Case Report: Disseminated Intravascular Coagulation Induced by Aortic Valve Fibroelastoma.AUTHORSThumma, Milanya M.; Northeast Ohio Medical University, College of Medicine, Rootstown, Ohio, United StatesAbu Alya, Walaa; Department of Internal Medicine, Cleveland Clinic Fairview Hospital, Cleveland, Ohio, United States.Corresponding Author: Abu Alya, Walaa; Department of Internal Medicine, Cleveland Clinic Fairview Hospital, Cleveland, Ohio, United States.INTRODUCTIONPapillary fibroelastomas are the second most common type of cardiac tumors. Although the exact mechanism for the development of fibroelastomas is unknown, it is theorized that small microthrombi coalesce on the cusps of the valve. Fibroelastomas are benign on their own; however, they significantly increase the risk of embolizations. This is due to the microthrombi that are at risk to dislodge from the physiological pressures of left ventricular ejection and spread throughout the body. Possible complications include stroke, transient ischemic attack, myocardial infarction, angina, etc.Transesophageal echocardiogram is the gold standard for diagnosing fibroelastoma. They typically appear as pedunculated, mobile masses with stippled edges and are typically less than 10 millimeters. According to one study, surgical resection is recommended for symptomatic cases greater than 10 mm (Kaplan, et al 2020). In addition, prophylactic anticoagulation therapy is initiated to protect against thrombi. This patient’s fibroelastoma wasn’t discovered until after her ischemic stroke. Due to her lack of surgical candidacy, she suffered many complications of the fibroelastoma.Case history and examinationThis patient is a female in her 70s with a past medical history of thrombocytopenia and bladder cancer, originally diagnosed in 2023 with recurrence in mid-2024. A couple months later, she was admitted to the hospital due to an ischemic stroke. During that admission, there was concern for an embolic origin of the stroke, which led to an echo for workup. A fibroelastoma measuring 12 mm x 9 mm was found on the right coronary cusp [images 1 A and B]. She was not a candidate for surgical resection of the fibroelastoma due to her thrombocytopenia. The patient was discharged on Eliquis.Two weeks later, her hematologist/oncologist held the patient’s Eliquis due to thrombocytopenia and concerns for thrombocytic thrombocytopenic purpura. That evening, the patient began experiencing abdominal pain and headaches. The pain and symptoms worsened overnight, resulting in the patient coming to the ED the next morning. She was hemodynamically stable, alert, and oriented at that time without focal deficit.In the ED, CT Abdomen/Pelvis showed acute splenic and bilateral renal infarcts [image 2 and 3]; CT Brain demonstrated a small hemorrhagic infarct in the right frontal/parietal cortex [image 4] . In addition, lab workup indicated a picture similar to DIC, as platelets were 26; fibrinogen was acutely decreased; d-dimer was elevated; and PT/INR and PTT were prolonged.Upon admission, anticoagulants were held initially due to hemorrhagic infarct. She was given platelet infusions and steroids for 3 days to address thrombocytopenia. Two days later, the patient had acute left homonymous hemianopia. MRI brain at that time showed acute right PCA infarct.Differential Diagnosis:Differential diagnoses include disseminated intravascular coagulation (DIC) in the setting of fibroelastoma, cancer-associated coagulopathy, immune thrombocytopenic purpura (ITP), and thrombotic thrombocytopenic purpura (TTP). Vasculitis is less likely given the absence of typical symptoms and rash.Outcome and follow upAfter a lengthy discussion with the patient and her spouse, the decision was made to re-initiate anticoagulation with heparin. The next day, the patient complained of upper quadrant abdominal pain. At that time, a CT abdomen scan showed acute thrombotic occlusions of the proximal jejunal and ileal jejunal branches of the superior mesenteric artery.At that time, the patient was transitioned to inpatient hospice care considering the rapid worsening of her condition.DISCUSSIONThis case illustrates the complex interplay of risk factors contributing to disseminated intravascular coagulation (DIC) and thrombotic events in a patient with aortic valve fibroelastoma. Although fibroelastomas are generally benign tumors, they significantly increase the risk of forming microthrombi, particularly in