Juvenile Pulmonary Hypertension Associated with Fibromuscular Dysplasia

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Author(s)

    • Yano Toshiaki
    • Department of Respirology, Graduate School of Medicine, Chiba University
    • Tanabe Nobuhiro
    • Department of Respirology, Graduate School of Medicine, Chiba University
    • Kitazono Miyako
    • Department of Respirology, Graduate School of Medicine, Chiba University
    • Yamauchi Keita
    • Department of Respirology, Graduate School of Medicine, Chiba University
    • Sakao Seiichiro
    • Department of Respirology, Graduate School of Medicine, Chiba University
    • Takiguchi Yuichi
    • Department of Respirology, Graduate School of Medicine, Chiba University
    • Tatsumi Koichiro
    • Department of Respirology, Graduate School of Medicine, Chiba University

Abstract

A 20-year-old female diagnosed as idiopathic pulmonary arterial hypertension at 7 years of age was referred with worsening dyspnea and chest pain. Several imaging studies and right cardiac catheterization showed multiple stenoses in the peripheral pulmonary arteries with severe pulmonary hypertension and multiple systemic arterial stenoses lacking in systemic hypertension. No evidence of inflammatory or autoimmune disease was detected. Fibromuscular dysplasia was clinically diagnosed because of the narrowed systemic and pulmonary arterial stenoses which included dilatation and aneurysms that appeared similar to a string of beads. Treatment with sildenafil yielded a temporary improvement in her disease state.<br>

Journal

  • Internal Medicine

    Internal Medicine 49(22), 2487-2492, 2010

    The Japanese Society of Internal Medicine

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