2005Journal of Investigative MedicineRequires access

78 CARDIAC PAPILLARY FIBROELASTOMA PRESENTS AS AN ACUTE EMBOLIC STROKE IN A YOUNG AFRICAN AMERICAN MALE

William Campbell, Ervin R. Fox

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Abstract

Transesophageal echocardiography (TEE) is an important diagnostic tool for young patients with stroke. Cardiac papillary fibroelastomas (CPFs) are relatively rare intracardiac tumors that can arise from any of a number of endocardial surfaces and potentially cause stroke. Cerebral embolization, coronary embolization, sudden death, pulmonary embolization and retinal embolization have all been reported with CPF in the medical literature. TEE has played a major role in the diagnosis and management of these cases. We report a case of a 35-year-old African American male with no prior medical history who presented with an acute onset of seizure activity associated with bladder incontinence, right-sided weakness and expressive aphasia. Physical examination was only remarkable for weakness in the right upper and lower extremities and expressive aphasia. Computed tomography scan of the head without contrast demonstrated a hypodensity in the cortex and underlying white matter within the left middle cerebral artery distribution. The carotid Doppler and transcranial Doppler results were unrevealing and the laboratory workup for a hypercoaguable state was unremarkable. Blood cultures were negative. A TEE was performed to search for a cardiac source of embolus. The TEE revealed a 0.5 cm × 0.5 cm mobile mass on the atrial side of the mitral valve attached to the posterior leaflet by a short stalk. The differential included papillary fibroelastoma, less likely vegetation or atypical myxoma. No other intracardiac source of embolus was noted. The patient underwent cardiothoracic surgery where a small frond-like mass was excised from the middle scallop of the posterior leaflet. Histology demonstrated a central avascular core of dense connective tissue surrounded by a peripheral layer of loose myxoid connective tissue. The mass was covered by a hyperplastic endocardial cell lining. These findings were typical for CPF. Fibrin deposition was noted at the tip of the tumor. The patient9s neurologic symptoms improved during the remainder of his hospital course. He was discharged on post-op day seven with outpatient physical and speech therapy. This case underscores the importance of TEE in identifying the cause of stroke in young patients. Furthermore the case shows the importance of including CPF in the differential for embolic sources of stroke as its diagnosis significantly affects management. Surgery is suggested for patients with CPF and stroke and for asymptomatic patients with left sided lesions or large tumors (> 1 cm) if the cumulative risk of suffering an embolic event outweighs the risk of cardiothoracic surgery.

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Transesophageal echocardiography (TEE) is an important diagnostic tool for young patients with stroke. Cardiac papillary fibroelastomas (CPFs) are relatively rare intracardiac tumors that can arise from any of a number of endocardial surfaces and potentially cause stroke. Cerebral embolization, coronary embolization, sudden death, pulmonary embolization and retinal embolization have all been reported with CPF in the medical literature. TEE has played a major role in the diagnosis and management of these cases. We report a case of a 35-year-old African American male with no prior medical history who presented with an acute onset of seizure activity associated with bladder incontinence, right-sided weakness and expressive aphasia. Physical examination was only remarkable for weakness in the right upper and lower extremities and expressive aphasia. Computed tomography scan of the head without contrast demonstrated a hypodensity in the cortex and underlying white matter within the left middle cerebral artery distribution. The carotid Doppler and transcranial Doppler results were unrevealing and the laboratory workup for a hypercoaguable state was unremarkable. Blood cultures were negative. A TEE was performed to search for a cardiac source of embolus. The TEE revealed a 0.5 cm × 0.5 cm mobile mass on the atrial side of the mitral valve attached to the posterior leaflet by a short stalk. The differential included papillary fibroelastoma, less likely vegetation or atypical myxoma. No other intracardiac source of embolus was noted. The patient underwent cardiothoracic surgery where a small frond-like mass was excised from the middle scallop of the posterior leaflet. Histology demonstrated a central avascular core of dense connective tissue surrounded by a peripheral layer of loose myxoid connective tissue. The mass was covered by a hyperplastic endocardial cell lining. These findings were typical for CPF. Fibrin deposition was noted at the tip of the tumor. The patient9s neurologic symptoms improved during the remainder of his hospital course. He was discharged on post-op day seven with outpatient physical and speech therapy. This case underscores the importance of TEE in identifying the cause of stroke in young patients. Furthermore the case shows the importance of including CPF in the differential for embolic sources of stroke as its diagnosis significantly affects management. Surgery is suggested for patients with CPF and stroke and for asymptomatic patients with left sided lesions or large tumors (> 1 cm) if the cumulative risk of suffering an embolic event outweighs the risk of cardiothoracic surgery.

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Available abstract

Transesophageal echocardiography (TEE) is an important diagnostic tool for young patients with stroke. Cardiac papillary fibroelastomas (CPFs) are relatively rare intracardiac tumors that can arise from any of a number of endocardial surfaces and potentially cause stroke. Cerebral embolization, coronary embolization, sudden death, pulmonary embolization and retinal embolization have all been reported with CPF in the medical literature. TEE has played a major role in the diagnosis and management of these cases. We report a case of a 35-year-old African American male with no prior medical history who presented with an acute onset of seizure activity associated with bladder incontinence, right-sided weakness and expressive aphasia. Physical examination was only remarkable for weakness in the right upper and lower extremities and expressive aphasia. Computed tomography scan of the head without contrast demonstrated a hypodensity in the cortex and underlying white matter within the left middle cerebral artery distribution. The carotid Doppler and transcranial Doppler results were unrevealing and the laboratory workup for a hypercoaguable state was unremarkable. Blood cultures were negative. A TEE was performed to search for a cardiac source of embolus. The TEE revealed a 0.5 cm × 0.5 cm mobile mass on the atrial side of the mitral valve attached to the posterior leaflet by a short stalk. The differential included papillary fibroelastoma, less likely vegetation or atypical myxoma. No other intracardiac source of embolus was noted. The patient underwent cardiothoracic surgery where a small frond-like mass was excised from the middle scallop of the posterior leaflet. Histology demonstrated a central avascular core of dense connective tissue surrounded by a peripheral layer of loose myxoid connective tissue. The mass was covered by a hyperplastic endocardial cell lining. These findings were typical for CPF. Fibrin deposition was noted at the tip of the tumor. The patient9s neurologic symptoms improved during the remainder of his hospital course. He was discharged on post-op day seven with outpatient physical and speech therapy. This case underscores the importance of TEE in identifying the cause of stroke in young patients. Furthermore the case shows the importance of including CPF in the differential for embolic sources of stroke as its diagnosis significantly affects management. Surgery is suggested for patients with CPF and stroke and for asymptomatic patients with left sided lesions or large tumors (> 1 cm) if the cumulative risk of suffering an embolic event outweighs the risk of cardiothoracic surgery.

Key concepts: Papillary fibroelastoma, Embolic stroke, Medicine, Cardiology, Stroke (engine), Internal medicine, Acute stroke, Cardiac Tumors

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