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Karla Quevedo, Mohamed Teleb, Syed H. Saad, Aamer Abbas, Tariq Siddiqui
(Department of Internal Medicine, Division of Cardiovascular Disease, Paul L. Foster School of Medicine, Texas Tech University Health Science Center, El Paso, TX, USA)
Med Sci Case Rep 2015; 2:5-9
Systemic amyloidosis is a rare multisystem disease caused by the deposition of insoluble proteins in various tissues and organs. Cardiac involvement, termed cardiac amyloidosis (CA), is observed in about 50% of patients with systemic amyloidosis, and is the major driver of mortality in patients with primary or light-chain amyloidosis (AL).
Case Report: A 52–year-old African-American woman presented with chest pain. Her past medical history consisted of hypertension, dyslipidemia, asthma, peripheral vascular disease, chronic kidney disease, and stage 1 diastolic dysfunction. Echocardiogram demonstrated left and right ventricular hypertrophy with diffuse valvular and inter-septal thickening consistent with infiltrative disorder. Right heart catheterization showed moderate pulmonary hypertension and low cardiac output and index. Due to these echocardiographic findings, biopsy specimens were obtained, which demonstrated amyloid deposition; electron microscopy showed AL (Lambda)-type amyloid deposition. This presentation defined very specific symptoms of amyloidosis resulting from systemic infiltrative involvement of the heart.
Conclusions: Amyloid cardiomyopathy results in a rapid decline in cardiac function inducing symptomatic heart failure. It is usually confounded with hypertensive heart disease or hypertrophic cardiomyopathy, given the nature of the clinical presentation and the echocardiographic findings, leading to a delay in the diagnosis. It is important to address the underlying cause of the AL amyloidosis, which will predict the clinical improvement of heart failure.
Keywords: Amyloidosis, Cardiomyopathy, Restrictive, Heart Failure