Kernaussagen
Die pulmonale Hypertonie wurde - basierend auf pathophysiologischen Prozessen an den
Lungengefäßen, die allen Formen gemeinsam sind - durch eine WHO-Konferenz 2003 neu
klassifiziert.
Die Diagnosestellung basiert auf den molekularen Mechanismen, die der Pathophysiologie
der IPAH sowie den sekundären pulmonalen Hypertonien bei angeborenen Herzfehlern und
bei alveolärer Hypoxämie zugrunde liegen. Hinweise auf eine PAH gibt die Anamnese.
Die Echokardiographie erlaubt die Diagnose und in vielen Fällen auch die Klärung der
Ursache. Die invasive Untersuchung des Lungengefäßbetts ist die Basis für die Therapiestrategie.
Die Therapie richtet sich nach dem klinischen Bild (Funktionsstadien). Eine vasodilatative
Therapie ist bei Kindern bei bis zu 20 - 40 % der Fälle, bei Erwachsenen jedoch nur
bei 10 % möglich. Mehr als 60 % der Patieneten bedürfen einer antiproliferativen Behandlung.
Behandlungserfolge sind eine Verlangsamung des fortschreitenden Krankheitsprozesses
mit einer Verbesserung der Leistungsfähigkeit. Neue antiproliferative Behandlungskonzepte
sind in Aussicht. Die Lungentransplantation ist noch keine Langzeitlösung.
Die Fortschritte im Gesamtmanagement der Erkrankung erfordern eine ständige Aktualisierung
aller Empfehlungen. Aber schon jetzt gibt es feststehende Abläufe und Prioritäten
zu Diagnostik und Therapie. Ziel ist es, die Diagnose „pulmonale Hypertonie” zu sichern,
sie entsprechend der neuen Klassifikation zuzuordnen, die geeignetste der möglichen
Therapieformen zu suchen und die Prognose einzuschätzen.
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Prof. Dr. med. Dietmar Schranz
Kinderherzzentrum
Justus-Liebig-Universität · Feulgenstr. 12 · 35392 Gießen
Fax: 0641/99-43461
Email: Dietmar.Schranz@paediat.med.uni-giessen.de
