SESSION TITLE: Monday Fellow Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Desmoid tumors are rare, fibrous tumors accounting for only 0.03% of neoplasms. They are locally invasive and associated with high reoccurrence rates. Left ventricular non-compaction (LVNC) is the arrest of normal cardiac compaction during embryonic development characterized by left ventricular trabeculae, intertrabecular recesses and a thin compacted layer. LVNC can result in left ventricular impairment, cardiac arrhythmia and embolic disease. LVNC has a prevalence of 0.05% in the adult population and is associated with genetic syndromes such as Noonan or Barth. CASE PRESENTATION: A 33-year-old male with a history of Gardner’s Syndrome, desmoid tumors, hypertension, non-ischemic cardiomyopathy is evaluated for heart failure. Patient’s symptoms were consistent with NYHA Class III. Patient was prescribed guideline-directed medical therapy (GDMT) and referred for echocardiogram. Echocardiogram revealed an ejection fraction of 40-45% with heterogeneous calcifications within the left ventricular myocardium. Cardiac MRI was ordered for further evaluation. MRI revealed mild dilation and global hypokinesis of left ventricle with ejection fraction of 31%. Trabeculations were seen at the distal aspect of the left ventricular myocardium along with focal areas of perfusion defects consistent with calcifications. Findings were concerning for LVNC. The MRI also detected a large anterior chest wall mass measuring 20x9cm consistent with a desmoid tumor (Figure 1). Patient was continued on GDMT for heart failure and referred to cardiothoracic surgery for evaluation of tumor removal. DISCUSSION: LVNC is diagnosed based on left ventricular morphology. Echocardiogram is the best initial test for evaluation but cardiac MRI provides a better assessment of the severity of LVNC. LVNC predisposes patients to left ventricular systolic failure, malignant arrhythmias, and thrombotic events. Management of LVNC involves goal directed medical therapy for heart failure, anticoagulation, and ICD based on guidelines for nonischemic cardiomyopathy. However no specific guidelines for treatment of LVNC exist. Management of desmoid tumors involve surgical resection to negative margins to minimize reoccurrences. Radiotherapy is used as adjuvant therapy in cases of incomplete resection. CONCLUSIONS: Although LVNC is associated with genetic syndromes, no connection to Gardner’s Syndrome or desmoid tumors could be found in the literature. The presence of these 3 rare genetic diseases in one individual suggest that there may be a genetic link. Reference #1: Akinseye OA, Ibebuogu UN, Jha SK. Left Ventricular Noncompaction Cardiomyopathy and Recurrent Polymorphic Ventricular Tachycardia: A Case Report and Literature Review. Perm J. 2017;21:17-045. Reference #2: Courtney E. Bennett and Ronald Freudenberger, “The Current Approach to Diagnosis and Management of Left Ventricular Noncompaction Cardiomyopathy: Review of the Literature,” Cardiology Research and Practice, vol. 2016, Article ID 5172308, 7 pages, 2016. https://doi.org/10.1155/2016/5172308. Reference #3: Bölke E, Krasniqi H, Lammering G, et al. Chest wall and intrathoracic desmoid tumors: surgical experience and review of the literature. Eur J Med Res. 2009;14(6):240-3. DISCLOSURES: no disclosure on file for Francecso Franchi; No relevant relationships by Aaron Richardson, source=Web Response No relevant relationships by Jose Rivas, source=Web Response