Multisystem Inflammatory Syndrome in Children Requiring ECMO: Anticoagulation strategies and Lessons Learned

Intro: Multisystem Inflammatory Syndrome in Children (MIS-C) is a post-infectious inflammatory response after exposure to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) which can cause acute cardiac dysfunction requiring mechanical circulatory support (MCS). MCS utilization for MIS-C is complicated by a propensity for thrombosis, which threatens circuit integrity. This study describes a cohort of MIS-C patients requiring MCS, their outcomes, and the anticoagulation strategies utilized. Method(s): A retrospective case series of patients diagnosed with MIS-C needing veno-arterial extracorporeal membrane oxygenation (VA-ECMO) at Children's Healthcare of Atlanta from March 1, 2020 to June 30, 2022. VA-ECMO variables, laboratory data, complications, and outcomes were collected. Result(s): Seven patients (all male) with severe MIS-C required VA-ECMO for acute cardiac dysfunction. Median age was 13 years (range 4-15 years). Median ICU stay was 13 days (range 6-17 days) with a median ECMO duration of 7 days (IQR 3-8 days) and median mechanical ventilation duration of 8 days (IQR 5-11 days). All seven patients survived to hospital discharge with good neurologic outcomes. Median time to qualitatively normal ventricular function by echocardiogram was 9.5 days (IQR 3-21 days). Heparin was initially used in 6 patients, bivalrudin initially used in 1 patient, and 1 patient converted from heparin to bivalirudin for refractory systemic thrombosis. Median heparin dose was 206u/kg/d (IQR 192-276u/kg/d) with median anti-Xa levels of 0.75 (IQR 0.1-1.1) and median daily PTT 102 seconds (IQR 83-107 seconds). Median daily PTT of patients receiving bivalirudin was 86 seconds (80-93 seconds). Median R-values by thromboelastography were 38 seconds (IQR 25-55 seconds). Two patients required catheter directed thrombolysis with tissue plasminogen activator (t-PA) for refractory intracardiac thrombi, both were initially started on heparin. Significant cannula thrombosis occurred in 2 patients, 1 initially started on heparin and 1 initially on bivalrudin. Bleeding resulting in compartment syndrome occurred in one patient on heparin requiring fasciotomy of the upper extremities, this patient was not receiving t-PA. Conclusion(s): Anticoagulation management for MIS-C patients requiring ECMO is fraught with challenges. A successful management strategy may necessitate higher heparin assay levels, the use of direct thrombin inhibitors for refractory thrombosis, and the deployment of catheter directed thrombolysis. In this case series, CDT was safely and successfully used in two patients. Further studies are required to understand the optimal anticoagulation strategy for these patients to minimize complications.

Profile of Cardiac Involvement in Children After Exposure to COVID-19

OBJECTIVE: To evaluate the incidence and pattern of cardiac involvement in children post-COVID (coronavirus disease) infection in a tertiary care referral hospital in India. METHOD(S): A prospective observational study was conducted including all consecutive children with suspected MIS-C referred to the cardiology services. RESULT(S): Of the 111 children with mean (SD) age was 3.5 (3.6) years, 95.4% had cardiac involvement. Abnormalities detected were coronary vasculopathy, pericardial effusion, valvular regurgitation, ventricular dysfunction, diastolic flow reversal in aorta, pulmonary hypertension, bradycardia and intra-cardiac thrombus. The survival rate post treatment was 99%. Early and short-term follow-up data was available in 95% and 70%, respectively. Cardiac parameters improved in majority. CONCLUSION(S): Cardiac involvement post COVID-19 is often a silent entity and may be missed unless specifically evaluated for. Early echocardiography aided prompt diagnosis, triaging, and treatment, and helps in favorable outcomes.

