Abstract Eosinophilic myocarditis is recognized by severe heart failure and marked eosinophilia infiltration resulting from different etiologies. Acute necrotizing eosinophilic myocarditis, the initial presentation of the disease, is rare and often fatal, with unique echocardiographic pictures, and followed by endocardial thrombosis and chronic endomyocardial fibrosis. We report a young female with acute lymphoblastic leukemia who presented fever and acute heart failure syndrome. The echocardiography showed severe left ventricle diastolic dysfunction with preserved ejection fraction. Systemic eosinophilia and the unique echocardiographic images made the diagnosis of acute necrotizing eosinophilic myocarditis. The patient survived after intensive cytotoxic chemotherapy including high-dose steroid

Chieh-Lin Teng

Chieh-Shou Su

Chih-Tai Ting

Fang-Yi Lin

Kuo-Yang Wang

Wei-Wen Lin

Ying-Chieh Liao

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Available online at www.sciencedirect.comJournal of the Chinese Medical Association 75 (2012) 536e538
www.jcma-online.comCase Report
Acute necrotizing eosinophilic myocarditis in a young woman
Ying-Chieh Liao a, Chieh-Shou Su a,c, Chieh-Lin Teng b, Kuo-Yang Wang a,d, Fang-Yi Lin a,e,
Chih-Tai Ting a,c, Wei-Wen Lin a,e,*
aCardiovascular Center, Taichung Veterans General Hospital, Taichung, Taiwan, ROC
bDivision of Hematology and Oncology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung, Taiwan, ROC
c Institute of Clinical Medicine, Cardiovascular Research Center and Department of Medicine, National Yang-Ming University School of Medicine, Taipei,
Taiwan, ROC
dDepartment of Medicine, Chung-Shan Medical University, Taichung, Taiwan, ROC
eDepartment of Medical Imaging and Radiological Sciences, Central Taiwan University of Science and Technology, Taichung, Taiwan, ROC
Received January 21, 2011; accepted June 8, 2011AbstractEosinophilic myocarditis is recognized by severe heart failure and marked eosinophilia infiltration resulting from different etiologies. Acute
necrotizing eosinophilic myocarditis, the initial presentation of the disease, is rare and often fatal, with unique echocardiographic pictures, and
followed by endocardial thrombosis and chronic endomyocardial fibrosis. We report a young female with acute lymphoblastic leukemia who
presented fever and acute heart failure syndrome. The echocardiography showed severe left ventricle diastolic dysfunction with preserved
ejection fraction. Systemic eosinophilia and the unique echocardiographic images made the diagnosis of acute necrotizing eosinophilic
myocarditis. The patient survived after intensive cytotoxic chemotherapy including high-dose steroid.
Copyright  2012 Elsevier Taiwan LLC and the Chinese Medical Association. All rights reserved.
Keywords: acute lymphoblastic leukemia; acute necrotizing eosinophilic myocarditis; eosinophilic myocarditis1. Introduction
Eosinophilic myocarditis is a unique cause of myocarditis,
usually presenting progressive heart deterioration and
evidence of eosinophilia.1 It may be secondary to leukemia,
parasite infection, allergic disease, granumatous disease,
connective tissue disease, vasculitis such as Churg-Strauss
syndrome, or primary hypereosinophilic syndrome.2 The
clinical picture ranges from asymptomatic endocardial infil-
tration and fulminate myocarditis to chronic restrictive
cardiomyopathy with or without apical thrombus formation.
Initially, the eosinophil infiltration produces endomyocardial
necrosis, inflammation and arteritis, representing acute* Corresponding author. Dr. Wei-Wen Lin, Cardiovascular Center, Taichung
Veterans General Hospital, 160, Section 3, Chung-Kang Road, Taichung 407,
Taiwan, ROC.
E-mail address: weinlin@vghtc.gov.tw (W.-W. Lin).
1726-4901/$ - see front matter Copyright  2012 Elsevier Taiwan LLC and the C
http://dx.doi.org/10.1016/j.jcma.2012.07.011necrotizing eosinophilic myocarditis. Then, the second
thrombotic phase and chronic endomyocardial fibrosis subse-
quently follow.3 Definite diagnosis is based on endomyo-
cardial biopsy; however, unique echocardiographic picture
with eosinophilia is essentially diagnostic.4
2. Case report
A 24-year-old Filipino woman was admitted due to fever
and back soreness for several days. There was no symptom or
sign related to heart failure at admission. Dry cough, orthop-
nea, and leg edema were first noted and progressing since the
second day of admission. Acute pulmonary edema was also
evident in the chest film and rapidly progressed to respiratory
failure. She was intubated on the third day. New systolic
murmur at apex was heard during auscultation. Blood tests
showed leukocytosis of 75.8 109/L (normal range
4e10 109/L), profound eosinophilia of 60.64 109/L
(normal range 50e350 109/L), elevated cardiac enzymeshinese Medical Association. All rights reserved.
537Y.-C. Liao et al. / Journal of the Chinese Medical Association 75 (2012) 536e538including creatine kinase (CK; 914 U/L, normal range
<160 U/L), CK-MB (17 U/L, normal range <16 U/L), and
troponin-I (10.3 ng/ml, normal range <0.034 U/L). The
patient’s electrocardiogram (ECG) revealed low voltage in
limb leads and poor R-wave progression in precordial leads.
Pulmonary artery wedge pressure was 39 mmHg, and the
cardiac index was 4.42 L/min/m2 under inotropic agent
support. Transthoracic echocardiogram (TTE) showed an
unusual thickening and high echogenicity of the endocardium,
which was asymmetrically distributed at the basal part of the
posterior wall and lateral wall of the left ventricle (LV). The
anterior wall of the LV, the interventricular septum and the
right ventricle (RV) were relatively spared. The posterior
leaflet of the mitral valve was also involved, resulting in
moderate eccentric mitral regurgitation. Pulse-wave Doppler
imaging of mitral inflow demonstrated that the ratio of E wave
to A wave was high and the deceleration time was short. The
