Download Multimodality imaging of Churg–Strauss myocarditis

Case Report
Heart Metab. (2014) 62:27–30
Multimodality imaging of
Churg–Strauss myocarditis
Amit Sud, Roberta L. Brum, Kelly Victor, Panagiotis Koudounis, Gerald S. Carr-White and Ronak Rajani
Department of Cardiology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
Correspondence: Dr Ronak Rajani, Department of Cardiology, St Thomas’ Hospital,
Westminster Bridge Road, London SE1 7EH, UK
Tel: +44 207 718 81004, fax: +44 208 399 4699, e-mail: [email protected]
Abstract
A 26-year-old man presented with a 1-month history of hemoptysis, fever and chest pain. Investigations
revealed a peripheral blood eosinophilia along with focal areas of lung consolidation on radiographic
imaging. Subsequent echocardiography confirmed cardiac involvement, with a reduction in systolic
function and a speckled appearance of the myocardium. The patient was diagnosed as having
eosinophilic granulomatosis with polyangiitis and was treated with intravenous cyclophosphamide
and corticosteroids. He made an excellent recovery and was subsequently discharged 4 weeks later.
We present the radiographic and cardiac imaging findings of this condition along with a review of the
value on cardiac screening in this patient population. L Heart Metab; 2014;62:27–30
Keywords: Cardiac magnetic resonance; Churg–Strauss syndrome; echocardiography; eosinophilic
granulomatosis polyangiitis; myocarditis.
Introduction
A 26-year-old man with a history of asthma presented
to our institute with a 1-month history of intermittent
hemoptysis, pleuritic chest pain and night sweats.
Clinical examination revealed a tachycardia of 112
beats per minute and pyrexia of 37.9°C. The full blood
count demonstrated an elevated total white cell count
(16.4 × 109) with an abnormally elevated eosinophil
count of 8.2 × 109. Serum biochemistry was normal
apart from elevated serum troponin T measurement
of 1366 ng/L. Given the clinical findings, the patient
underwent an urgent 12-lead ECG and a chest X-ray.
The ECG showed saddle-shaped ST-segment elevation suggestive of pericarditis, while the chest X-ray
showed patchy consolidation in the left mid zone and
coarse reticular marking in the mid zones bilaterally
(Figure 1).
A computed tomographic pulmonary angiogram
excluded the presence of pulmonary emboli but confirmed the presence of extensive lung parenchymal
changes. The proximal airways were dilated with some
opacification and distal dilatation. In addition, there was
associated thickening of the bronchi and multifocal
areas of ground glass opacification (Figure 2). These
findings were suggestive of pulmonary hemorrhage
along with eosinophilic pneumonia.
A subsequent transthoracic echocardiogram
confirmed concomitant pericardial and myocardial
involvement (Figure 3). There was a mild pericardial
effusion and the myocardium was thickened (14 mm)
with a heterogeneous speckled appearance indicative of edema. Left ventricular systolic function was
mildly reduced at 40% and there was evidence of
reduced long axis function.
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Ronak Rajani
Multimodality imaging of Churg–Strauss myocarditis
Heart Metab. (2014) 62:27–30
Abbreviations
ACE: angiotensin converting enzyme; ANCA: antineutrophil cytoplasmic antibodies; CMR: cardiac magnetic resonance; EGPA: eosinophilic granulomatosis
with polyangiitis; FFS: five-factor score
Fig. 2 Computed tomography pulmonary angiogram showing (a)
multifocal areas of ground glass opacification with more focal consolidation and (b) peripheral ground glass changes seen within the
lower lobe of the left lung (black arrows).
Fig. 1 Chest X-ray. Initial chest X-ray showing patchy areas of
consolidation within the lower lobe of the left lung (white arrows).
Immunology demonstrated an elevated serum
IgE level of 902 KU/L (normal range 0–81). There
were no myeloperoxidase, anti proteinase-3, glomerular basement membrane, extractable nuclear
antigens or antineutrophil cytoplasmic antibodies
(ANCA). Given the elevated eosinophil count,
elevated serum IgE level and imaging findings, a
diagnosis of Churg–Strauss syndrome with myocardial involvement was made. The patient was
promptly started on corticosteroids, an angiotensin
converting enzyme (ACE) inhibitor and a β-blocker.
Unfortunately, shortly thereafter he developed a
right foot drop secondary to partial right common
peroneal nerve damage, which initiated the start
of intravenous cyclophosphamide. A cardiac magnetic resonance (CMR) scan performed 1 week
later showed an improvement in systolic function
to 55% and confirmed the myocardial involvement
suggested by the transthoracic echocardiogram.
On T2-weighted imaging there was evidence of circumferential enhancement indicative of myocardial
edema. Following the administration of gadolinium,
patchy areas of late enhancement were noted
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Fig. 3 Transthoracic echocardiogram showing thickening of the left
ventricular walls, a “speckled” texture to the myocardium in the
parasternal long axis (a) and apical four-chamber views (b). (c) A
pulsed Doppler sample taken at the tips of the mitral valve leaflets
demonstrating restrictive diastolic filling of the left ventricle. (d) A
reduction in myocardial systolic velocity at the lateral mitral valve
annulus indicative of reduced longitudinal axis function. LA, left
atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.
within the apex, anterior, septal and inferior walls
with a transmurality of 25%. In addition, there were
patches of intramyocardial late gadolinium enhancement within the mid ventricular septal and inferior
walls (Figure 4).
The patient remained in hospital for a further 4
weeks during which his symptoms gradually improved.