A Persistent Nightmare: A Case of Recurrent Brachial Artery Thrombosis

Background Brachial artery thrombosis can be seen with thromboembolism, hypercoagulability, and arterial thoracic outlet syndrome. Case A 33-year-old healthy female construction worker presented with right hand discoloration and pain. She suffered a COVID-19 infection 8 weeks prior with hand symptoms developing shortly thereafter. She could no longer work due to the pain. Duplex ultrasound and CTA of the right upper extremity (Figure) demonstrated localized thrombosis of the right brachial artery. The workup yielded no aortic or intracardiac thrombus, and cardiac event monitor showed no atrial arrhythmia. She underwent thrombectomy with brachial artery stenting and was found, during surgery, to have distal ulnar artery occlusion. Two days post-op, she had recurrent pain and was found to have brachial artery recurrent thrombosis. She underwent urgent brachial-brachial bypass. Arm pain continued despite graft patency, so ulnarpalmar bypass was performed. Decision-making Hypercoagulability workup, including antiphospholipid antibody, protein C, protein S, homocysteine, and Lp(a), was negative. Neither central thrombus on TEE nor evidence of thoracic outlet syndrome was found. As a diagnosis of exclusion, brachial artery thrombosis was ascribed to COVID infection. Despite rivaroxaban, the patient developed gangrene (Panel C) requiring partial digit amputation. Conclusion We present a case of COVID-19-induced recurrent brachial artery thrombosis despite surgical intervention. [Formula presented]Copyright © 2023 American College of Cardiology Foundation

Looks Like a Vegetation but It Is Not Endocarditis

Background Myxomas are the second most common primary cardiac tumor (PCT) but overall have a low incidence rate. They usually arise from the interatrial septum whereas infective endocarditis (IE) vegetations frequently develop where there is turbulent blood flow, i.e., on the atrial side of the atrioventricular valves. Case A 75 year old male presented with fatigue, shortness of breath (SOB), myalgias and lower extremity edema for 2 weeks. His vital signs were stable and he was afebrile. Blood cultures were negative, WBC was normal, COVID-19 test was negative, and troponin was mildly elevated. TEE showed an ejection fraction of 20% with a large mitral valve (MV) mass (Figure 1A,B). Decision-making The mass was surgically resected and the MV was replaced (Figure 1C). On pathologic evaluation, the mass was confirmed to be a myxoma. The patient was later discharged without complication. Conclusion Clinical features of myxoma can overlap with IE including fever, malaise, SOB, and other signs of valvular obstruction or embolization. About 5% of myxomas originate from the MV and the differential diagnosis for an intra-atrial mass should include IE, PCT, metastatic tumors, and intracardiac thrombus. On echocardiography, myxomas appear irregularly frond-like or grape-cluster in shape. They are typically nonhomogeneous and can have areas of calcifications (Figure 1A). Both TEE and TTE are the mainstay for diagnosis of intracardiac masses and TEE specifically assists in guiding surgical excision. [Formula presented]Copyright © 2023 American College of Cardiology Foundation

THROMBUS IN TRANSIT CAPTURED BY BEDSIDE ULTRASOUND: A LIFE-SAVING TOOL

SESSION TITLE: Thrombosis Jamboree: Rare and Unique Cases SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/19/2022 12:45 pm - 1:45 pm INTRODUCTION: Point of care ultrasound used by the provider is rapidly expanding in internal medicine. Thrombus in transit (TIT) is defined on ultrasound as mobile echogenic material temporarily present in the right heart chambers to the pulmonary circulation via the tricuspid valve or systemic circulation via an atrial septal defect. In this case, we were able to identify echogenic material traversing the tricuspid valve into the pulmonary circulation, which confirmed the diagnosis of pulmonary embolus [1] CASE PRESENTATION: This is a 71-year-old female with a history of hypertension who presented to the emergency room with 4-day pleuritic chest pain, productive cough, fever, and exertional dyspnea. She was hemodynamically stable, afebrile, tachycardic, and tachypneic. Initial diagnostic workup demonstrated elevated cardiac enzymes and creatinine, ground-glass opacities on chest CT, positive COVID PCR, and sinus tachycardia with nonspecific T wave abnormalities. Given her renal insufficiency, CTA was initially held off. The patient was found to have right lower extremity deep venous thrombosis, and a heparin infusion was started while waiting for a V/Q scan. Shortly after admission, she had a syncopal episode after using the bathroom. CPR was initiated for suspected cardiac arrest, and a bedside ultrasound demonstrated a sizeable mobile thrombus in the right atrium traversing the tricuspid valve into the right ventricle. Given this finding, we elected to move forward with CTA chest, and this study confirmed extensive bilateral PE with right heart strain. DISCUSSION: TIT is a rare emergency in PE (4%) with a staggering mortality rate twice as high as PE without TIT [2]. The gold standard for diagnosis of PE is CT angiogram, and early echocardiography is a cornerstone in diagnosis and risk stratification. However, patients similar to the one discussed in this care may present with conditions preventing timely utilization of these tools. POCUS allows for the rapid assessment and implementation of time-sensitive treatments. Historically, it has been a must-have skill set among ER and critical care physicians. Only 35% of internal medicine residency programs have fully integrated formal diagnostic POCUS within the past decade despite increasing interest among trainees. The expeditious medical decision made for our patient was possible following a focused echocardiogram performed by an internal medicine resident. In patients with massive PE, only 35% of echocardiograms obtained within 24 hours were done in the ER, and still, 1 in 6 happened after 6 hours [3]. CONCLUSIONS: As with any operator-dependent skill, proficiency in POCUS is a prerequisite for reliable findings and time-sensitive medical decision-making. POCUS only becomes a lifesaving tool in experienced hands. Hence, it is imperative that internal medicine residency programs consider this tool an essential component of resident training. Reference #1: Arboine-Aguirre L, Figueroa-Calderón E, Ramírez-Rivera A, et al. Thrombus in transit and submassive pulmonary thromboembolism successfully treated with tenecteplase. Gac Med Mex. 2017;153(1):129–33. Reference #2: Casazza F, Bongarzoni A, Centonze F, Morpurgo M. Prevalence and prognostic significance of right-sided cardiac mobile thrombi in acute massive pulmonary embolism. Am J Cardiol. 1997;79(10):1433-1435. doi:10.1016/s0002-9149(97)00162-8 Reference #3: Torbicki A, Galié N, Covezzoli A, et al. Right heart thrombi in pulmonary embolism: results from the International Cooperative Pulmonary Embolism Registry. J Am Coll Cardiol. 2003;41(12):2245-2251. doi:10.1016/s0735-1097(03)00479-0 DISCLOSURES: No relevant relationships by Varinder Bansro No relevant relationships by Olayiwola Bolaji No relevant relationships by clarence findley No relevant relationships by Faizal Ouedraogo

