Recurrent Syncopal Episodes Research Articles

8424 Complex Management Of Adrenal Insufficiency In Pregnancy: A Challenging Case

Abstract Disclosure: T. Avanessian: None. H. Alsamarraie: None. Background: Physiologic changes, including changes in the HPA axis and RAAS, during pregnancy pose challenges in the management of adrenal insufficiency in pregnant women. This case report discusses the management of adrenal insufficiency in a young primigravida female with Addison’s disease. Clinical Case: At 19.5 weeks of gestation, a 26-year-old primigravida, previously diagnosed with Addison’s disease 5 years ago, presented to an acute care center for syncopal episodes. The pregnancy had been complicated by persistent nausea and vomiting, labeled as hyperemesis gravidarum, and recurrent syncopal episodes, resulting in 6 ED visits and 3 hospitalizations, with symptoms improving each time after IV hydration and then returning within a few days. At presentation, the patient reported lightheadedness, sweating, palpitations, dyspnea, fatigue, nausea, vomiting, and a 10 lb weight loss since the beginning of pregnancy. The pre-pregnancy regimen was hydrocortisone 10 mg in the morning and 5 mg in the afternoon, with fludrocortisone 0.1mg daily. At 8 weeks of gestation, due to increased fatigue and hypotension, the hydrocortisone dose was doubled, while fludrocortisone was continued the same. During admission, blood pressure ranged from 67/37 to 90/56 mmHg, and heart rate ranged between 93-103 bpm. On labs, electrolytes were normal, and the serum renin level was elevated at 5.803 ng/ml/hr (0.167-5.380). Treatment included IV hydration and hydrocortisone 40 mg in the morning and 20 mg in the afternoon. Despite symptom improvement, blood pressure remained low. Thus, fludrocortisone was increased to 0.1 mg twice daily. Within 3 days, all symptoms improved, allowing for the down-titration of the hydrocortisone dose. The patient was discharged with hydrocortisone 30 mg at 8 AM, 10 mg at 3 PM, 5 mg at 7 PM, and fludrocortisone at 0.1 mg twice daily. Conclusion: Managing symptoms of adrenal insufficiency during pregnancy is challenging due to the overlap of symptoms and signs with physiologic manifestations of pregnancy. The use of biochemical testing is limited, given the physiological activation of RAAS and progressive increases in renin, CRH, ACTH (from both pituitary and placental sources), and cortisol during pregnancy. Monitoring mineralocorticoid requirements is further complicated by physiological changes in blood pressure and interstitial fluid status. While some literature suggests that adjusting fludrocortisone may not be necessary, as an increase in glucocorticoid dose can compensate, determining the correct dose of glucocorticoid and mineralocorticoid replacement should be tailored to each patient and their specific symptoms.

Procedural heart rate variability and its effect on short-term symptoms after cardioneuromodulation procedure

Abstract Background Syncope is a miscellaneous clinical condition that affects a large portion of the population every year all over the world and responsible for a considerable amount of hospital and particularly emergency department admissions (1) The use of a right-sided approach in catheter ablation of ganglion plexi (GP) has been applied to reduce syncope episodes caused by vasovagal syncope (VVS) and decrease the overall burden of syncope (2). Purpose Our aim is to show that right-sided GP ablation which is known as cardioneuromodulation (CNM) is a new and promising way to cease recurrent syncope episodes and assess its effects on heart rate (HR), P-P intervals, ventricular depolarisation/repolarization parameters (QRS,QTc,Tpe durations, QTc/Tpe ratio), recurrence of syncope episodes, and patients symptoms such as fatigue, dizziness, etc. We also aimed to identify changes between pre and post procedural heart rates and its effects on short-term symptoms during follow-up. Methods A group of 55 patients with positive tilt table response or recurrent vasovagal syncope with pauses >2 sec in holter monitoring was enrolled this study. During the EP study, GPs determined both anatomically and by EP stimulation then autonomic denervation was performed focused by radiofrequency ablation. All patients followed-up for 12 months. Results Right-sided ablation was performed during the procedure and HR increased significantly in all patients after the procedure compared to before. Syncope episodes of the patients decreased significantly after the procedure. The patients who did not complain of syncope at the first, second and third appointment, respectively, 76.4% (n:42), 90.9% (n:50) and 92.7% (n:51). Patients had complaints at most within the first 4 months after the procedure (Table-1). At the first appointment, 4 (7.3%) patients had syncope and 9 (16,4%) patients had sinus tachycardia. Patient group with heart rate variability varying more or less than 20% during the procedure were examined, 81% of those with HR variability <20% did not have any symptoms, while 64.7% of the group with HR variability above 20% did not have any symptoms at first appointment (p:0,461) (Table.2). Tachycardia was seen in 23.5% of those with HR variability >20%, while it was determined as 9.5% in those with a HR variability <20 % Although these comparisons do not show a statistically significant difference due to the number of few patients, it is clinically significant. The same relationship was not observed between HR variability and symptoms in the second and third appointments. Conclusion Cardioneuromodulation is a remarkable treatment modality and it should be taken into consideration that more symptoms may develop in the early post-procedural period in the group who has post-procedure heart rate variability over 20%, and a cut-off should be determined for the target heart rate during the procedure.Table-1Table-2

