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Churg-Strauss syndrome associated with rapid deterioration of left ventricular diastolic dysfunction and conduction
Jung Yeon Chin1, Jeong Eun Yi, Ho-Joong Youn
1Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Insights
Churg-Strauss syndrome (CSS) can cause fatal eosinophilic endomyocarditis. This case highlights rapid worsening of heart dysfunction and conduction issues despite treatment for hypereosinophilia.
Area of Science:
- Cardiology
- Rheumatology
- Pathology
Background:
- Cardiac involvement in Churg-Strauss syndrome (CSS) significantly increases mortality risk.
- Eosinophilic endomyocarditis is a rare but severe manifestation of CSS.
Observation:
- A 75-year-old woman presented with symptoms suggestive of cardiac compromise.
- Diagnostic workup revealed intraventricular conduction delay, impaired biventricular diastolic function, apical thickening, and thrombi.
Findings:
- Endomyocardial biopsy confirmed eosinophilic infiltration, indicative of eosinophilic endomyocarditis secondary to CSS.
- Despite successful steroid treatment for hypereosinophilia, left ventricular diastolic function rapidly progressed to a restrictive pattern.
- The patient developed a ventricular escape rhythm and died on day 14.
Implications:
- This case underscores the potential for rapid and fatal cardiac deterioration in CSS-related eosinophilic endomyocarditis.
- Early recognition and aggressive management of cardiac manifestations are crucial for improving outcomes in CSS patients.
- The rapid progression of diastolic dysfunction and conduction abnormalities presents a unique challenge in managing this condition.
Abstract:
Cardiac involvement in Churg-Strauss syndrome (CSS) is a major cause of mortality. Here we report a case of a 75-year-old woman with eosinophilic endomyocarditis due to CSS. An electrocardiogram showed intraventricular conduction delay, and echocardiography showed an impaired relaxation pattern and biventricular apical thickening. Magnetic resonance imaging revealed subendocardial delayed enhancement with biventricular apical thrombi. Endomyocardial biopsy showed perivascular eosinophilic infiltration. Despite resolution of the hypereosinophilia after steroid therapy, her left ventricular (LV) diastolic function worsened into a restrictive pattern and she died with a ventricular escape rhythm on her 14th day in the hospital. This case is unusual in that there was rapid progression of the LV diastolic dysfunction and conduction disturbance due to CSS.
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