Endomyocardial Fibrosis Clinical Presentation

Updated: Dec 29, 2020
  • Author: Ali A Sovari, MD, FACP, FACC; Chief Editor: Henry H Ooi, MD, MRCPI  more...
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Presentation

History

Typically, endomyocardial fibrosis (EMF) has an insidious onset, and symptoms relate to the specific chambers and valves where the disease is most extensive, including the following:

  • When right ventricular involvement or tricuspid regurgitation predominates, lower extremity swelling, increasing abdominal girth, and nausea may be expected.

  • With left ventricular involvement, dyspnea is the predominant symptom, especially exertional dyspnea. Additionally, fatigue, paroxysmal nocturnal dyspnea, and orthopnea may be present.

  • Thromboembolic complications may occur in EMF.

  • Rarely, patients may present early in the course of the disease with an acute febrile illness with symptoms of cardiac insufficiency mimicking myocarditis.

  • Angina-like chest pain was reported in a patient with EMF involving the left ventricle. Patients with EMF may also present with arrhythmia symptoms such as syncope, near syncope, and palpitations.

  • Fibrosis is a substrate for arrhythmia and, therefore, ventricular and atrial arrhythmias can potentially be among the manifestations of the disease.

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Physical Examination

Physical findings in patients with endomyocardial fibrosis (EMF) are also dependent on the extent and distribution of disease and may include the following:

  • In those with right ventricular involvement, jugular venous pressure elevation, ascites, and edema may be present.

  • The presence of ascites may appear out of proportion to the amount of peripheral edema. This may occur because of the concomitant presence of a protein-losing enteropathy and subsequent hypoalbuminemia. In 1 retrospective study of patients with symptomatic EMF, ascites was observed in approximately half of the cases and was associated with greater involvement of the right ventricle and with a longer duration of the disease, thus being a characteristic of a worse prognosis. [18]

  • Patients with tricuspid regurgitation may have giant V waves observed in the jugular venous pulsations.

  • A third or fourth heart sound and tachycardia may be present.

  • Signs of pulmonary congestion are present in patients with left-sided disease.

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