Atrio-Ventricular Conduction Abnormalities in Multisystem Inflammatory Syndrome in Children

Purpose of study Since mid-April 2020 in Europe and North America, clusters of pediatric cases with a newly described severe systemic inflammatory response with shock have appeared. Patients had persistent fevers >38.5 C, hypotension, features of myocardial dysfunction, coagulopathy, gastrointestinal symptoms, rash, and elevated inflammatory markers without other causes of infection. The World Health Organization, Centers for Disease Control, and Royal College of Paediatrics associated these symptoms with SARS-CoV-2 as multisystem inflammatory syndrome in children (MIS-C). Cardiac manifestations include coronary artery aneurysms, left ventricular systolic dysfunction evidenced by elevation of troponin-T (TnT) and pro-B-type naturietic peptide (proBNP), and electrocardiogram (ECG) abnormalities. We report the clinical course of three children with MIS-C while focusing on the unique atrioventricular (AV) conduction abnormalities. Case #1:19-year-old previously healthy Hispanic male presented with abdominal pain, fever, and non-bloody diarrhea for three days. He was febrile and hypotensive (80/47 mmHg) requiring fluid resuscitation. Symptoms, lab findings, and a positive COVID-19 antibody test were consistent with MIS-C. Methylprednisolone, intravenous immunoglobulin (IVIG), and enoxaparin were started. He required epinephrine for shock and high flow nasal cannula for respiratory distress. Initial echocardiogram demonstrated a left ventricular ejection fraction (LVEF) of 40% with normal appearing coronaries. Troponin and proBNP were 0.41 ng/mL and proBNP 15,301 pg/mL respectively. ECG showed an incomplete right bundle branch block. He eventually became bradycardic to the 30s-50s and cardiac tracing revealed a complete AV block (figure 1a). Isoproterenol, a B1 receptor agonist, supported the severe bradycardia until the patient progressed to a type 2 second degree AV block (figure 1b). A second dose of IVIG was administered improving the rhythm to a type 1 second degree AV block. An IL-6 inhibitor, tocilizumab was given as the rhythm would not improve, and the patient soon converted to a first-degree AV block. Cardiac magnetic resonance imaging showed septal predominant left ventricular hypertrophy and subepicardial enhancement along the basal inferior/anteroseptal walls typical for myocarditis. Case #2: 9-year-old previously healthy Hispanic male presented after three days of daily fevers, headaches, myalgias, diffuse abdominal pain, and ageusia. He was febrile, tachycardic, and hypotensive (68/39 mmHg). Hypotension of 50s/20s mmHg required 3 normal saline boluses of 20 ml/kg and initiation of an epinephrine drip. Severe hypoxia required endotracheal intubation. After the MIS-C diagnosis was made, he was treated with IVIG, mehtylprednisolone, enoxaparin, aspirin, and ceftriaxone. Due to elevated inflammatory markers by day 4 and patient's illness severity, a 7-day course of anakinra was initiated. Initial echocardiogram showed mild tricuspid and mitral regurgitation with a LVEF of 35-40%. Despite anti-inflammatory therapy, troponin and proBNP were 0.33 ng/mL and BNP of 25,335 pg/mL. A second echocardiogram confirmed poor function so milrinone was started. Only, after two doses of anakinra, LVEF soon normalized. Despite that, he progressively became bradycardic to the 50's. QTc was prolonged to 545 ms and worsened to a max of 592 ms. The aforementioned therapies were continued, and the bradycardia and QTc improved to 405 ms. Patient #3: 9-year-old African American male presented with four days of right sided abdominal pain, constipation, and non-bilious non-bloody emesis. He had a negative COVID test and unremarkable ultrasound of the appendix days prior. His history, elevated inflammatory markers, and positive COVID- 19 antibody were indicative of MIS-C. He was started on the appropriate medication regimen. Initial ECG showed sinus rhythm with normal intervals and echocardiogram was unremarkable. Repeat imaging by day three showed a decreased LVEF of 50%. ECG had since changed to a right bundle branch block. Anakinra as started and steroid dosing was increased. By day 5, he became bradycardic to the 50s and progressed to a junctional cardiac rhythm. Cardiac function normalized by day 7, and anakinra was subsequently stopped. Thereafter, heart rates ranged from 38-48 bpm requiring transfer to the pediatric cardiac intensive care unit for better monitoring and potential isoproterenol infusion. He remained well perfused, with continued medical management, heart rates improved. Methods used Retrospective Chart Review. Summary of results Non-specific T-wave, ST segment changes, and premature atrial or ventricular beats are the most often noted ECG anomalies. All patients initially had normal ECGs but developed bradycardia followed by either PR prolongation or QTc elongation. Two had mild LVEF dysfunction prior to developing third degree heart block and/or a junctional escape rhythm;one had moderate LVEF dysfunction that normalized before developing a prolonged QTc. Inflammatory and cardiac markers along with coagulation factors were the highest early in disease course, peak BNP occurred at approximately hospital day 3-4, and patient's typically had their lowest LVEF at day 5-6. Initial ECGs were benign with PR intervals below 200 milliseconds (ms). Collectively the length of time from initial symptom presentation till when ECG abnormalities began tended to be at day 8-9. Patients similarly developed increased QTc intervals later in the hospitalization. When comparing with the CRP and BNP trends, it appeared that the ECG changes (including PR and QTc elongation) occurred after the initial hyperinflammatory response. Conclusions Although the mechanism for COVID-19 induced heart block continues to be studied, it is suspected to be secondary to inflammation and edema of the conduction tissue. Insufficiency of the coronary arterial supply to the AV node and rest of the conduction system also seems to play a role. Although our patients had normal ECG findings, two developed bundle branch blocks prior to more complex rhythms near the peak of inflammatory marker values. Based on the premise that MIS-C is a hyperinflammatory response likely affecting conduction tissue, our group was treated with different regimens of IVIG, steroids, anakinra, and/or tocilizumab. Anakinra, being an IL-1 inhibitor, has been reported to dampen inflammation in viral myocarditis and tocilizumab has improved LVEF in rheumatoid arthritis patients. Based on our small case series, patient's with MISC can have AV nodal conduction abnormalities. The usual cocktail of IVIG and steroids helps;however, when there are more serious cases of cardiac inflammation, adjuvant immunosuppresants like anakinra and toculizumab can be beneficial. (Figure Presented).

