Therapiealgorithmus der pulmonalarteriellen Hypertonie

Ekkehard Grünig; Katrin Milger; Albert Omlor; Andreea Florea; Benjamin Egenlauf; Dennis Ladage; Dirk Skowasch; David Pittrow; Lars Harbaum; Michael Halank; Oktay Tutarel; Panna K. Csonka; Satenik Harutyunova; Silvia Ulrich; Tobias J. Lange; Thomas Sonnweber; Vera Hess; Nikolaus Kneidinger; Hans Klose

doi:10.1055/a-2625-4905

Pneumologie, Inhaltsverzeichnis

Pneumologie 2025; 79(10): 775-786
DOI: 10.1055/a-2625-4905

Übersicht

Therapiealgorithmus der pulmonalarteriellen Hypertonie

Treatment Algorithm of Pulmonary Arterial Hypertension

Authors

Ekkehard Grünig

¹Zentrum für Pulmonale Hypertonie, Thoraxklinik Heidelberg gGmbH am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
Katrin Milger

²Universitätsklinik für Innere Medizin, Klinische Abteilung für Pneumologie, Lung Research Cluster, Medizinische Universität Graz, Graz, Österreich
Albert Omlor

³Klinik für Innere Medizin V, Universitätsklinikum des Saarlandes, Homburg/Saar, Deutschland
Andreea Florea

¹Zentrum für Pulmonale Hypertonie, Thoraxklinik Heidelberg gGmbH am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
Benjamin Egenlauf

¹Zentrum für Pulmonale Hypertonie, Thoraxklinik Heidelberg gGmbH am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
Dennis Ladage

⁴Klinik für Pneumologie, Kliniken Maria Hilf, Mönchengladbach, Deutschland
Dirk Skowasch

⁵Klinik für Pneumologie, Herzzentrum des Universitätsklinikums Bonn, Bonn, Deutschland
David Pittrow

⁶Institut für Klinische Pharmakologie, Medizinische Fakultät, Technische Universität, Dresden, Deutschland
Lars Harbaum

⁷Abteilung für Pneumologie, II. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland
Michael Halank

⁸Spezialambulanz Pulmonale Hypertonie, Universitätsklinikum Carl Gustav Carus Dresden, Dresden, Deutschland
Oktay Tutarel

⁹Klinik für angeborene Herzfehler, Deutsches Herzzentrum der Charité, Charité – Universitätsmedizin Berlin, Berlin, Deutschland
Panna K. Csonka

¹Zentrum für Pulmonale Hypertonie, Thoraxklinik Heidelberg gGmbH am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
Satenik Harutyunova

¹Zentrum für Pulmonale Hypertonie, Thoraxklinik Heidelberg gGmbH am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
Silvia Ulrich

¹⁰Klinik für Pneumologie, Universitätsspital Zürich, Zürich, Schweiz
Tobias J. Lange

¹¹Abteilung für Pneumologie und Beatmungsmedizin, Kreisklinik Bad Reichenhall, Deutschland und Universität Regensburg, Lehrstuhl Innere Medizin II, Fakultät für Medizin, Regensburg, Deutschland
Thomas Sonnweber

¹²Klinik für Innere Medizin II, Medizinische Universität Innsbruck, innsbruck, Österreich
Vera Hess

¹Zentrum für Pulmonale Hypertonie, Thoraxklinik Heidelberg gGmbH am Universitätsklinikum Heidelberg, Heidelberg, Deutschland
Nikolaus Kneidinger^*

²Universitätsklinik für Innere Medizin, Klinische Abteilung für Pneumologie, Lung Research Cluster, Medizinische Universität Graz, Graz, Österreich

¹³Medizinische Klinik und Poliklinik V, LMU Klinikum, LMU München, Comprehensive Pneumology Center (CPC-M) Deutsches Zentrum für Lungenforschung (DZL), München, Deutschland
Hans Klose^*

⁷Abteilung für Pneumologie, II. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland

Abstract

Zusammenfassung

Die gezielte Therapie der pulmonalarteriellen Hypertonie hat einen positiven Einfluss auf die Symptomatik und Prognose der Erkrankung. Eine gezielte und früh eingesetzte Kombinationstherapie zeigt dabei größere Verbesserungen im Vergleich zu einer Monotherapie. Für Therapieentscheidungen ist die Einschätzung des Mortalitätsrisikos anhand der Risikostratifikation sowie das Vorhandensein von Komorbiditäten essenziell. Ein niedriger Risikostatus ist anzustreben, sichert jedoch nicht immer einen langfristigen klinisch stabilen Verlauf. Zur Kontrolle des Therapieansprechens sollten daher frühzeitige und regelmäßige Reevaluationen erfolgen, bei denen die therapeutischen Konsequenzen im Sinne einer Therapieanpassung eruiert werden.

Neuartige Wirkstoffe und die Erschließung neuer Wirkmechanismen haben die Therapiemöglichkeiten sehr erweitert und verbessert, müssen aber in Langzeitstudien weiter evaluiert werden. Die Definition neuer Therapieziele, z. B. Erreichen einer Remission der PAH, sollten unter den neuen therapeutischen Optionen kritisch diskutiert und durch prospektive Studien weiter evaluiert werden [1].

Abstract

Targeted therapy for pulmonary arterial hypertension has a positive impact on the symptoms and prognosis of the disease. Targeted and early combination therapy demonstrates greater improvements compared to monotherapy. For treatment decisions, assessing the mortality risk based on risk stratification and the presence of comorbidities is essential. A low-risk status is desirable, but does not always guarantee long-term clinically stable disease. Therefore, early and regular reevaluations should be performed to monitor treatment response, determining the therapeutic consequences for treatment adjustment.

Novel agents and the discovery of new mechanisms of action have greatly expanded and improved treatment options, but require further evaluation in long-term studies. The definition of new treatment goals, such as achieving remission of PAH, should be critically discussed among the new therapeutic options and further evaluated through prospective studies [1].

Schlüsselwörter

pulmonale Hypertonie - Therapie - Rechtsherzdysfunktion

Keywords

pulmonary hypertension - therapy - right heart insufficiency

Volltext

Referenzen

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