J Cardiovasc Ultrasound.  2015 Mar;23(1):40-43. 10.4250/jcu.2015.23.1.40.

Cardiac Involvement of Churg-Strauss Syndrome as a Reversible Cause of Dilated Cardiomyopathy

Affiliations
  • 1Department of Cardiovascular Medicine, Chonnam National University Hospital, Gwangju, Korea. christiankyehun@hanmail.net
  • 2Department of Radiology, Chonnam National University Hospital, Gwangju, Korea.

Abstract

A 31-year-old male who had been treated for Churg-Strauss syndrome (CSS) presented with sudden onset of dysarthria. Brain magnetic resonance imaging (MRI) showed acute multifocal bilateral cerebral infarctions suggesting embolic causes. Cardiac MRI showed dilated cardiomyopathy with severe biventricular dysfunction with intracardiac thrombi, and multiple high signal intensity spots in myocardium of the left ventricle with multifocal delayed enhancement suggesting multifocal myocarditis due to small vessel vasculitis associated with CSS. After anticoagulation therapy, treatments for heart failure, and immunosuppressive therapy including parenteral steroids and cyclophosphamide to control CSS, the symptoms and signs of heart failure and cardiac function of the patient were improved. Considering the pathophysiologic mechanism of cardiac involvement in CSS, immunosuppressive therapy to control the disease activity of CSS should be taken into account, besides usual management for heart failure.

Keyword

Churg-Strauss syndrome; Dilated cardiomyopathy; Prognosis

MeSH Terms

Adult
Brain
Cardiomyopathy, Dilated*
Cerebral Infarction
Churg-Strauss Syndrome*
Cyclophosphamide
Dysarthria
Heart Failure
Heart Ventricles
Humans
Magnetic Resonance Imaging
Male
Myocarditis
Myocardium
Prognosis
Steroids
Vasculitis
Cyclophosphamide
Steroids

Figure

  • Fig. 1 Magnetic resonance image (MRI) findings of the brain. MRI image findings of the brain reveals multi-focal acute cerebral infarctions (indicated by white arrows).

  • Fig. 2 Electrocardiogram findings. Electrocardiogram findings on admission (A) and at discharge (B).

  • Fig. 3 Chest X-ray and echocardiographic findings. Chest X-ray and echocardiographic findings before (A-D) and after treatment (E-H). Cardiomegaly and pulmonary infiltrates on chest X-ray, and left ventricular (LV) dysfunction on echocardiography were improved after 3 months of medical therapy. Asterisk (*) indicated LV thrombi.

  • Fig. 4 Magnetic resonance image findings of the heart. Magnetic resonance imaging of the heart reveals multiple high signal intensity in subepicardial and subendocardial area of left ventricular myocardium with multifocal delayed enhancement (arrowheads). Consolidative lesions with high signal intensity in right middle lobe were also noted (asterisk).


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