Prevalence and diagnostic significance of novel 12-lead ECG patterns following COVID-19 infection in elite soccer players

Background: Identification of athletes with cardiac inflammation following COVID-19 can prevent exercise fatalities. The efficacy of pre and post COVID-19 infection electrocardiograms (ECGs) for detecting athletes with myopericarditis has never been reported. We aimed to assess the prevalence and diagnostic significance of novel 12-lead ECG patterns following COVID-19 infection in elite soccer players. Method(s): We conducted a multicentre study over a 2-year period involving 5 centres and 34 clubs and compared pre COVID and post COVID ECG changes in 455 consecutive athletes. ECGs were reported in accordance with the International recommendations for ECG interpretation in athletes. The following patterns were considered abnormal if they were not detected on the pre COVID-19 infection ECG: (a) biphasic T-waves;(b) reduction in T-wave amplitude by 50% in contiguous leads;(c) ST-segment depression;(d) J-point and ST-segment elevation >0.2 mV in the precordial leads and >0.1 mV in the limb leads;(e) tall T-waves >=1.0 mV (f) low QRS-amplitude in >3 limb leads and (g) complete right bundle branch block. Athletes exhibiting novel ECG changes underwent cardiovascular magnetic resonance (CMR) scans. One club mandated CMR scans for all 28 (6%) athletes, despite the absence of cardiac symptoms or ECG changes. Result(s): Athletes were aged 22+/-5 years (89% male and 57% white). 65 (14%) athletes reported cardiac symptoms. The mean duration of illness was 3+/-4 days. The post COVID ECG was performed 14+/-16 days following a positive PCR. 440 (97%) athletes had an unchanged post COVID- 19 ECG. Of these, 3 (0.6%) had cardiac symptoms and CMRs resulted in a diagnosis of pericarditis. 15 (3%) athletes demonstrated novel ECG changes following COVID-19 infection. Among athletes who demonstrated novel ECG changes, 10 (67%) reported cardiac symptoms. 13 (87%) athletes with novel ECG changes were diagnosed with inflammatory cardiac sequelae;pericarditis (n=6), healed myocarditis (n=3), definitive myocarditis (n=2), and possible/probable myocarditis (n=2). The overall prevalence of inflammatory cardiac sequelae based on novel ECG changes was 2.8%. None of the 28 (6%) athletes, who underwent a CMR, in the absence of cardiac symptoms or novel ECG changes revealed any abnormalities. Athletes revealing novel ECG changes, had a higher prevalence of cardiac symptoms (67% v 12% p<0.0001) and longer symptom duration (8+/-8 days v 2+/-4 days;p<0.0001) compared with athletes without novel ECG changes. Among athletes without cardiac symptoms, the additional yield of novel ECG changes to detect cardiac inflammation was 20% (n=3). Conclusion(s): 3% of elite soccer players demonstrated novel ECG changes post COVID-19 infection, of which almost 90% were diagnosed with cardiac inflammation during subsequent investigation. Most athletes with novel ECG changes exhibited cardiac symptoms. Novel ECGs changes contributed to a diagnosis of cardiac inflammation in 20% of athletes without cardiac symptoms.

