Allergy Asthma Respir Dis.  2015 Mar;3(2):159-163. 10.4168/aard.2015.3.2.159.

Heart transplantation in a patient with eosinophilic granulomatosis with polyangiitis known as Churg-Strauss syndrome

• Park S
• Kim T
• Kim HJ
• Shin B
• Park SY
• Kwon HS
• Moon HB
• Cho YS
• Kim TB

• KMID: 2168511
• DOI: http://doi.org/10.4168/aard.2015.3.2.159

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a rare systemic necrotizing vasculitis affecting small- to medium-sized vessels. EGPA is associated with severe asthma and eosinophilia. The most frequently involved organs are skin and peripheral nerves; however, EGPA may involve other organs, such as the gastrointestinal tract, kidney, and heart. Antineutrophil cytoplasm antibodies (ANCAs)-related abnormal immune reactions are known to be associated with EGPA, but only 30%-40% of patients have a positive marker of ANCA. ANCA-negative patients are at higher risk of cardiac involvement than ANCA-positive patients. Cardiac involvement is one of the leading causes of mortality and could be resistant to conventional treatment. Early treatment with steroid plus cyclophosphamide is important because it could give chances of restoration of cardiac function. For patients undergoing heart transplantation, we should consider the severity of cardiac disease and the presence of systemic diseases, including vasculitis. Here, we report a case of a 25-year-old EGPA patient with cardiac involvement who eventually received heart transplantation for progressive heart failure, although treated with systemic corticosteroid with cyclophosphamide. EGPA patients undergoing heart transplantion are rarely reported worldwide, and this is the first case report in Korea.

Keyword

Eosinophilicgranulomatous vasculitis; Churg-Strauss syndrome; Heart transplantation; Eosinophilia

MeSH Terms

Adult
Antibodies
Antibodies, Antineutrophil Cytoplasmic
Asthma
Churg-Strauss Syndrome*
Cyclophosphamide
Cytoplasm
Eosinophilia
Eosinophils*
Gastrointestinal Tract
Heart
Heart Diseases
Heart Failure
Heart Transplantation*
Humans
Kidney
Korea
Mortality
Peripheral Nerves
Skin
Vasculitis
Antibodies
Antibodies, Antineutrophil Cytoplasmic
Cyclophosphamide

Figure

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  Fig. 1 Initial electrocardiogram shows atrial fibrillation with rapid ventricular response and right bundle branch block.

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  Fig. 2 (A) Initial chest x-ray: Image shows cardiomegaly, increased pulmonary vascularity and small amount of pleural effusion. (B) Posttransplantation chest x-ray: Cardiomegaly is markedly improved.

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  Fig. 3 (A) Preoperative echocardiography shows severely dilated left ventricle, global hypokinesia with atrial fibrillation. (B) Postoperative echocardiography shows normal findings with ejection fraction of 57%.

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  Fig. 4 Trends of blood eosinophil levels and brain natriuretic peptide (BNP). EF, ejection fraction; QOD, every other day; bid, twice a day.

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