restrictive mitral inflow pattern suggested severe diastolic
dysfunction (Fig. 1). The chamber sizes were normal, and the
LV systolic function was preserved. There was no thrombus at
the apex of the LV or RV. By profound eosinophilia and the
unique echocardiographic picture, acute necrotizing eosino-
philic myocarditis was diagnosed.
Intensive chemotherapy including high-dose steroid and
antibiotics were prescribed soon after acute lymphoblastic
leukemia was diagnosed by bone marrow biopsy.Fig. 1. Echocardiography of the patient. (A) Parasternal long-axis view showed a l
posterior segment (arrow) and involved posterior leaflet of the mitral valve (arro
infiltration (arrow), predominantly on the posterior wall, extending to inferior and l
moderate eccentric mitral regurgitation (arrow). Note the endocardial infiltration of
wave Doppler of mitral inflow. The E/A ratio was about 2 with short decelerationHemodynamics was supported by inotropic agent. The patient
survived after neutropenic sepsis following chemotherapy, and
was discharged in 2 months.
3. Discussion
Eosinophilia, which is caused by many diseases, can
involve multiple organs,5 including the heart, characterized as
eosinophilic cardiomyopathy.6 It was first discovered in 1936
by Löffler and called “fibroplastic parietal endocarditis with
blood eosinophilia”. Heart involvement usually happens in
eosinophil counts exceeding 1.5 109/L for at least 6
months.3 As described by Löffler, the subacute and chronic
forms of eosinophilic myocarditis, characterized by variable
degree of fibrosis and thrombosis and chronically progressive
heart failure, are more well-known. Cytotoxic agents and
corticosteroid may improve outcome in these patients.7 In
contrast, acute necrotizing eosinophilic endocarditis, the initial
presentation of eosinophilic myocarditis, is extremely rare and
is always fatal as a medical emergency.8 Eosinophil infiltration
and degranuation causing profound necrosis and arteritis are
the hallmarks in the disease.9 No effective treatment is proven
yet. Cardiac support and treating the underlying disease is
generally considered.
The hallmarks of echocardiography of eosinophilic
myocarditis are endocardial infiltration, apical thrombusocalized thickening endocardium with high echogenicity, mainly in the basal-
whead). (B) Parasternal short axis view showed the asymmetric endocardial
ateral wall. (C) The color Doppler image of apical four-chamber view showed
LV lateral wall also involved the posterior leaflet of the mitral valve. (D) Pulse-
time, which suggested severe diastolic dysfunction.
538 Y.-C. Liao et al. / Journal of the Chinese Medical Association 75 (2012) 536e538formation and endocardial fibrosis as the disease progresses.10
The geometrics of heart involvement is uniquely confined to
the LV basal segment of the posterior wall, and further impairs
the motion of the posterior mitral leaflet to cause eccentric
mitral regurgitation. Diastolic dysfunction is always seen, and
restrictive cardiomyopathy is evident in the chronic stage. The
image of eosinophilic myocarditis in the chronic stage is well
documented, characterized by the obliteration of the apex in
RVor LV by thrombosis and fibrosis.2 However, reports in the
literature of acute necrotizing eosinophilic endocarditis are
extremely rare. In our case, the endocardium of RV and
interventricular septum were spared, and LV endocardial
biopsy in the hemodynamically unstable patient was very
risky. Noninvasive TTE showed a specific and typical picture
without thrombus or fibrosis. Combined with the rapidly
progressive cardiac course and systemic eosinophilia, the
diagnosis of acute necrotizing eosinophilic cardiomyopathy is
convincing without endomyocardial biopsy.
No randomized controlled trial has yet evaluated the
treatment of eosinophilic myocarditis. Some observational
trials reported that high-dose steroid and cytotoxic agents may
be beneficial.11 The experience of treating acute necrotizing
eosinophilic cardiomopathy is more limited. The therapy is
usually focused on cardiac support and the underlying etiology
of eosinophilia. Some literature reported dramatic improve-
ment after steroid treatment.12 However, due to the rapidly
progressive course and diagnostic difficulty, many patients
were diagnosed by autopsy without prompt treatment before
their death. Quick and correct diagnosis is essential to treat the
disease and prevent fatality, but is rarely achieved. Thus,
endocardial biopsy should not be delayed if the imaging is not
diagnostic with clinical suspicion.13 In our patient, typical
echocardiography provided precise diagnosis in a noninvasive
manner, and made the strong link between clinical heart
failure and acute lymphoblastic leukemia-related eosinophilia.
It was also an important guide for the therapy in the initially
fulminant course.
In conclusion, acute necrotizing eosinophilic myocarditis is
extremely rare, with poor prognosis. It should be suspected in
all patients with eosinophilia and unexplained heart failure,
especially with a rapidly progressive course. To identify the
unique echocardiographic picture helps produce the diagnosis
early and may improve outcome. The case we have presented
showed a typical clinical course and specific imaging.Although rare, the echocardiographic image brought a strong
clue to the diagnosis and lead to successful treatment in our
case.
Acknowledgments
This study was supported in part by the National Science
Council Grants, 98-2314-B-075A-010-33-MY2, 98-2314-B-
075A-011-MY2, Taipei, Taiwan, and by the Yen Tjing Ling
Medical Foundation (CI-97-12), Taipei, Taiwan, ROC.
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Acute necrotizing eosinophilic myocarditis in a young woman

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Acute necrotizing eosinophilic myocarditis in a young woman

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