TOPIC: Cardiovascular Disease TYPE: Fellow Case Reports INTRODUCTION: Papillary fibroelastomas are rare and arise through an overgrowth of intracardiac endothelium; pathogenicity varies based on their size, proximity/adherence to valvular structures and the presence of comorbid diseases. Separately, myxedema coma is a known complication of chronic hypothyroidism and classically manifests with altered mentation, bradycardia and hypothermia. And while each is rare individually, these conditions are likely interconnected. CASE PRESENTATION: A 64 year-old female with a history of hypothyroidism presented to the hospital after being found unresponsive at home. Initial assessment under emergent care demonstrated an intact airway but a generally unresponsive patient; hemodynamics were significant for a heart rate of 57, temperature of 95.7 degrees Fahrenheit and respirations of 11 per minute. Laboratory studies were normal apart from a thyroid-stimulating hormone of 126 with a T4 of <0.25. Suspecting myxedema coma, providers placed the patient empirically on intravenous levothyroxine as well as corticosteroids, which led to an expected TSH down-trend and improved mentation. Seven days later, however, the patient again became altered, prompting magnetic resonance imaging of her brain, which demonstrated embolic strokes affecting multiple vascular territories, suggesting a central source. Subsequent transthoracic echocardiography revealed a dense, mobile structure on the aortic side of the left cusp measuring 0.37 cm, most consistent with a fibroelastoma. Definitive surgical intervention in the form of fibroelastoma resection and resuspension of the AV commissures was performed but the patient declined post-operatively after a Vfib arrest, ultimately prompting the family to pursue comfort measures. DISCUSSION: Although a rare entity, there is data in the available literature to support an association between myxedema coma with papillary fibroelastoma. One group under Suzuki et al. suggested that EMO syndrome, which consists of exophthalmos, myxedema of the pretibial region and osteoarthropathy, may itself contribute to the development of fibroelastoma. Researchers here specifically noted that, on microscopic analysis, Alcian blue-positive mucinous regions were found in both the pretibial soft tissue and resected fibroelastoma of their patient. And again while uncommon clinically, this phenomenon may suggest an infiltrative process into exta-thryoidal soft/cartilagenous tissues in states of systemic thryoid-mediated stress. CONCLUSIONS: The presence of aortic valvulopathy in pre-existing states of hypothyroidism, particularly in myxedema, should at minimum raise suspicion for papillary fibroelastoma. If confirmed, fibroelastomas must be resected while maintaining patient's hemodynamic stability and thyroid hormone metabolism. Tissue from both the tumor and from any suspected extra-thyoroidal sites should also be sampled and cross-compared on histopathology. REFERENCE #1: Gopaldas RR, Atluri PV, Blaustein AS, Bakaeen FG, Huh J, Chu D. Papillary fibroelastoma of the aortic valve: operative approaches upon incidental discovery. Tex Heart Inst J. 2009;36(2):160-163. REFERENCE #2: Suzuki H, Shimura H, Haraguchi K, Harii N, Endo T, Hosaka S, Yoshii S, Tada Y, Onaya T. Exophthalmos, pretibial myxedema, osteoarthropathy syndrome associated with papillary fibroelastoma in the left ventricle. Thyroid. 1999 Dec;9(12):1257-60. doi: 10.1089/thy.1999.9.1257. PMID: 10646668. REFERENCE #3: Kurmann RD, El-Am EA, Sorour AA, Ahmad A, Lee AT, Scott CG, Bois MC, Maleszewski JJ, Klarich KW. Papillary Fibroelastoma Growth: A Retrospective Follow-Up Study of Patients With Pathology-Proven Papillary Fibroelastoma. J Am Coll Cardiol. 2021 Apr 27;77(16):2154-2155. doi: 10.1016/j.jacc.2021.02.027. PMID: 33888255. DISCLOSURES: No relevant relationships by Samuel Reynolds, source=Web Response
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