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Cardiovascular Disease |

Dyspnea as the Presenting Symptom in a Patient With Cardiac Amyloidosis

Kevin Charles, MD; Jocelyn Taylor, MD; Muhammad Ebrahim Khan, MD; Shebey Thomas, MD; Govindarajan Venkatesh, MD
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Guthrie Clinic/Robert Packer Hospital, Sayre, PA


Chest. 2014;146(4_MeetingAbstracts):102A. doi:10.1378/chest.1994419
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Abstract

SESSION TITLE: Cardiovascular Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Amyloidosis is understood as an infiltrative disease in which extracellular tissue deposition of insoluble protein fibrils causes organ dysfunction. In primary amyloidosis, deposited proteins stem from immunoglobulin light chain fragments from an undelying plasma cell dyscrasia. Infiltrative deposition in the heart, although rare, can cause a restrictive cardiomyopathy causing heart failure associated dyspnea

CASE PRESENTATION: A 67 year old male presented complaining of exertional dyspnea and lower extremity swelling. Initial history and clinical examination was consistent with congestive heart failure. EKG revealed low voltage QRS complexes and left ventricular characteristics on echocardiogram included delayed relaxation pattern, 60% ejection fraction and concentric hypertrophy. Diastolic heart failure was secondary to infiltrative disease and was treated with ramipril, aldactone and lasix. Right heart catheterization revealed an elevated wedge pressure of 21 mmHg and diagnostic ventricular pressure tracings suggested restrictive cardiac physiology. Serum and urine protein analysis, and immunofixation studies confirmed IgG lambda gammopathy. Bone marrow biopsy showed 10% plasma cells with light chain restriction and beta-2 microglobulin was elevated (3.34 mg/L). These findings confirmed patient's plasma cell myeloma which was treated with bortezomib, lenalidomide and dexamethasone. New onset atrial tachyarrhythmia enused and patient died from a cardiac arrest. Complications were secondary to cardiogenic shock in the setting of an infiltrative disease associated restictive cardiomyopathy.

DISCUSSION: In a patient with plasma cell dyscrasia, cardiac involvement is a marker of poor prognosis. Treatment of cardiac amyloidosis is two fold. Chemotherapy or stem cell transplantation for the plasma cell disorder and optimal heart failure management. Cardioverter-defibrillators and heart transplantation are being considered, although their efficacy remains controversial.

CONCLUSIONS: Plasma cell myeloma with concurrent diastolic heart failure suggests a restrictive cardiomyopathy secondary to infiltration. Autologous bone marrow transplants with stem cell rescues is the most effective myeloma treatment. In regards to the heart failure, further research on the effectiveness of aggressive treatment strategies such as, heart transplantation and cardioverter defibrillators is required, to better understand management options.

Reference #1: Brenner DA, Jain M, Pimentel DR, et al. Human amyloidogenic light chains directly impair cardiomyocyte function through an increase in cellular oxidant stress. Circ Res 2004; 94:1008

Reference #2: Baccouche H, Maunz M, Beck T, et al. Differentiating cardiac amyloidosis and hypertrophic cardiomyopathy by use of three-dimensional speckle tracking echocardiography. Echocardiography 2012; 29: 668

Reference #3: Lin G, Dispenzieri A, Kyle R, et al. Implantable cardioverter defibrillators in patients with cardiac amyloidosis. J Cardiovas Electrophysiol 2013; 24:793

DISCLOSURE: The following authors have nothing to disclose: Kevin Charles, Jocelyn Taylor, Muhammad Ebrahim Khan, Shebey Thomas, Govindarajan Venkatesh

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