PPP1R13L-Related Cardiomyopathy Stability Following IL1 Inhibition

Introduction: Variants in PPP1R13L are associated with severe childhood-onset cardiomyopathy resulting in rapid progression to death or cardiac transplantation. PPP1R13L is proposed to encode a protein that limits the transcriptional activity of the NFkappaB pathway leading to elevated IL-1, IL-6, and TNF-alpha production in murine models. Optimal medical management for PPP1R13L-related cardiomyopathy is unknown. Here we report usage of a targeted anti-IL-1 immuno-modulatory therapy resulting in cardiac stabilization in a pediatric patient with congenital cardiomyopathy secondary to PPP1R13L variants. Case Report: A 4-year-old boy presented acutely with fever in the setting of persistent abdominal pain, vomiting, fatigue, and decreased appetite for two months following a mild COVID-19 related illness. Echocardiogram revealed severely depressed biventricular systolic function with an ejection fraction of 30%. Due to acute decompensated heart failure symptoms with hemodynamic instability, he was intubated and placed on continuous inotropic infusions with aggressive diuresis. Cardiac MRI demonstrated extensive subepicardial to near transmural fibrosis by late gadolinium enhancement in right and left ventricles. An implantable cardioverter-defibrillator (ICD) was placed due to frequent runs of polymorphic non-sustained ventricular tachycardia. Testing for viral pathogens was positive for rhino/enterovirus. Initial genetic testing was non-diagnostic (82-gene cardiomyopathy panel) but given the patient's significant presentation whole genome sequencing was pursued that showed two separate PPP1R13L variants in trans (c.2167A>C,p.T723P and c.2179_2183del,p. G727Hfs*25, NM_006663.4). Patient serum cytokine testing revealed elevations in IL-10 (4.7 pg/mL) and IL-1beta (20.9 pg/mL). Given the patient's tenuous circumstances and concern for continued progression of his cardiac disease, a trial of IL-1 inhibition via anakinra dosed at 3 mg/kg or 45 mg daily was initiated following hospital discharge. With approximately 6 months of therapy, the patient's cardiac function is stable with normalization of IL-10 and IL-1beta serum levels. Notably, the ventricular arrhythmia decreased after initiation of anakinra with no ICD shocks given. Therapy overall has been well tolerated without infectious concerns. Conclusion(s): In patients with PPP1R13L-related cardiomyopathy, immuno-modulatory therapies should be considered in an attempt to slow cardiac disease progression.Copyright © 2023 Elsevier Inc.

Ahead of the Curve - Experience from the Uk's First Dedicated Specialist Crm Clinic for Achd Patients

Background: The population of Adults with Congenital Heart Disease (ACHD) is expanding. A significant number will require Cardiac Rhythm Management (CRM) devices. In current UK practice, these patients are routinely seen in non-specialist CRM clinics and little is published regarding best-practice CRM programming and management in the ACHD population. Objective(s): Our objective was to establish a new model of patient-centred/-specific care delivered by specialist CRM physiologists, supported by an EP consultant (with a special interest in ACHD) in a dedicated clinic. We hoped to set new standards of care and patient experience, and improve efficiency and outcomes. Method(s): Data was collected from the electronic record system and CRM device database. A control group of non-ACHD patients was selected at random at our institution over the same period (2018-2022). Result(s): The clinic population n = 468 had a sex ratio of 0.92 (M:F) and mean age of 44 years (range 16 - 86). Mean time since primary implant was 9 years. All device types were represented: loop recorder (52), pacemaker (262), cardioverter defibrillator (116) and cardiac resynchronisation therapy devices (38). The underlying ACHD condition was: simple 46%, moderate 28% and complex 26%. Outcomes of appointments (n = 1,234) are shown vs controls (n = 126) (figure 1). Appointment and patient numbers rose year-on-year (100 to 226 patients, 281 to 367 appointments). There was a lower incidence of 'no review / reprogramming ' in ACHD CRM clinic appointments compared to the non-ACHD population, as well as a higher incidence of programming changes, however the trend over time within the ACHD group showed an increase in 'no review / reprogramming' and a decrease in reviews / reprogramming events. In contrast, non-ACHD patients had an increase in medical reviews and reprogramming required between 2018/19 and 2021/22. This is likely due to the COVID pandemic and deferred time to appointments and review. Conclusion(s): Our data demonstrate that the ACHD CRM population require additional input from the medical and scientist teams when compared to non-ACHD patients, however over time there has been a reduction in major programming/review and a commensurate increase in minor programming/discussion and no review. A reverse trend was observed in the non-ACHD patients pre- and post- COVID. These data support the proposal that specialised clinics provide the optimal management ACHD CRM clinics and should be delivered by dedicated practitioners. [Formula presented]Copyright © 2023

Incidence of ventricular arrhythmias related to COVID infection and vaccination in patients with Brugada syndrome: Insights from a large Italian multicenter registry based on continuous rhythm monitoring.