Comparison between catheter ablation versus permanent pacemaker implantation as an initial treatment for tachycardia–bradycardia syndrome patients: a prospective, randomized trial

BackgroundClinical outcomes after catheter ablation (CA) or pacemaker (PM) implantation for the tachycardia–bradycardia syndrome (TBS) has not been evaluated adequately. We tried to compare the efficacy and safety outcomes of CA and PM implantation as an initial treatment option for TBS in paroxysmal atrial fibrillation (AF) patients.MethodsSixty-eight patients with paroxysmal AF and TBS (mean 63.7 years, 63.2% male) were randomized, and received CA (n = 35) or PM (n = 33) as initial treatments. The primary outcomes were unexpected emergency room visits or hospitalizations attributed to cardiovascular causes.ResultsIn the intention-to-treatment analysis, the rates of primary outcomes were not significantly different between the two groups at the 2-year follow-up (19.8% vs. 25.9%; hazard ratio (HR) 0.73, 95% confidence interval (CI) 0.25–2.20, P = 0.584), irrespective of whether the results were adjusted for age (HR 1.12, 95% CI 0.34–3.64, P = 0.852). The 2-year rate of recurrent AF was significantly lower in the CA group compared to the PM group (33.9% vs. 56.8%, P = 0.038). Four patients (11.4%) in the CA group finally received PMs after CA owing to recurrent syncope episodes. The rate of major or minor procedure related complications was not significantly different between the two groups.ConclusionCA had a similar efficacy and safety profile with that of PM and a higher sinus rhythm maintenance rate. CA could be considered as a preferable initial treatment option over PM implantation in patients with paroxysmal AF and TBS.Trial registrationKCT0000155.

Predictors of pacemaker requirement in patients with implantable loop recorder and unexplained syncope: A systematic review and meta-analysis.

Identifying the underlying cause of unexplained syncope is crucial for appropriate management of recurrent syncopal episodes. Implantable loop recorders (ILRs) have emerged as valuable diagnostic tools for monitoring patients with unexplained syncope. However, the predictors of pacemaker requirement in patients with ILR and unexplained syncope remain unclear. In this study, we shed light on these prognostic factors. PubMed/MEDLINE, EMBASE, Web of Science, and Cochrane CENTRAL were systematically searched until May 04, 2023. Studies that evaluated the predictors of pacemaker requirement in patients with implantable loop recorder and unexplained syncope were included. The "Quality In Prognosis Studies" appraisal tool was used for quality assessment. The pooled odds ratio (OR) with 95% confidence intervals (CIs) was calculated. The publication bias was evaluated using Egger's and Begg's tests. Ten studies (n = 4200) were included. Right bundle branch block (OR: 3.264; 95% CI: 1.907-5.588, p < .0001) and bifascicular block (OR: 2.969; 95% CI: 1.859-4.742, p < .0001) were the strongest predictors for pacemaker implantation. Pacemaker requirement was more than two times in patients with atrial fibrillation, sinus bradycardia and first degree AV block. Valvular heart disease, diabetes mellitus, and hypertension were also significantly more in patients with pacemaker implantation. Age (standardized mean difference [SMD]: 0.560; 95% CI: 0.410/0.710, p < .0001) and PR interval (SMD: 0.351; 95% CI: 0.150/0.553, p = .001) were significantly higher in patients with pacemaker requirement. Heart conduction disorders, atrial arrhythmias and underlying medical conditions are main predictors of pacemaker device implantation following loop recorder installation in unexplained syncopal patients.

Therapeutic options for syncope: a meta-analysis of trials with and without placebo control

