• Chest · Feb 2022

    Case Reports

    Two Siblings With Peripheral Pulmonary Arterial Stenosis: Pulmonary Angiography of Advanced and Early Stages.

    • Kohsaku Goto, Shun Minatsuki, Kanna Fujita, Norifumi Takeda, Masaru Hatano, and Issei Komuro.
    • Department of Cardiovascular Medicine, Graduate School of Medicine, Tokyo, Japan. Electronic address: kosh1107raku@gmail.com.
    • Chest. 2022 Feb 1; 161 (2): e75-e80.

    AbstractPeripheral pulmonary arterial stenosis (PPAS) is known to cause pulmonary hypertension (PH). Although adult patients at advanced stage have been increasingly reported, there are few reports on clinical characteristics and pulmonary angiography (PAG) findings of early stage PPAS. We present two Japanese siblings with PPAS with homozygosity of RNF213 p.Arg4810Lys-one with advanced stage and the other with early stage. The latter case was an asymptomatic 37-year-old woman with mild PH. Notably, her PAG demonstrated nonthrombotic stenosis in the subsegmental branches of the pulmonary arteries with varying degrees of stenosis among lung segments. Taken together with a family history, genetic analysis, and cerebral angiography, the obtained images were considered as showing PPAS with early stage. This result is clinically informative to diagnose PPAS at an early stage and is also important to understand the pathogenesis of PPAS.Copyright © 2021 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

      Pubmed     Full text   Copy Citation     Plaintext  

      Add institutional full text...

    Notes

     
    Knowledge, pearl, summary or comment to share?
    300 characters remaining
    help        
    You can also include formatting, links, images and footnotes in your notes
    • Simple formatting can be added to notes, such as *italics*, _underline_ or **bold**.
    • Superscript can be denoted by <sup>text</sup> and subscript <sub>text</sub>.
    • Numbered or bulleted lists can be created using either numbered lines 1. 2. 3., hyphens - or asterisks *.
    • Links can be included with: [my link to pubmed](http://pubmed.com)
    • Images can be included with: ![alt text](https://bestmedicaljournal.com/study_graph.jpg "Image Title Text")
    • For footnotes use [^1](This is a footnote.) inline.
    • Or use an inline reference [^1] to refer to a longer footnote elseweher in the document [^1]: This is a long footnote..

    hide…