右心室隆起:诊断中的临床难题。

IF 1.2 4区 医学 Q3 ACOUSTICS Journal of Clinical Ultrasound Pub Date : 2024-09-20 DOI:10.1002/jcu.23848
Richard P Allwood
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引用次数: 0

摘要

致心律失常性右室心肌病(ARVC)是一种遗传性疾病,其特点是心肌进行性纤维脂肪组织替代。无症状者通常首次出现急性心脏症状,如晕厥、室性心律失常或心脏性猝死(SCD),可发生在年轻人和运动员中。在遗传性心肌病领域,ARVC 是最具诊断挑战性的疾病之一,因为它的表达方式多变、渗透性不完全,而且缺乏具体、独特的诊断标准。在没有其他临床发现或背景的情况下,目前的成像模式无法明确区分 ARVC 和其他疾病实体。右心室(RV)结构变化可导致突出的 ARVC 特征。2010 年修订的任务组标准(TFC)的一个重要组成部分是通过超声心动图评估右心室室壁运动收缩;然而,这可能很难评估。本病例报告探讨了 ARVC 的诊断标准以及 RV 室壁运动收缩在诊断中的作用。
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A right ventricular bulge: A clinical dilemma in diagnosis.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by progressive fibrofatty tissue replacement of the myocardium. Asymptomatic individuals can often present for the first time with acute cardiac symptoms, such as syncope and ventricular arrhythmias or sudden cardiac death (SCD), which can occur in young and athletic populations. In the field of inherited cardiomyopathies, ARVC is one of the most challenging to diagnose due to its variable expressivity, incomplete penetrance, and lack of specific, unique diagnostic criteria. Without additional clinical findings or context, current imaging modalities are unable to definitively distinguish ARVC from other disease entities. Right ventricular (RV) structural changes can lead to prominent ARVC features. An important component of the 2010 revised task force criteria (TFC) is the assessment of RV wall motion contraction by echocardiography; however, this can be difficult to assess. This case report explores the diagnostic criteria used for ARVC and the role of RV wall motion contraction in the diagnosis.

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来源期刊
CiteScore
1.90
自引率
0.00%
发文量
248
审稿时长
6 months
期刊介绍: The Journal of Clinical Ultrasound (JCU) is an international journal dedicated to the worldwide dissemination of scientific information on diagnostic and therapeutic applications of medical sonography. The scope of the journal includes--but is not limited to--the following areas: sonography of the gastrointestinal tract, genitourinary tract, vascular system, nervous system, head and neck, chest, breast, musculoskeletal system, and other superficial structures; Doppler applications; obstetric and pediatric applications; and interventional sonography. Studies comparing sonography with other imaging modalities are encouraged, as are studies evaluating the economic impact of sonography. Also within the journal''s scope are innovations and improvements in instrumentation and examination techniques and the use of contrast agents. JCU publishes original research articles, case reports, pictorial essays, technical notes, and letters to the editor. The journal is also dedicated to being an educational resource for its readers, through the publication of review articles and various scientific contributions from members of the editorial board and other world-renowned experts in sonography.
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