patients with pre-existing conditions that predispose them to a hypercoagulable state. In this patient, the presence of bladder cancer and thrombocytopenia further complicated her clinical picture.Anticoagulation management was a pivotal concern throughout her care. The decision to hold anticoagulants after her hemorrhagic stroke appeared prudent due to her thrombocytopenic state; however, this allowed thrombotic complications to escalate. While fibroelastomas typically present incidentally, they can lead to significant complications when symptomatic (Gopaldas et al., 2009). This is especially relevant in patients with malignancies, where a hypercoagulable state is often present due to various factors, including the release of procoagulant substances from tumor cells (Kaplan et al., 2020). In this case, the fibroelastoma likely exacerbated her hypercoagulability, leading to a paradoxical increase in thromboembolic events despite anticoagulation with Eliquis.The rapid onset of organ infarcts in this patient is particularly notable. Her CT findings of acute splenic, renal, and jejunal infarcts, coupled with the MRI results of a right PCA infarct, underscores the urgency and severity of her condition. The interplay between the fibroelastoma and her malignancy may have led to a vicious cycle of thrombosis and resultant end-organ damage. This case raises critical questions about the timing and management of anticoagulation in patients with fibroelastoma complicated by several comorbidities and emphasizes the importance of individualized treatment plans.In light of her declining condition and limited treatment options, transitioning to hospice care became a necessary consideration. This shift underscores the importance of a patient-centered approach in managing advanced malignancies and associated complications, focusing on quality of life over aggressive interventions when the prognosis is poor.CONCLUSIONSThis case underscores the intricate relationship between malignancies, benign cardiac tumors, and hypercoagulable states, emphasizing the need for careful monitoring and management of anticoagulation in such patients. The fibroblastoma’s contribution to thromboembolic events, especially in the context of this patient’s malignancy and thrombocytopenia, illustrates the challenges faced in clinical decision-making. As highlighted by Kaplan et al. (2020), timely recognition and intervention for symptomatic fibroelastomas are crucial to prevent serious complications. This case serves as a reminder of the importance of individualized care, balancing the risks of anticoagulation against the potential for life-threatening thrombotic events. Future studies may benefit from investigating the mechanisms linking fibroelastomas and malignancies to guide more effective management strategies and improve patient outcomes in similar cases.Authors’ Contributions:Thumma, Milanya M: Literature review and writing the case. Abu Alya, Walaa: reviewed the case and corresponding author.INFORMED CONSENTWritten informed consent was obtained from patient to publish this report in accordance with the journal’s patient consent policyACKNOWLEDGEMENTSWe would like to express our heartfelt gratitude to the patient and her husband for their willingness to share the medical details of her case in the hope of helping others. We also extend our thanks to the medical team, who provided exceptional care and support throughout her management. May the patient’s soul rest in peace.Key Clinical Message:This case highlights the complex interactions between aortic valve fibroelastoma, malignancy, and hypercoagulability, emphasizing the critical need for individualized management of anticoagulation in patients with such conditions. Fibroelastomas, although benign, can contribute to significant thromboembolic events, especially in patients with underlying malignancies and conditions like thrombocytopenia. Careful monitoring and timely intervention are essential to mitigate the risk of serious complications, such as disseminated intravascular coagulation and organ infarction.REFERENCESGopaldas 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):160-3. PMID: 19436815; PMCID: PMC2676606.Kaplan JG, Kanwal A, Bahoora J, Berquist J, Hunyadi V, Keirn R. Papillary fibroelastoma presenting with multi-organ symptoms. J Community Hosp Intern Med Perspect. 2020 Oct 29;10(6):597-599. doi: 10.1080/20009666.2020.1811067. PMID: 33194138; PMCID: PMC7598949.