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Pulmonale Hypertonie

Mögliche genetische Ursachen und therapeutische Optionen

Pulmonary hypertension

Possible genetic causes and therapeutic options

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Zusammenfassung

Die pulmonale Hypertonie (PH) ist eine schwere Krankheit, die oft viel zu spät erkannt wird. Sie ist primär durch Luftnot und Rechtsherzinsuffizienz gekennzeichnet. Erbliche Faktoren spielen insbesondere für die seltene pulmonalarterielle Hypertonie (PAH) und die PAH bei angeborenen Herzfehlern eine Rolle. In diesem Artikel werden die allgemeinen und supportiven Therapien für den Lungenhochdruck sowie die gezielte medikamentöse Behandlung der PAH und der nichtoperablen chronisch thromboembolischen PH nach dem aktuellsten Stand dargestellt. Um eine gute Prognose der Patienten zu erreichen, ist eine frühe Überweisung an ein Expertenzentrum zur weiterführenden Diagnostik und Therapie essenziell.

Abstract

Pulmonary hypertension (PH) is a severe disease, which is usually only recognized at a late stage. It is characterized by dyspnea and right heart insufficiency. In some forms of pulmonary hypertension, such as the rare pulmonary arterial hypertension (PAH) and PAH associated with congenital heart defects, genetic factors have been identified. This article summarizes the general and supportive therapies for PH, targeted pharmaceutical treatment for PAH and non-operable chronic thromboembolic pulmonary hypertension. To achieve acceptable survival rates, it is essential to transfer patients to an expert center at an early stage for further differential diagnostics and therapy.

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Die Autoren erklären, dass sie sich bei der Erstellung des Beitrags von keinen wirtschaftlichen Interessen leiten ließen. Die Autoren legen folgende potenzielle Interessenkonflikte offen: EG ist Mitglied eines nationalen und internationalen Advisory Boards von Bayer HealthCare, Novartis, Actelion, Pfizer, GlaxoSmithKline und hat von Bayer HealthCare, Novartis, Actelion, Pfizer, United Therapeutics, AOP Orphan, Gilead, GlaxoSmithKline Honorare für Vorträge bekommen. NE erhielt Vortragshonorare von Actelion, Pfizer und Bayer HealthCare. WHS erhielt Honorare für Beratertätigkeit von Siemens, Terumo, Covidien sowie Vortragshonorare von Actelion, GSK, Novartis, Pfizer, AOP Orphan. Der Verlag erklärt, dass die inhaltliche Qualität des Beitrags von zwei unabhängigen Gutachtern geprüft wurde. Werbung in dieser Zeitschriftenausgabe hat keinen Bezug zur CME-Fortbildung. Der Verlag garantiert, dass die CME-Fortbildung sowie die CME-Fragen frei sind von werblichen Aussagen und keinerlei Produktempfehlungen enthalten. Dies gilt insbesondere für Präparate, die zur Therapie des dargestellten Krankheitsbilds geeignet sind.

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Authors and Affiliations

  1. Zentrum für Pulmonale Hypertonie, Thoraxklinik, Universitätsklinikum Heidelberg, Amalienstr. 5, 69126, Heidelberg, Deutschland

    E. Grünig, C.A. Eichstaedt & N. Ehlken

  2. Sektion Pneumologie, Universitätsklinikum Eppendorf, Hamburg, Deutschland

    E. Mayer

  3. Abteilung für Thoraxchirurgie, Kerckhoff-Klinik, Bad Nauheim, Deutschland

    H. Klose

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  1. E. Grünig
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  2. C.A. Eichstaedt
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  3. N. Ehlken
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  4. E. Mayer
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  5. H. Klose
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Correspondence to E. Grünig.

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Grünig, E., Eichstaedt, C., Ehlken, N. et al. Pulmonale Hypertonie. Herz 40, 457–470 (2015). https://doi.org/10.1007/s00059-015-4230-6

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