Abstract 18070: Amyloid Ambush: A Shocking Encounter With AL Cardiac Amyloidosis

Introduction: We present a case of a 44-year-old female with multiple comorbidities with a catastrophic course of cardiac AL amyloidosis.

Case Presentation: The patient initially presented with symptoms, including epigastric pain, nausea, vomiting, and chest discomfort. Notably, she had a previous diagnosis of carpal tunnel syndrome. Soon after, she developed cardiogenic shock, sustained ventricular tachycardia, and was transferred to our center.

Diagnostic Workup: The echocardiogram showed mildly reduced LVEF and moderately depressed RVSF. Initial RHC revealed significantly elevated right and left filling pressures and a low cardiac index. Cardiac MRI exhibited abnormal gadolinium kinetics and parametric mapping consistent with cardiac amyloidosis. Endomyocardial biopsy with light chain mass spectrometry confirmed the presence of AL amyloidosis. The kappa/lambda ratio was 60, and a bone marrow biopsy showed 30% plasma cells.

Treatment and Outcome: The patient's cardiogenic shock worsened despite intensive support with an intra-aortic balloon pump and multiple iono-pressors. Unfortunately, her deteriorating condition rendered her ineligible for chemotherapy, transplantation, and left ventricular assist device (LVAD) placement.

Conclusion: This case unveils the elusive nature of AL cardiac amyloidosis, where cardiogenic shock can be the initial presentation. Clinicians must remain vigilant, recognizing LV hypertrophy coupled with a low-voltage EKG and a preceding history of carpal tunnel syndrome. We underscore the rapid progression and limited treatment options.