THROMBUS IN TRANSIT: UNSUCCESSFUL ANTICOAGULATION IN A PATIENT WITH COVID-19

SESSION TITLE: Bedside Ultrasound Cases: Beyond Our Sight SESSION TYPE: Case Reports PRESENTED ON: 10/19/2022 09:15 am - 10:15 am INTRODUCTION: A thrombus-in-transit (TT), although rare, occurring in approximately 4-18% of pulmonary embolism (PE) cases, carries a high risk of mortality. One study commenting on 80-100% without treatment;therefore, TT should be considered a medical emergency and treated immediately. CASE PRESENTATION: A 64 years old female patient with history of Hypertension and morbid obesity presented to the Emergency department complaining of shortness of breath for 2 weeks with rapid worsening of symptoms and new chest pain in the previous 2 days. Patient blood pressure was 110/70, heart rate 160 irregularly irregular saturating 91% on room air, respiratory rate of 25. Patient tested positive for SARS-CoV-2, and her basic blood work showed elevated BNP and troponin with significant elevation of D-Dimer. The patient never smoked, had no recent travel and not taking OCPs. Bed side point of care echocardiogram showed large right atrial thrombus floating between the right atrium and right ventricle. Subsequent lower extremity ultrasound showed extensive left femoral thrombosis and pulmonary CT Angiogram showed a PE. The treatment options were discussed with the patient including giving full or half dose thrombolytics, or just anticoagulation with heparin. The patient opted for anticoagulation alone. Unfortunately, the patient had a cardiac arrest few hours later. Thrombolytics were given during CPR but the patient passed away. DISCUSSION: TT refers to free-floating right heart thrombi that travel from a venous source in the lower extremities to the pulmonary arteries. Although rare, the presence of a right heart thrombi in the setting of PE predicts a worse prognosis with a high mortality rate and thus, should be treated as a medical emergency. The diagnostic test of choice for TT is an echocardiogram, which shows an elongated right-sided mass illustrating high and chaotic motility with changing shape that continuously prolapses in and out of the right ventricle. Management of TT is still not well established. Options include anticoagulation with heparin, thrombolysis, or surgical removal. A particular study done by Greco et al. in 1999 used recombinant tissue plasminogen activator (rt-PA) with continuous echocardiogram monitoring, that revealed complete lysis of heart clots in all 7 patients within 24 hours. It also showed no changes in symptoms and ultimately showed improvement in blood pressure and heart rate. CONCLUSIONS: Available treatment options include anticoagulation alone, thrombolysis, or surgical embolectomy. Although anticoagulation can prevent clot propagation, it carries a mortality rate of up to 29%, comparable to surgical intervention. Surgical embolectomies could be an alternative option if contraindications to thrombolytics exist. Ultimately, no significant difference was found among the treatment options, suggesting the need for further research and clinical trials. Reference #1: Bikdeli B, Madhavan MV, Jimenez D, et al. COVID-19 and thrombotic or thromboembolic disease: Implications for prevention, antithrombotic therapy, and follow-up: JACC state-of-the-art review. J Am Coll Cardiol. 2020;75(23):2950–73. Reference #2: Cameron, James, et al. "Right Heart Thrombus: Recognition, Diagnosis and Management.” Journal of the American College of Cardiology, vol. 5, no. 5, 1985, pp. 1239–1243., https://doi.org/10.1016/s0735-1097(85)80031-0. Reference #3: Greco, Francesco, et al. "Successful Treatment of Right Heart Thromboemboli with IV Recombinant Tissue-Type Plasminogen Activator during Continuous Echocardiographic Monitoring.” Chest, vol. 116, no. 1, 1999, pp. 78–82., https://doi.org/10.1378/chest.116.1.78. DISCLOSURES: no disclosure on file for Ahmad alkhatatneh;No relevant relationships by Mohammad Alnabulsi No relevant relationships by Mohd Hazem Azzam No relevant relationships by Kelianne Comitalo

