{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,7]],"date-time":"2025-10-07T01:13:35Z","timestamp":1759799615998,"version":"build-2065373602"},"reference-count":54,"publisher":"Georg Thieme Verlag KG","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pneumologie"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Die pulmonale Hypertonie (PH) stellt einen h\u00e4modynamisch definierten pathologischen Zustand dar, welcher durch einen mittleren pulmonalarteriellen Druck (mPAP) &gt;\u200a20\u200ammHg  charakterisiert ist. Die differenzialdiagnostische Zuordnung erfolgt anhand des pulmonalarteriellen Wedge-Drucks (PAWP) und des pulmonalen Gef\u00e4\u00dfwiderstands (PVR): pr\u00e4kapill\u00e4re PH: PAWP \u2264\u200a15\u200ammHg bei gleichzeitig erh\u00f6htem PVR &gt;\u200a2 Wood Units (WU), isolierte postkapill\u00e4re PH: PAWP &gt;\u200a15\u200ammHg, PVR \u2264\u200a2\u200aWU und kombinierte post- und pr\u00e4kapill\u00e4re PH: PAWP &gt;\u200a15\u200ammHg und PVR &gt;\u200a2\u200aWU.<\/jats:p><jats:p>Die belastungsinduzierte PH ist definiert als normwertiger mPAP in Ruhe, jedoch pathologischer Drucksteigerung unter k\u00f6rperlicher Belastung, definiert durch eine Steigung des mPAP relativ zum Herzzeitvolumen (HZV) von &gt;\u200a3\u200ammHg\/L\/min.<\/jats:p><jats:p>Die etablierte klinische Klassifikation der PH bleibt in ihrer Grundstruktur mit 5 Hauptgruppen erhalten. Modifikationen beinhalten: Re-Etablierung der Subgruppe der Langzeitresponder auf Kalziumkanalblocker innerhalb der idiopathischen pulmonalarteriellen Hypertonie (IPAH), Erweiterung der Gruppe 2 durch zus\u00e4tzliche Subkategorien bei PH infolge Linksherzerkrankungen und verfeinerte Untergliederung der Gruppe 3 auf Basis spezifischer pulmonaler Grunderkrankungen statt rein funktioneller Einschr\u00e4nkungen. Die Wirkstoffe Mitomycin-C und Carfilzomib wurden neu in die Liste der Pharmaka mit gesicherter Assoziation zur Entwicklung einer PAH aufgenommen.<\/jats:p>","DOI":"10.1055\/a-2625-4769","type":"journal-article","created":{"date-parts":[[2025,10,6]],"date-time":"2025-10-06T22:42:36Z","timestamp":1759790556000},"page":"723-731","source":"Crossref","is-referenced-by-count":0,"title":["Definition und Klassifikation der pulmonalen Hypertonie"],"prefix":"10.1055","volume":"79","author":[{"given":"Philipp","family":"Douschan","sequence":"additional","affiliation":[{"name":"Abteilung f\u00fcr Pulmonologie, Lung Research Cluster, Medizinische Universit\u00e4t Graz, Graz, \u00d6sterreich"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ann-Sophie","family":"Kaemmerer-Suleiman","sequence":"additional","affiliation":[{"name":"Herzchirurgische Klinik, Universit\u00e4tsklinikum Erlangen, Erlangen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Benjamin","family":"Egenlauf","sequence":"additional","affiliation":[{"name":"Zentrum f\u00fcr pulmonale Hypertonie, Thoraxklinik am Universit\u00e4tsklinikum Heidelberg, Heidelberg, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hans-J\u00fcrgen","family":"Seyfarth","sequence":"additional","affiliation":[{"name":"Abteilung Pneumologie, Medizinische Klinik und Poliklinik f\u00fcr Onkologie, Gastroenterologie, Hepatologie und Pneumologie der Universit\u00e4t Leipzig, Leipzig, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katarina","family":"Zeder","sequence":"additional","affiliation":[{"name":"Abteilung f\u00fcr Pulmonologie, Lung Research Cluster, Medizinische Universit\u00e4t Graz, Graz, \u00d6sterreich"},{"name":"University of Maryland, Institute for Health Computing, Bethesda, MD, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laura","family":"Mayer","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Pneumologie, Universit\u00e4tsspital Z\u00fcrich, Z\u00fcrich, Schweiz"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Max","family":"Wissm\u00fcller","sequence":"additional","affiliation":[{"name":"Klinik III f\u00fcr Innere Medizin \u2013 Allgemeine und interventionelle Kardiologie, Elektrophysiologie, Angiologie, Pneumologie und internistische Intensivmedizin, Uniklinik K\u00f6ln, K\u00f6ln, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Melanie","family":"Heberling","sequence":"additional","affiliation":[{"name":"Medizinische Klinik I \u2013 Pneumologie, Universit\u00e4tsklinikum Dresden, Dresden, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Huntgeburth","sequence":"additional","affiliation":[{"name":"Internationales Zentrum f\u00fcr Erwachsene mit angeborenen Herzfehlern (EMAH), M\u00fcnchen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michaela","family":"Barnikel","sequence":"additional","affiliation":[{"name":"LMU Klinikum M\u00fcnchen, Medizinische Klinik und Poliklinik V, M\u00fcnchen, Deutschland. 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