Abstract Funding Acknowledgements Type of funding sources: None. Background 8% of the population suffer recurrent episodes of syncope which can cause physical injury and psychological morbidity. The extent to which therapeutic options effectively reduce syncope recurrence beyond placebo remain unclear. Methods We performed a meta-analysis of randomised trials including pharmacological, cardiac pacing and physical interventions for syncope. We assessed the effect of these therapeutic interventions on clinical syncope recurrence. We stratified the trials by use of placebo control. Results There were 50 eligible trials: 22 pacing, 19 pharmacological and 9 of physical interventions including 3,975 patients. Trials assessing syncope recurrence with placebo control were neutral for Beta-Blockers (RR 1.11, 95% CI 0.85 to 1.45, p=0.36), Fludrocortisone, (RR 0.83, 95% CI: 0.63 to 1.11 p=0.83) and conventional dual-chamber pacing (RR 0.81, 95% CI 0.61 to 1.09 p=0.287). However, they were favourable for Selective Serotonin Reuptake Inhibitors [SSRIs] (RR 0.40, 95% CI 0.25 to 0.63 p&amp;lt;0.001), Midodrine (RR 0.70, 95% CI 0.53 to 0.94, p=0.016) and Closed-Loop-Stimulation (CLS) pacing (RR 0.21, 95% CI 0.12 to 0.34, p &amp;lt; 0.001). Non-placebo-controlled trials reported significant benefit for every category of therapy including conventional dual-chamber pacing, medicines (Beta-Blockers, Midodrine and Oral rehydration salts) and physical interventions including tilt-training. Three categories of therapy have been trialled with and without placebo control: the non-placebo-controlled studies showed significantly different results from their placebo-controlled counterparts (beta-blockers p=0.024, midodrine p=0.006, dual-chamber pacing p=0.017) (figure 2). Conclusion Under placebo-controlled conditions, CLS pacing reduces risk of syncope recurrence by ~75%, whereas conventional pacing does not. SSRIs and midodrine also show significant efficacy under placebo-controlled conditions. Without placebo control, trials consistently show artefactually larger benefits. Future non-placebo-controlled trials for syncope should not be conducted.

P453 RECURRENT SYNCOPAL EPISODES IN A YOUNG PATIENT: TO IMPLANT THE PM OR NOT TO IMPLANT THE PM, ALWAYS A DIFFICULT DECISION!

Abstract Introduction Syncope represents one of the main causes of access to the Emergency Department and outpatient visits, accounting for up to 13% of hospital admissions. Although usually benign, syncopal episodes are a source of considerable distress for the patient and family members, particularly when dealing with otherwise healthy young patients. We report the case of a young patient who presented with recurrent syncopal episodes with important psychological and professional implications. Case Report A 33–year–old man comes to our Syncope Unit with a long history of repeated syncopal episodes preceded by fleeting warning signs, sometimes with minor trauma, occurring during daily activities, in conjunction with minor injuries or post–voiding but never during exertion. The patient began to limit his activities for fear of syncope and was inhibited from driving work vehicles and performing tasks at height. The various cardiological examinations performed over the years were normal. The study of syncope evoked a syncope with a 13 sec pause on carotid sinus massage while the Head–up Tilt Test was negative. A Loop Recorder (ILR) was then implanted to correlate the syncopal episodes with any slowing or pathological pauses in the heart rhythm. At remote monitoring of the ILR, an asystole of about 16 seconds symptomatic for syncope was documented. In relation to the recurring syncopal episodes, the important professional limitations imposed on the patient, as well as at the explicit request of the patient himself, despite the lacking scientific evidences availables, we proceeded to implant a dual–chamber pacemaker with a frequency response algorithm. In the 4 years after the implant there were no syncopal recurrences and the patient resumed his normal daily and professional activities with a marked improvement also in his psychological setting. After 2.5 years, however, the ventricular lead broke and required its extraction and reimplantation. Conclusions The decision to implant the device was not easy, precisely in relation to the possible complications over the years, but this allowed the patient to resume a normal private and professional life. However, an accurate risk–benefit assessment is required, sharing the "weight" of the decision with the patient and his family.

C67 ATRIOVENTRICULAR BLOCK DUE TO CARDIAC CONDUCTION TISSUE INFILTRATION: A CLINICAL PRESENTATION OF TRANSTHYRETIN CARDIAC AMYLOIDOSIS

Abstract Background In cardiac amyloidosis, the deposition of insoluble amyloid fibrils can affect the cardiac conduction tissue, causing bradyarrhythmic clinical manifestations, often in the advanced stages of the disease. In this clinical case, atrioventricular block represented the clinical onset of transthyretin cardiac amyloidosis. Clinical case A 68–year–old man was admitted to our ward for recurrent syncopal episodes. He had in his medical history bilateral carpal tunnel syndrome. The electrocardiogram documented sinus rhythm with preserved QRS voltages, right bundle branch block, left anterior hemiblock, and first and second degree atrioventricular block with mean ventricular rate of 38 beats/min. A color–Doppler echocardiogram revealed increased left and right ventricular thickness (maximum thickness 16 and 10 mm, respectively), diastolic dysfunction, and preserved ejection fraction (60%). The patient underwent a coronary angiography, which was normal, and an endomyocardial biopsy of the left ventricle, which revealed the presence of hypotrophic and degenerated cardiomyocytes surrounded by large areas of amorphous substance that exhibited apple–green birefringence in polarized light after staining with Congo red, suggesting the diagnosis of cardiac amyloidosis. Sections of conduction tissue were included in the specimens, which appeared on histology as small myocytes positive by immunohistochemistry for HCN4, suggestive of conduction tissue cardiomyocytes. The conducting tissue was almost completely replaced by amyloid deposits positive by immunohistochemistry for transthyretin. The affected patient underwent a genetic test which revealed the mutation of the TTR gene (p.Thr79Lys). The patient subsequently underwent pacemaker implantation and started treatment with Tafamidis due to the absence of neurological involvement. Conclusion In patients presenting clinically with syncope and conduction abnormalities, the diagnosis of cardiac amyloidosis should always be considered, because they may represent the clinical onset of the disease.

