Papillary Fibroelastoma - An Unusual Cause of Angina Pectoris

Introduction

Papillary fibroelastoma is a rare heart disease that is classified as one of the primary benign cardiac tumours. The tumour originates from the endocardium, most commonly from the area of the valves of the left heart. Its typical sea anemone-like appearance results from the forked surface with numerous fronds sometimes covered by tiny mural thrombi ([Fig. 1]) [[1], [2], [3], [4]].

Fig. 1 Extirpated papillary fibroelastoma - a typical sea anemone-like appearance.

Papillary fibroelastoma may be found incidentally on echocardiographic examination or at autopsy. Systemic tumour embolisation is frequently a cause of severe symptoms. Tumour fragments or mural thrombi embolise predominantly into the cerebral or coronary arteries [[1], [3], [4], [5]]. Due to its location on the valve endocardium, the tumour may interfere with the valve function, and, in the case of aortic valve involvement, myocardial ischaemia may occur from intermittent obturation of a coronary artery ostium [[4], [5]]. For these reasons, early surgical treatment is recommended, even in a case of incidental diagnosis in asymptomatic patients [[3]].

References

• 1 Kuroczynski W, Hake U, Pruefer D. et al . Aortic valve papillary fibroelastoma.  J Heart Valve Dis. 2005;  14 140-142
• 2 Travis W D, Brambilla E, Müller-Hermelink H K. et al .Tumours of the Lung, Pleura, Thymus and Heart. WHO Classification of Tumours. Lyon; IARC Press 2004: 263-265
• 3 Yamamoto S, Fuchimoto K, Tanaka A. et al . Papillary fibroelastoma of the aortic valve: report of a case.  Surg Today. 2002;  32 354-358
• 4 Vagefi P A, Bates N R, Ptaszek L M. et al . Valve-sparing excision of aortic valve papillary fibroelastoma.  J Thorac Cardiovasc Surg. 2006;  131 1177-1180
• 5 Boulmier D, Ecke J E, Verhoye J P. Recurrent myocardial infarction due to obstruction of the RCA ostium by an aortic papillary fibroelastoma.  J Invasive Cardiol. 2002;  14 686-688