INTRODUCTION: Brugada syndrome (BrS) has a dynamic ECG pattern that might be revealed by certain conditions such as fever. We evaluated the incidence and management of ventricular arrhythmias (VAs) related to COVID-19 infection and vaccination among BrS patients carriers of an implantable loop recorder (ILR) or implantable cardioverter-defibrillator (ICD) and followed by remote monitoring. METHODS: This was a multicenter retrospective study. Patients were carriers of devices with remote monitoring follow-up. We recorded VAs 6 months before COVID-19 infection or vaccination, during infection, at each vaccination, and up to 6-month post-COVID-19 or 1 month after the last vaccination. In ICD carriers, we documented any device intervention. RESULTS: We included 326 patients, 202 with an ICD and 124 with an ILR. One hundred and nine patients (33.4%) had COVID-19, 55% of whom developed fever. Hospitalization rate due to COVID-19 infection was 2.76%. After infection, we recorded only two ventricular tachycardias (VTs). After the first, second, and third vaccines, the incidence of non-sustained ventricular tachycardia (NSVT) was 1.5%, 2%, and 1%, respectively. The incidence of VT was 1% after the second dose. Six-month post-COVID-19 healing or 1 month after the last vaccine, we documented NSVT in 3.4%, VT in 0.5%, and ventricular fibrillation in 0.5% of patients. Overall, one patient received anti-tachycardia pacing and one a shock. ILR carriers had no VAs. No differences were found in VT before and after infection and before and after each vaccination. CONCLUSIONS: From this large multicenter study conducted in BrS patients, followed by remote monitoring, the overall incidence of sustained VAs after COVID-19 infection and vaccination is relatively low.

Application of Novel Technologies in Cardiac Electrotherapy to Prevent Complications.

(1) Background: Cardiac electrotherapy is developing quickly, which implies that it will face a higher number of complications, with cardiac device-related infective endocarditis (CDRIE) being the most frequent, but not the only one. (2) Methods: This is a retrospective case study followed by a literature review, which presents a patient with a rare but dangerous complication of electrotherapy, which could have been prevented if modern technology had been used. (3) Results: A 34-year-old female was admitted with suspicion of CDRIE based on an unclear echocardiographic presentation. However, with no signs of infection, that diagnosis was not confirmed, though an endocardial implantable cardioverter-defibrillator (ICD) lead was found folded into the pulmonary trunk. The final treatment included transvenous lead extraction (TLE) and subcutaneous ICD (S-ICD) implantation. (4) Conclusions: With the increasing number of implantations of cardiac electronic devices and their consequences, a high index of suspicion among clinicians is required. The entity of the clinical picture must be thoroughly considered, and various diagnostic tools should be applied. Lead dislocation into the pulmonary trunk is an extremely rare complication. Our findings align with the available literature data, where asymptomatic cases are usually effectively treated with TLE. Modern technologies, such as S-ICD, can effectively prevent lead-related problems and are indicated in young patients necessitating long-term ICD therapy.

COVID-19 associated Brugada pattern electrocardiogram: Systematic review of case reports