Large retrosternal non-hodgkin’s lymphoma presenting with recurrent syncope episodes and chest asymmetry: A case report

In this case we report a 42-year-old woman who presented to Syrian Heart Lung Center (SHLC) with recurrent syncope attacks, general fatigue and weight loss.

Carotid Sinus Syndrome in a Patient of Nasopharyngeal Carcinoma Treated with Palliative Radiotherapy

Carotid sinus syndrome leading to bradycardia, unconsciousness and eventually cardiac arrest is a rare condition. Its pathophysiology is not fully understood. Here, we present a rare case of a 79-year-old male with left cervical lymph node metastasis originating from primary carcinoma of nasopharynx. He was admitted to hospital with history of recurrent syncopal episodes at home. Paroxysms of extreme bradycardia (Heart rate of &lt; 40) were detected during hospital stay. CT-scan and MRI of neck revealed compression of left neck vasculature. This patient was treated with palliative radiotherapy. He showed favorable response in that he became asymptomatic.

JS-NG-8: CASE REPORT: POSSIBLE NEW CHANNELOPATHY ASSOCIATED WITH ELEVATED ALDOSTERONE LEVELS

Background: Potassium channelopathies are associated with prolonged QT cardiac syndrome. New potassium channelopathies in somatic cells, including KCNJ5 mutations, also underlie some forms of primary hyperaldosteronism. We present a first-in-human report of an association between a KCNQ1 loss of function mutation manifesting with life threatening-ventricular tachycardia (VT), severe hypokalemia and presumed hyperaldosteronism. Case Presentation: The patient is a 51-year-old Asian woman known for obesity, Wolff-Parkinson-White post ablation, and recurrent syncopal episodes with polymorphic VT due to prolonged QTc, requiring an implantable cardioverter defibrillator. On initial presentation for syncope, spontaneous hypokalemia was discovered at 3.1 mmol/L. She had multiple hospitalizations for torsade storms and persistent hypokalemia resistant to aggressive potassium supplementation until addition of spironolactone. Investigations revealed a suppressed plasma renin activity &lt; 0.05 ng/L/s, serum aldosterone concentration at 302 pmol/L, and an aldosterone to renin ratio (ARR) &gt; 6040. Repeated ARR was &gt; 10980. Confirmatory saline suppression testing or adrenal vein sampling were not performed due to risk of recurrent VT associated with spironolactone cessation. A comprehensive evaluation for the etiology of this patient's clinical presentation revealed a mutation in the KCNQ1 gene type 1. Literature review: The voltage-dependent K(+) channel responsible for activating delayed K(+) current is composed of pore-forming KCNQ1 and regulatory KCNE1/3 subunits which loss of function predispose to sustained torsade de pointes. Animal models demonstrate that KCNQ1 and KCNE1 mRNAs are expressed in the zona glomerulosa of adrenal glands where potassium channels directly participate in the control of aldosterone production. Although KCNE3 is not expressed in mouse adrenals, its deletion is thought to cause activation of lymphocytes targeting adrenal glands, leading to hyperaldosteronism. Furthermore, administration of spironolactone to KCNE3 knockout mice ameliorates their QT prolongation and predisposition to VT. There have been no case reports of an association between KCNQ1/E1/E3 loss of function and hyperaldosteronism in humans. Conclusion: This raises the question if this mechanism can also be a component of human KCNQ1 and KCNE1/3-linked arrhythmogenesis and hyperaldosteronism. Alternatively, we can further speculate that patients with hyperaldosteronism who develop malignant arrhythmias during hypokalemia are subjects with impaired repolarization reserve as a consequence of variants in genes known to cause long QT syndrome. The variants, leading to subclinical ion channel dysfunction, are typically silent until unmasked by electrolytes abnormalities such as hypokalemia. To our knowledge, this case is a first report of a potentially novel channelopathy associated with hyperaldosteronism in humans. These findings are hypothesis generating and require further investigations.

Arrhythmic risk in single or recurrent episodes of unexplained syncope with complete bundle branch block