ASSOCIATION OF ELECTROCARDIOGRAM FINDINGS WITH MORTALITY IN COVID-19 PATIENTS

Objective: The COVID-19 virus has greatly debilitated a lot of patients in the Philippines. Baseline demographic characteristics and other parameters such as baseline electrocardiographic (ECG) findings were investigated to act as predictors for mortality among admitted patients who are RT-PCR positive for COVID. This study aims to determine the association of baseline ECG findings including both normal and abnormal variants such as those suggestive of acute cardiac injury and hypertrophy with mortality of these patients. Design and method: This is a retrospective study of patients admitted from March 2020 to May 2021 who were diagnosed to have COVID-19 thru an RT-PCR swab. Inclusion criteria include a recorded baseline ECG. Demographic characteristics were also included. Patients were divided according to their ECG findings as normal and abnormal. Among those with abnormal ECG findings, they were further subdivided into those with ischemic changes, hypertrophy, and miscellaneous (bundle branch blocks). Results: A total of one hundred thirty-six (136) patients were included in this study. Among the abnormal ECG findings obtained, the miscellaneous group particularly those with right bundle branch block was noted to be associated with mortality (OR = 4.86, 95% CI 1.04-22.7, p = 0.044). Other ECG findings such as ischemic changes and hypertrophy showed no significant association. Conclusion: Among the different classifications of ECG findings, those with baseline ECG findings of right bundle branch block showed a positive association with mortality. Other parameters such as demographic features are also noted to have no association with mortality. Further investigation and a higher population number are suggested to further conclude these findings.

Dynamic change of an electrocardiogram in myocarditis following mRNA COVID-19 vaccination

Background and Aim: Coronavirus disease (COVID-19) is a pandemic infectious disease caused by the SARS-CoV-2 virus. At the begin-ning of 2021, the Food and Drug Administration (FDA) of the United States issued an emergency use authorization (EUA) for the Pfizer-BioNTech COVID-19 vaccine to prevent COVID-19 infection. Cases of myocarditis and pericarditis after the vaccination in adolescents and young adults have been reported, especially more often after the second dose. There are very few case reports in Asia. We aim to present a case of confirmed myocarditis after the Pfizer-BioNTech COVID-19 vaccine in Thailand and the remarkable dynamic change in an electrocardiogram (ECG). Method(s): Case report. We reported the case and reviewed serial electrocardiogram and echocardiographic findings, including car-diac magnetic resonance imaging (MRI) in a case of confirmed myocarditis after the Pfizer-BioNTech COVID-19 vaccine. Result(s): A previously healthy 13-year-old boy presented with chest pain and shortness of breath within 20 hours following the second dose of the Pfizer-BioNTech COVID-19 vaccine. Electrocardiogram (ECG) revealed diffuse ST-segment elevation with significant improvement within 3 hours. The peak level of high sensitivity cardiac troponin T was 1,546 ng/L. No alternative etiology of myocarditis was identified. Echocardiogram revealed mildly depressed left ventricular septal wall motion. Cardiac MRI showed abnormal native T1, T2 mapping, and extracellular volume (ECV) that were compatible with myocarditis. His symp-toms were relieved with ibuprofen. He was discharged on the fifth day of admission. In a 1-week follow-up, the ECG showed an incomplete right bundle branch block. Conclusion(s): This case illustrates the potential of myocarditis follow-ing mRNA COVID-19 vaccination with striking dynamic change and transition of the ECG. The possible mechanism for myocar-ditis after vaccination is molecular mimicry between viral spike protein and cardiac protein. More data and long-term follow-up are needed to understand the association between the COVID-19 vaccine and myopericarditis.

