Multimodality imaging in the case of fibroelastoma
Doolub G, Dayer M, Gosling O.
Department of cardiology, Musgrove Park Hospital
A 59-year-old lady with a background of parietal infarct and paroxysmal atrial fibrillation presented to cardiology clinic with exertional breathlessness and chest tightness. In view of her family history of ischaemic heart disease she was referred for a CT coronary angiogram. This revealed a mass lesion on the right coronary cusp of the aortic valve. Coronaries were normal.
A transoesophageal echocardiogram confirmed a 9 x 9mm aortic valve fibroelastoma. There was no evidence of aortic regurgitation or stenosis, and the remaining aortic valve leaflets and valves were normal. Left ventricular systolic function was mildly impaired (ejection fraction 49%). She was started on a beta-blocker and ACE-inhibitor, and after MDT discussion, was referred for surgery.
Fibroelastomas are rare primary cardiac masses affecting mainly the cardiac valves. They are the third most frequent type of primary cardiac tumour, after atrial myxomas and lipomas1. Characteristically these tumours have elongated papillary fronds, reminiscent of a sea anemone2. Layers of mucopolysaccarides and connective tissue are typical histological features.2 They tend to affect the aortic valve more commonly (44.4%) than the mitral valve (36.4%)2.
Figure 1. Transoesophageal imaging in long (A) and short (B) axis of a large aortic valve mass (arrow) subsequently identified as a fibroelastoma. ECG-gated contrast-enhanced CT (C) reveals a pedunculated low attenuation mass whereas reconstructed CT image (D) demonstrates the papillary fronds.
Multiple choice questions
- What are the differentials for valvular masses?
- Lambl’s excrescence
- Infective vegetation
- All of the above
CORRECT ANSWER: E-All of the above should be considered in patients presenting with a new valvular mass.
- What are the main complications arising from fibroelastomas?
- Coronary ischaemia
- Coronary occlusion
- Sudden cardiac death
- All of the above
CORRECT ANSWER: E.
Although fibroelastomas are benign histologically, there is a high predilection for systemic embolization, giving rise to all of the above complications. Sudden cardiac death can result from a ‘ball valve’ phenomenon at the coronary ostium.3
- What are the two most common presentations associated with aortic valve fibroelastoma?
- Sudden death
- Limb ischaemia
- Myocardial infarction
CORRECT ANSWERS: B and D
Aortic valve involvement and a highly mobile fibroelastoma are associated with higher incidence of embolization and death2. Stroke is the most common presentation related to mitral valve fibroelastomas.2
- Michael H. Crawford MD, FACC, John P. DiMarco MD, PhD, Walter J. Paulus(2009) Cardiology. 3rd Philadelphia: Elsevier
- De Visser RN1, van Mieghem C, van Pelt NC, Weustink AC, Kerker JP, Galema TW. Papillary fibroelastoma of the aortic valve and coronary artery disease visualized by 64-slice CT. Nat Clin Pract Cardiovasc Med. 2008 Jun;5(6):350-3.
- Gowda RM et al. (2003) Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J146: 404–410.