Introduction and objectivesPatients with a single syncopal episode (SSE) and complete bundle branch block (cBBB) are frequently managed more conservatively than patients with recurrent episodes (RSE). The objective of this study was to analyze if there are differences between patients with single or recurrent unexplained syncope and cBBB in arrhythmic risk, the diagnostic yield of tests, and clinical outcomes. MethodsCohort study of consecutive patients with unexplained syncope and cBBB with a median follow-up time of 3 years. The patients were evaluated via a stepwise workup protocol based on electrophysiological study (EPS) and long-term follow-up with an implantable cardiac monitor. ResultsOf the 503 patients included in the study, 238 (47.3%) had had only 1 syncopal episode. The risk of an arrhythmic syncope was similar in both groups (58.8% in SSE vs 57.0% in RSE; P=.68), also after adjustment for possible confounding variables (HR, 1.06; 95%CI, 0.81-1.38; P=.674). No significant differences between the groups were found in the EPS results and implantable cardiac monitor diagnostic yield. A total of 141 (59.2%) patients with SSE and 154 (58.1%) patients with RSE required cardiac device implantation (P=.797). After appropriate treatment, 35 (7%) patients had recurrence of syncope. The recurrence rate and mortality were also similar in both groups. ConclusionsPatients with cBBB and unexplained syncope are at high risk of an arrhythmic etiology, even after the first syncopal episode. Patients with SSE and RSE have a similar arrhythmic risk and similar outcomes, and therefore there is no clinical justification for not managing them in the same manner.

1011 HETEROGENEOUS ARRHYTHMIC PHENOTYPE IN A CASE OF LEFT VENTRICULAR NON COMPACTION CARDIOMYOPATHY

Abstract A young 47 years old female patient was hospitalized in our department for recurrent traumatic syncopal episodes in order to undergo Head Up Tilt Test and Electrophysiology Study (EPS). Her ECG was normal and also transthoracic echocardiography didn't show pathological findings. 24 hour Holter ECG recording showed a short run of polymorphic ventricular tachycardia. Regarding familiar history her mother had died at a young age of sudden death. Head-up tilt test resulted negative for induced-syncope. During hospitalization the patient complained onset of malaise rapidly exiting in syncope: telemetry showed two pauses of 3.8 and 5.1 sec respectively, due to paroxysmal III degree atrio-ventricular block. The day after, the patient underwent electrophysiological study. During programmed ventricular stimulation from RV apex with train and V extrastimolous S1 300 S2 220 induction of syncopal ventricular fibrillation was observed, promptly treated with external DC shock. Cardiac MRI was performed showing diagnostic signs of Left Ventricular non-Compaction Cardiomyopathy. Considering the history of traumatic syncope the patient underwent dual chamber ICD implantation and was discharged. No clinical events in a 6 month follow-up. Genetical analysis was performed and we are waiting for the results. The term left ventricular non-compaction (LVNC) identifies a cardiomyopathy, characterized by intrauterine arrest of the compaction process of the ventricular myocardium during the end of the fourth week, thus leading to the development of prominent trabeculae of the left ventricle, deep intertrabecular recesses and a ventricular wall divider into two different layers of myocardium, the first compact, the other not. This nosological entity is difficult to classify (the European Society of Cardiology considers it as not classifiable among other cardiomyopathies, unlike the American Heart Association) mostly from the point of view of clinical implications, often unpredictable. In fact, we may have completely asymptomatic forms, which, according to some authors, should be considered as normal and completely benign variants, and various manifestations, in which the lack of compaction represents a morphological trait shared by phenotypically distinct forms of cardiomyopathy, such as hypertrophic, dilated and restrictive. LVNC carries an increased risk of ventricular dysfunction resulting in chronic heart failure, thromboembolic events and especially arrhythmic, often ventricular and life-threatening manifestations. A large spectrum of arrhythmias has been observed in patients with non-compact ventricles: in particular, supraventricular and ventricular tachycardias, Wolff-Parkinson-White (WPW) syndrome, but also bradyarrhythmias, which include sinus bradycardia, Sick Sinus Syndrome and various degrees of atrioventricular block, up to complete block. Ventricular tachycardias, including those that progress to ventricular fibrillation, occur in 38–47% of adult patients with LVNC, hence sudden death is often the cause of clinical presentation. Seen the features of the pathology, early diagnosis and family clinical evaluation are necessary aspects in patient management.