"BUT I AM TOO YOUNG FOR THIS": EARLY PRESENTATION OF USUAL INTERSTITIAL PNEUMONIA

SESSION TITLE: Diagnosis of Lung Disease through Pathology Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Usual interstitial pneumonia (UIP) is a histological term used to describe a pattern of interstitial fibrosis with alternating areas of the normal lung with temporal fibrosis and architectural alteration due to chronic scarring or honeycomb change. It is a subset of idiopathic interstitial pneumonias (IPF) that usually presents in the sixth and seventh decades of life with progressive dyspnea on exertion and productive cough. CASE PRESENTATION: We present a 46 y/o man with a history of thyroid disease, hypertension and a former smoker of 20 pack-year smoking. Presented to ED complaining of low oxygen saturation with pulse oximetry at home with readings between 60-80%. Accompanied with progressive dyspnea on exertion and unintentional weight loss of 80 pounds in the last year. Also referred productive cough of white sputum that was worse in the morning. Home nebulized Albuterol therapy did not provide improvement. Denied recent viral respiratory infections, night sweats, environmental exposures nor family history of lung disease. DISCUSSION: Physical exam demonstrated bilateral expiratory dry crackles and pulse oximetry oxygen saturation at room air of 78%. RBBB evidenced on EKG. Bloodwork showed polycythemia with hemoglobin of 17.8;ABG's with pH: 7.40, Pco2: 42.2, PO2: 59.8, HCO3: 26, O2 sat: 90.8 and ideal PO2: 85.6 consistent with metabolic alkalosis with BMP CO2 of 30, A/a gradient: 43.0. Mycoplasma IgM, Influenza A & B and COVID-19 antigen test were negative. CXR with increased vascular markings, chest CT demonstrated small pericardial effusion, bilateral coarse interstitial pulmonary markings and bronchiectasis suggestive of chronic interstitial lung disease with no specific pattern. Left heart catheterization revealed right ventricular hypertrophy, normal EF >55%, and no evidence of coronary disease. Alpha-1 antitrypsin: 158, EPO: 6.5, HIV, and hepatitis panel were all negative. Rheumatology work up with only an ANA antibody positive, with titer 1:160. Patient underwent VATS procedure with wedge biopsy of the right upper and middle lobe that revealed usual interstitial pneumonia pattern. Patient improved and was discharged on home oxygen 3L. At follow-up, treatment was started with Nintedanib and Sildenafil Citrate. He had clinical improvement and oxygen requirements decreased to intermittent oxygen. CONCLUSIONS: Patients with interstitial pulmonary fibrosis experience slow progressive decline with typical clinical presentation over 60 years of age. This case remarks the importance of the need for stratification of interstitial lung disease classification, when pattern and history are non specific, with the use of VATS procedure for early start of treatment. Our patient with no environmental exposure or connective tissue disease had an uncommon early presentation of usual interstitial pneumonia. Reference #1: Tibana, R.C.C., Soares, M.R., Storrer, K.M. et al. Clinical diagnosis of patients subjected to surgical lung biopsy with a probable usual interstitial pneumonia pattern on high-resolution computed tomography. BMC Pulm Med 20, 299 (2020). https://doi.org/10.1186/s12890-020-01339-9 DISCLOSURES: No relevant relationships by Jesse Aleman No relevant relationships by Carlos Martinez Crespí no disclosure submitted for Jean Ramos;No relevant relationships by Alexandra Rodriguez Perez No relevant relationships by Paola Vazquez No relevant relationships by Nahomie Veguilla Rivera

