Fibroelastoma: a rare cause of cardioembolic stroke in the young

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Abstract

Objectives

The investigation and management of stroke in a young person remains challenging. The majority do not have traditional risk factors and the mechanism of stroke can be unclear. We present a case of a 34-year-old East Timorese woman who had major sequential strokes caused by a cardiac fibroelastoma. We reviewed the current literature on this rare condition and its cardioembolic stroke propensity.

Methods

We searched PubMED/OVID databases with the term ‘fibroelastoma’. We evaluated the epidemiology, diagnosis, complications and treatment of fibroelastoma.

Results

Fibroelastoma is the second most common cardiac tumour arising from or attached to valvular structures.1 Autopsy series estimate an incidence of 0.33%.2 With advances and availability of cardiac imaging, these tumours are more readily detected even in the asymptomatic patient. Transoesophageal echocardiogram (TOE) and cardiac MRI have greater resolution in identifying the tumour, as a proportion are missed on transthoracic echocardiogram (TTE).3 Fibroelastoma has great thrombogenic tendency; systemic embolisation is high with stroke risk estimated at 30%–40% per annum3,4, making stroke the most frequently encountered presentation of fibroelastoma followed by arrhythmia, heart failure and sudden cardiac death. High risk tumour features are those that are (1) freely mobile, (2) greater than 1 cm and (3) located on the left side of heart.2 In symptomatic patients there is compelling evidence to support surgical excision of the tumour in a timely manner. In those asymptomatic, the data is unclear. However given the relatively safe surgical profile and detrimental consequences, it is advisable to operate. Anticoagulation is prudent while awaiting surgery.

Conclusions

Fibroelastoma is a rare but important cause of cardioembolic stroke. Often these tumours are missed on routine TTE. A high index of suspicion is needed and young patients with cardioembolic strokes should be considered for TOE and cardiac MRI. Treatment with surgical resection is warranted to reduce future risk of stroke.

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