Providência, Rui

Abstract Arrhythmogenic cardiomyopathy (ACM) is addressed in international guidelines and expert consensus statements. To assist clinicians in their routine practice and support decision-making, we performed a systematic review of the most relevant guidelines and recommendations for ACM diagnosis and management. Our search, covering MEDLINE, EMBASE, and resources from scientific societies over the last 10 years, identified two guidelines and three consensus statements that met rigorous inclusion criteria for detailed analysis. In the examined documents, key areas of agreement included the critical role of cardiac imaging for initial diagnosis and ongoing monitoring, genetic testing in index patients, ventricular arrhythmia management, catheter ablation indications, heart failure treatment strategies, and exercise recommendations. However, significant differences were found in definitions and diagnostic criteria for ACM, interpretation of phenocopies, management of family members, and criteria for implantable cardioverter defibrillator implantation. Additional discrepancies emerged regarding the role of multidisciplinary teams, non-cardiac surgical considerations, atrial fibrillation management, and reproductive issues. Crucially, there remain considerable gaps in evidence, especially in areas such as the management and follow-up of patients with potential or borderline ACM diagnoses, as well as the care of their relatives. The clinical implications of genetic findings, along with the clinical management of left-dominant, biventricular phenotypes, and hot phases of disease, are also insufficiently addressed. Furthermore, a critical shortfall is the lack of externally validated risk assessment tools to guide clinical decision-making. Bridging these gaps could help guiding future research and guideline development towards improving patient outcomes.

Heart failure with reduced ejection fraction is a major cause of morbidity and mortality. Cardiac resynchronization therapy (CRT) improves outcomes but faces challenges such as unsuccessful lead placement and suboptimal responses. The Wireless Stimulation Endocardially for CRT (WiSE-CRT) system, a novel leadless left ventricular (LV) pacing technology, uses ultrasonic energy to deliver electrical stimulation directly to the LV endocardium, potentially improving the response rates. A comprehensive literature search was performed across multiple databases from inception until August 2024. The outcomes of interest included the New York Heart Association (NYHA) functional score, LV ejection fraction (LVEF), LV end-diastolic volume (LVEDV), LV end-systolic volume (LVESV), and QRS duration. Statistical analysis involved pooling the mean differences (MDs) and their corresponding 95% confidence intervals (CIs) from each study using the inverse-variance random-effects model in RevMan version 5.4.1 software. Seven studies, including 468 patients with heart failure treated with the WiSE-CRT system, were included. The pooled analysis showed significant improvements in NYHA functional score (MD: −0.44; 95% CI: −0.67 to −0.21; P = 0.0002; I 2 = 77%), LVEF (MD: 5.71; 95% CI: 4.54 to 6.87; P < 0.001), LVEDV (MD: −24.49; 95% CI: −33.24 to −15.73; P < 0.001), and LVESV (MD: −24.47; 95% CI: −32.80 to −16.14; P < 0.001). The QRS duration also decreased significantly (MD: −38.62; 95% CI: −46.76 to −30.49; P < 0.001; I 2 = 60%). The WiSE-CRT system significantly improved NYHA functional score, LVEF, QRS duration, LVEDV, and LVESV in patients with heart failure. This leadless pacing technology offers a promising alternative for ineligible candidates or for those who do not respond to conventional CRT.

Abstract Aims Causes of death remain largely unexplored in the atrial fibrillation (AF) population. We aimed to (i) thoroughly assess causes of death in patients with AF, especially those associated with sudden cardiac death (SCD) and (ii) evaluate the potential association between AF and SCD. Methods and results Linked primary and secondary care United Kingdom Clinical Practice Research Datalink dataset comprising 6 529 382 individuals aged ≥18. We identified 214 222 patients with newly diagnosed AF, and an equivalent number of non-AF patients matched for age, sex and primary care practice. The underlying primary cause of death for each patient was assessed in the form of International Classification of Diseases Tenth Revision (ICD-10) codes and also as part of broader disease categories (i.e. ICD-10 chapters). Findings Over a median follow-up of 2.7 (interquartile range: 0.7–6.0) years, 124 781 (58.25%) patients with AF died. Sudden cardiac death occurred in 13 923 patients with AF [6.50% patients with AF vs. 2.01% non-AF patients; odds ratio (OR) = 3.38, 95% confidence interval (CI): 3.27–3.50, P &lt; 0.0001], contributing to 11.05% of all AF mortality. Diseases of the circulatory system, neoplasms and respiratory diseases explained 45% of AF mortality. Sudden cardiac death occurred more frequently in males (OR = 1.87, 95% CI: 1.80–1.93, P &lt; 0.0001), and females with AF died more often of diseases of the circulatory, respiratory, digestive, and genitourinary system and less often of neoplastic disorders. Interpretation Conditions of the circulatory system are the main driver of mortality in the AF population. Females with AF experience higher cardiovascular and respiratory mortality but die less frequently of neoplasms. The risk of SCD is higher in the AF population, occurring more frequently in males.

