Author/Authors :
Chin, Re-I Saint Louis University School of Medicine, Saint Louis, USA , Monda, John J. Saint Louis University School of Medicine, Saint Louis, USA , Sheth, Maulik Department of Internal Medicine - SSM St. Mary’s Health Center, USA , Ogle, William Department of Cardiothoracic Surgery - SSM St. Mary’s Health Center, USA , Merenda, Gloria Department of Pathology - SSM St. Mary’s Health Center, USA , De, Debapriya Department of Internal Medicine - SSM St. Mary’s Health Center, USA
Abstract :
We describe a case of a young male without stroke risk factors who presented with a sudden onset of left-sided weakness, left hand
numbness, and left eye blurriness. CT scan of the head without contrast and diffusion-weighted MRI of the brain with contrast
revealed an ischemic stroke in the right middle cerebral artery distribution. Transesophageal echocardiography (TEE) revealed a
mobile pedunculated mass on the posterior surface of the mitral valve. This mass was resected and pathology showed a cardiac
papillary fibroelastoma (CPFE), which was determined to be the cause of the patient’s cardioembolic stroke. Further workup also
found that patient had microcytic anemia secondary to 𝛽-thalassemia intermedia, a rare hematologic disorder due to defective
hemoglobin synthesis. Recently, another case report suggested 𝛽-thalassemia major may underlie the pathogenesis of CPFE. 𝛽-
Thalassemia major causes a state of chronic inflammation and endothelial damage, which can mediate CPFE formation. Based
on literature review, this is the first case report of a CPFE in a patient with 𝛽-thalassemia intermedia. This hypothesis-generating
case report calls attention to the need for elucidating the relationship between CPFE and 𝛽-thalassemia in future studies to better
understand the diagnosis and management of a rare cardiac tumor.