IDIOPATHIC ISOLATED RIGHT VENTRICULAR DYSFUNCTION IN THE SETTING OF RECENT COVID-19 INFECTION: A CASE REPORT

CASE: We describe a case of isolated acute right ventricular (RV) strain not attributable to pulmonary embolism (PE) or Acute Respiratory Distress Syndrome (ARDS) in the setting of recent COVID-19 infection. A 77-year-old male with medical history notable for type 2 diabetes, obesity, chronic kidney disease, obstructive sleep apnea, and chronic hypoxemic respiratory insufficiency with a last known left ventricular ejection fraction (LVEF) of 77% on admission with preserved RV function, and recent COVID-19 infection was admitted for septic shock secondary to a post-viral MRSA pneumonia 12 days after diagnosis with COVID-19. On day 5 of admission, after completion of antibiotic therapy and resolved shock, the patient developed relative hypotension and an oliguric acute kidney injury with creatinine of 1.9 (previously 1.0) and urine microscopy findings consistent with acute tubular necrosis. EKG at the time showed new incomplete right bundle branch block. On day 8 of admission, relative hypotension continued with an uptrend in creatinine to 4.8 despite adequate fluid resuscitation. EKG showed new complete right bundle branch block with high-sensitivity troponin peaking at 550 (previously 15). A transthoracic echocardiogram showed enlarged RV and isolated severe hypokinesis of the RV mid-free wall consistent with “McConnel's sign” and acute right heart strain, with poorly visualized left ventricle, but without regional wall motion abnormalities. CTA Chest evaluating through the segmental arteries ruled out acute PE. Acute coronary syndrome was ruled out with traditional and right-sided EKG. Oxygen requirements remained unchanged throughout the course of his admission. The patient was transferred to the ICU for undifferentiated shock requiring triple pressor therapy and eventually died from acute renal failure and volume overload. IMPACT/DISCUSSION: While RV strain secondary to ARDS and PE has been implicated in COVID-19 and found to be an independent predictor of mortality, there is limited literature describing isolated RV dysfunction in their absence. Increasing reports showing cardiac microthrombi in autopsies of COVID-19 patients suggest alternate etiologies of RV injury and suggest potential utility of empiric therapeutic anticoagulation in all patients presenting with COVID-19. Alternatively, direct viral injury isolated to the RV may be unique in COVID19. Additionally, “McConnell's sign” combined with enlarged RV is traditionally considered a specific marker of PE, with reported specificity of 94% in the original report. However, there are increasing reports shedding doubt on the specificity of this finding. CONCLUSION: This case demonstrates the need to consider alternate etiologies for RV dysfunction in COVID-19, including microthrombi and direct viral injury. Additionally, this case adds to the growing literature demonstrating the limitation of “McConnell's sign,” even in patients with high suspicion for PE.

Next-Day Discharge Following Transcatheter Aortic Valve Implantation: An Australian Tertiary Hospital Experience

Background: With increasing utilisation of transcatheter aortic valve implantation (TAVI) for aortic stenosis, there is a need to explore the safety of next-day discharge. We aimed to evaluate the safety and outcomes of next-day discharge following TAVI. Methods: We performed a retrospective analysis of patients who underwent TAVI at a tertiary centre between 2020 and 2021. Included patients were those discharged the next day after TAVI as routine care. Data collected included baseline demographics, Society of Thoracic Surgeons (STS) score, perioperative complications and 30-day mortality rates. Results: Thirty-three patients (33% female, median age 82 years;interquartile range [IQR], 77–84) were discharged the next day post-TAVI. Median STS score was 2.3% (IQR, 1.7–3.6). On pre-TAVI ECG, two patients (6%) had right bundle branch block (QRS duration 147–154 ms). All patients demonstrated well-seated aortic valve prosthesis with no aortic regurgitation on same-day transthoracic echocardiogram. Six patients (18%) had new conduction abnormalities post-TAVI (five transient left bundle branch block, one atrial fibrillation which self-resolved). There were no significant procedural complications including no pericardial effusion or vascular injury. All patients were discharged directly home without the need for subacute care. Two patients (6%) were re-hospitalised within 30 days of discharge: one admitted with presyncope of unclear cause and one required a pacemaker for tachy-brady syndrome. All patients were alive and well at 30 days. Conclusion: We have demonstrated that next-day discharge TAVI is safe in selected patients with an uncomplicated procedure. In the era of COVID, implementation of next-day discharge can reduce unnecessary length of stay and may improve hospital resource allocation.

British Cardiovascular Society Annual Conference, '100 years of Cardiology'

The proceedings contain 226 papers. The topics discussed include: mitral valve abnormalities in decedents of sudden cardiac death due to hypertrophic cardiomyopathy and idiopathic left ventricular hypertrophy;prevalence and diagnostic significance of novel 12-lead ECG patterns following COVID-19 infection in elite soccer players;clinical outcomes and myocardial recovery in energetics, perfusion and contractile function after valve replacement surgery in severe aortic stenosis patients with diabetes comorbidity;brain phenotype of takotsubo syndrome;improving the diagnostic accuracy of apical hypertrophic cardiomyopathy;investigating a novel role for nesprin-1 and the linc complex in cardiomyocyte mechanotransduction;transcatheter aortic valve implantation in patients with right bundle branch block: should prophylactic pacing be undertaken?;and disease penetrance in asymptomatic carriers of familial cardiomyopathy variants.