Abstract Aims The viability of cardiac resynchronization therapy (CRT) in inotrope-dependent heart failure (HF) has been a matter of debate. Methods and results We searched Medline, EMBASE, Scopus, and the Cochrane Library until 31 December 2022. Studies were included if (i) HF patients required inotropic support at CRT implantation; (ii) patients were ≥18 years old; and (iii) they provided a clear definition of ‘inotrope dependence’ or ‘inability to wean’. A meta-analysis was performed in R (Version 3.5.1). Nineteen studies comprising 386 inotrope-dependent HF patients who received CRT (mean age 64.4 years, 76.9% male) were included. A large majority survived until discharge at 91.1% [95% confidence interval (CI): 81.2% to 97.6%], 89.3% were weaned off inotropes (95% CI: 77.6% to 97.0%), and mean discharge time post-CRT was 7.8 days (95% CI: 3.9 to 11.7). After 1 year of follow-up, 69.7% survived (95% CI: 58.4% to 79.8%). During follow-up, the mean number of HF hospitalizations was reduced by 1.87 (95% CI: 1.04 to 2.70, P &lt; 0.00001). Post-CRT mean QRS duration was reduced by 29.0 ms (95% CI: −41.3 to 16.7, P &lt; 0.00001), and mean left ventricular ejection fraction increased by 4.8% (95% CI: 3.1% to 6.6%, P &lt; 0.00001). The mean New York Heart Association (NYHA) class post-CRT was 2.7 (95% CI: 2.5 to 3.0), with a pronounced reduction of individuals in NYHA IV (risk ratio = 0.27, 95% CI: 0.18 to 0.41, P &lt; 0.00001). On univariate analysis, there was a higher prevalence of males (85.7% vs. 40%), a history of left bundle branch block (71.4% vs. 30%), and more pronounced left ventricular end-diastolic dilation (274.3 ± 7.2 vs. 225.9 ± 6.1 mL). Conclusions CRT appears to be a viable option for inotrope-dependent HF, with some of these patients seeming more likely to respond.

Introduction: The impact of COVID-19 was felt across cardiology as resources were redirected to critical care, delaying elective cardiology procedures. Hypothesis: To evaluate differences in outcomes of cardiac implantable electronic device (CIED) procedures in 2020 (height of COVID) vs. 2016-2019 (pre-COVID). Methods: Adults >= 18 years undergoing CIED procedures were identified from National Inpatient Sample Databases (NIS) 2016-2020. Complications and mortality were assessed based on the type of device: Permanent Pacemaker (PPM); Implantable Cardioverter Defibrillator (ICD); Cardiac Resynchronization Therapy with Pacemaker (CRT-P) or defibrillator (CRT-D): Leadless PPM; Generator explant (GE), and Lead extraction (LE). Results: A 13.8% drop in CIED admissions was recorded between 2019 and 2020 (p<0.01). Fewer procedures involving PPM, ICD, CRT-P, CRT-D, LE, and GE were observed between 2019 and 2020. Leadless PPM utilization rose consistently between 2016-2020 (47.6% increase during 2020 vs 2019)(Figure). In 2020, a higher proportion of patients were classified with a Charlson Comorbidity Index score of ≥3 (severe) (47.4% in 2020 vs. 42.9% in 2016-2019, p<0.01). The table shows significantly higher complications during CIED implants with no changes in mortality related to procedures. Conclusions: Patients admitted for CIED implants in 2020 had more severe comorbidities and expressed considerable differences in complications than pre-COVID era (2016-2019), but mortality remained unchanged. Leadless PPM use increased during pandemic, as against a decrease in the use of other CIEDs.

