1355494 - Coronary Artery Ostial Obstruction Due to Papillary Fibroelastoma of the Aortic Valve: A Case Report

Description of Clinical Presentation: Cardiac papillary fibroelastoma (CPF) is a benign primary cardiac neoplasm, commonly found in men above 40 years old. The clinical presentation of CPF ranges from asymptomatic to embolism-related complications such as stroke, myocardial ischemia, infarction, or ventricular fibrillation. The acute coronary syndrome is a rare complication of CPF, which was reported only in a few cases in the medical literature. Hence, we report a case of a 50-year-old female with a CPF on the right coronary cusp of the aortic valve diagnosed with multi-modality imaging with a definitive diagnosis through histopathologic confirmation. The patient presented with acute onset of fatigue, diaphoresis, and vomiting. An initial electrocardiogram (ECG) demonstrated T wave inversion in aVL. Repeated ECG two hours later showed persistent T wave inversion in aVL with new T wave inversions in lead I and ST depression in V2-V6. Troponin levels were elevated from 3.6 ng/L to 1503 ng/L but the patient did not report chest pain, abdominal pain, or dyspnea.

Diagnostic Techniques and Their Most Important Findings: Computed tomography coronary angiography did not show any significant coronary stenosis but revealed a low attenuation node with 7 × 6 mm in dimension attached to the right coronary cusp of the aortic valve.  Cardiac magnetic resonance imaging (CMR) demonstrated elevated T1 maps of 1621 ms, T2 maps of 57 ms, and extracellular volume fraction value of 47 % in the inferior basal wall, inferior middle, and apical region. T1 and T2 maps delineated an area of myocardial edema in the right coronary territory with subendocardial myocardial infarct on the late gadolinium enhancement image. Transesophageal echocardiogram (TEE) showed a mobile pedicled tumor on the right coronary cusp of the aortic valve. Treatment was discussed among a multidisciplinary team and the CPF was surgically removed.

Learning Points from this Case: The acute coronary syndrome is a rare, but potentially fatal complication of cardiac papillary fibroelastoma (CPF). Multi-modality imaging is valuable in delineating the evaluation of the exact position, dimensions, nature of cardiac masses, diagnostic workup, and preliminary assessment before the surgery. There are no clear guidelines for the treatment of CPF.