Beyond the Common Diagnosis: How CMR Unmasks a Rare Biventricular Endomyocardial Fibrosis in a Heart Failure Patient

Presenting Author(s)

• 

  Marina R. Cavallari, MD

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

Primary Author(s)

• 

  Marina R. Cavallari, MD

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

Co-Author(s)

• LP

  Luciano G. Possa, MD

  Doctor
  Instituto Dante Pazzanese de Cardiologia, Brazil
• 

  Natassia SSC Gomes, MD

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

  Gustavo R. Modolo, MD

  Doctor
  Instituto Dante Pazzanese de Cardiologia, Brazil
• EB

  Everton R. Barbosa, MD

  MD
  Instituto Dante Pazzanese de Cardiologia, Brazil
• 

  Camilla F. Reimberg, MD

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

  Alice Cunha Darzé, MD

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

  Amanda Marsiaj Rassi, MD

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

  João Marcelo Caneschi, MD

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

  Rafael de Castro Hendges, MD, PhD

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

  Paul Salvador Salvador Morales, MD

  Cardiologist
  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
• CP

  Carlos EE Prazeres, MD

  Doctor
  Instituto Dante Pazzanese de Cardiologia, Brazil
• 

  Tiago Senra, MD, PhD

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

  Ibraim Masciarelli Pinto, MD, PhD

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

Description of Clinical Presentation: A 61-year-old male, former smoker and former alcohol user, originally from the interior of Pernambuco, Brazil, presented with his first episode of acutely decompensated heart failure. Initial echocardiography showed a left ventricular ejection fraction of 42%. After clinical stabilization, he was referred to a tertiary cardiology center for etiological investigation.

Diagnostic Techniques and Their Most Important Findings: Coronary CTA ruled out significant obstructive coronary artery disease but revealed left chamber dilation, increased LV trabeculation with apical calcification, and signs of intramyocardial fat. Cardiac MRI (3T) was performed for definitive characterization. Cine SSFP sequences confirmed LV dilation and severe systolic dysfunction with apical akinesia and diffuse hypokinesia. First-pass perfusion revealed a large apical filling defect without perfusion. Late gadolinium enhancement (LGE) sequences were pivotal, demonstrating: 1) Extensive, dense LGE in the LV apex, mid inferior, and inferolateral segments with a characteristic "V-shaped" morphology, consistent with fibrosis; 2) Subendocardial LGE in the inflow tract and papillary muscles; 3) LGE in the right ventricular apex, indicating biventricular involvement; 4) A non-enhancing apical mass adjacent to the fibrotic area, consistent with a mural thrombus.

Learning Points from this Case: This case exemplifies the critical role of CMR with LGE in diagnosing endomyocardial fibrosis (EMF). CMR provides a non-invasive "virtual histology," accurately characterizing the typical pattern of apical fibrosis and differentiating it from thrombus, which is a common complication. The identification of biventricular involvement and extensive fibrosis has significant prognostic and surgical implications, guiding referral for potential endocardiectomy and long-term management in a specialized cardiomyopathy clinic. This case highlights EMF as an important differential diagnosis in patients from endemic regions presenting with heart failure and preserved coronary arteries.

Coronary CTA. Myocardial calcification -Yellow arrows. Fibrotic obliteration - White arrows.
First-pass perfusion and Cine SSFP sequences. Large apical filling defect - white arrows. Apical mural thrombus - red arrows.
Late gadolinium enhancement sequences. Extensive, dense LGE in the LV apex, mid inferior, and inferolateral segments with a characteristic "V-shaped" morphology - Yellow arrows; Subendocardial LGE in the inflow tract and papillary muscles - Pink arrow; LGE in the right ventricular apex - Blue arrow; A non-enhancing apical mural thrombus - White arrows.