Sunday, August 07, 2022

Fibrose Endomiocárdica

 

Multimodality Imaging in Endomyocardial Fibrosis: Diagnosis and Assessment of the Extent of the Disease

Originally published7 Jan 2021https://doi.org/10.1161/CIRCIMAGING.120.012093Circulation: Cardiovascular Imaging. 2021;14:e012093

Endomyocardial fibrosis remains an important cause of restrictive cardiomyopathy despite the unsolved questions regarding the cause and therapeutic strategies. Worldwide prevalence is estimated at 10 to 12 million in 2008.1 Echocardiography is the standard modality for endomyocardial fibrosis diagnosis. Ventricular endocardial fibrosis with organized thrombus is the hallmark of advanced disease.2

In this case, a 70-year-old male patient was admitted with symptoms of right heart failure. ECG demonstrated atrial fibrillation and right bundle-branch block. Transthoracic, 2-dimensional, and 3-dimensional transesophageal echocardiography evidenced obliteration of the right ventricular (RV) apex, severe right atrial enlargement with a prominent aneurysm of fossa ovalis, and inferior vena cava dilatation (Figure, Movies I and II in the Data Supplement). Myocardial contrast echocardiography revealed marked RV apex and subtle left ventricular apex subendocardial delayed perfusion and a small perfusion defect over the RV endocardium (Figure, Movie III in the Data Supplement). Late gadolinium enhancement cardiac magnetic resonance imaging showed mild RV systolic dysfunction, apical thickening and obliteration, hypoperfusion at rest, and typical late double V enhancement, compatible with subendocardial fibrosis and thrombus (Figure, Movie IV in the Data Supplement). Left ventricular early involvement was demonstrated by the presence of hypoperfusion at rest and late subendocardial enhancement in its apex and apical lateral segment, as well as involvement of the mitral valve (Figure, Movie IV in the Data Supplement).

Figure.

Figure. Echocardiography and cardiac magnetic resonance (CMR) images.A, Transthoracic echocardiography and (B) Transesophageal echocardiography (TEE) evidenced obliteration of the right ventricular (RV) apex (white arrows). C, Three-dimensional TEE confirmed the obliteration of RV apex (red arrow) and showed a prominent aneurysm of fossa ovalis (white arrow). D and E, Myocardial contrast echocardiography evidenced a subtle left ventricular (LV) apex and marked RV apex subendocardial delayed perfusion associated with a perfusion defect over the RV endocardium (white arrows). F, CMR showed RV apical thickening and obliteration, typical late double V enhancement compatible with subendocardial fibrosis and thrombus, besides the presence of LV late subendocardial enhancement in its apex and apical lateral segment (white arrows).

Echocardiographic findings are highly concordant with the surgical and autopsies´ ones, reinforcing this noninvasive technique as the choice for diagnosis of endomyocardial fibrosis, especially in endemic areas.3 In this patient, myocardial contrast echocardiography added information about the extent of myocardial involvement, as well as the presence of a possible thrombus. Multimodality imaging approach can provide complementary information. Cardiac magnetic resonance imaging provides an early diagnostic advantage compared with transthoracic echocardiography and can provide differential diagnosis.2 The overall prognosis remains poor and treatment options remain limited. Accurate diagnosis with referral to experienced centers gives patients the best chance at improving their survival.1

Sources of Funding

None.

Disclosures None.

Footnotes

The Data Supplement is available at https://www.ahajournals.org/doi/suppl/10.1161/CIRCIMAGING.120.012093.

Daniela do Carmo Rassi, MD, PhD, Echocardiography, Cardiology São Francisco de Assis Hospital, Rua 9-A, Setor Aeroporto. Goiânia -GO, CEP: 74075-250. Email 

References

posted by Aloyzio Achutti at 9:04 PM

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