Abstract 4135215: Eosinophilic Coronary Periarteritis (ECPA): An Under-recognized Coronary Disease with Fatal Consequences

Clinical presentation: A 50-year-old male with a paraesophageal hernia and grade D esophagitis presented to the ER with complaints of heartburn. He subsequently went into cardiac arrest and was diagnosed with a hemopericardium with tamponade physiology from a ruptured right coronary artery (RCA). Imaging Findings: Initial CT of the chest found a 1.5x1.5 cm dilation of the proximal RCA (Fig1A). Post cardiac arrest, repeat CT of the chest revealed an interval increase of the proximal RCA measuring 3.5x3.5 cm and a moderate-to-large hemopericardium (Fig1B). An emergent limited TEE demonstrated a large circumferential hemopericardium and biventricular standstill. Decision-making: In the ER, the initial symptoms resolved and he remained under observation. Hours later, he became bradycardic with a heart in the 20s and was found unresponsive. ACLS was activated and the patient was emergently cannulated and ECMO was initiated due to refractory cardiac arrest. Despite efforts from a multidisciplinary team including cardiology, cardiothoracic surgery and cardiac critical care, ROSC could not be achieved thus portending a poor prognosis. The family decided to withdraw care and the patient expired. Autopsy revealed a 925 mL hemopericardium from a ruptured 3.5 cm severely aneurysmal RCA and other remarkable findings included a 2 cm proximal aneurysm of left anterior descending artery and a 1 cm proximal aneurysm of left circumflex artery (Fig2A,B). Genetic testing for connective tissue disease was negative. Histopathology showed an eosinophilic predominant inflammatory infiltrate of the adventitia suggesting periarteritis (Fig2C) without signs of vasculitis in other organs. Conclusion: ECPA (eosinophilic coronary periarteritis) is a fatal entity with reported cases of sudden cardiac death and findings of vessel medial degeneration and coronary dissection. This case raises awareness of an under-recognized condition with limited guidance in diagnostic and therapeutic interventions.