Myocardial Infarction as the Initial Presentation of Cardiac Papillary Fibroelastoma　― Detection of a Mottled Mass Using Intravascular Ultrasound ―

A 76-year-old woman presented with sudden-onset chest pain. Electrocardiography demonstrated ST-segment elevation in the precordial leads and aV_R. Coronary angiography revealed an eccentric, severe stenosis at the left main trunk (LMT) ostium (Figure A). Thrombectomy was unsuccessful, and significant stenosis with an intravascular filling defect persisted after deployment of a stent to the LMT. Intravascular ultrasound (IVUS) revealed a critical stenosis caused by an iso-high-echoic mass with multiple hypoechoic “mottled” spots. Notably, the mass extended from the LMT ostium to the left coronary sinus of Valsalva (Figure B, Supplementary Movie). Transesophageal echocardiography demonstrated the mass originating from the aortic cusps (Figure C). The patient was referred for surgery. Intraoperative examination revealed a tumor originating from the commissure between the left and non-coronary aortic cusps, extending to the LMT ostium and entrapped within the stent (Figure D). Both the tumor and the stent were surgically resected, followed by aortic valve replacement. Gross and microscopic examinations confirmed the diagnosis of cardiac papillary fibroelastoma (PFE) (Figure E,F). At the 2-year follow-up, the patient remained free of disease recurrence.

Figure.

(A) Coronary angiography. (B) Intravascular ultrasound of the intracoronary (Left) and extracoronary (Right) arteries. An iso-high-echoic mass with multiple hypoechoic “mottled” spots (white arrows) is visualized. (C) Transesophageal echocardiography. (D) Intraoperative images. (E) The gross specimen of the tumor has a sea anemone-like appearance. (F) Microscopic examination reveals multiple avascular papillary fronds.

Although most cardiac PFE patients are asymptomatic, it can cause acute myocardial infarction. Coronary angiography can pose diagnostic challenges, particularly in differentiating cardiac PFE from thrombus and detecting tumors causing intermittent obstruction, as in the case reported by Kim et al.¹ in which myocardial infarction caused by cardiac PFE was diagnosed using optical coherence tomography (OCT). Although OCT offers high-resolution imaging, it is limited to the coronary lumen and requires blood displacement during examination, whereas IVUS allows continuous evaluation from the sinus of Valsalva to the coronary artery, as in this case.

Supplementary Files

Supplementary Movie

Please find supplementary file(s);

https://doi.org/10.1253/circj.CJ-24-0529

Reference

• 1.   Kim WC, Sumaya W, Elkhateeb OE. Fibroelastoma presenting with recurrent myocardial infarction and diagnosed with intracoronary imaging: A novel intracoronary image of fibroelastoma. Eur Heart J 2022; 43: 167.