{"title":"Isolated unilateral absence of the pulmonary artery (IUAPA) presenting with progressive interstitial shadows: A case report.","authors":"Tomohito Takeshige, Ryo Koyama, Makiko Kohmaru, Takashi Akimoto, Junko Watanabe, Toshifumi Yae, Kenji Kido, Kazuhisa Takahashi","doi":"10.1002/rcr2.70048","DOIUrl":null,"url":null,"abstract":"<p><p>Unilateral absence of the pulmonary artery (UAPA) is a rare congenital condition. When UAPA occurs without associated congenital heart disease, it is referred to as isolated unilateral absence of the pulmonary artery (IUAPA). IUAPA is frequently not diagnosed until adulthood. A 61-year-old female patient presented with right chest pain and a worsening interstitial shadow over 9 years. Contrast-enhanced computed tomography revealed the absence of blood flow from the pulmonary artery in the right lung, leading to the diagnosis of IUAPA. In this case, the diagnosis of UAPA was established after approximately 40 years. This case underscores the importance of identifying vascular abnormalities to differentiate this condition in patients presenting with nonspecific symptoms and interstitial shadows.</p>","PeriodicalId":45846,"journal":{"name":"Respirology Case Reports","volume":null,"pages":null},"PeriodicalIF":0.8000,"publicationDate":"2024-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11480744/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Respirology Case Reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1002/rcr2.70048","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/10/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}
引用次数: 0
Abstract
Unilateral absence of the pulmonary artery (UAPA) is a rare congenital condition. When UAPA occurs without associated congenital heart disease, it is referred to as isolated unilateral absence of the pulmonary artery (IUAPA). IUAPA is frequently not diagnosed until adulthood. A 61-year-old female patient presented with right chest pain and a worsening interstitial shadow over 9 years. Contrast-enhanced computed tomography revealed the absence of blood flow from the pulmonary artery in the right lung, leading to the diagnosis of IUAPA. In this case, the diagnosis of UAPA was established after approximately 40 years. This case underscores the importance of identifying vascular abnormalities to differentiate this condition in patients presenting with nonspecific symptoms and interstitial shadows.
期刊介绍：
Respirology Case Reports is an open-access online journal dedicated to the publication of original clinical case reports, case series, clinical images and clinical videos in all fields of respiratory medicine. The Journal encourages the international exchange between clinicians and researchers of experiences in diagnosing and treating uncommon diseases or diseases with unusual presentations. All manuscripts are peer-reviewed through a streamlined process that aims at providing a rapid turnaround time from submission to publication.
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