbaren medikamentösen und supportiven Maßnahmen die Lungentransplantation eine wichtige Therapieoption dar.

Abstract

Pulmonary hypertension (PH) is classified into five distinct groups according to the fifth world conference in Nice 2013. Pulmonary arterial hypertension (PAH) comprises idiopathic PAH, hereditary PAH, drug-induced and associated PAH. Right heart catheterization is essential for the diagnosis of PH and should precede initiation of a targeted PAH therapy. Besides general measures and supportive therapy, four different classes of targeted drugs have been approved for the treatment of PAH. Combination therapy, either sequential or initial (up-front), is increasingly gaining recognition. Risk stratification and treatment goals have been defined to guide therapeutic decisions. However, cure is still far from reach and lung transplantation is an important treatment option for patients with end-stage disease under optimal supportive and targeted drug therapy.

• Literatur

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