Endomyocardial Fibrosis Clinical Presentation

Updated: Nov 24, 2014
  • 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.
  • Anginalike 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.
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Physical

Physical findings 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. [13]
  • 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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Causes

A specific single etiology of EMF has not been established. Suggested potential causes include the following:

  • Infectious causes, such as parasites (eg, helminths) and protozoans (eg, toxoplasmosis, malaria)
  • Inflammatory causes: Eosinophilia
  • Nutritional causes, such as general malnutrition, a high-tuber diet, Ce toxicity, and hypomagnesemia
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