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Medikamentöse Therapie der pulmonal arteriellen Hypertonie

Pharmacotherapy of pulmonary arterial hypertension

  • Leitthema
  • Published:
Der Pneumologe Aims and scope

Zusammenfassung

Die medikamentöse Therapie der pulmonal arteriellen Hypertonie (PAH) hat in den vergangenen Jahren eine bemerkenswerte Entwicklung durchlaufen. Neben der „supportiven Therapie“, die Antikoagulation, Diuretika, Sauerstoff, Antiarrhythmika und ggf. Antidepressiva einschließt, ist die sog. gezielte medikamentöse Therapie von größter Bedeutung. Hierfür liegt Evidenz aus größeren kontrollierten Studien vor. Die Therapie sollte sich in streng strukturierter Form an definierten Therapiezielen orientieren. Die regelmäßige Beurteilung des Ansprechens und der Verträglichkeit sind erforderlich. Werden die Therapieziele nicht erreicht, kommt einer Kombinationstherapie und der Lungentransplantation eine große Bedeutung zu. Eine wachsende Anzahl an Substanzen ist für die gezielte PAH-Therapie zugelassen und neu gewonnene Erkenntnisse verbessern den Einsatz von supportiven Therapien. Die gezielten Medikamente umfassen Kalziumkanalantagonisten (hochdosiert), Endothelinrezeptorantagonisten, Phosphodiesterase-5-Inhibitoren, Guanylatzyklasestimulatoren und Prostanoide. Sie bewirken eine Verbesserung der Symptomatik und Leistungsfähigkeit sowie eine Verzögerung des Eintritts einer klinischen Verschlechterung.

Abstract

The pharmaceutical treatment of pulmonary arterial hypertension (PAH) has been the subject of continuous development over the last years. A growing number of targeted therapies are available as well as new evidence for the implementation of supportive therapy. Evidence for the use of targeted therapies comes from larger controlled trials. Treatment should be guided in a structured manner by predefined treatment goals. A repeated evaluation of the treatment response and tolerability is mandatory. In the case of an unsatisfactory response the treatment algorithm recommends combination therapy and if necessary lung transplantation. The targeted therapies consist of calcium channel blockers, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, guanylate cyclase stimulators and prostanoids. Supportive therapy comprises optimization and modulation of anticoagulation, diuretics, antiarrhythmic therapy and antidepressive treatment. The current treatment results in a reduction of morbidity and improvement of event-free survival.

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Abb. 1

Abbreviations

6MWD:

6-Minuten-Gehstrecke

APAH:

Assoziierte pulmonal arterielle Hypertonie

CCB:

Kalziumkanalblocker

cAMP:

Zyklisches Adenosinmonophosphat

cGMP:

Zyklisches Guanosinmonophosphat

eNOS:

Endotheliale Stickstoffmonoxidsynthase

EMA:

European Medicines Agency

ETA:

Endothelinrezeptor Typ A

ETB:

Endothelinrezeptor Typ B

ESC:

European Society of Cardiology

ERA:

Endothelin Rezeptor Antagonist

ERS:

European Respiratory Society

HPAH:

Hereditäre pulmonal arterielle Hypertonie

IPAH:

Idiopathische pulmonal arterielle Hypertonie

NT-proBNP:

N-terminal prohormone of brain natriuretic peptide

NO:

Stickstoffmonoxid

PAH:

Pulmonal arterielle Hypertonie

PDE:

Phosphodiesterase

PGI:

Prostazyklin/Prostaglandin I2

PH:

Pulmonale Hypertonie

sGC:

Lösliche Guanylatzyklase

WHO-FC:

WHO-Funktionsklasse

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Einhaltung ethischer Richtlinien

Interessenkonflikt. L. Harbaum gibt an, dass Reisekosten durch Actelion Pharmaceuticals Deutschland GmbH, Bayer Vital GmbH und GlaxoSmithKline GmbH & Co. KG übernommen worden sind. H. Olschewski gibt an, dass er Honorare für Vorträge, Beratertätigkeiten oder Forschungsunterstützung von Actelion, Bayer, Gilead, GSK, Lilly, Novartis und Pfizer erhalten hat. H. Klose gibt an, dass er Honorare für Vorträge und Beratertätigkeiten von Actelion Pharmaceuticals Deutschland GmbH, Bayer Vital GmbH und GlaxoSmithKline GmbH & Co. KG und Pfizer Deutschland GmbH erhalten hat. Zusätzlich hat er von Actelion Pharmaceuticals Deutschland GmbH und GlaxoSmithKline GmbH & Co. KG finanzielle Forschungsunterstützung erhalten.

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Authors and Affiliations

  1. Zentrum für Pulmonal Arterielle Hypertonie, Martin Zeitz Zentrum für Seltene Erkrankungen, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland

    L. Harbaum &  H. Klose

  2. Sektion Pneumologie, II. Medizinische Klinik und Poliklinik, Onkologisches Zentrum, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Deutschland

    L. Harbaum &  H. Klose

  3. Klinische Abteilung für Pulmonologie, Universitätsklinik für Innere Medizin, LKH Universitätsklinikum Graz, Graz, Österreich

    H. Olschewski

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  1. L. Harbaum
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Correspondence to H. Klose.

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Harbaum, L., Olschewski, H. & Klose, H. Medikamentöse Therapie der pulmonal arteriellen Hypertonie. Pneumologe 12, 390–400 (2015). https://doi.org/10.1007/s10405-015-0881-5

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