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Cardiac involvement in idiopathic hypereosinophilic syndrome
  1. R Lorenzoni,
  2. L Cortigiani,
  3. A Melosi

  1. lorenzoni.r{at}tin.it

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A 38 year old man was referred for dyspnoea. Transthoracic and transoesophageal echocardiography revealed the presence of deposits at the apex of the left and right ventricle (arrows). Routine blood tests showed an increased eosinophil count (18 × 109/litre). A bone marrow biopsy demonstrated an eosinophilic myeloproliferative disease. The patient was diagnosed with hypereosinophilic syndrome, in which cardiac involvement is common.


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The patient was treated with alpha interferon plus oral methylprednisolone. Concomitant treatment with diuretics and oral anticoagulants was also instituted. His symptoms improved and no further increase of the endomyocardial deposits was observed.

There are three stages in hypereosinophilic syndrome: necrotic, thrombotic, and fibrotic. Common echocardiographic findings in this disease are mural thrombus with apical cavity obliteration (which was the main manifestation in our patient) and thickening of the myocardium. Atrioventricular valves are often involved in the fibrotic process with subsequent valvar regurgitation. A restrictive pattern is typical of the late stage of the disease.

However, in our patient no sign of a restrictive pattern was evident. In fact, Doppler (pulmonary veins and transmitral flow) and nuclear (peak filling rate and time to peak filling rate) indexes of left ventricular compliance were normal.

According to the normal appearance of the spared myocardium and the atrioventricular valves, the fibrotic stage has yet to occur in this patient. Early institution of, and favourable response to, treatment may halt the fibrotic process and improve symptoms and prognosis in these patients.

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