Background: Fever is a common clinical manifestation of COVID-19 infection. Fever has also been associated with unmasking Brugada pattern ECG in patients and may result in life-threatening arrhythmia. Little is known regarding COVID-19 associated Brugada pattern ECG. There is paucity of data and guidance in how to manage these patients. Method(s): To identify all published case reports, the latest Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist was followed. A literature search was conducted using PubMed, EMBASE, and Scopus through September 2021. A systematic review was performed to identify the incidence, clinical characteristics, and management outcomes of COVID-19 patients with a Brugada pattern ECG. Result(s): A total of 18 cases were collected. The mean age was 47.1 years and 11.1% were women. No patient had prior confirmed diagnosis of Brugada syndrome. The most common presenting clinical symptoms were fever (83.3%), chest pain (38.8%), shortness of breath (38.8%), and syncope (16.6%). All 18 patients presented with type 1 Brugada pattern ECG. Four patients (22.2%) underwent left heart catheterization, and none demonstrated the presence of obstructive coronary disease. The most common reported therapies included antipyretics (55.5%), hydroxychloroquine (27.7%), and antibiotics (16.6%). One patient (5.5%) died during hospitalization. Three patients (16.6%) who presented with syncope received either an implantable cardioverter defibrillator or wearable cardioverter defibrillator at discharge. At follow up, thirteen patients (72.2%) had resolution of type 1 Brugada pattern ECG. Conclusion(s): COVID-19 associated Brugada pattern ECG is rare. Most patients may see resolution of the ECG pattern once their symptoms have improved. Increased awareness and timely use of antipyretics is warranted in this population.

Mortality And Morbidity Burden Of Covid-19 Infection In Left Ventricular Assist Device Patients

Introduction: COVID-19 pandemic has resulted in more than 6.1 million deaths and more than 480 million infections worldwide (1). Left ventricular assist device patients (LVAD) with their multiple co-morbidities are at high risk for morbidity and mortality from the COVID-19 infection. Few studies and case reports demonstrating the outcomes of COVID-19 infection in LVAD patients have been published, with the most recent study in 2021 (2-4). However, none of these studies spanned the entire stretch of the pandemic. Hypothesis: : COVID-19 infection would result in significant mortality and multi-system complications among patients with an LVAD. Method(s): IRB approval was obtained for our retrospective cohort study. 225 LVAD patients across two large centers in Texas, USA were screened for COVID-19 infection from December 1, 2019 to February 28, 2022. 68 events of COVID-19 infection were identified among 64 patients. One patient was excluded due to false positive test and 3 patients were infected twice and counted as separate events. Outcomes including mortality, respiratory failure, bleeding, and thromboembolic complications were assessed. Result(s): Baseline characteristics and results are summarized in Table 1. 51% of the patients needed hospitalization or emergency department visit for COVID infection. Five patients were intubated (7.4%). 6 patients developed chronic hypoxic respiratory failure requiring outpatient supplemental oxygen. 4 patients suffered from ventricular tachycardias while three other patients had Implantable cardioverter Defibrillator (ICD) shocks during COVID infection. 9 patients had epistaxis or gastrointestinal bleeding within 1 month of testing COVID positive. One HM2 patient had confirmed LVAD outflow cannula thrombus on CT heart and another patient with HeartWare had confirmed inflow cannula thrombus requiring emergent exchange to HM3 due to pump stoppage. Three patients suffered a stroke (5%). No events of pulmonary emboli or DVTs were noted. The mortality rate among this cohort was 14% (9 out of 64 patients). Four patients died during the same hospitalization. 33% had HM2 and 67% had HM3 LVADs, making a mortality rate of 37% (3 out of 8) for HM2 patients and 9% for HM3 (6 out of 55). 88% were males, 56% were African Americans, 67% had NICM, and 78% had at least moderate RV dysfunction at baseline. Conclusion(s): COVID-19 infection resulted in significant mortality and complications including stroke, pump thrombus, arrhythmias, respiratory failure, and bleeding events among LVAD patients.Copyright © 2022

Effects of lockdown on physical activity and arrhythmia burden in patients with an implantable cardioverter-defibrillator: The COVID-19 Lockdown ICD-Carelink (CLIC) study

Background: Both COVID-19 and the measures taken to control the pandemic may significantly affect cardiovascular health. The effects of a lockdown on physical activity and its potential consequences for arrhythmia burden remain largely unknown. Purpose(s): In this study, we investigated the effect of the lockdown during the first COVID-19 wave on patients' physical activity and arrhythmia burden. Method(s): All patients with an ICD connected to a Carelink homemonitoring system from two Dutch hospitals were included. Anonymized data on physical activity, heart rate, and occurrence of ventricular tachycardia/ fibrillation (VT/VF), and atrial fibrillation/tachycardia (AF/AT) were obtained and were compared between March-April 2020 (lockdown) and March-April 2019 (reference) within each patient. The study was approved by the local ethics committee. Result(s): The ICDs of 531 patients registered significantly less activity during de lockdown period compared to the reference period (210+/-104 min vs 182+/-103 min, p<0.0001, Figure 1, panels A and B), while weather conditions improved (1A). Daytime and nighttime heart rates were significantly lower during lockdown compared to the reference period (71.3+/-9 bpm vs 72.6+/-9 bpm, p<0.0001 and 63.4+/-9 vs 63.8+/-9, p=0.02, respectively). AF/AT burden increased (Figure 2A) while number of VT/VF episodes decreased (2B). There was no significant difference in number of NSVT episodes. Conclusion(s): During the lockdown in the first COVID-19 wave, the Carelink system revealed significantly less activity, increase in AF/AT burden and decrease in VT/VF episodes. Further investigation is needed to understand the relationship between physical activity and the occurrence of arrhythmias in ICD patients. (Figure Presented).

