European Journal of Echocardiography 2005 6(5):379-381; doi:10.1016/j.euje.2004.11.010
Copyright © 2005, The European Society of Cardiology
Two cases of endomyocardial disease with hypereosinophilia in Turkey
Özgül Uçara,*,
Zehra Gölba
b and
Nesligül Yldrmb
aNumune Education and Research Hospital, Cardiology Department, Ankara, Turkey
bAnkara TürkiyeYüksek 
htisas Hospital, Cardiology Department, Ankara, Turkey
Received 23 August 2004; received in revised form 22 November 2004; accepted after revision 29 November 2004.
ozgul_ucar{at}yahoo.com
* Corresponding author. Keklikpnar Mah, 463/7 Dikmen, Ankara 06450, Turkey. Tel.: +90 3124769578; fax: +90 3123103460.
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Abstract
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Endomyocardial disease is characterized by fibrothrombotic thickening
of apical endocardium and subvalvular regions of atrioventricular
valves. The disease is uncommon in Turkey. In this report two
cases of endomyocardial disease with hypereosinophilia which
were medically managed are presented.
Keywords: Endomyocardial disease; Endomyocardial fibrosis; Löffler endocarditis; Hypereosinophilic syndrome; Restrictive cardiomyopathy
Endomyocardial disease is characterized by fibrothrombotic thickening
of apical endocardium and subvalvular regions of atrioventricular
valves resulting in restrictive physiology.
1 The disease is
uncommon in Turkey and to our knowledge this is the first case
report presenting patients with typical features of endomyocardial
disease.
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Case 1
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A 35-year-old woman was admitted for abdominal distention. On
physical examination, the vital signs were stable. No cardiac
murmurs could be heard. She had ascites and peripheral edema.
The blood eosinophil count at presentation was 1.5
x10
9/L (13%
of total white blood cells). There were nonspecific T wave abnormalities
in ECG and a pleural effusion in the right costophrenic sinus
in chest X-ray. Transthoracic echocardiography revealed left
ventricular apical obliteration, endocardial thickening together
with mobile thrombi extending to the left ventricular cavity.
Right ventricular apical obliteration and biatrial enlargement
were also present. Fibrothrombotic tissue reached out the supporting
apparatus of atrioventricular valves without affecting leaflet
mobility (
Fig. 1). Left ventricular end-diastolic and end-systolic
diameters were reduced (3.4cm and 2.1cm, respectively) and ejection
fraction was 68%. Inferior vena cava was dilated with no inspiratory
collapse. The left ventricular Doppler diastolic filling pattern
was consistent with a pseudonormal pattern. No specific cause
could be defined for hypereosinophilia. The patient was given
heart failure therapy with furosemide, spironolactone, betablocker
as well as corticosteroid therapy. A control echocardiogram
was performed every two weeks. Although the blood eosinophil
count decreased to 1.6
x10
8/L three months after steroid therapy,
the echocardiographic findings remained unchanged and the patient
died from refractory heart failure five months after diagnosis.
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Case 2
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A 53-year-old woman with complaints of appetite loss, fever,
abdominal pain, shortness of breath and productive cough was
admitted. On cardiac auscultation an apical pansystolic murmur
could be heard. She had hepatosplenomegaly and mild lower extremity
edema. ECG revealed sinusal tachycardia and nonspecific ST segment
changes. Bilateral pleural effusion was detected in chest X-ray.
She had hypereosinophilia with an eosinophil count of 1.45
x10
9/L
(15% of total white blood cells). Bone marrow biopsy revealed
increased eosinophils. No parasitic, allergic, malignant or
systemic etiology explaining hypereosinophilia could be defined.
On transthoracic echocardiography, the apices of both ventricles
were obliterated with fibrothrombotic tissue which extended
through subvalvular regions of atrioventricular valves resulting
in moderate mitral and tricuspid regurgitation on color Doppler
due to restricted leaflet motion. Left and right ventricular
cavity volumes were reduced and atria were enlarged (
Fig. 2).
Peak systolic pulmonary artery pressure estimated from tricuspid
regurgitant velocity was 53mmHg. Doppler studies detected restrictive
type diastolic filling with an E/A ratio greater than two and
decreased deceleration time. Medical therapy with acetylsalicylic
acid, subcutaneous nadroparine, furosemide, enalapril, warfarin
sodium and steroids was initiated. Although the symptoms were
relieved, no change in echocardiographic findings was detected
in three control examinations performed every two weeks. Unfortunately
the patient died after two months.
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Discussion
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Endomyocardial disease has two types; endomyocardial fibrosis
(EMF) and Löffler endocarditis. EMF mostly occurs in tropical
countries and is less associated with hypereosinophilia, whereas
the latter is seen in temperate countries and is accompanied
with hypereosinophilia.
1 Löffler endocarditis is accepted
to be one of the manifestations of the idiopathic hypereosinophilic
syndrome. As Turkey is a temperate country and both of our patients
had increased blood eosinophils, we diagnosed them as having
Löffler endocarditis. The pathophysiology of endomyocardial
disease is thought to be infiltration of myocardium (especially
apices of ventricles) by abnormal eosinophils and subsequent
necrosis caused by toxic degranulation. The initial necrosis
is followed by thrombosis and fibrosis.
2 Typical echocardiographic
findings are endocardial thickening, fibrothrombotic obliteration
of the ventricular apices and valvular regurgitation due to
limited motion of the posterior mitral leaflet. Doppler and
hemodynamic studies reveal the findings of restrictive cardiomyopathy.
3 There still exists no definite therapy for endomyocardial disease
and the prognosis is poor. Corticosteroids have proven benefit
in the short term and have become a standard therapy.
4,5 Although
we observed clinical improvement and a decrease in the peripheral
eosinophil count with steroid therapy, echocardiographic findings
remained unchanged. In steroid resistant cases, hydroxyurea
and interferon-alpha are also recommended.
6 Future treatment
may involve the tyrosine kinase inhibitor imatinib mesylate
or anti-interleukin-5 agent mepolizumab.
7,8 Surgical therapy
is a promising alternative to medical therapy.
9 Since the disease
is rarely seen in our country, the patients could not be evaluated
for surgery due to the absence of experienced centers.
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References
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Case 1