281 CARDIAC RESYNCHRONIZATION TERHAPY IN PERSISTENT LEFT SUPERIOR VENA CAVA WITH A BRIDGING INNOMINATE VEIN

Abstract Background The Persistent Left (PL) Superior Vena Cava (SVC) is a congenital malformation of the thoracic venous return that has an incidence of 0.2-3% in the general population and an incidence of about 0.5% in patients undergoing cardiac implantable electronic device. In this case, the placement of the leads, especially for the Right Ventricle (RV) and Left Ventricle (LV) through one of the branches could be challenging. Methods A 66-year-old man with hypertension, diabetes and dyslipidemia, in optimal pharmacologic therapy for congestive heart failure (NYHA II), hospitalized for recurrent syncopal episodes, with extreme sinus bradycardia, first-degree AV block and Left Bundle Branch Block, The patient had a LV hypertrophy and mild global systolic function (LV ejection fraction of 40%) for these we opted for a pacemaker CRT device implantation. Results Isolation of left cephalic vein was used as venous access for right atrium (RA) and RV leads while Seldinger technique via subclavian vein was used as venous access for LV lead. The guidewire through the subclavian vein pointed out the presence of PLSVC. Left subclavian venogram confirmed PLSVC and revealed a bridging innominate vein. To pass through the innominate vein and reach the SVC, a 0.035-inch hydrophilic guidewire was used. A 6Fr-sheath was advanced through the hydrophilic guidewire, subsequently a second metallic guidewire was advanced through the same introducer by removing the dilator. The RV and RA leads were placed respectively on low right interventricular septum and RA appendage passing through bridging innominate vein. Electrical parameters were normal. For the placement of the LV lead a 9.5Fr sheath introducer was advanced over the guidewire through the PLSVC. A venogram showed a lateral and a postero-lateral branch of the coronary sinus with acute angulation. With a 6Fr quadripolar Josephson catheter the postero-lateral branch was entered, eventually a 9Fr peelable outer guide catheter was advanced through the electrophysiology catheter becoming selective with the interested branch. A quadripolar S shaped lead was used, it was advanced into the targeted vein through a 0,014fr hydrophilic guidewire. Adequate electrical parameters and stability were achieved. Conclusion Lead implantation in patient with PLSVC is challenging. Placing the RA and RV leads through SVC ensure a better stability along time. The cannulation of CS is easily achieved by approach via PLSVC while selective vein cannulation can be difficult due to the modified acute angulation.

Non-islet Cell Tumor-related Hypoglycemia in a Case of Metastatic Gastrointestinal Stromal Tumor: A Rare Paraneoplastic Syndrome: A Case Report

Introduction: Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract. Clinical presentations depend upon the site of involvement. It can lead to obstruction, bleeding, perforation, or symptoms due to mass effects. Tumor-induced hypoglycemia (nonislet cell tumor-related hypoglycemia [NICTH]) is a rare manifestation of GIST. Case Report: We herewith present the case of a 46-year-old female who presented with multiple abdominal lumps and recurrent episodes of syncope due to NICTH. On investigations, she was diagnosed to have unresectable GIST on biopsy. Her hypoglycemic episodes could be explained by rare paraneoplastic syndrome due to the raised levels of insulin-like growth factor-2. After treatment with imatinib (800 mg once daily), her hypoglycemic episodes subsided, which supported the diagnosis. Conclusion: NICTH is a rare paraneoplastic manifestation of GIST; targeted therapy can avert neuroglycopenic symptoms.

SYSTEMIC MASTOCYTOSIS WITH CONCURRENT ALPHA-GAL ASSOCIATED ANAPHYLAXIS

<h3>Introduction</h3> We present a case of a patient, diagnosed with systemic mastocytosis (SM), who was also found to have anaphylaxis to galactose-α-1,3-galactose (alpha-gal) several years after his initial SM diagnosis. <h3>Case Description</h3> The patient initially presented in his 20s with recurrent episodes of hypotension, pre-syncope, and syncope. An extensive cardiac and neurologic workup did not elucidate an etiology. He moved to Mississippi and had a six-year period with no episodes. In 2015, he was admitted to the intensive care unit due to presumed septic shock secondary to an infected pilonidal cyst. A tryptase level during hospitalization was elevated at >30, and a bone marrow biopsy confirmed the diagnosis of indolent SM. After starting antihistamines, the patient was initially free of anaphylaxis episodes. The patient then began experiencing weekly anaphylactic episodes, often with severe hypotension, sometimes requiring multiple doses of epinephrine. Episodes persisted despite increased antihistamine regimen. After extensive testing, he was found to have elevated alpha-gal specific IgE and was diagnosed with alpha-gal-associated anaphylaxis. With mammalian meat avoidance, he had no further anaphylactic episodes. <h3>Discussion</h3> Our reports highlight the importance of considering food allergens, particularly alpha-gal, in locations with a high incidence of lone star tick bites, as possible triggers for recurrent anaphylaxis in patients with SM. Given the delayed presentation of alpha-gal reactions and the inherent propensity for patients with SM to experience anaphylaxis, this diagnosis can easily be overlooked. The case also suggests the possibility that underlying SM may be associated with more severe manifestations of alpha-gal reaction.

S2659 Splenic Artery Pseudoaneurysm as a Rare Cause of Upper Gastrointestinal Bleeding

