Key result
Surgical debulking and mitral valve replacement were performed for a primary left atrial intimal sarcoma causing severe mitral stenosis, but the patient expired from acute congestive heart failure.
Why the study?
To describe an uncommon presentation of primary left atrial intimal sarcoma disguised as functional mitral stenosis, along with its surgical and medical management.
Case Report (n=1)
No
This case highlights a rare presentation of primary left atrial intimal sarcoma causing functional mitral stenosis and rapid progression to fatal heart failure.
Consider malignancy in atypical LA masses causing mitral stenosis; leaves open optimal therapy for MDM2-positive cardiac sarcomas.
A 66-year-old female with a 1-month history of increasing fatigue, dyspnea on exertion, and palpitations presented with clinical signs of heart failure. Chest computed tomography (CT) revealed a large, mobile left atrial (LA) mass attached to the mitral valve causing severe mitral stenosis and mitral regurgitation. The mass was surgically debulked and the mitral valve was replaced. Pathology revealed a poorly differentiated malignant spindle cell neoplasm with diffuse nuclear positivity of MDM2 and multifocal positivity of CDK4, consistent with intimal sarcoma. This case seeks to describe an uncommon presentation of a rare malignancy, and the surgical and medical management of the disease.
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Visinoni et al. (2023) conducted a case report in Primary Left Atrial Intimal Sarcoma (n=1). Surgical debulking and mitral valve replacement was evaluated on Clinical course and survival. Surgical debulking and mitral valve replacement were performed for a primary left atrial intimal sarcoma causing severe mitral stenosis, but the patient expired from acute congestive heart failure.
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