Chango Azanza Diego Xavier, Fernández Tirado Ruth Lizbeth, López Pillaga Valeria Verenisse, Tello Ochoa José David, Pinos Vásquez Javier Fernando
Hospital del Rio, Cuenca, Ecuador. Hospital del Rio Cuenca Ecuador.
Cardiológico del Austro, Cuenca, Ecuador. Cardiológico del Austro Cuenca Ecuador.
Arch Peru Cardiol Cir Cardiovasc. 2022 Dec 31;3(4):220-225. doi: 10.47487/apcyccv.v3i4.249.. eCollection 2022 Oct-Dec.
Systemic light chain amyloidosis is a disease characterized by the accumulation of amyloid protein in multiple organs and systems. We present the case of a 52-year-old male patient with a diagnosis of systemic light chain amyloidosis associated with cardiac and renal involvement. A renal biopsy showed the presence of renal amyloidosis associated with proteinuria, and the patient was referred for cardiovascular evaluation. The baseline electrocardiogram showed micro voltage in frontal leads that were discordant with the left ventricular hypertrophy evidenced in the transthoracic echocardiogram (TTE). Cardiac magnetic resonance imaging (CMR) confirmed the presence of cardiac amyloid infiltration with a pattern of extensive ventricular late-gadolinium enhancement. Despite being referred and receiving specific systemic chemotherapy treatment, the evolution was not favorable after four months of follow-up with worsening cardiac infiltration, increasing values of biomarkers, and progression of dyspnea. The TTE was useful in revealing the unfavorable evolution and worsening of diastolic function parameters and increased wall thickness in the context of infiltration. The electrocardiogram and echocardiogram were easily accessible tools that allowed the monitoring of the response to treatment.
系统性轻链淀粉样变性是一种以淀粉样蛋白在多个器官和系统中积聚为特征的疾病。我们报告一例52岁男性患者,诊断为系统性轻链淀粉样变性,伴有心脏和肾脏受累。肾活检显示存在与蛋白尿相关的肾淀粉样变性,该患者被转诊进行心血管评估。基线心电图显示额面导联低电压,与经胸超声心动图(TTE)显示的左心室肥厚不一致。心脏磁共振成像(CMR)证实存在心脏淀粉样浸润,表现为广泛的心室晚期钆增强。尽管转诊并接受了特定的全身化疗,但随访四个月后病情进展不利,心脏浸润加重,生物标志物值升高,呼吸困难加重。TTE有助于揭示舒张功能参数的不利演变和恶化,以及浸润情况下室壁厚度增加。心电图和超声心动图是易于获取的工具,可用于监测治疗反应。