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Cardiac involvement in a 44-year-old lady with Churg-Strauss Syndrome

clinical, diagnostic and pathophysiological challenges

Case Presentation:

A 44-year-old lady suffered from neck pain for about four weeks. In spite of treatment with analgetics, her symptoms worsened and she started to suffer from joint pain and headache that was sometimes accompanied by visual disturbances. Presentation at a neurological department revealed normal findings regarding cerebral magnetic resonance imaging as well as liquor diagnostics. However, routine blood analysis revealed an elevated troponin level and the patient was immediately transferred to our hospital for further cardiac work-up with the suspicion of a troponin-positive acute coronary syndrome.

Pericardial Disease

A thorough cardiac anamnesis revealed the presence of angina pectoris (AP) at rest and on exercise in addition to recurrent palpitations primarily occurring at rest for a couple of weeks. She had a history of pneumonia, recurrent sinusites and suspected asthma with known intolerance to medications such as acetylsalicylic acid (ASS) and metamizole. Her family history was positive for coronary artery disease (CAD), since both, her father (with 63 yrs of age) and her sister (with 50 yrs of age), had experienced an acute myocardial infarction (MI).
During physical examination, her heart rate was 110 bpm, her blood pressure 115/80 mmHg and she had no elevated temperature. Her physical exam was normal – apart from a slightly swollen eye lid. Blood analyses revealed an elevated erythrocyte sedimentation rate (75 mm/h; normal <15 mm/h), an elevated leukocyte count (16.700 /mm³) with 3.9% eosinophilic granulocytes (normal 1% to 6%) and an elevated C-reactive protein (CRP) level of 10.6 mg/dL (normal 0.1 to 0.5 mg/dL), respectively. Regarding her cardiac enzymes, the total creatine kinase level was 391 U/L (normal <190 U/L), the creatine kinase-MB fraction level was 58 U/L (normal <25 U/L), the troponin T level was 1.03 ng/mL (normal <0.03 ng/mL), and the N-terminal pro-brain natriuretic peptide level was 9,779 pg/mL (normal <450 pg/mL). Her resting ECG (at presentation) demonstrated a tachycardic sinus rhythm with ST-segment depression in the inferolateral leads.

Description of the problems

Obviously, this patient presented with a non-ST-elevation myocardial infarction (NSTEMI) and was suffering from typical as well as atypical symptoms of AP. Considering her positive family history of CAD, an ischemic atherosclerotic event had to be suspected. However, since the patient had also a history of asthma, joint pain and recurrent sinusitis in addition to an elevated leukocyte count and erythrocyte sedimentation rate, Churg-Strauss Syndrome (CSS) with potential cardiac involvement was also suspected according to the diagnosis criteria of the American College of Rheumatology.


What was the cause of NSTEMI in this patient?
Why did this patient suffer from cardiac (and other) symptoms?
Which diagnostic approach had to be chosen in such a clinical constellation?
Which diagnostic procedures were appropriate regarding further diagnostic work-up?

Answers and results

Since this patient presented with a NSTEMI and recurrent resting chest pain, she was first taken to the catheterization lab and underwent diagnostic coronary angiography. Coronary angiograms demonstrated normal coronary arteries without any significant luminal obstructions. Since coronary vasomotion disorders such as epicardial coronary spasm or microvascular dysfunction are important differential diagnoses for atypical as well as typical chest pain in young female patients without CAD, an additional coronary vasomotion test with intracoronary acetylcholine (ACH) provocation was performed. With increasing doses of ACH infusion into the left coronary artery (LCA) the patient felt the same chest pain as she did at home while diffuse coronary spasm in the LCA was observed (Fig. 1) that was accompanied by increased ST-segment depressions in the inferolateral leads.


Figure 1: LCA angiograms pre- (left) and post-acetylcholine infusion

After intracoronary infusion of nitroglycerin, the patient’s symptoms relieved, the diffuse coronary spasm disappeared and ECG changes normalised. Hence, while obstructive CAD was ruled out, coronary spasm was identified as a potential mechanism causing chest pain in this patient.
Since coronary epicardial spasm and/or microvascular dysfunction can occur in case of acute myocardial inflammation or in case of chronic fibrotic diseases with progressive perivascular and myocardial fibrosis, a cardiovascular magnetic resonance (CMR) study was performed in order to further evaluate the presence of inflammatory and/or structural myocardial changes. The CMR study was performed on a clinical scanner (1.5-T Aera, Siemens, Germany). CMR cine images demonstrated a mildly hypertrophied left ventricle (interventricular septum diameter 13mm) with reduced left ventricular systolic function (ejection fraction 49%) and rather diffuse hypokinesia. Contrast imaging was performed after intravenous application of 0.15 mmol/kg gadopentetate-meglumine (Magnograf) by use of an inversion-recovery gradient-echo technique. Extensive areas of late gadolinium enhancement (LGE) indicative of myocardial necrosis and/or fibrosis were detected in the subendocardium of the left ventricular wall as well as in the septum (distributed in the subendocardial and intramural myocardium) as demonstrated in Fig. 2. Such a LGE pattern is not typical for myocardial inflammation due to (viral) myocarditis, but similar patterns have been recently described in patients with CCS. However, the exact underlying pathomechanism for such a distribution of myocardial damage in CCS patients is still unclear.


Figure 2: CMR images

Considering the paramount therapeutic value of proving cardiac involvement in case of CCS, endomyocardial biopsy was performed in this patient. Histopathological biopsy results revealed a severe myocardial inflammation with eosinophilic infiltration confirming the suspected diagnosis of CCS with cardiac involvement (Fig. 3). Moreover, intramyocardial arterioles with extensive perivascular fibrosis and media proliferation were observed (Fig. 4) suggesting a chronic microvasculopathy in addition to the diagnosis of eosinophilic myocarditis. Hence, an immunosuppressive therapy with steroids was started. Moreover, the patient was put on an ACE-inhibitor and statin aiming at improving myocardial and coronary wall remodelling. In addition, a low-dose ß-blocker therapy (due to the reduced left ventricular function) as well as a symptomatic therapy with nitrates was recommended. Only a few days later, leukocyte counts and CRP level had normalised and her chest pain already improved. A follow-up CMR study was scheduled.


Cardiac involvement in patients with CCS may not only be associated with eosinophilic myocarditis but also with chronic microvasculopathy with arteriolar vessel wall fibrosis. Consequently, patients with CCS and cardiac involvement may suffer from typical as well as atypical chest pain due to a) myocardial inflammation and/or b) coronary spasm and/or c) microvascular dysfunction. We hypothesize that subendocardial and/or intramural myocardial damage which is a characteristic finding in patients with CCS (and that can be non-invasively detected by CMR), is not only caused by eosinophilic myocardial inflammation but also due to coronary vasomotion disorders such as coronary spasm and/or microvascular dysfunction.

Notes to editor

Presented by:
Sonja Sieprath, Karin Klingel, Reinhard Kandolf, Udo Sechtem and Ali Yilmaz
Cardiology Department.
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.