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肺動脈高血圧における骨型遺伝子タンパク質受容体2型変異:右心室の見方

Cathelijne E van der Bruggen1, Chris M Happé1, Peter Dorfmüller1

  • 1From Department of Pulmonology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands (C.E.E.V.D.B., C.M.H., P.T., O.A.S., N.R., A.V.N., H.J.B., F.S.d.M.); Department of Physiology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands (C.M.H., N.R., F.P.H., J.V.D.V., F.S.d.M.); Univ. Paris-Sud, Le Kremlin-Bicêtre, France (P.D., B.G., .M.H.); AP-HP, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital Bicêtre, Le Kremlin-Bicêtre, France (P.D., B.G., M.H.); INSERM999, LabEx LERMIT, Centre Chirurgical Marie Lannelongue, Le Plessis Robinson, France (P.D., B.G., M.H.); Department of Clinical Genetics, VU University Medical Center, Amsterdam, The Netherlands (A.C.H.); Department of Physics and Medical Technology, VU University Medical Center, Amsterdam, The Netherlands (J.T.M.); Department of Thoracic and Vascular Surgery and Heart-Lung Transplantation, Hôpital Marie-Lannelongue, Le Plessis Robinson, Paris-Sud University, France (O.M.); Department of Cardiology, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands (M.L.H.); and Department of Molecular Cell Biology, Laboratory of Experimental Cardiology, Leiden University Medical Center, Leiden, The Netherlands (M.-J.G.).

Circulation
|March 18, 2016
PubMed
まとめ

BMPR2遺伝子変異を有する肺動脈高血圧の患者は,同様の肺動脈圧にもかかわらず,右心房機能が悪化します. この研究では,肺動脈高血圧における右心房機能に対するBMPR2変異の影響を調査した.

キーワード:
遺伝学心不全肺高血圧

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