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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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.


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.