Covid-19 survivors may have deterioration in frontal plane qrs-t angle and the other electrocardiogram parameters

Background: COVID-19 infection is known to damage myocardial tissue and increase arrhythmic events. However, the data in the literature on permanent attachments are limited. In our study, we planned to investigate possible arrhythmic damages in COVID-19 survivors using the frontal plane QRS-T [f(QRS)-T] angle and some other ECG parameters. Patients & Methods: 269 patients who recovered from COVID-19 between April 2020 and January 2021 were included into the study. Pre-admission electrocardiograms and first-month outpatient clinic control ECGs of the patients were compared. Results: After COVID-19, left bundle branch block (p<0.001), right bundle branch block (p<0.001), right bundle branch block (p<0.001), atrial fibrillation (p<0.001) rates had increased. Prolongation was detected in QRS duration (p<0.001), QT interval (p=0.014), adjusted QT interval (p =0.007) and Tpe interval (p=0.012). F(QRS)-T angle (p<0.001) and fragmented QRS rate (p<0.001) were increased. Conclusions: It was observed in our study that;even if patients survive after COVID-19, permanent deterioration in ECG parameters may occur.

A MULTIMODALITY IMAGING DIAGNOSIS OF A CHALLENGING CASE OF CONCOMITANT GROUP 1 PULMONARY HYPERTENSION AND PARADOXICALLY LOW- FLOW, LOW-GRADIENTAORTIC STENOSIS

A 76 year old woman was admitted to our hospital for self-limiting dyspnoea (NYHA class III) in oxygen dependence and frequent lipothymia following Valsalva manoeuvres. She was previously admitted to a Spoke Centre for heart failure (HF) with preserved ejection fraction (EF) and a new diagnosis of “pre-capillary pulmonary hypertension (PH)”. Despite a diagnosis of PH of unclear aetiology, she was started on macitentan without being reassessed for functional capacity due to Covid emergency;because of worsening symptoms, she was admitted to our Hub Centre. Resting ECG showed right axis deviation, right ventricle (RV) hypertrophy, first-degree atrioventricular block and right bundle branch block. Transthoracic echocardiography (TTE) showed reduced left ventricular (LV) volume with preserved EF (diastolic volume= 37 ml, EF=88%), severe right atrial and RV dilation with flattening of the interventricular septum, estimated pulmonary artery systolic pressure (PASP) of 124 mmHg, and moderate calcific aortic stenosis (peak aortic velocity 3.3 m/s, mean gradient 25 mmHg, valve area 1.1 cm2). Right and left heart catheterization showed severe pre-capillary PH (mean pulmonary pressure 60 mmHg, mean wedge 11 mmHg, pulmonary vascular resistance 14.41 WU), a severe aortic valve stenosis (aortic valve area 0.68 cmq and peak-to-peak gradient 25 mmHg, slight reduction of cardiac index 2.04 l/min/mq) and no significant coronary artery disease. The degree of aortic stenosis was considered as moderate-severe by integrating data of transesophageal echocardiography (planimetric area 1cm2) and assessment of calcium score (1615 Agatson units). Pneumological causes, chronic thromboembolic PH, rheumatologic diseases, HIV infection, paraneoplastic origin and veno-occlusive disease were all ruled out as potential PH causes and a diagnosis of Idiopathic pulmonary arterial hypertension (IPAH) was finally made. The Heart Team established the best therapeutic option was a transcatheter aortic valve replacement (TAVI) allowing better haemodynamic tolerability of PH therapy. The patient underwent TAVI and was started on PH therapy;a complete atrio-ventricular block developed after the procedure, requiring permanent pacemaker (PM) implantation. Unfortunately, few days later, the patient died following pacemaker's lead dislocation. Conclusion: PH has a diverse aetiology, and prognosis is generally poor, especially in patients with severe comorbidities. (Figure Presented).

