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American Journal of TransplantationImages in Transplantation – Continuing Medical Education (CME)

Each month, the American Journal of Transplantation will feature Images in Transplantation, a journal-based CME activity, chosen to educate participants on current developments in the science and imaging of transplantation. Participants can earn 1 AMA PRA Category 1 CreditTM per article at their own pace.

This month's feature article is titled: “Abnormal Finding on a Screening Endomyocardial Biopsy.”

Accreditation and Designation Statement

This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of Blackwell Futura Media Services, the American Society of Transplant Surgeons and the American Society of Transplantation. Blackwell Futura Media Services is accredited by the ACCME to provide continuing medical education for physicians.

Blackwell Futura Media Services designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM. Physicians should only claim credit commensurate with the extent of their participation in the activity.

Statement of Need

Amyloidosis is a relatively common disease, which presents specific diagnostic and therapeutic challenges. There often is a considerable delay between onset of symptoms and the correct diagnosis in patients with amyloidosis. There are newer approaches to amyloidosis treatment and knowledge of these treatments is in need of dissemination. This activity reviews key features of cardiac amyloidosis and provides physicians with the opportunity to update their knowledge in a succinct format. This activity is designed to improve competence (ability to apply knowledge to practice).

Educational Objectives

Upon completion of this educational activity, participants will be better able to:

  • • 
    Identify patients who may suffer from amyloidosis
  • • 
    Describe the common types of cardiac amyloidosis
  • • 
    Summarize the clinical and pathological features of amyloidosis
  • • 
    Recognize that amyloidosis can recur in transplanted organs

Target Audience

This activity has been designed to meet the educational needs of physicians and surgeons in the field of transplantation.

Disclosures

No commercial support has been accepted related to the development or publication of this activity. Blackwell Futura Media Services has reviewed all disclosures and resolved or managed all identified conflicts of interest, as applicable. The following authors, editors, and staff reported no relevant financial relationships with respect to this activity.

Editor-in-Chief

Allan D. Kirk, MD, PhD, FACS

Editors

Sandy Feng, MD, PhD

Douglas W. Hanto, MD, PhD

Authors

Josef Stehlik, MD, Mohamed Labedi, MD, Dylan Miller, MD and Monica P. Revelo, MD

ASTS Staff

Mina Behari, Director of Education

This manuscript underwent peer review in line with the standards of editorial integrity and publication ethics maintained by the American Journal of Transplantation. The peer reviewers have no relevant financial relationships to disclose. The peer review process for the American Journal of Transplantation is blinded. As such, the identities of the reviewers are not disclosed in line with the standard accepted practices of medical journal peer review.

Instructions on Receiving CME Credit

This activity is designed to be completed within an hour. Physicians should claim only those credits that reflect the time actually spent in the activity. This activity will be available for CME credit for twelve months following its publication date. At that time, it will be reviewed and potentially updated and extended for an additional twelve months.

Follow these steps to participate, answer the questions and claim your CME credit:

  • • 
  • • 
    Read the target audience, educational objectives, and activity disclosures.
  • • 
    Read the article in print or online format.
  • • 
    Reflect on the article.
  • • 
    Access the CME Exam, and choose the best answer to each question.
  • • 
    Complete the required evaluation and print your CME certificate.

This patient is a 59-year-old woman who presented with symptoms of heart failure and an echocardiogram suggestive of restrictive cardiomyopathy (Figure 1) 11 years ago. An endomyocardial biopsy was obtained and showed extensive myocardial deposits with randomly arranged, nonbranching 10–12 nm fibrils typical of amyloid (Figure 2). Serum and urine immunofixation electrophoresis showed the presence of a monoclonal IgG kappa light chain monoclonal protein. Bone marrow biopsy showed 5% mainly kappa light chain-restricted plasma cells, confirming the diagnosis of plasma cell dyscrasia and resulting monoclonal immunoglobulin kappa light-chain amyloidosis. The patient was treated with melphalan and prednisone chemotherapy and achieved sustained remission of amyloidosis, confirmed by serial examination of peripheral blood and bone marrow indices. Despite remission of the amyloidosis, there was a slow progression of her cardiomyopathy and she required heart transplantation 2 years ago. The patient had an unremarkable posttransplant course and has done well since. She now presents for routine annual exam that includes an echocardiogram, an angiogram and a screening biopsy. The echocardiogram showed normal biventricular function and normal echogenicity of the myocardium, the angiogram demonstrated normally appearing epicardial coronary arteries. The endomyocardial biopsy is shown in Figure 3.

Questions:

  1. Top of page
  2. Questions:
  • 1
    Amyloidosis is caused by:
    • a.   
      Noncaseating granulomas
    • b.   
      Proteins which misfold and deposit in extracellular space
    • c.   
      Mycobacterium amyloidi
    • d.   
      Byproducts of lymphocytes in acute leukemia
  • 2
    The following are characteristic features of cardiac amyloidosis:
    • a.   
      Insidious onset of right heart failure symptoms
    • b.   
      Progressive obstructive coronary disease
    • c.   
      Marked dilation of both ventricles
    • d.   
      All of the above
  • 3
    The electrocardiogram in patients with cardiac amyloidosis typically shows:
    • a.   
      Complete heart block
    • b.   
      Acute current of injury
    • c.   
      Low-voltage QRS
    • d.   
      Deep ST-segment depressions
  • 4
    The following regimen may be appropriate in treatment of amyloidosis:
    • a.   
      Melphalan and prednisone chemotherapy
    • b.   
      High-dose melphalan therapy followed by autologous stem cell transplantation
    • c.   
      Chemotherapy and heart transplantation in carefully selected patients
    • d.   
      All of the above
  • 5
    Figure 3 shows the following:
    • a.   
      Acute cellular rejection
    • b.   
      Acute antibody-mediated rejection
    • c.   
      Ischemic graft injury
    • d.   
      Recurrence of amyloid in the cardiac allograft

To complete this activity and earn credit, please go to http://www.amjtrans.com/cme