Effect of COVID-19 on cardiac electrophysiology practice: a systematic review of literature.

The authors conducted a systematic review on the effect of coronavirus disease 2019 on electrophysiology (EP) practice and procedure volume in various settings. This review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. PubMed/MEDLINE, Scopus, Web of Science, CINAHL, and Embase were examined with combinations of medical subject headings terms for identification of the relevant studies. After excluding duplicates, irrelevant, and ineligible studies, 23 studies were included for full qualitative analysis. The overall study-level volume reduction of EP procedures ranged from 8 to 96.7%. All studies reported an overall reduction in EP physiology procedures being carried out except one in Poland, which reported an overall increase in the total EP procedures carried out in 2020. This study still reported a decrease in EP procedure volume during the first lockdown phase. Procedural volume reduction was seen most commonly for cardiovascular implantable electronic device placement (20/23 studies, 86.9%), electrophysiology studies (11/23 studies, 47.8%), and ablations (9/23, 39.1% studies). The most common reason stated for the observed decline in EP procedures was the cancellation and postponement of nonurgent elective cases in the hospitals (15/23 studies, 65.2%). There has been an overall reduction in EP procedure volume across different centers. The impact of the decline in EP procedures will be seen only after the services resume to prepandemic levels, but an increase in-patient volume and procedure waiting time is expected. This review will provide insights into improving healthcare service delivery in times of unprecedented public health emergencies.

Clinical Outcomes of Patients with COVID-19 Infection and Cardiac Implantable Electronic Devices

BACKGROUND: Existing cardiac disease contributes to poor outcome in patients with coronavirus disease 2019 (COVID-19). Little information exists regarding COVID-19 infection in patients with a cardiac implantable electronic device (CIED). OBJECTIVE(S): To assess the association between CIEDs and severity of COVID-19 infection. METHOD(S): We performed a retrospective analysis including 13,000 patients > 18 years old with COVID-19 infection between January and December 2020. Patients with COVID-19 who had a permanent pacemaker or defibrillator were matched 1:4 based on age and sex followed by univariate and multivariate analyses. Baseline characteristics and clinical outcomes were assessed. RESULT(S): Forty patients with CIED and 160 patients without CIED were included in the current analysis. Mean age was 72.6 +/- 13 years, and approximately 50% were females. Majority of the patients in the study arm had a pacemaker (63%), whereas only 15 patients (37%) had a defibrillator. Patients with COVID-19 and CIED presented more often with atrial fibrillation, coronary artery disease, heart failure, hypertension, diabetes, and chronic kidney disease. They were more likely to be hospitalized in the intensive care unit (ICU) and required more ventilatory support (35% vs. 18.3%). Thirty-day mortality (22.5% vs. 13.8%) and 1-year mortality (25% vs. 15%) were higher among patients with COVID-19 and CIED. CONCLUSION(S): Patients with COVID-19 and CIED had a significantly higher prevalence of co-morbidities that were associated with increased mortality. Although, CIED by itself was not found as an independent risk factor for morbidity and mortality, it may serve as a warning for severe illness with COVID-19.

