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A 51 year old male patient had been followed for endomyocardial fibrosis (EMF) in another hospital for 19 years. He had been oligosymptomatic, with clinical manifestations of right side cardiac failure, and was taking diuretics and an angiotensin receptor blocker. At admission to our institution, the echocardiogram (panel A) showed severe right ventricular apical obliteration, characteristic of EMF, with an extremely enlarged right atrium. The left ventricle presented with normal dimensions, ejection fraction, and diastolic function, with mild to moderate mitral regurgitation. Pulsed wave tissue Doppler echocardiography of the left ventricle showed normal myocardial velocities of the septum, lateral, anterior, and posterior mitral annuli, and an E/E’ of 2.5, reflecting normal left ventricular end diastolic pressure. However, the systolic and diastolic velocities of the lateral wall of the right ventricle were low. In addition, the inferior vena cava was dilated without respiratory variation. Magnetic resonance imaging with gadolinium (panel B) enabled the detection of the typical fibrous tissue deposition in the apex of the right ventricle. After our evaluation, the patient agreed to undergo surgical treatment.
EMF affects only the heart and the cause is still unknown. Usually, systolic function is well preserved and diastolic dysfunction is responsible for the severe heart failure. The most appropriate time for surgical intervention of these patients is a debated issue. Subgroup analyses have shown that right ventricular fibrotic tissue compromise indicates a worse prognosis. These patients may be oligosymptomatic and must be followed closely to detect clinical manifestations of right sided heart failure. In this case, the patient promptly underwent surgery to avoid severe liver and kidney failure.
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