C19. Cardiac Amyloidosis: A Great Pretender of Left Ventricular Hypertrophy with Systemic Manifestation

Abstract

Abstract Background Systemic amyloidosis encompasses an underdiagnosed disorder characterized by the extracellular deposition of amyloid in one or more organs, including the heart. The therapeutic strategy depends on the characterization of amyloid protein type and the extent of the disease. When founding a pathological left ventricular hypertrophy, initial clues provided by electrocardiography and echocardiography combined with other clinical manifestations could support a suspicion of cardiac amyloidosis. Case Summary A 54-year-old male patient was repeatedly admitted to a local hospital for recurrent heart failure and refractory bilateral pleural effusions of unknown cause. There was intolerance of any heart failure medications and repeated thoracocentesis failed to control the pleural effusion. The echocardiogram revealed severely impaired left ventricular function with phenotypic overlap between hypertrophic and restrictive cardiomyopathy. In contrast, we found a low voltage electrocardiogram. After an extensive diagnostic workup, including speckle tracking echocardiography, cardiac magnetic resonance, serum immunofixation and free light chain, bone marrow biopsy, and also Congo Red staining for amyloid, the final diagnosis was light chain (AL) amyloidosis. Discussion Cardiac involvement in systemic amyloidosis is the leading cause of morbidity and mortality. Frequently, myocardial wall thickening is incorrectly referred to as hypertrophy which may potentially lead to the wrong diagnosis and therapy. However, a need remains for improved awareness and expertise, while a multidisciplinary approach is important in making the correct diagnosis and management of this debilitating disease.