The evolving landscape of pulmonary arterial hypertension clinical trials

Although it is a rare disease, the number of available therapeutic options for treating pulmonary arterial hypertension has increased since the late 1990s, with multiple drugs developed that are shown to be effective in phase 3 randomised controlled trials. Despite considerable advancements in pulmonary arterial hypertension treatment, prognosis remains poor. Existing therapies target pulmonary endothelial dysfunction with vasodilation and anti-proliferative effects. Novel therapies that target proliferative vascular remodelling and affect important outcomes are urgently needed. There is need for additional innovations in clinical trial design so that all emerging candidate therapies can be rigorously studied. Pulmonary arterial hypertension trial design has shifted from short-term submaximal exercise capacity as a primary endpoint, to larger clinical event-driven trial outcomes. Event-driven pulmonary arterial hypertension trials could face feasibility and efficiency issues in the future because increasing sample sizes and longer follow-up durations are needed, which would be problematic in such a rare disease. Enrichment strategies, innovative and alternative trial designs, and novel trial endpoints are potential solutions that could improve the efficiency of future pulmonary arterial hypertension trials while maintaining robustness and clinically meaningful evidence.Copyright © 2022 Elsevier Ltd

Impact of Nationwide COVID-19 Lockdowns on the Implantation Rate of Cardiac Implantable Electronic Devices.

AIM: The COVID-19 pandemic resulted in a significant decrease in the number of hospital admissions for severe emergent cardiovascular diseases during lockdowns worldwide. This study aimed to determine the impact of both the first and the second Danish nationwide lockdown on the implantation rate of cardiac implantable electronic devices (CIEDs). METHODS: We retrospectively analysed the number of CIED implantations performed in Denmark and stratified them into 3-week intervals. RESULTS: The total number of de novo CIED implantations decreased during the first lockdown by 15.5% and during the second by 5.1%. Comparing each 3-week interval using rate ratios, a significant decrease in the daily rates of the total number of de novo and replacement CIEDs (0.82, 95% CI [0.70, 0.96]), de novo CIEDs only (0.82, 95% CI [0.69, 0.98]), and non-acute pacemaker implantations (0.80, 95% CI [0.63, 0.99]) was observed during the first interval of the first lockdown. During the second lockdown (third interval), a significant decrease was seen in the daily rates of de novo CIEDs (0.73, 95% CI [0.55, 0.97]), and of pacemakers in total during both the second (0.78, 95% CI [0.62, 0.97]) and the third (0.60, 95% CI [0.42, 0.85]) intervals. Additionally, the daily rates of acute pacemaker implantation decreased during the second interval (0.47, 95% CI [0.27, 0.79]) and of non-acute implantation during the third interval (0.57, 95% CI [0.38, 0.84]). A significant increase was observed in the number of replacement procedures during the first interval of the second lockdown (1.70, 95% CI [1.04, 2.85]). CONCLUSIONS: Our study found only modest changes in CIED implantations in Denmark during two national lockdowns.

Safety of same-day discharge versus overnight stay strategy following cardiac device implantations: a high-volume single-centre experience.

BACKGROUND: The strategy of cardiac implantable electronic device (CIED) implantations performed as day-case admissions has gained a wider acceptance overtime; however, data on safety are still limited. This study aims to investigate the safety of a same-day discharge protocol introduced in our hospital for the postprocedural management of patients undergoing CIED implantation. METHODS: Α prospective, non-interventional, non-randomised study performed in a single high-volume implanting centre for a 16-month period (March 2020 to June 2021). At total of 821 of 965 (85.1%) patients scheduled for elective CIED implantation were considered to be eligible for inclusion in the Short-stay Device Management Protocol. These patients were compared with a historical group of 932 patients, meeting the same inclusion criteria. RESULTS: Procedure was successful in 812 patients (98.9%), committed to same-day discharge versus 921 of 932 patients (98.8%) admitted for overnight stay (p = 0.87). Overall, 90-day complication rate was comparable in both groups (4.14% vs 4.07%, p = 0.95), as was major (1.46% vs. 1.82%, p = 0.55) and minor (2.67% vs. 2.25%, p = 0.64) complication rates. The composite early post-procedural complication rates and late post-procedural complication rates were comparable among groups (0.97 vs 1.18%, p = 0.70 and 0.73% vs 0.64%, p = 0.83, respectively). Six hundred sixty-seven patients (84%) preferred the same-day discharge strategy. Finally, a reduction of 792 bed-days was recorded, resulting in possible financial Health System benefits. CONCLUSIONS: Same-day discharge is feasible and safe in the majority of patients referred for CIED implantation. Additionally, same-day discharge is preferred by patients and may reduce procedure-related costs due to significant bed-day reductions.

Remote monitoring of pacemakers and defibrillators: Effective and safe in Brazil?

