Right ventricle endomyocardial fibrosis subdiagnosis

Presenting Author(s)

• 

  Maira Azevedo Ximenes

  Fellowship in Cardiac Imaging Department DASA + Unifesp
  Universidade Federal de São Paulo - Unifesp
  São Paulo, Sao Paulo, Brazil

Primary Author(s)

• 

  Maira Azevedo Ximenes

  Fellowship in Cardiac Imaging Department DASA + Unifesp
  Universidade Federal de São Paulo - Unifesp
  São Paulo, Sao Paulo, Brazil

Co-Author(s)

• 

  Gilberto Szarf, MD, PhD

  Radiologist
  Hospital Israelita Albert Einstein
  Sao Paulo, Sao Paulo, Brazil
• MS

  Maria Eduarda Menezes de Siqueira, MD,PhD

  Assistant
  Sociedade Brasileira de Cardiologia

Description of Clinical Presentation: Endomyocardial fibrosis is a cardiac disease whose etiopathogenesis is still not fully understood. It may be related to a state of chronic eosinophilia, primarily affecting populations in tropical countries. Due to the fibrous component of the subendocardium and myocardium, the resulting cardiomyopathy has restrictive characteristics and distortion of the ventricular and valve structure. It may present with clinical features similar to other heart diseases and nonspecific cardiological examinations, making diagnosis difficult.

A 62-year-old woman with hypertension, diabetes, and a former smoker, originally from Pernambuco, Brazil, had previously been referred to São Paulo. In 2019, she began experiencing sporadic episodes of dyspnea associated with chest pain and palpitations, with slow and progressive clinical worsening. Previous imaging studies had only revealed right atrial dilation. During 2020-2022, outpatient visits became infrequent. She returned for follow-up in July 2024, when she was admitted to the emergency room with severe, squeezing chest pain radiating to the left upper limb and dyspnea unrelated to exertion. An electrocardiogram showed ST-segment depression in leads DI, V5, and V6. A previous cardiac catheterization in 2021 revealed only discrete coronary atheromatous plaques. Cardiac magnetic resonance imaging (CMR) was performed.

Diagnostic Techniques and Their Most Important Findings: CMR displayed significant pericardial effusion, reduced right ventricular systolic function, significant biatrial enlargement, hyperintensity in the interventricular septum on Steady State Free Precession sequences suggestive of myocardial liposubstitution, and late gadolinium enhancement sequences demonstrated a pattern of apical intracavitary myocardial fibrosis, suggestive of endomyocardial fibrosis. The cardiomyopathy presented components of external and internal restriction, with progression to right ventricular failure. The patient developed mixed shock (cardiogenic and septic) and died on July 15, 2024. Autopsy to evaluate the heart showed areas of biventricular myocardial fibrosis, organized mainly at the apical segments, distributed in the pattern of endomyocardial fibrosis, confirming the diagnosis.

Learning Points from this Case: Endomyocardial fibrosis can present in the right ventricle, but due to the fewer incidence compared to the left ventricle, this clinical hypothesis is only raised based on more complex imaging tests such as CMR. As in this case report, detailed visualization of the cardiac right chambers was an advantage of CMR, combined with the use of paramagnetic contrast for characterizing structural alterations. In the diagnosis of endomyocardial fibrosis, these alterations may present with specific patterns and guide clinical management. In patients with little improvement or progressive clinical worsening, presenting symptoms of right ventricular failure in areas with a high incidence of the disease, complementary investigation with CMR is well indicated.

Steady State Free Precession sequences
Late Gadolinium Enhancement sequences