快速进展性心脏淀粉样变性的诊断和治疗难题:基于病例报告的文献综述。

IF 2 Q2 EMERGENCY MEDICINE International Journal of Emergency Medicine Pub Date : 2024-10-21 DOI:10.1186/s12245-024-00750-x
Nahid Senobari, Roozbeh Nazari, Pouya Ebrahimi, Hamidreza Soleimani, Maryam Taheri, Kaveh Hosseini, Homa Taheri, Robert J Siegel
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引用次数: 0

摘要

导言心脏淀粉样变性是一种罕见的、可能致命的全身性疾病变种。早期诊断有可能显著改善临床预后:一名 56 岁的女性因呼吸困难和心悸前来就诊。她的体格检查和心脏磁共振成像(CMRI)无创评估显示她患有局限性心肌病,骨髓活检结果显示她患有全身性淀粉样变性:讨论:心脏淀粉样变性的诊断并不总是那么简单,延误诊断会导致病情恶化,增加发病和死亡风险。心电图、超声心动图、心脏磁共振成像和组织病理学评估是诊断心脏淀粉样变性的主要方法。治疗包括控制心力衰竭症状和改变病情的干预措施,包括内科和外科治疗方法:临床学习要点(结论):心脏受累是全身性淀粉样变性的主要死因。早期怀疑、诊断和治疗是提高患者生存率的关键。CMRI 在诊断心脏淀粉样变性中可发挥重要作用。为便于直观总结,本文提供了图表摘要。
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Diagnostic and therapeutic challenges in rapidly progressing cardiac amyloidosis: a literature review based on case report.

Introduction: Cardiac amyloidosis is a rarely reported and potentially fatal variant of the systemic disease. Its early diagnosis could potentially lead to significantly improved clinical outcomes.

Case presentation: A 56-year-old female presented with dyspnea and palpitations. Her physical exam and non-invasive evaluation with cardiac magnetic resonance imaging (CMRI) revealed restrictive cardiomyopathy, and the bone marrow biopsy results showed systemic amyloidosis.

Discussion: The diagnosis of cardiac amyloidosis is not always straightforward, and delay can cause the progression of the disease and an increased risk of morbidity and mortality. Electrocardiograms, echocardiograms, cardiac magnetic resonance imaging, and histopathologic evaluation are the main methods for diagnosing cardiac amyloidosis. The treatment consists of controlling heart failure symptoms and disease-modifying interventions, including medical and surgical therapeutic methods.

Clinical learning point (conclusion): Cardiac involvement is the main cause of death in systemic amyloidosis. Early suspicion, diagnosis, and treatment are crucial in improving patients' survival. CMRI can play an essential role in the diagnosis of cardiac Amyloidosis. A graphical abstract is provided for visual summary.

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来源期刊
CiteScore
4.60
自引率
0.00%
发文量
63
审稿时长
13 weeks
期刊介绍: The aim of the journal is to bring to light the various clinical advancements and research developments attained over the world and thus help the specialty forge ahead. It is directed towards physicians and medical personnel undergoing training or working within the field of Emergency Medicine. Medical students who are interested in pursuing a career in Emergency Medicine will also benefit from the journal. This is particularly useful for trainees in countries where the specialty is still in its infancy. Disciplines covered will include interesting clinical cases, the latest evidence-based practice and research developments in Emergency medicine including emergency pediatrics.
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