Cardiac Magnetic Resonance Imaging in the Diagnosis of Endomyocardiofibrosis

Presenting Author(s)

• 

  Amanda Marsiaj Rassi, MD

  Cardiologist
  Instituto Dante Pazzanese de cardiologia
  São Paulo, Sao Paulo, Brazil

Co-Author(s)

• 

  Alice Cunha Darzé, MD

  Cardiologist
  Instituto Dante Pazzanese de Cardiologia
  São Paulo, Sao Paulo, Brazil

Primary Author(s)

• 

  Amanda Marsiaj Rassi, MD

  Cardiologist
  Instituto Dante Pazzanese de cardiologia
  São Paulo, Sao Paulo, Brazil

Co-Author(s)

• 

  Ibraim Masciarelli Pinto, MD, PhD

  Director, Non Invasive Cardiology
  Instituto Dante Pazzanese de Cardiologia
  Sao Paulo, Sao Paulo, Brazil
• 

  Marina R. Cavallari, MD

  Doctor
  Instituto Dante Pazzanese de Cardiologia
  São Paulo, Sao Paulo, Brazil
• 

  Natassia SSC Gomes, MD

  Doctor
  Instituto Dante Pazzanese de Cardiologia
  São Paulo, Sao Paulo, Brazil
• 

  Antonio Tito 3. Paladino Filho, MD, PhD

  Cardiologist
  Hospital Vila Nova Star, Rede D’or São Paulo-SP
  Sao Paulo, Sao Paulo, Brazil
• 

  Camilla F. Reimberg, MD

  Doctor
  Instituto Dante Pazzanese de Cardiologia
  São Paulo , Sao Paulo, Brazil
• GM

  Gustavo R. Modolo, MD

  Doctor
  Instituto Dante Pazzanese de Cardiologia, Brazil
• LP

  Luciano G. Possa, MD

  Doctor
  Instituto Dante Pazzanese de Cardiologia, Brazil
• 

  Paul Salvador Salvador Morales, MD

  Cardiologist
  Instituto Dante Pazzanese de Cardiologia
  São Paulo, Sao Paulo, Brazil
• 

  Tiago Senra, MD, PhD

  Physician
  Dante Pazzanese Institute of Cardiology
  Sao Paulo, Sao Paulo, Brazil
• EB

  Everton R. Barbosa, MD

  MD
  Instituto Dante Pazzanese de Cardiologia, Brazil
• RH

  Rafael Hendges, MD

  cardiologist
  instituto dante pazzanese de cardiologia, Brazil
• CP

  Carlos Eduardo E E D Prazeres, MD

  Doctor
  Instituto Dante Pazzanese de Cardiologia, Brazil

Description of Clinical Presentation: A 38-year-old female patient with hypertension sought care with recent-onset dyspnea. She reported a diagnosis of atrial fibrillation and left ventricular thrombus, and was started on oral anticoagulation. Upon admission, cardiac magnetic resonance imaging (CMRI) was performed. The findings were suggestive of endomyocardial fibrosis. Medical treatment for heart failure was initiated, in addition to continued anticoagulant therapy.

Diagnostic Techniques and Their Most Important Findings: Cardiac magnetic resonance imaging (CMR) showed filling of the left ventricular cavity with fibrotic tissue in the apical portion and in the mid-inferior and inferolateral segments, causing apical obliteration and subendocardial delayed enhancement in the apical portion (“V-shaped morphology”). In addition, left atrial enlargement, preserved left ventricular ejection fraction, and mitral regurgitation were observed.

Learning Points from this Case: Endomyocardial fibrosis (EMC) is a form of restrictive cardiomyopathy of unknown etiology, characterized by endocardial fibrosis of the apices and inflow tracts of one or both ventricles, leading to obliteration of the ventricular cavity and regurgitation of the atrioventricular valves. It is prevalent in tropical and subtropical regions, affecting younger populations. Dietary, environmental, and infectious factors may also be associated, leading to an inflammatory and hypereosinophilic process. The hallmark of the disease is collagen deposition and fibroblast proliferation, leading to fibrotic obliteration of the ventricle and consequent restrictive cardiomyopathy. CMR images can provide cardiac volumes and ventricular systolic function, distinguishing EMF from other restrictive cardiomyopathies by the presence of subendocardial fibrosis and apical obliteration. They can also demonstrate the typical pattern of delayed enhancement and the presence of thrombus in the ventricular apex. Delayed enhancement extends from the subvalvular region to the ventricular apex, where it is most prominent. It has a 3-layered appearance (double V sign), with an inner layer lacking perfusion due to the presence of thrombus, a middle layer reflecting fibrotic tissue, and an outer layer of nearly normal myocardium.

mitral regurgitant jet
filling of the apical cavity of the left ventricle with fibrotic tissue, causing its obliteration
Subendocardial LGE with "V" morphology