Introduction: Upper gastrointestinal bleeding (UGIB) is one of the most common causes for hospital admission in the US. Common etiologies for UGIB include peptic ulcer disease, erosive esophagitis, varices, and less commonly malignancies. Splenic artery pseudoaneurysm (PSA) is a known complication of pancreatitis. It may be asymptomatic or present with left upper quadrant or epigastric pain, and rarely as hemorrhagic shock. Splenic artery PSA presenting with UGIB is extremely unusual. Case Description/Methods: We present the case of a 57-year-old male with alcohol use disorder complicated by a prior episode of alcoholic pancreatitis, who presented with 3 days of hematemesis, melena, and recurrent syncopal episodes. In the ED his vitals and physical exam were notable for tachycardia, hypotension, and melena. Labs revealed a hemoglobin of 4.2. Non-contrast CT of the abdomen and pelvis showed evidence of blood in the antrum and a 2.0 x 1.6 cm out-pouching from the greater curvature of the stomach which was postulated to be a gastric diverticulum (image 1). Upper endoscopy revealed fresh blood in the antrum and duodenum, as well as a massive adherent clot in the fundus. Since no single site of active bleeding was identified, the patient was started on IV PPI with continued close monitoring in the ICU. He continued to have melena, and increased PRBC requirements. Repeat EGD was performed but was unchanged from prior. A CT angiogram of the abdomen and pelvis demonstrated a 1.5 cm actively bleeding PSA arising from a first divisional branch of the splenic artery communicating with the fundus of the stomach (images 2 & 3). The patient underwent successful coil embolization (image 4). Hemoglobin remained stable throughout the remainder of his admission with no further evidence of bleeding. Discussion: This case demonstrates PSA as a rare cause of UGIB. Lesions are most commonly caused by chronic pancreatitis, pancreaticobiliary surgery, and trauma. This patient’s PSA was likely secondary to his prior episode of alcoholic pancreatitis. Prompt diagnosis of PSA is essential given the high mortality rate associated with rupture. Clinicians should maintain a high index of suspicion for gastrointestinal PSA in patients with a history of pancreatitis who present with UGIB for whom no obvious source of bleeding can be identified on endoscopy.Figure 1.: CT Thx/Abd/Pel demonstrating a 2.0 x 1.6 cm out-pouching from the greater curvature of the stomach (arrow), postulated to be a gastric diverticulum, 2. CT Angiogram Abd/Pel demonstrating the splenic artery PSA (arrow), 3. Fluoroscopic imaging re-demonstrating the splenic artery PSA with extravasation of contrast into the gastric lumen, 4. Splenic Artery PSA after successful coil embolization.

“Blocked broken heart syndrome”: an unusual case of a complete atrioventricular block complicating a Takotsubo cardiomyopathy

BackgroundComplete heart block is considered a unique and rare complication of Takotsubo cardiomyopathy, an otherwise self-resolving disease. When this occurs, there is a double clinical dilemma: first to find out which triggered the other and second, to decide whether or not to implant a permanent pacemaker.Case presentationWe present a case of a 77 years-old female patient, with previous medical history of arterial hypertension, diabetes mellitus, dyslipidemia and bifascicular block known since 2013. She came to the emergency department after recurrent syncopal episodes. At admission a complete heart block was diagnosed, and it was implanted a single chamber temporary pacemaker. The patient remained in disproportional acute decompensated heart failure despite pacemaker implantation. She denied chest pain although referring an episode of self-limiting chest pain 2 days before, after an argument with the family. Blood analysis showed an important rise in NTproBNP and troponin levels. Transthoracic echocardiogram showed a dilated left ventricle with akinesia of apical and mid segments, hyperkinesia of basal segments and severely depressed left ventricle ejection fraction. Coronary angiography showed no significant lesions and the diagnosis of Takotsubo cardiomyopathy was suspected. During the following days, she recovered her own intrinsic rhythm. Electrocardiogram evolved with deep T-wave inversion and prolonged QT interval and transthoracic echocardiogram showed resolution of the previous alterations. Despite complete reversion of rhythm alteration, it was decided to implant a permanent pacemaker.ConclusionsWe describe a rare, life-threatening and often underdiagnosed complication of the stress cardiomyopathy. Furthermore, we performed a literature revision of this rare complication and discussed the therapeutic challenge encountered in such patients.

EnSite NavX mapping system guided implantation of a dual-chamber permanent pacemaker in a 41-year-old pregnant woman with a 4-year follow-up

BackgroundX-ray fluoroscopy has been the primary cardiac imaging modality in permanent pacemaker implantation (PPI) operations, but it inevitably results in radiation exposure for both operators and patients. Fluoroscopy is considered a contraindication, especially in certain circumstances, such as gestation, during which the fetus is most sensitive to radiation exposure. Therefore, measures to avoid radiation exposure are necessary, and a more safe and feasible approach is needed for this procedure. Since the EnSite NavX mapping system (ENMS) can create the required geometric contours of those relevant cardiac structures and chambers, it can be used as an alternative to X-ray fluoroscopy in PPI. In addition, because the displacement of atrial leads is a common complication of PPI, lead displacement may occur more readily without fluoroscopic guidance. Therefore, reliable measures are required to prevent leads from displacement.Case introductionA 41-year-old woman at the 15th week of gestation was referred to our department with recurrent episodes of syncope and amaurosis fugax for 2 years. Holter monitoring showed sinus rhythm, Mobitz Type II atrioventricular block and high-grade atrioventricular block with ventricular arrest up to 4945 ms. A dual-chamber PPI was performed successfully for the patient under the guidance of the ENMS instead of fluoroscopy. Displacement of atrial lead was effectively avoided by bending the top of atrial lead before implantation and making it a U-shape during operation, which left space for possible subsequent external pulling stress.ConclusionsFor PPI, ENMS is a feasible and reliable alternative to traditional X-ray fluoroscopy, especially when performing operations on pregnant patients. By bending the top of the active-fixation atrial lead into a U-shape during operation, the displacement of atrial lead may be avoided.

