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When a dilated heart is not dilated cardiomyopathy: an atypical case of endomyocardial fibrosis
Session:
Sessão Speaker’s Corner - Casos clínicos desafiantes… em Miocardiopatias
Speaker:
Joana Silva Ferreira
Congress:
CPC 2022
Topic:
D. Heart Failure
Theme:
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Subtheme:
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Session Type:
Sessão de Casos Clínicos
FP Number:
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Authors:
Joana Silva Ferreira; Sara Gonçalves; Tatiana Duarte; José Maria Farinha; Marta Fonseca; Ana Fátima Esteves; Rui Coelho; Rui Caria
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">Background: </span></span></span><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">Endomyocardial fibrosis (EMF) is a common cause of heart failure (HF) in equatorial regions but is rare in developed countries. Its etiology is not yet fully understood but it is characterized by the presence of fibrosis in the endocardium, typically resulting in a restrictive cardiomyopathy. We report an atypical case of EMF, initially misdiagnosed as dilated cardiomyopathy, where cardiac magnetic resonance (CMR) was crucial to establish the diagnosis.</span></span></span></p> <p style="text-align:justify"> </p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">Case report: </span></span></span><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">A 29-year-old male of Angolan origins, with no relevant medical history, presented in a Portuguese hospital with nausea, fatigue and peripheral oedema. </span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">Blood tests revealed elevated NT-proBNP levels, elevated liver enzymes and serologic tests suggesting active hepatitis B, with low viral load. Cardiac troponin and eosinophil levels were normal.</span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">Echocardiography showed a dilated left ventricle (LV) with severe systolic and diastolic dysfunction (LV ejection fraction of 18%), as well as right ventricular dilation, severe atrial dilation and pulmonary hypertension.</span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">Coronary angiography excluded significant coronary disease.</span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">A working diagnosis of dilated cardiomyopathy was assumed, with previous myocarditis being the most likely etiology. After decongestion, he was discharged on neurohormonal HF medication </span><span style="font-family:"Arial",sans-serif">with an early follow-up in the HF clinic.</span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">Echocardiographic reassessment months later showed an improved LV ejection fraction (45%) and an apical thrombus obliterating the LV apex (Fig. 1A). CMR also revealed subendocardial late gadolinium enhancement (LGE) with a double-V aspect of fibrosis plus thrombus in the LV apex (Fig. 1B).</span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">The origins of the patient together with the presence of ventricular thrombus without severe dysfunction, the apical obliteration and the typical “double-V” fibrosis/thrombus pattern led to the diagnosis of EMF.</span></span></span></p> <p style="text-align:justify"> </p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">Conclusion: </span></span></span><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-family:"Arial",sans-serif">This is a very rare case of EMF mimicking dilated cardiomyopathy. It highlights the importance of pursuing a thorough investigation of all HF patients, including multimodality imaging whenever the diagnosis is uncertain.</span></span></span></p>
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