Cardiac amyloidosis presenting as refractory heart failure: Role of GLS in diagnosis and on follow up.

Chigullapalli Sridevi, Susheel Kumar Malani, Prakyath Shetty
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Abstract

Background: Cardiac amyloidosis represents a challenging diagnosis in patients with multiple myeloma, often presenting as refractory heart failure with subtle early manifestations that can delay appropriate treatment. Case: We report a 55-year-old male with a three-year history of multiple myeloma who presented with progressive dyspnea and bilateral lower limb edema. Despite being normotensive and non-diabetic, he exhibited clinical signs of volume overload with elevated jugular venous pressure and bilateral crepitations. Electrocardiography revealed left ventricular hypertrophy with strain pattern, left axis deviation, and left bundle branch block, raising suspicion for infiltrative cardiomyopathy. Transthoracic echocardiography demonstrated moderate concentric left ventricular hypertrophy with reduced ejection fraction (40%) and severe grade III diastolic dysfunction. Global longitudinal strain analysis revealed markedly reduced GLS at -9.7% with characteristic apical sparing pattern, creating the pathognomonic "cherry-on-top" appearance. Despite optimal heart failure management and continued multiple myeloma treatment, six-month follow-up demonstrated dramatic GLS deterioration to -1.9% , indicating progressive cardiac amyloidosis and poor prognosis. Conclusion: Global longitudinal strain serves as both a sensitive diagnostic tool and powerful prognostic marker in cardiac amyloidosis secondary to multiple myeloma, supporting its integration into routine cardiac surveillance protocols for patients with plasma cell disorders to enable earlier diagnosis and better risk stratification.

以难治性心力衰竭为表现的心脏淀粉样变性:GLS在诊断和随访中的作用。
背景:心脏淀粉样变在多发性骨髓瘤患者中是一个具有挑战性的诊断,通常表现为难治性心力衰竭,早期表现微妙,可能延迟适当的治疗。病例:我们报告一名55岁男性,患有三年多发性骨髓瘤病史,表现为进行性呼吸困难和双侧下肢水肿。尽管血压正常,非糖尿病患者,但他表现出容量超载的临床症状,颈静脉压升高,双侧心悸。心电图示左心室肥厚伴应变型,左心轴偏曲,左束支阻滞,怀疑浸润性心肌病。经胸超声心动图显示中度左心室同心性肥厚伴射血分数降低(40%)和严重的III级舒张功能不全。全球纵向应变分析显示GLS显著降低-9.7%,具有典型的根尖保留模式,形成典型的“樱桃顶”外观。尽管心衰管理和多发性骨髓瘤持续治疗,6个月的随访显示GLS急剧恶化至-1.9%,表明心脏淀粉样变性进展和预后不良。结论:整体纵向应变是多发性骨髓瘤继发的心脏淀粉样变性的敏感诊断工具和强大的预后标志物,支持将其纳入浆细胞疾病患者的常规心脏监测方案,以实现早期诊断和更好的风险分层。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Global Cardiology Science & Practice
Global Cardiology Science & Practice Medicine-Cardiology and Cardiovascular Medicine
CiteScore
1.60
自引率
0.00%
发文量
20
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