Hamostaseologie 2008; 28(04): 225-230
DOI: 10.1055/s-0037-1617105
Original Article
Schattauer GmbH

Antikoagulation bei pulmonal arterieller Hypertonie

Anticoagulation in pulmonary arterial hypertension
E. Grünig
1   Thoraxklinik Heidelberg
,
N. Ehlken
1   Thoraxklinik Heidelberg
,
Ch. Nagel
2   St. Vincentius Krankenhaus Karlsruhe
› Author Affiliations
Further Information

Publication History

Publication Date:
29 December 2017 (online)

Zusammenfassung

Die pulmonal arterielle Hypertonie (PAH) wird häufig von Thrombose und Thromboembolien vorzugsweise der kleinen Lungenarteriolen begleitet. Diese sind eine Folge der endothelialen Dysfunktion v. a. der kleinen Pulmonalarteriolen, die mit einer prothrombotischen und verminderten antifibrinolytischer Aktivität einhergeht. Auch die Funktion der Blutplättchen ist gestört. Zudem haben die meisten Patienten mit PAH eine Rechtsherzinsuffizienz mit vergrößertem rechten Ventrikel und verlangsamten Blutfluss. Neben diesen pathopysiologischen Befunden begründen vier nicht randomisierte klinische Studien, die eine verbesserte Prognose durch die Therapie mit Marcumar vermuten lassen, die Rationale zur Antikoagulation bei PAH. In diesem Übersichtsartikel sollen die wichtigsten Studien zur Antikoagulation bei PAH sowie die derzeitigen Empfehlungen aus den Leitlinien zusammengefasst werden.

Summary

In pulmonary arterial hypertension (PAH), thrombosis and thromboembolism occurs as a consequence of pulmonary microvasculopathy with a change of pulmonary vascular microenviroment toward a procoagulant, prothrombotic and antifibrinolytic pattern. Circulating antiphospholipid antibodies, increased plasma levels of platelet aggregating agents (serotonin, thromboxane), adhesion molecules (P selectin, von Willebrand factor), antifibrinolytic enzymes (plasminogen activator inhibitor 1) and prothrombotic cytokines have been identified in PAH patients so far. Thrombogenic pulmonary vasculopathy has been documented in many patients with PAH. Furthermore, most patients will not be diagnosed until right heart enlargement and impaired right ventricular function has developed. Thus, there is clear rationale for a treatment with anticoagulation. In four uncontrolled studies Warfarin improved the prognosis of patients with idiopathic and other forms of PAH. However, so far there are no prospective randomised studies evaluating the role of anticoagulants in the treatment of PAH. This review summarizes the current data and guidelines concerning anticoagulation in PAH.

 
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