Paradoxical Left Ventricular Hypertrophy by Echocardiogram and Low Voltage ECG: A Key Clue in the Diagnostic Workup of Two Distinct Presentations of Cardiac AL Amyloidosis

超声心动图和低电压心电图显示的矛盾性左心室肥厚:两种不同表现的心脏AL淀粉样变性诊断的关键线索

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Abstract

Cardiac amyloidosis remains a rare disease caused by the extracellular deposition of abnormal proteins-amyloids in the myocardium. These protein structures in the myocardium are associated with high morbidity and mortality, with prognosis hinging on early detection and treatment. Three main types of cardiac amyloidosis have been identified: light chain (AL), familial or senile (ATTR), and secondary amyloidosis which is associated with chronic inflammation. Cardiac amyloidosis classically presents as diastolic heart failure with symptoms of volume overload low voltage on electrocardiogram (ECG) and echocardiographic features of diastolic dysfunction and paradoxical left ventricular hypertrophy (paradoxical with respect to low voltage on ECG). Early suspicion should trigger additional laboratory and imaging workup to facilitate early detection. Early detection remains critical to prognosis. Herein, we present two patients admitted to a safety-net hospital within one month of each other with distinct presentations yet important, overlapping characteristics that led to the diagnosis of AL amyloidosis in both patients.

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