Nicole Rollins
68 y/o man was referred to cardiology in 2007 for worsening DOE and fatigue Echocardiogram showed decreased systolic function, EF of 35%, LVH, a small heart, large left atrium, Pulmonary HTN, and aortic, tricuspid, and mitral regurgitation Heart Catheterization 2007 showed non- diseased coronaries and EF of 50%.
MRI in 2008 showed hypertrophic cardiomyopathy and EF 49% Biventricular Pacemaker and ICD placed in 2008 after complete heart failure No improvement in symptoms Sent to Boston for a second opinion in 2009 Echocardiogram – showed speckled pattern of reflectance Sent for an endomyocardial biopsy Current Cardiac Medication Regimen Vasotec, Diovan, Coreg, Aldactone, and Metalozone
Increased JVD 2+ Pedal Edema bilaterally Lungs were clear to auscultation bilaterally Regular heart rate and rhythm with soft aortic insufficiency murmur
Restrictive infiltrating cardiomyopathy Typical presents after age 35 Typically associated with renal dysfunction, multiple myeloma, peripheral neuropathy, and pulmonary emboli Two main etiologies Primary – mutation in immunoglubin light chains Secondary – malfunction in the serum amyloid A protein
Restrictive Cardiomyopathy Fluid retention, peripheral edema, hepatomegally, and Increased JVD Systolic heart failure Orthostatic hypotension Conduction System Disease Typically atrial fibrillation or complete heart block
Other causes of Restrictive Cardiomyopathy Hemochromoatosis or Scleroderma Congestive Heart Failure Atrial Fibrillation
Electrocardiogram Echocardiogram MRI Heart Catheterization Abdominal Fat Pad Biopsy Endomyocardial Biopsy Used to confirm suspected diagnosis Positive Congo-red stain
Poor prognosis Heart Transplant with simultaneous bone marrow transplant Pacemaker with ICD Diuretic
68 y/o man with 2 years of worsening DOE and fatigue underwent an endomyocardial biopsy with the pathology results confirming the diagnosis of amyloidosis. The patient’s current therapy includes a diuretic regimen and the placement of a biventricular pacemaker with ICD. Patient was referred to Boston for better management of his condition.