He was discharged home 5 weeks after his initial presentation. At follow-up 3 months later, he had received
six infusions of cyclophosphamide, was on a reducing
dose of prednisolone and was being maintained on an
ACE inhibitor and β-blocker. His chest X-ray (Figure 5)
and echocardiographic findings (Figure 6) had resolved
and his foot drop had improved.
Heart Metab. (2014) 62:27–30Ronak
Rajani
Multimodality imaging of Churg–Strauss myocarditis
Fig. 4 Cardiac magnetic resonance scan showing some residual
thickening of the myocardium in diastole in the four-chamber (a)
and two-chamber (b) views along with a small rim of pericardial
fluid (PE); (c) and (d) show areas of late gadolinium enhancement
at the basal mid septal, basal inferior, apical and apical anterior
segments (white arrows).
Discussion
In 1951, Churg and Strauss described a syndrome characterized by asthma and a “strikingly
uniform clinical picture of fever and eosinophilia,
and symptoms of cardiac failure, renal damage
and peripheral neuropathy resulting from vascular
embarrassment in various systems and organs”
[1]. This entity was known as “Churg–Strauss
syndrome” for many years but this has now been
replaced by “eosinophilic granulomatosis with
polyangiitis” (EGPA) [2].
The American College of Rheumatology has identified six criteria for the disease [3]. When at least four of
these six criteria are met (asthma, eosinophilia >10%,
neuropathy, non-fixed pulmonary infiltrates, paranasal
sinus abnormalities and extravascular eosinophil infiltration) a vasculitis can be classified as being EGPA with
a sensitivity of 85% and specificity of 99.7% [3]. EGPA
is classically described as having three main sequential
phases [4]. The first phase, or prodromal phase, usually
consists of allergic rhinitis, nasal polyposis and airway
irritability. The second phase, or eosinophilic phase, is
characterized by peripheral blood eosinophilia organ
involvement. The third phase, or vasculitic phase, is
accompanied by clinical manifestations as a result of
systemic necrotizing vasculitis.
Fig. 5 Chest X-ray performed 6 weeks after the start of treatment
demonstrating an improvement of the initial radiographic appearances.
Fig. 6 Transthoracic echocardiogram performed 3 months after
the start of treatment demonstrating a normal wall thickness of the
myocardium and myocardial texture (a and b). Return to normal of
diastolic filling (c) and longitudinal axis function (d). LA, left atrium;
LV, left ventricle; RA, right atrium; RV, right ventricle.
Cardiac involvement
Cardiac involvement can occur in up to 62% of
patients with EGPA [5] and is more common in
ANCA-negative patients [6, 7]. Cardiac manifestations include restrictive or dilated cardiomyopathy,
myocarditis, arrhythmias, valvular abnormalities,
arrhythmias and sudden death. Dennert et al studied
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Ronak Rajani
Multimodality imaging of Churg–Strauss myocarditis
32 consecutive patients in remission and found that
62% of patients had evidence of cardiac involvement.
Clinical symptoms were present in 25%, major ECG
abnormalities in 13%, echocardiographic abnormalities in 50% and CMR abnormalities in 62% of
patients. The commonest ECG abnormality was the
presence of T-wave changes (50%), while on echocardiography wall motion abnormalities (41%). CMR
imaging was concordant with the echocardiographic
findings in demonstrating wall motion abnormalities
in 47% of patients. In addition, there was evidence of
regional fibrosis in 22% and global fibrosis in 25% of
patients. Fibrosis was only found in patients who had
other cardiac abnormalities, and there was a good
agreement between echocardiography and CMR
imaging when fibrosis was excluded (sensitivity 88%
and specificity 81%, respectively).
In general, the prognosis of EGPA is considered
to be good, with an overall 10-year survival rate of
81–92% [8]. Up to 50% of EGPA mortality is caused
by cardiac involvement, with up to 39% of patients
dying during the acute phase of the disease [8]. In a
recent study of 383 patients with EGPA followed up
for a mean duration of 66.8 months, cardiac involvement determined by clinical evaluation, an ECG and
echocardiography remained the greatest predictor of
death with a hazard ratio of 4.11 (95% CI 1.96–8.60)
[6]. Although there remains considerable interest in
the detection of subclinical cardiac involvement with
CMR and positron emission tomography, further
longitudinal studies are required to determine their
prognostic value [9]. Until these data are available,
early clinical, ECG and echocardiographic screening
is indicated for all patients with EGPA to detect early
cardiac involvement.
Treatment
The choice of initial therapy is usually determined
by the patient’s prognostic profile, as defined by the
five-factor score (FFS) [10]. This includes cardiac,
gastrointestinal and central nervous system involvement with proteinuria greater than 1 g/24 hours
and creatinine greater than 140 μM/L. Patients with
an FFS score of 1 or greater usually have a worse
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Heart Metab. (2014) 62:27–30
prognosis and are treated with corticosteroids and
immunosuppressants. Corticosteroids are usually
used in isolation for patients with an FFS score of
0. Although remission rates remain high with this
approach, controversy still exists as to how many
pulses of intravenous cyclophosphamide should
be used, and relapse rates remain high for those
patients receiving reducing doses of corticosteroids
alone. There are no systematic trials evaluating
the use of ACE inhibitors and β-blockers in EGPAassociated cardiac involvement.
Conclusions
The current case reports the multimodality imaging
findings of a patient who presented with EGPA and
demonstrates the value of early echocardiographic
screening in these patients to identify early cardiac
involvement to guide treatment.
REFERENCES
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