Systemic leak capillary syndrome with myocardial involvement and cardiogenic shock: a case report

BackgroundSystemic capillary leak syndrome (SCLS) is a potentially fatal disorder characterized by relapses of hypovolemic shock episodes.Case SummaryWe present a case of a 58-year-old man who presented to the Emergency Department with a history of recurrent episodes of syncope in the last hours. A few days before medical contact the patient complained of sore throat, fever, and flu-like symptoms. He was initially admitted with a diagnosis of suspected myopericarditis. Forty-eight hours later, the haemodynamic status suddenly deteriorated to a mixed cardiogenic and shock; an endomyocardial biopsy showed localized inflammatory infiltrates and areas of necrosis of cardiomyocytes with positive viral search for parvovirus B19 (PVB19), therefore the patient was treated with methylprednisolone pulses. Based on the concurrent presence of the typical triad of hypotension, hypoalbuminaemia, and haemoconcentration we suspected systemic leak capillary syndrome potentially triggered by the PVB19 infection with acute myocarditis. The clinical conditions further deteriorated with rhabdomyolysis and acute kidney injury: we started continuous veno-venous haemofiltration adding a cytokines adsorber. In the following hours, we observed a significant clinical improvement. The patient was discharged 1 month later and 5 months after discharge he experienced a new attack of SCLS, this time without myocardial involvement and with prompt symptoms resolution.ConclusionSystemic capillary leak syndrome is a potentially fatal disorder: early recognition of this entity and prompt initiation of supportive therapy are warranted, therefore, it is paramount that an emergency physician thinks of SCLS in patients with signs of cardiogenic shock and the classical triad of hypotension, hypoalbuminia, and haemoconcentration.

P1490: CHILDREN WITH SICKLE CELL DISEASE AND MOYAMOYA SYNDROME: A 10-YEAR EXPERIENCE

Background: Sickle cell anemia (SCD) is a multiorgan disease. Involvement of the central nervous system (CNS) is associated with increased mortality. Cerebrovascular accidents, especially when associated with permanent neurological deficits, are one of the most devastating potential outcomes of SCD. SCD can be complicated by Moyamoya syndrome (MMS), which is characterized by progressive stenosis of the supraclinoid segment of carotid arteries and development of typical collaterals, resulting in increased risk for both ischemic and hemorrhagic strokes. Aims: We describe the clinical characteristics of children with SCD and MMS treated at our center over the last 10 years and define the prevalence of MMS in our setting. Methods: We conducted a retrospective, descriptive study of all patients with SCD who were diagnosed with cerebrovascular disease and with MMS in our institution from 2012 to 2021. Indications for neuroimaging [(magnetic resonance imaging (MRI) or magnetic resonance angiography (MRA)] in our center include recurrent episodes of syncope, epileptic seizures, neurocognitive changes, time-averaged maximum mean velocity (TAMMV) of the middle cerebral or intracranial internal carotid arteries (ICA) measured ≥200 cm/s or before starting regular transfusion regime. Relevant clinical data was collected and summarized using descriptive statistics. Results: The study included 109 patients with SCD and 19,3% of them (21 patients) required neuroimaging. Within these patients, 6 presented with acute focal neurological signs that resulted in a diagnosis of ischemic stroke in four patients, and posterior reversible encephalopathy syndrome diagnosis in the other two. Besides these, 2 patients (9,5%) were diagnosed by neuroimaging with MMS and 1 (4,8%) had a pattern suggestive of MMS on brain MRI not confirmed by angiography. Furthermore, whereas four (19%) patients had no imaging findings suggestive of cerebrovascular disease, they presented with TAMMV ≥200cm/s, so they were proposed to regular transfusion regimen for primary prevention of stroke. Regarding the patients diagnosed with SCD and MMS, MMS was diagnosed at 9 years old in one patient and at 10 years old in another. Both patients had a SS genotype and were diagnosed following an abnormal surveillance transcranial doppler study, since they were asymptomatic for the disease. One of the patients was under erythrocytapheresis protocol and achieved a HbS level <20%. Currently she is without critical stenosis or cerebrovascular events, maintaining only imaging follow-up. The other patient was under regular transfusion regimen with HbS levels between 40 and 50%, and has been proposed for sibling bone marrow transplantation and encephaloduroarteriosynangiosis. Summary/Conclusion: The prevalence of MMS in our center (1,8%) is lower than described in other studies. Recent guidelines recommend at least a one-time MRI screening in early-school-age children to detect cerebrovascular disease earlier and reduce the burden of CNS complications in children and adults with SCD. By following these new recommendations, we expect an earlier and more frequent diagnosis of MMS, thus reducing the morbidity of these patients.