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.