Role of Dexmedetomidine in Perioperative Management of Pheochromocytoma

The prevalence of Pheochromocytoma in pat ient with hypertension is 0.1 -0.6%. These types of tumours are known for unpredictable perioperative course and hemodynamic instability. Various different drugs and anaesthesia techniques can be used to tackle these situations. Dexmedetomidine is emerged as newer agent with better hemodynamic stability, reducing requirement of other anaesthesia drugs, blunting of sympathoadrenal response in resection of Pheochromocytoma. We report four cases operated between January 2021 to June 2021.Preoperative preparation was done with α and β blockade. Dexmedetomidine was used during induction as 1 mcg/kg over 10 mins followed by 0.7mcg/kg/hr intraoperatively. Combination of Dexmedetomidine, Fentanyl, NTG, Isoflurane and Epidural analgesia was used. IF needed boluses of Esmolol and Labetalol were used during tumor manipulation. All the patients had an uneventful perioperative course. Dexmedetomidine with pre-operative α and β blockade reduce the need of other drugs intraoperatively and can be used as anaesthetic adjunct to maintain steady hemodynamic.

Inherited arrhythmias in children with infectious diseases

Introduction: The connection of a number of inherited arrhythmias with febrile body temperature is proved. Due to connection between fever and clinical manifestations (including ECG changes) of inherited arrhythmias there are additional opportunities for diagnostics of these life-threatening arrhythmias in infectious patients (including Covid -19). Methods: 3584 ECGs of children with infectious diseases (average age 8.5±5.3 years old;boys - 57.5%, girls - 42.5%) were analyzed. Patients (pts) with QTc>440 ms or QTc<320 ms, complete right bundle branch block, left bundle branch block or its branches, atrioventricular block, ST elevation in the right precordial leads were given additional examination depending on the intended diagnosis (inherited arrhythmias): daily 12-channel Holter ECG monitoring, stress test, echocardiography. The family history was also clarified (cases of sudden cardiac death, syncope). The diagnosis was made on the basis of generally accepted diagnostic criteria and confirmed by molecular genetic analysis. Results: ECG changes, which are typical for Brugada syndrome (BrS), type 1, were detected in 2 pts (0.05%). Long QT syndrome (LQTS) was detected in 2 pts too. Mutations in the SCN5A gene (exon 16 Arg893Cys, R878H) were identified in pts with BrS and in the KCNQ1 (exon 9 Trp379Ter) with LQTS. In pts with LQTS, sinus tachycardia was registered with the background of increased body temperature, which allowed to reveal long QT interval. 1 pt with LQTS is a female athlete. 1pt with BrS had been previously observed by a cardiologist in connection with grade I atrioventricular block. An increase in body temperature leads to disruption of the sodium ion channels which underlie the development of the BrS. In the case of LQTS, in our study, the increase in the QTc is most likely due to a change in heart rate rather than a direct effect of an increase in body temperature on the ion channels. Conclusions: 1. BrS (type 1) was detected in 2 pts (0.05%) and LQTS in 2 pts (0.05%) at first. We consider that when taking an ECG from pts with fibril body temperature, this percentage may be higher. 2. ECG registration in pts with fever (including athletes) raises the probability of timely inherited arrhythmias diagnosis.

THE MANAGEMENT AND SCREENING OF FAMILIAL ARRHYTHMOGENIC CARDIOMYOPATHY

Background: Arrhythmogenic cardiomyopathy (ACM) is a hereditary condition associated with VT and sudden cardiac death (SCD). Currently, only 50% of individuals diagnosed with ACM have identifiable gene variants. Case: A 55-year-old woman presented with rapid palpitations, and recurrent syncope and presyncope. Her past medical history included Ehlers-Danlos syndrome and spontaneous carotid artery dissection. Her family history included MI in her father and cardiac disorders - including SCD - in many non-first-degree relatives. Her ECG revealed sinus rhythm with RBBB and LAFB. Decision-making: Our team performed an EP study in which atrial fibrillation and two beats of AVNRT were induced, and an ILR was implanted. A 28 beat run of symptomatic VT at 166 bpm was detected during the COVID-19 pandemic when elective procedures were suspended. This prompted the use of a wearable defibrillator to prevent SCD. When elective procedures resumed, the patient underwent CTA (negative) and cardiac MRI revealing localized RV apical free wall akinesia and mild RV enlargement. The Mount Sinai Morningside EP team concluded the patient met the task force criteria for ACM (class IIa per HRS consensus) and recommended the use of an ICD which was implanted two days later. Additionally, genetic testing was performed and was negative for identifiable gene variants (Invitae Arrhythmogenic Cardiomyopathy Panel). Her three daughters also underwent EP studies and ILR implantations as a result of rSR’ patterns seen on their ECGs. The eldest daughter had 6 beats of VT and a cardiac MRI found diffuse cardiomyopathy (but not ACM), the youngest had SVT but no ACM, and the middle daughter had no arrhythmia/ACM. Workup and management are in progress and involve the same approach received by the incident patient. Conclusion: This case demonstrates that during a pandemic lockdown wearable defibrillators can be used as an effective bridge to definitive treatment of VT. It also illustrates that additional ACM gene mutations have yet to be discovered since a genetic component is likely involved in this pedigree. Close follow-up with the cardiac team will ensure safe and effective management of this potentially lethal, but treatable condition.

