Zusammenfassung
Die pulmonal-arterielle Hypertonie (PAH) betrifft häufig junge Patienten, für die eine lebenslange Antikoagulation eine einschneidende Entscheidung darstellen kann. Daher ist es wichtig, die zum Teil widersprüchlichen klinischen und wissenschaftlichen Befunde zu dieser Therapie zu analysieren. PAH wird häufig aufgrund der endothelialen Dysfunktion, vor allem der kleinen Pulmonalarteriolen mit prothrombotischer und verminderter antifibrinolytischer Aktivität, von Thrombose und Thromboembolien der Lungenstrombahn begleitet. Messbare Veränderungen von Gerinnungsfaktoren sind von prognostischer Bedeutung. Zudem ist die Funktion der Thrombozyten gestört. Die meisten Patienten mit PAH haben darüber hinaus eine Rechtsherzinsuffizienz mit vergrößertem rechtem Ventrikel und verlangsamtem Blutfluss. Neben diesen pathophysiologischen Befunden begründen 4 nichtrandomisierte klinische Studien, die eine verbesserte Prognose durch Phenprocoumontherapie vermuten lassen, die Rationale zur Antikoagulation bei PAH und die Empfehlungen der nationalen und internationalen Leitlinien. Klinische Studien zu Alternativen wie der Gabe von Acetylsalicylsäure stehen aus. Es sollten alle Anstrengungen unternommen werden, die Erkrankung früher zu diagnostizieren, um durch eine frühe Therapie u. a. die Veränderung der Gerinnung und Thrombozytenfunktion rasch korrigieren zu können.
Abstract
Pulmonary arterial hypertension (PAH) often affects young patients for whom life-long anticoagulation can represent an incisive decision. Therefore, it is important to analyze the sometimes conflicting clinical and scientific results for this therapy. PAH is often accompanied by thrombosis and thrombo-embolisms in the pulmonary circulation, in particular the small pulmonary arterioles with prothrombotic and reduced antifibrinolytic activity due to endothelial dysfunction. Measurable alterations of coagulation factors are of prognostic significance. Thrombocyte function is also disrupted. Furthermore, most patients with PAH have a right-sided cardiac insufficiency with an enlarged right ventricle and reduced circulation. In addition to these pathophysiologic findings four non-randomized studies, which allow the assumption of improved prognosis by phenprocoumon therapy, have confirmed the rational of anticoagulation for PAH and the recommendations of national and international guidelines. Clinical studies on alternatives, such as the administration of acetylsalicylic acid have not yet been carried out. Efforts should be made to diagnose the disease as early as possible in order to correct alterations in coagulation and thrombocyte function by an early therapy.
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Grünig, E. Antikoagulation bei pulmonaler arterieller Hypertonie. Pneumologe 6, 390–398 (2009). https://doi.org/10.1007/s10405-009-0322-4
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DOI: https://doi.org/10.1007/s10405-009-0322-4