Severe Hypertriglyceridemia as a Cause of Arterial Thrombosis with Peripheral Embolic Complications

Lead Author's Financial Disclosures: Nothing to disclose. Study Funding: None. Background/Synopsis: Extensive evidence exists in support of a causal association of elevated triglyceride-rich lipoprotein (TRL) levels with the risk of atherosclerosis progression. Hypertriglyceridemia has been established as a risk factor for venous thrombosis, including a 2- fold increase in the risk of venous thrombosis in postmenopausal women. However, there is limited data on the role of hypertriglyceridemia in the arterial thrombosis. Objective/Purpose: Not Applicable. Methods: Case description: A 51-year-old white female with hypertension and type 2 diabetes (hemoglobin A1C, 7.4%) was transferred for further management of newly diagnosed bilateral renal and splenic infarcts. No risky habits were elicited except for the use of combined hormonal contraceptives over the past two years to control menorrhagia. Family history was significant for hypertriglyceridemia. Her physical exam was unremarkable. Testing for COVID-19 was negative. An extensive hypercoagulable and autoimmune work-up was unremarkable. Fasting lipid profile was significant for elevated levels of triglycerides, 1,274 mg/dL (replicated on two separate occasions), very low-density lipoprotein-cholesterol, 255 mg/dL, and non-high-density lipoprotein-cholesterol, 214 mg/dL, directly measured low-density lipoprotein cholesterol, 39 mg/dL and lipoprotein(a), 6 mg/dL. There was no structural pathology on the echocardiogram, including no interatrial shunt or intracardiac thrombus. Her whole-body computed tomography angiography revealed a focal calcified protruding thrombus in the distal thoracic aorta. No significant plaque was seen elsewhere in the aorta. Results: Decision-making. The posterior thrombus in the distal thoracic and proximal abdominal aorta was determined as a culprit for the visceral organ infarcts. Over the course of the hospital stay her abdominal pain gradually resolved. Treatment with low dose aspirin and therapeutic dose of low-molecular weight heparin was initiated followed by apixaban and aspirin on discharge. She was started on atorvastatin 40 mg, fenofibrate 145 mg, icosapent ethyl 4 g, resulting in a 70% reduction in the triglycerides levels (306 mg/dL). In 3 months, her repeat CT angiography showed significant resolution of the aortic atherothrombosis with no signs of aortic wall inflammation. At the 6-month follow-up visit she was switched to dual antiplatelet therapy with a plan to repeat imaging in 6 months. Conclusions: This case illustrates challenges in managing patients with arterial thrombosis in the setting of familial hypertriglyceridemia. Apart from severely elevated triglycerides no other etiology was evident. We propose further investigation of the prothrombotic properties of TRL and the role of targeted triglyceride-lowering therapies on atherothrombotic outcomes.

A Case of Right Ventricular Thrombus in a Patient With Recent COVID-19 Infection.

