Papillary fibroelastoma of the aortic valve: occlusion of the left main coronary artery.

Abstract

A 53-year-old woman with palpitations and respiratory distress as her main symptoms, which had resulted in a cardiac arrest, was referred to the emergency room. Coronary angiography (CAG) with percutaneous cardiopulmonary support (PCPS) was performed, but no severe stenosis was found. The patient was diagnosed with vasospastic angina, treated medically, and discharged to her home. Six months later, however, during hospitalization for sick sinus syndrome, she suddenly developed runs of ventricular tachycardia. Therefore, CAG with PCPS was again carried out and revealed an occlusion of the left main trunk (LMT) of the coronary artery (Panel A, arrow). Intravascular ultrasound imaging was also performed (Panel B), and a plausible thrombus formation associated with coronary spasm was diagnosed. An emergency surgical intervention was therefore carried out. Intraoperative transoesophageal echocardiography imaging showed both a large mass at the aortic valve that may have occluded the LMT (Panel C, Supplementary data online, Video S1), and a previously undiagnosed severe aortic valve regurgitation caused by the mass. Aortic cross-clamping and a cardiac arrest were conducted; a huge mass originating from the right coronary cusp (RCC) of the valve was found (Panels D and E, arrowheads) and resected. No thrombus formation in the coronary artery was observed. Post-operatively, the patient's angina resolved without any sign of aortic regurgitation. The pathology report revealed a papillary fibroelastoma (PFE). This series of symptoms was caused by embolization within the coronary artery and the presence of a huge PFE, which together caused this rare condition.