Background: The remote monitoring (RM) of cardiac implantable electronic devices (CIEDs) has become a common method of in-home monitoring and follow-up in high-income countries given its effectiveness, safety, convenience, and the possibility of early intervention. However, in Brazil, RM is still underutilized. Objectives: This observational study aims to demonstrate our experience of using RM in Brazil and the predictive factors of RM of CIED follow-up in Brazil. Methods: This was a prospective cohort study of patients with a CIED. Event rates are reported and clinical responses to those findings and outcomes based on the detection of RM. A logistic regression model was performed to identify predictors of more events, with P < .05 for statistical significance. Results: This study evaluated consecutive 119 patients: 30.2% with pacemakers, 42.8% with implantable cardioverter-defibrillator, 22.7% with cardiac resynchronization therapy (CRT) with defibrillator, and 3.3% with CRT with pacemaker. Events were detected in 63.9% of the cases in 29.5 ± 23 months of follow-up. The outcomes found were that 44.5% needed elective evaluation in medical treatment and 23.5% needed immediate evaluation in therapy. Logistic regression analysis showed that the groups with CRT or CRT with defibrillator (75.0%), reduced ejection fraction (76.5%), and New York Heart Association functional class ≥II (75.0%) had the highest RM event rates. Conclusions: RM proved to be effective and safe in the follow-up of patients with CIEDs in Brazil, allowing early interventions and facilitating therapeutic management.

Development of a patient and clinician co-led education program to promote living well with an implantable cardioverter defibrillator: Insights from a pilot project.

Objective: To evaluate a pilot education program designed to improve patients' experience of living well with an implantable cardioverter-defibrillator (ICD). Methods: Patient Partners with previously implanted ICD and clinicians collaboratively performed monthly education sessions for potential and recent ICD recipients. Curriculum development was informed by current evidence of ICD patients' unique educational needs; delivery format transitioned to a virtual platform following the onset of COVID-19. Participants' experience was evaluated using a tailored questionnaire to explore preliminary insights. Results: 126 participants (median age: 62 years; women: 30%) attended 24 sessions. In-person participants (n = 62, 49.2%) reported sessions as helpful (n = 56, 94%) with regards to format and Patient Partner interactions. Virtual participants 64 (50.8%) completed an electronic survey (n = 27, 45%); reporting sufficient information for most topics with the exception of potential psychological effects of ICD implantation. Patient Partners as collaborative session leaders was perceived to be very helpful (n = 22, 82%) or somewhat helpful (n = 5, 18%). Conclusion: This novel educational partnership met the learning needs of patients at the vulnerable time of new cardiac device implantation of both in-person and virtual formats. Innovation: The inclusion of Patient Partners in co-led cardiac education informs novel approach to care that may improve patients' experiences of living well with complex technology.

Ventricular arrhythmia burden in patients with implantable cardioverter defibrillator and remote patient monitoring during different time intervals of the COVID-19 pandemic.

PURPOSE: The current study investigated whether the changes in patient care in times of the COVID-19 pandemic, especially the reduction of in-person visits, would result in a deterioration of the arrhythmic and clinical condition of patients with an implantable cardioverter defibrillator (ICD) and remote patient monitoring. METHODS: Data were obtained from a local ICD registry. 140 patients who received ICD implantation at our department and had remote patient monitoring were included. The number of patients with ventricular arrhythmias, appropriate ICD therapy, the number of visits to our outpatient clinic and hospitalization due to acute coronary syndrome, stroke or heart failure were compared during three time intervals of the COVID-19 pandemic (first (LD1) and second (LD2) national lockdown in Germany and the time after the first lockdown (postLD1)) and a time interval 1 year before the pandemic began (preCOV). Each time interval was 49 days long. RESULTS: Patients had significantly fewer visits to our outpatient clinic during LD1 (n = 13), postLD1 (n = 22) and LD2 (n = 23) compared to the time interval before the pandemic (n = 43, each p ≤ 0.05). The number of patients with sustained ventricular arrhythmias, appropriate ICD therapy and clinical events showed no significant difference during the time intervals of the COVID-19 pandemic and the time interval 1 year prior. CONCLUSIONS: The lockdown measures necessary to reduce the risk of infection during the COVID-19 pandemic, led to a reduction of in-person patient visits, but did not result in a deterioration of the arrhythmic and clinical condition of ICD patients with remote patient monitoring.

