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Twin Diseases: A Case of Cardiomyopathy Resembling ...
Twin Diseases: A Case of Cardiomyopathy Resembling Cardiac Sarcoidosis- Histologically Confirmed as Giant Cell Myocarditis
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This case report describes a 56-year-old previously healthy woman who initially presented with syncope, seizure-like activity, and arrhythmias including paroxysmal SVT, ventricular tachycardia, and complete heart block. She later developed chest pain, a new left bundle branch block, and elevated troponin. Cardiac catheterization showed non-obstructive coronary disease and reduced left ventricular ejection fraction (30–35%). Cardiac MRI revealed heterogeneous late gadolinium enhancement strongly suggestive of cardiac sarcoidosis, so she was treated with an ICD, high-dose prednisone, and amiodarone.<br /><br />Despite treatment, she rapidly worsened. Within a week she developed volume overload, then refractory VT storm, followed by cardiogenic shock requiring intra-aortic balloon pump support, ventilation, inotropes, ECMO, and ultimately orthotopic heart transplantation. Histology of the explanted heart showed myocyte dropout, granulation tissue, hemosiderin-laden macrophages, and T-cells, consistent with resolving giant cell myocarditis (GCM), with no evidence of cardiac sarcoidosis (CS).<br /><br />The report emphasizes that GCM and CS have major clinical and imaging overlap, especially on cardiac MRI and PET, but are distinct diseases. GCM typically progresses rapidly with severe heart failure and poor prognosis, while CS more often presents with conduction disease, arrhythmias, and slower progression. Definitive diagnosis requires endomyocardial biopsy. Histologic clues favoring GCM include disproportionate necrosis relative to inflammation and absence of well-formed granulomas; granulomas support CS.<br /><br />The authors conclude that early differentiation is critical because treatment and prognosis differ: CS is usually managed with prednisone, whereas GCM often requires steroid-sparing immunosuppressive therapy and may progress to transplant.
Asset Subtitle
Anam Ahmad
Meta Tag
Author List
Anam Ahmad, Balakrishna Ravella, Chinenye Udokwu, Edward J. Hurley, Keith A. Bernstein
Category
Clinical Vignettes
Concept
Giant Cell Myocarditis
Concept
Cardiac Sarcoidosis
Concept
Cardiac Biopsy
Concept
Corticosteroids
Concept
Nonischemic Cardiomyopathy
Distinguished
Non-Finalist
Presenter Organization
St. Lukes Hospital Chesterfield Missouri
Presenting Author
Anam Ahmad
Track
Adult
Keywords
giant cell myocarditis
cardiac sarcoidosis
ventricular tachycardia
complete heart block
cardiogenic shock
cardiac MRI
late gadolinium enhancement
endomyocardial biopsy
heart transplantation
immunosuppressive therapy
Giant Cell Myocarditis
Cardiac Sarcoidosis
Cardiac Biopsy
Corticosteroids
Nonischemic Cardiomyopathy
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