Multiple aortic valve papillary fibroelastomas causing recurrent cerebrovascular events

Abstract

Introduction

Papillary fibroelastomas (PFE) are benign, rare primary cardiac tumors often discovered incidentally during cardiac imaging or as part of a workup for thromboembolic or obstructive events. We present a case of multiple PFE on the aortic valve's right coronary and non-coronary leaflets, presenting as recurrent cerebrovascular events managed by resection with valve preservation.

Case Summary

A 53-year-old male presented with an acute right frontal cerebrovascular accident and a history of recurrent transient ischemic attacks. A transesophageal echocardiogram (TEE) revealed a 10 × 8 mm mass on the right coronary cusp of the aortic valve. Differential diagnosis included PFE versus thrombus. Following a neurology evaluation, the patient was anticoagulated and scheduled for urgent cardiac surgery. During surgery, an 8 × 7 mm mass was sharply excised from the right coronary leaflet's anterior surface. Further examination revealed additional masses: a 3 mm mass on the underside of the right coronary leaflet, a 2 mm mass on the non-coronary leaflet, and another 2 mm mass on its underside. All masses were excised, and pathology confirmed the diagnosis of PFE. The patient's post-operative course was unremarkable, and a pre-discharge transthoracic echocardiogram (TTE) demonstrated complete removal of the aortic valve masses with normal ventricular and aortic valve function.

Discussion

PFE are rare benign cardiac tumors with a significant risk of thromboembolic or obstructive events, including stroke. Grossly, they are attached to the endocardium by a short fibrous pedicle with multiple papillary fronds resembling a sea anemone. More than 95 % originate from the left heart, most commonly affecting the aortic valve. Diagnosis is primarily through echocardiography, with TEE being more sensitive than TTE. Surgical excision is curative, offering an excellent long-term prognosis.

Conclusion

All patients with suspected embolic cerebrovascular events should undergo echocardiographic evaluation to assess for potential cardiac etiologies. Surgical resection of PFE results in uniformly excellent clinical outcomes, with aortic valve preservation achievable in over 98 % of cases.