{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,8]],"date-time":"2025-09-08T06:13:48Z","timestamp":1757312028743},"reference-count":31,"publisher":"Georg Thieme Verlag KG","issue":"05","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pneumologie"],"published-print":{"date-parts":[[2022,5]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>\n          Einleitung\u2002Die Polycythaemia vera (PV) ist eine seltene Ursache einer Pulmonalen Hypertonie (PH) Klasse 5 nach Nizza-2018. Anhand von Kasuistiken werden die unterschiedlichen Verl\u00e4ufe, zum einen mit prim\u00e4rer Manifestation einer PH und nachfolgend einer PV, zum anderen mit Entwicklung einer PH im Rahmen einer PV, dargestellt.<\/jats:p><jats:p>\n          Kasuistiken\u20021) Bei Erstkontakt 75-j\u00e4hrige Patientin mit progredienter Dyspnoe und Nachweis einer Belastungs-PH im RHK. Im Verlauf Entwicklung einer Ruhe-PH bis 70\u200ammHg systolisch trotz initialer Mono- und nachfolgend dualer Therapie der PH. Nach 5 Jahren Diagnose einer Polycythaemia vera und Einleitung einer Hydroxycarbamid-Therapie und nachfolgend auch Aderl\u00e4sse. Im weiteren Verlauf zunehmende kardiale Dekompensation und Versterben. 2) 74-j\u00e4hrige Patientin bei Diagnosestellung einer chronisch megakaryozyt\u00e4ren-granulozyt\u00e4ren Myelose. Nach 7 Jahren Nachweis einer Polycythaemia vera (V617F-Mutation im JAK2-Gen) und einer monoklonalen Gammopathie. Bei Splenomegalie Milzbestrahlung und nach 1 Jahr Therapie mit Ruxolitinib. 2 Jahre sp\u00e4ter, bei zunehmender Dyspnoe, Nachweis einer pulmonalen Hypertonie (CTEPH) mit einem PA-mean von 43\u200ammHg und PVR von 4,5 WE. Unter Antikoagulation und Riociguattherapie nur passager Besserung der Belastbarkeit und der PA-Drucke. Innerhalb eines Jahres Entwicklung einer zunehmend restriktiven Ventilationsst\u00f6rung mit Hypox\u00e4mie und einer Herzinsuffizienz (EF 45\u200a%), mit Rechtsherzdekompensation und kardio-renalem Syndrom. Unter Dialyse nur kurzfristige Rekompensation, sodass die Patientin verstarb.<\/jats:p><jats:p>\n          Diskussion\u2002Die Kasuistiken zeichnen sich durch eine Kombination aus PV und PH, bei unterschiedlicher zeitlicher Sequenz, sowie eine nur geringe passagere Beeinflussbarkeit durch eine PH-spezifische Therapie, mit nachfolgend progredienter kardialer Dekompensation aus. Sie spiegeln somit die verschiedenen \u00c4tiologien, klinischen Manifestationsformen und die geringe therapeutische Beeinflussbarkeit einer PH bei myeloproliferativen Erkrankungen wider. Unklar ist weiterhin der Stellenwert einer PH-spezifischen Therapie insbesondere in Anbetracht unterschiedlicher Pathomechanismen in der Genese der PH.<\/jats:p><jats:p>\n          Schlussfolgerung\u2002Patienten mit myeloproliferativen Erkrankungen bed\u00fcrfen eines Screenings auf eine PH. Auch im Verlauf einer PH kann sich eine myeloproliferative Erkrankung demaskieren oder entwickeln. Die therapeutische Beeinflussbarkeit der PH ist eingeschr\u00e4nkt.<\/jats:p>","DOI":"10.1055\/a-1775-6424","type":"journal-article","created":{"date-parts":[[2022,4,5]],"date-time":"2022-04-05T22:54:14Z","timestamp":1649199254000},"page":"345-353","source":"Crossref","is-referenced-by-count":2,"title":["Pulmonale Hypertonie und Polycythaemia vera"],"prefix":"10.1055","volume":"76","author":[{"given":"Michael","family":"Westhoff","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Pneumologie, Schlaf- und Beatmungsmedizin, Lungenklinik Hemer, Zentrum f\u00fcr Pneumologie und Thoraxchirurgie, Hemer"},{"name":"Universit\u00e4t Witten\/Herdecke University, Witten"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patric","family":"Litterst","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Pneumologie, Schlaf- und Beatmungsmedizin, Lungenklinik Hemer, Zentrum f\u00fcr Pneumologie und Thoraxchirurgie, 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