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揭示弯刀综合征的一个独特病例:巴基斯坦的临床意义及多学科管理挑战

Unveiling a Unique Case of Scimitar Syndrome: Clinical Significance and Multidisciplinary Management Challenges in Pakistan.

作者信息

Shamal Gulalay, Khan Anam Israr, Ali Ahsan, James Neha, Ghulam Moula

机构信息

Internal Medicine, Rehman Medical Institute, Peshawar, PAK.

General Medicine, Rehman Medical Institute, Peshawar, PAK.

出版信息

Cureus. 2024 Feb 8;16(2):e53874. doi: 10.7759/cureus.53874. eCollection 2024 Feb.

DOI:10.7759/cureus.53874
PMID:38465183
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10925003/
Abstract

Scimitar syndrome, a rare congenital cardiac anomaly, involves abnormal pulmonary vein drainage into systemic veins, leading to distinct imaging features resembling a curved-blade sword. This case report presents a unique instance of scimitar syndrome in Pakistan, emphasizing its clinical importance and the challenges of management. A 26-year-old female with a history of recurrent pulmonary infections and respiratory symptoms since childhood was diagnosed with scimitar syndrome. Radiological assessments, including chest X-rays, computed tomography pulmonary angiograms (CTPA), and transthoracic echocardiography, confirmed the presence of a curved vessel originating from the right hemidiaphragm and connecting with the inferior vena cava (IVC). The patient and her medical team opted for conservative management, involving multidisciplinary care, tailored treatment for infections, and regular monitoring. The rarity of Scimitar syndrome necessitates careful diagnosis and management decisions. While surgical intervention is often recommended, this case demonstrates the complexities of choosing conservative management based on patient preferences and the evolving clinical course. A literature review reveals varied outcomes of surgical and conservative approaches, emphasizing the need for personalized strategies. Radiological techniques, such as CTPA and MRI, play pivotal roles in diagnosis and monitoring. This case report underscores the clinical significance of scimitar syndrome, particularly in regions with limited reported cases, like Pakistan. The multidisciplinary management approach, the decision-making process regarding conservative treatment, and the unique radiological findings contribute to the medical community's understanding of this rare condition.

摘要

弯刀综合征是一种罕见的先天性心脏异常,涉及肺静脉异常引流至体静脉,导致出现类似弯刀的独特影像学特征。本病例报告展示了巴基斯坦一例独特的弯刀综合征病例,强调了其临床重要性及管理挑战。一名26岁女性,自幼有反复肺部感染和呼吸道症状病史,被诊断为弯刀综合征。包括胸部X光、计算机断层扫描肺动脉造影(CTPA)和经胸超声心动图在内的影像学评估证实,有一条弯曲血管起源于右半膈肌并与下腔静脉相连。患者及其医疗团队选择了保守治疗,包括多学科护理、针对感染的个体化治疗以及定期监测。弯刀综合征的罕见性需要仔细的诊断和管理决策。虽然通常建议进行手术干预,但本病例显示了根据患者偏好和不断变化的临床病程选择保守治疗的复杂性。文献综述揭示了手术和保守治疗方法的不同结果,强调了个性化策略的必要性。CTPA和MRI等放射学技术在诊断和监测中发挥着关键作用。本病例报告强调了弯刀综合征的临床意义,特别是在像巴基斯坦这样报告病例有限的地区。多学科管理方法、保守治疗的决策过程以及独特的放射学发现有助于医学界对这种罕见疾病的理解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2699/10925003/8d30a0b6fda7/cureus-0016-00000053874-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2699/10925003/86726405907b/cureus-0016-00000053874-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2699/10925003/8d30a0b6fda7/cureus-0016-00000053874-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2699/10925003/86726405907b/cureus-0016-00000053874-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2699/10925003/8d30a0b6fda7/cureus-0016-00000053874-i02.jpg

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本文引用的文献

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Treatment and prognosis of Scimitar syndrome: A retrospective analysis in a single center of East China.弯刀综合征的治疗与预后:中国东部单中心回顾性分析
Front Cardiovasc Med. 2022 Aug 24;9:973796. doi: 10.3389/fcvm.2022.973796. eCollection 2022.
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Surgical management of the scimitar syndrome.弯刀综合征的外科治疗。
J Card Surg. 2021 Oct;36(10):3770-3795. doi: 10.1111/jocs.15857. Epub 2021 Aug 16.
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Anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR.弯刀综合征的 CT 和 MRI 解剖关联和影像学特征。
J Cardiovasc Comput Tomogr. 2018 Jul-Aug;12(4):286-289. doi: 10.1016/j.jcct.2018.02.001. Epub 2018 Feb 3.
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Ann Thorac Surg. 2018 Feb;105(2):592-598. doi: 10.1016/j.athoracsur.2017.06.061. Epub 2017 Oct 18.
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Scimitar syndrome: the curved Turkish sabre.弯刀综合征:形似弯曲的土耳其马刀。
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2014;17(1):56-61. doi: 10.1053/j.pcsu.2014.01.003.
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Scimitar syndrome and evolution of managements.弯刀综合征及其治疗进展
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Scimitar syndrome: incidence, treatment, and prognosis.弯刀综合征:发病率、治疗及预后
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Scimitar syndrome.弯刀综合征
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