Congenital unilateral pericardial agenesis presenting as an isolated chest pain in an adolescent: a case report and comprehensive review.

IF 1.5 4区 医学 Q3 CARDIAC & CARDIOVASCULAR SYSTEMS
Farshad Jafari, Maryam Taheri, Pouya Ebrahimi, Maedeh Soflaee, Reyhaneh Alipore Rafie, Mohsen Anafje
{"title":"Congenital unilateral pericardial agenesis presenting as an isolated chest pain in an adolescent: a case report and comprehensive review.","authors":"Farshad Jafari, Maryam Taheri, Pouya Ebrahimi, Maedeh Soflaee, Reyhaneh Alipore Rafie, Mohsen Anafje","doi":"10.1186/s13019-025-03364-3","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Congenital pericardial agenesis is a rare congenital anomaly resulting from the incomplete development of the pleuropericardial membranes during embryogenesis, leading to the partial or complete absence of the pericardial sac. Although this condition usually remains asymptomatic, it can present with various misleading symptoms such as chest pain (add some other, maybe 2, more prevalent presenting symptoms), making diagnosis challenging. Advanced imaging techniques are crucial for accurate diagnosis and management, especially when usual diagnostic modalities do not achieve a definite diagnosis.</p><p><strong>Case presentation: </strong>The history and diagnostic process of a 16-year-old female who presented with isolated, non-exertional chest pain are detailed. A comprehensive diagnostic work-up was initiated, including chest X-ray (CXR), transthoracic echocardiogram (TTE), CT angiography (CTA), and cardiac magnetic resonance imaging (CMRI). These advanced imaging modalities unveiled the rare and elusive diagnosis of left-sided pericardial agenesis, decisively ruling out other potential causes and shedding light on an extraordinary case that challenges conventional diagnostic pathways.</p><p><strong>Conclusion: </strong>Unilateral pericardial agenesis, though typically benign and often shrouded in clinical obscurity, can manifest with enigmatic symptoms such as isolated chest pain, necessitating a meticulous and comprehensive diagnostic approach. Multimodal imaging is essential for accurate diagnosis and for ruling out complications such as cardiac herniation or coronary artery compression. Considering the absence of significant complications, conservative management was chosen in this case, with the patient being discharged with instructions to monitor for any warning signs.</p><p><strong>Clinical key message: </strong>Clinicians should consider congenital pericardial agenesis as one of the potential causes of unexplained chest pain, particularly when the initial investigations are inconclusive. Advanced imaging techniques (such as CXR and MRI) are vital for confirming the diagnosis and subsequently appropriate and timely management and preventing potential complications.</p>","PeriodicalId":15201,"journal":{"name":"Journal of Cardiothoracic Surgery","volume":"20 1","pages":"127"},"PeriodicalIF":1.5000,"publicationDate":"2025-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11829426/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Cardiothoracic Surgery","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1186/s13019-025-03364-3","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
引用次数: 0

Abstract

Introduction: Congenital pericardial agenesis is a rare congenital anomaly resulting from the incomplete development of the pleuropericardial membranes during embryogenesis, leading to the partial or complete absence of the pericardial sac. Although this condition usually remains asymptomatic, it can present with various misleading symptoms such as chest pain (add some other, maybe 2, more prevalent presenting symptoms), making diagnosis challenging. Advanced imaging techniques are crucial for accurate diagnosis and management, especially when usual diagnostic modalities do not achieve a definite diagnosis.

Case presentation: The history and diagnostic process of a 16-year-old female who presented with isolated, non-exertional chest pain are detailed. A comprehensive diagnostic work-up was initiated, including chest X-ray (CXR), transthoracic echocardiogram (TTE), CT angiography (CTA), and cardiac magnetic resonance imaging (CMRI). These advanced imaging modalities unveiled the rare and elusive diagnosis of left-sided pericardial agenesis, decisively ruling out other potential causes and shedding light on an extraordinary case that challenges conventional diagnostic pathways.

Conclusion: Unilateral pericardial agenesis, though typically benign and often shrouded in clinical obscurity, can manifest with enigmatic symptoms such as isolated chest pain, necessitating a meticulous and comprehensive diagnostic approach. Multimodal imaging is essential for accurate diagnosis and for ruling out complications such as cardiac herniation or coronary artery compression. Considering the absence of significant complications, conservative management was chosen in this case, with the patient being discharged with instructions to monitor for any warning signs.

Clinical key message: Clinicians should consider congenital pericardial agenesis as one of the potential causes of unexplained chest pain, particularly when the initial investigations are inconclusive. Advanced imaging techniques (such as CXR and MRI) are vital for confirming the diagnosis and subsequently appropriate and timely management and preventing potential complications.

求助全文
约1分钟内获得全文 求助全文
来源期刊
Journal of Cardiothoracic Surgery
Journal of Cardiothoracic Surgery 医学-心血管系统
CiteScore
2.50
自引率
6.20%
发文量
286
审稿时长
4-8 weeks
期刊介绍: Journal of Cardiothoracic Surgery is an open access journal that encompasses all aspects of research in the field of Cardiology, and Cardiothoracic and Vascular Surgery. The journal publishes original scientific research documenting clinical and experimental advances in cardiac, vascular and thoracic surgery, and related fields. Topics of interest include surgical techniques, survival rates, surgical complications and their outcomes; along with basic sciences, pediatric conditions, transplantations and clinical trials. Journal of Cardiothoracic Surgery is of interest to cardiothoracic and vascular surgeons, cardiothoracic anaesthesiologists, cardiologists, chest physicians, and allied health professionals.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信