PAPILLARY FIBROELASTOMA ON THE AORTIC VALVE – POTENTIAL RISK OF SUDDEN DEATH

An 80 year old woman was admitted to hospital with chest pain and syncopes. The diagnosis of acute myocardial infarction was made.Echocardiography showed hypokinesis of the diaphragmatic wall and apical akinesis Coronary angiogramy did not reveal any serious stenosis. A few months later, having chest pain, she calls for an ambulance. Subsequently she falls into unconsciousness with asystoly . Cardiopulmonary resuscitation was unsuccessful. Myocardial infarction is considered as a cause of death.
 The autopsy revealed a mass of  papillary fibroelastoma (PF) 18mm in size connected to the posterior aortic cusp.The tumour was composed of papillary projections whose appearance resembles a sea anemone.The tumour was very mobile and could reach both right and left coronary ostium easily. There were myofibrotic changes in the posterolateral myocardial wall and both papillary muscles.
 Cardiac PF is a benign tumour comprising 10% of all primary cardiac tumours. Mean age of the patients is 60 years. 90 % of PF at cardiac valves.
The majority have been observed on the aortic and mitral valves. The clinical diagnosis of PF can be difficult, because it can mimic a variety of underlying diseases. Tumour on the right or left coronary cups of the aortic valve can cause angina, syncope or sudden death.
In our case the PF was located on the aortic valve.The tumour was on a stalk long enough to reach both coronary ostia easily.Then possibly the myocardial infarction of posterolateral wall was evoked by occluded coronary artery by the tumour mass. The infarction of the posterolateral wall was also noticed in the autopsy. There were no serious coronary stenosis found in the coronarography 6 months before death and there were also no occlusions of coronary arteries found during the autopsy. We reached the conclusion that the sudden death was caused by papillary fibroelastoma.