Utilisation of home monitoring in children with cardiac devices in the COVID-19 ERA

Background and Aim: The covid-19 pandemic created huge chal-lenges for healthcare worldwide, in the UK reiterating the popu-lations need for an efficient national health system. With a device implantation rate of 1700/million population (UK), regular mon-itoring is a significant undertaking. We focus on a single tertiary cardiac centre for children, serving an 8 million population, and their ability to deliver such a service at one of the most challenging times in the history of the NHS. Method(s): We retrospectively gathered data for the period April 2020-April 2021 for all children under our service with;perma-nent pacing system (PPM), implantable defibrillator (ICD), loop recorder (LINQ). Data was gathered from our secure cardiac data-base including demographics, device particulars, settings and lon-gevity. First hand accounts from families were gathered by one clinician using a standardized structured set of questions. The responses were tabulated to reveal common themes and key words. Result(s): Pre-pandemic 122 patients had devices, with 42 (34%) having monitoring capability at home. As of April 2021 136 patients had devices in situ, with 87 (64%) on home monitoring. See table for data. Conclusion(s): Early in the pandemic parents of children with cardiac conditions were justifiably anxious, unclear as to whether their child was at an increased risk given their chronic condition. More complex children were asked to shield initially which meant their medical appointments became impractical. At a time when new ways of working became a necessity as opposed to a novelty our centre utilised the technology available to keep children and their families out of hospital. Home monitoring providing autonomy for our patients and families, and a link to those caring for them at a time of ongoing uncertainty. However there were limitations, 6% of our PPM systems don't have a compatible home monitoring system and those with poor network signal in the home aren't eligible. Furthermore demand for home monitoring has rocketed, which device companies have struggled to meet meaning we must prioritise our most complex and high risk patients.

Spanish implantable cardioverter-defibrillator registry. 18th official report of the Heart Rhythm Association of the Spanish Society of Cardiology (2021).

INTRODUCTION AND OBJECTIVES: This article presents the data corresponding to implantable cardioverter-defibrillator (ICD) implantations in Spain in 2021. METHODS: The data were drawn from implanting centers, which voluntarily completed a data collection sheet during the procedure. RESULTS: In 2021, 7496 implant data sheets were received, compared with 7743 reported by Eucomed (European Confederation of Medical Suppliers Associations), indicating that data were collected from 96.8% of the devices implanted in Spain. Data completion ranged from 99.9% for "name of implanting hospital" to 8.9% for "implanting hospital". In 2021, 199 hospitals participated in the registry, exceeding the figures of previous years, with around 170 participating hospitals. The total rate of registered implants was 158/million inhabitants (163 according to Eucomed), making 2021 the year with the highest activity. However, the registry continues to show significant differences among the various autonomous communities and the lowest implantation rate of all the European countries participating in Eucomed. CONCLUSIONS: The Spanish implantable cardioverter-defibrillator registry for 2021 recorded an increase in the number of ICD implantations, reflecting the recovery of hospital activity after the initial impact of the COVID-19 pandemic in 2020. Although the total number of implants has increased in Spain, figures are still much lower than the European Union average, with differences persisting among Spanish autonomous communities.