Coronavirus disease 2019 (COVID-19) is a viral respiratory disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The respiratory system is the main target of the virus; however, apart from lung disease, a relatively large proportion of patients develop thrombosis as well. We present the case of a 19-year-old male who was admitted after contracting community-acquired right-sided pneumonia. The patient had a history of COVID-19 infection four weeks before admission. The echocardiographic assessment revealed a 16 x 6-mm right ventricular (RV) thrombus. He underwent a cardiovascular magnetic resonance (CMR) study, which confirmed the findings. After ruling out the most common causes of hypercoagulability, COVID-19 was judged to be the cause of the thrombus. The patient was treated with warfarin. Follow-up imaging with echocardiography and CMR six months later revealed complete resolution of the thrombus. Hypercoagulability is a major complication of COVID-19 and in situ thrombosis can occur both in the arterial and venous circulation. The recognition of intracardiac thrombi even in low-risk patients with a history of COVID-19 infection and the immediate initiation of antithrombotic treatment to minimize the risk of embolization is of paramount importance. Advanced imaging techniques are often required to establish the diagnosis of this condition.

Respiratory symptoms of the 2019 novel coronavirus when the source of the symptoms is intra-cardiac thrombus not the pneumonia

Case Report The 2019 Novel Coronavirus (COVID-19) is currently causing a global pandemic. Common symptoms are fever, cough, myalgia, fatigue, headache, dyspnea, sore throat, vomiting, and diarrhea. Patients may present with end-organ failure, ARDS, shock, acute kidney injury, or even death. We present a case of COVID-19 with shortness of breath caused by an intra-cardiac thrombus. Case presentation An 84-year-old woman with COPD and diastolic heart failure presented with shortness of breath. She had hypoxemia on room air upon presentation. Lungs were clear on physical examination. COVID-19 PCR was positive. Her chest radiograph demonstrated no pulmonary infiltrates. Transthoracic echocardiography (TTE) demonstrated a large, irregularly shaped echogenic mass in both the right atrium and right ventricle consistent with a large thrombus. The mass in the right atrium was 3.9∗3.6 cm;the portion in the ventricle was 3.2∗2.2 cm. A previous TTE study in this patient did not reveal an intra-cardiac thrombus. No deep venous thrombosis was found. She was begun on anticoagulation and refused catheter-directed therapy. She improved and was discharged to her home. Discussion Thromboembolic complications of COVID-19 have been described in the literature. The most common are deep venous thrombosis and pulmonary embolism in critically ill patients despite the use of prophylactic anticoagulation. Several studies have reported post-mortem biopsies with widespread microthrombi. Arterial thrombosis with stroke and limb ischemia has also been described. Our case had an unusual presentation since the cause of her shortness of breath was the intra-cardiac thrombus. The pathogenesis beyond the hypercoagulability in COVID is not well understood. Some studies propose direct endothelial injury by the COVID-19 virus, causing microvascular inflammation, endothelial exocytosis, and endothelitis. Some experts propose a hypercoagulable state in COVID-19 patients based on elevated factor VIII, elevated fibrinogen, circulating prothrombotic microparticles, and neutrophil extracellular traps (NETs). Yet, no definitive mechanism has been identified. (Figure Presented).

Appearing and disappearing right-heart thrombus: A case reporto in COVID- 19 patient

Aims: Venous thromboembolism represents frequent complication of patients with severe COVID-19 disease. Several reports about atypical thrombosis are described, rarely it has been described a right venticular thrombus during the course of infection. We report a case of right endoventricular thrombosis in a patient with SARSCov- 2 pneumonia. Methods and results: A 58-year-old man was admitted to our ward for severe respiratory failure in interstitial pneumonia. The nasopharyngeal swab for COVID-19 resulted positive. Steroids and prophylaxis with LMWHwere started, associated to CPAP to maintain good gas exchange. During hospitalization a venous ECD was performed with evidence of left popliteal thrombosis despite the therapy. D-Dimer was 44±3 ng/ml. A new onset AF was documented at the telemetry, without troponin elevation. A cardiac ultrasound was performed showing a right endoventricular lesion of 1.8 cm adhering to the free wall of the right ventricle. A CT-pulmonary angiogram (CTPA) resulted negative for pulmonary embolism and confirmed suspected right ventricular thrombus. Treatment with fondaparinux 7.5mg was started. After 10 days, cardiac ultrasound shown complete resolution of thrombosis, and CT confirmed the disappearing of the mass. Dabigatran 150 mg twice/day was started. Patient clinically improved and he was discharged after 20 days of hospitalization. Conclusions: SARS-CoV-2 infection may cause inflammation with cytokine storm and hypercoagulability leading to venous thromboembolism. Atypical thrombus formation was reported, including right-ventricle free wall. Early caridac ultrasound was critical to make diagnosis and starting prompt treatment, therefore routine cardiac ultrasound is mandatory in severe COVID-19 patients.