Diagnosis and robotic-assisted, minimally invasive, autologous pericardial patch repair of a sinus venosus atrial septal defect (ASD) with repair of partial anomalous pulmonary venous connection

Case Report Sinus venosus ASDs are usually associated with one or more anomalous right sided pulmonary veins. Diagnosis by transthoracic echocardiogram (TTE) and confirmation with transesophageal echocardiogram (TEE) and right heart catherization can lead to a multidisciplinary approach for appropriate surgical correction. Case A 21-year-old male with no PMH presented to clinic for a routine physical to return to collegiate athletics post COVID-19 infection. His only complaint during this time was residual dyspnea (NYHA Class 1). An ECG was obtained and showed an incomplete right bundle branch block and TTE revealed an ASD with moderate RV dilation. Repeat TTE at our institution showed an interatrial shunt on injection of agitated saline via the right arm within three beats after injection. Subsequent right heart catheterization with shunt series revealed a step in oxygen saturation from 75% in the superior vena cava (SVC) to 88% in the right atrium. Additional imaging obtained with TEE confirmed a sinus venosus ASD. Cardiovascular surgery was engaged and further imaging with computed tomography angiography (CTA) of the chest confirmed a large superior sinus venosus ASD measuring 16 mm in diameter as well as partial anomalous right pulmonary venous drainage into the SVC. The heart team decided on a minimally invasive robotic approach and performed an autologous pericardial patch repair of the ASD with redirection of the right and superior pulmonary veins into the left atrium. Intra-op TEE showed no residual shunt across the interatrial septum. The patient had an uncomplicated post-operative course and was discharged home on day 4. Decision-Making Sinus venosus ASDs and associated anomalous pulmonary veins are often missed on TTE. In our patient, TEE and CTA assisted in the detection of anomalous pulmonary venous connection. A multidisciplinary heart team approach helped determine and tailor the best option for surgical correction in our patient's case. Conclusion Sinus venosus defects account for up to 10% of ASDs and can lead to pulmonary hypertension if left uncorrected. TTE remains the first imaging modality in assessing for ASDs, but TEE, RHC, and CTA can assist in comprehensive diagnosis and planning for procedural correction. Surgical closure in patients less than 25 years old without pulmonary hypertension is associated with low postoperative mortality, and a multidisciplinary approach can help ensure the most optimal method of surgical correction. (Figure Presented).

Pathophysiology of thromboembolic risk in SARS-CoV-2 pneumonia

Background: SARS-CoV-2 disease may be associated with a state of hypercoagulability and an increased risk of both venous and arterial thromboembolism. Clinical and biological evidence has documented a high thromboembolic risk in the acute phase of the infection, but the incidence of the risk in the late phase of the disease requires further investigation. Description of the case: A hypertensive and diabetic patient comes to the emergency room for dyspnea and fever. It is hypotensive, tachycardic and hypoxemic. Elevated D-dimer and troponin. ECG: sinus tachycardia and right bundle branch block. Echocardiogram: hypertensive heart disease and right overload. Chest CT: bilateral interstitial pneumonia, with filling defects at the bifurcation of the right pulmonary artery. Nasopharyngeal swab for SARSCoV- 2: positive. Conclusions: SARS-CoV-2 is configured as a multidimensional disease whose characteristic physiopathological and clinical aspects are being defined: a) an increased immunological and inflammatory response with activation of a cytokine storm and consequent coagulopathy, which favours both venous thromboembolism events and thrombosis in situ in the pulmonary arterioles and alveolar capillaries;b) a high intrapulmonary shunt, which accounts for the severity of respiratory failure, attributed to a reduced hypoxic pulmonary vasoconstriction with pulmonary neoangiogenic phenomena. These patients may benefit from anticoagulant therapy initiated as early as the diagnosis of SARS-CoV-2 pneumonia.