DILATED CARDIOMYOPATHY WITH CARDIOGENIC SHOCK SECONDARY TO COVID-19 INFECTION

SESSION TITLE: Extraordinary Cardiovascular Reports SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 01:35 pm - 02:35 pm INTRODUCTION: Acute myocarditis from COVID-19 has been well documented, but there are few cases of COVID -19 patients developing dilated cardiomyopathy (3). We present a case of COVID-19 induced dilated cardiomyopathy leading to cardiogenic shock. CASE PRESENTATION: A 49-year-old African-American female presented to the emergency department (ED) with shortness of breath. She was diagnosed with COVID-19 infection four weeks prior to presentation, and since that time she experienced continuously worsening dyspnea, congestion, and weakness. In the ED, the patient was found to have pulmonary edema and bilateral pleural effusions on chest x-ray, as well as acute kidney injury with a creatinine level of 2.85 mg/dL. An echocardiogram revealed a new diagnosis of dilated cardiomyopathy with reduced ejection fraction of 10-15% with a large left ventricular thrombus. Heparin infusion was initiated and intravenous furosemide was administered for diuresis. Her renal function continued to worsen, which was attributed to cardiorenal syndrome. She became hypotensive and was found to be in cardiogenic shock, which required intensive care unit admission with the initiation of continuous renal replacement therapy (CRRT). The patient improved with CRRT, however her renal function did not recover and she continued to require hemodialysis. She was able to be transferred out of the intensive care unit, and the heparin was bridged to warfarin. Goal-directed medical therapy was initiated for her heart failure. She was eventually discharged home with an external cardioverter-defibrillator vest. A follow-up echocardiogram three months later revealed the left ventricular thrombus had resolved, however, her ejection fraction had improved to only 15-20% despite medication compliance. An implantable cardioverter-defibrillator (ICD) was placed and the patient continues to be followed closely by cardiology. DISCUSSION: Viral infection is a well-documented cause of myocarditis with some patients developing dilated cardiomyopathy (1). Inflammatory dilated cardiomyopathy occurs most commonly in patients infected with Coxsackie B virus, Human Parvovirus B19, Adenovirus, Human Immunodeficiency Virus, Hepatitis C Virus, Cytomegalovirus, Epstein-Barr Virus, and Human Herpes Virus 6 (1). The proposed mechanism of inflammatory cardiomyopathy includes infection of the myocytes, incomplete viral elimination, and persistent retained viral components in the myocytes(2). This may cause direct viral injury or chronic myocardial inflammation leading to remodeling (2). It is documented in the literature that COVID-19 can lead to myocarditis and various types of acute cardiomyopathy (3). However, there have been only a few reported cases of COVID - 19 induced dilated cardiomyopathy (3). CONCLUSIONS: While rarely reported thus far, it should be established that COVID-19 alone can cause dilated cardiomyopathy and lead to heart failure (3). Reference #1: Schultheiss H-P, Baumeier C, Pietsch H, Bock C-T, Poller W, Escher F. Cardiovascular consequences of viral infections: from COVID to other viral diseases. Cardiovascular Research. Published online October 5, 2021. doi:10.1093/cvr/cvab315 Reference #2: Kühl U, Pauschinger M, Seeberg B, et al. Viral Persistence in the Myocardium Is Associated With Progressive Cardiac Dysfunction. Circulation. 2005;(13):1965-1970. doi:10.1161/circulationaha.105.548156 Reference #3: Komiyama M, Hasegawa K, Matsumori A. Dilated Cardiomyopathy Risk in Patients with Coronavirus Disease 2019: How to Identify and Characterise it Early? European Cardiology Review. Published online May 27, 2020. doi:10.15420/ecr.2020.17 DISCLOSURES: No relevant relationships by Amanda Cecchini No relevant relationships by Austin Richardson No relevant relationships by Krupa Solanki

COVID-19 Infection Concomitant With Infective Tricuspid Valve Endocarditis

The COVID-19 pandemic, together with its complications and management, has been a significant issue worldwide since March 2020. Concomitant infections in vulnerable patients with preexisting cardiovascular diseases are not uncommon. Sharing information about the diagnostic management and treatment of these comorbidities has a prominent role in unveiling some of this pandemic's challenges. We herein describe a young adult with a history of implantable cardioverter-defibrillator implantation diagnosed with COVID-19 infection and infective endocarditis. (Iranian Heart Journal 2022;23(4): 102-108). Copyright © 2022, Iranian Heart Association. All rights reserved.

Spanish implantable cardioverter-defibrillator registry. 18th official report of the Heart Rhythm Association of the Spanish Society of Cardiology (2021)

Introduction and objectives: This article presents the data corresponding to implantable cardioverter-defibrillator (ICD) implantations in Spain in 2021. Methods: The data were drawn from implanting centers, which voluntarily completed a data collection sheet during the procedure. Results: In 2021, 7496 implant data sheets were received, compared with 7743 reported by Eucomed (European Confederation of Medical Suppliers Associations), indicating that data were collected from 96.8% of the devices implanted in Spain. Data completion ranged from 99.9% for “name of implanting hospital” to 8.9% for “implanting hospital”. In 2021, 199 hospitals participated in the registry, exceeding the figures of previous years, with around 170 participating hospitals. The total rate of registered implants was 158/million inhabitants (163 according to Eucomed), making 2021 the year with the highest activity. However, the registry continues to show significant differences among the various autonomous communities and the lowest implantation rate of all the European countries participating in Eucomed. Conclusions: The Spanish implantable cardioverter-defibrillator registry for 2021 recorded an increase in the number of ICD implantations, reflecting the recovery of hospital activity after the initial impact of the COVID-19 pandemic in 2020. Although the total number of implants has increased in Spain, figures are still much lower than the European Union average, with differences persisting among Spanish autonomous communities.