Introduction: While Coronavirus disease 2019 (COVID-19) primarily affects the respiratory system but can impact the cardiovascular system can triggering various complications. Hypothesis: To investigate the trend in age-adjusted mortality rates (AAMRs) in patients with atrial fibrillation(AF) among COVID-19-positive patients (CPP) by race and sex. Methods: Trends in AAMRs (per 100,000) from all-cause in patients with AF between April to December 2016-2020 were evaluated via the National Inpatient Sample and the US Census Bureau. The AAMRs among CPP with AF in 2020 was also evaluated between racial and sex cohorts. Results: A gradual rise in AAMR(all causes) in patients with AF was observed between 2016-2019, with a 49.4% overall rise between 2019(271 per 100,000) and 2020(405 per 100,000) ( Table 1a and Figure ). Around 250475 COVID-19-positive patients had AF, and 24.2% of cases did not survive their hospitalization (vs. 10.9% in those without AF, p<0.01, aOR 1.457, 95% CI 1.438-1.477). Among CPP males and females with AF that died, White females and males were the oldest, while Black males and females reported the highest mean Charlson Comorbidity Index (CCI) score ( Table 1b ).Furthermore, among the CPP with AF in 2020, Hispanics reported a higher AAMR (119.6 per 100,000) than Whites(111.1 per 100,000) and Blacks(101.9 per 100,000). AAMRs in males were higher in Hispanic males (144.8 per 100,000), followed by White males (129.7 per 100,000), and Black males (110 per 100,000). However, the AAMRs in females were highest in Black females (91.3 per 100,000), followed by White females (88.3 per 100,000) and Hispanic females (87.0 per 100,000). ( Table 1c ) Conclusions: AAMRs in patients with AF are on the rise and a sharp growth was seen during the first year of the pandemic. AAMRs among CPP patients with AF were highest among Black females and Hispanic males, while White males and females were oldest and Black males and females reported the highest CCI scores.

Abstract Technological advances have made diagnosis of heart rhythm disturbances much easier, with a wide variety of options, including single-lead portable devices, smartphones/watches to sophisticated implantable cardiac monitors, allowing accurate data to be collected over different time periods depending on symptoms frequency. This review provides an overview of the novel and existing heart rhythm testing options, including a description of the supporting evidence for their use. A description of each of the tests is provided, along with discussion of their advantages and limitations. This is intended to help clinicians towards choosing the most appropriate test, thus improving diagnostic yield management of patients with suspected arrhythmias.

Background Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder affecting multiple systems, particularly the cardiovascular system. The leading causes of death in MFS are aortopathies and valvular disease. We wanted to identify the frequency of arrhythmia and postural orthostatic tachycardia syndrome, length of hospital stay, health care–associated costs (HAC), and in‐hospital mortality in patients with MFS. Methods and Results The National Inpatient Sample database from 2005 to 2014 was queried using International Classification of Diseases, Ninth Revision, Clinical Modification ( ICD‐9‐CM ) codes for MFS and arrhythmias. Patients were classified into subgroups: supraventricular tachycardia, ventricular tachycardia (VT), atrial fibrillation, atrial flutter, and without any type of arrhythmia. Data about length of stay, HAC, and in‐hospital mortality were also abstracted from National Inpatient Sample database. Adjusted HAC was calculated as multiplying HAC and cost‐to‐charge ratio; 12 079 MFS hospitalizations were identified; 1893 patients (15.7%) had an arrhythmia; and 4.9% of the patients had postural orthostatic tachycardia syndrome. Median values of length of stay and adjusted HAC in VT group were the highest among the groups (VT: 6 days, $18 975.8; supraventricular tachycardia: 4 days, $11 906.6; atrial flutter: 4 days, $11 274.5; atrial fibrillation: 5 days, $10431.4; without any type of arrhythmia: 4 days, $8336.6; both P =0.0001). VT group had highest in‐patient mortality (VT: 5.3%, atrial fibrillation: 4.1%, without any type of arrhythmia: 2.1%, atrial flutter: 1.7%, supraventricular tachycardia: 0%; P <0.0001) even after adjustment for potential confounders (without any type of arrhythmia versus VT; odds ratio [95% CI]: 3.18 [1.62–6.24], P =0.001). Conclusions Arrhythmias and postural orthostatic tachycardia syndrome in MFS were high and associated with increased length of stay, HAC, and in‐hospital mortality especially in patients with VT.

Abstract Recent technological advances have facilitated and diversified the options available for the diagnosis of cardiac arrhythmias. Ranging from simple resting or exercise electrocardiograms to more sophisticated and expensive smartphones and implantable cardiac monitors. These tests and devices may be used for varying periods of time depending on symptom frequency. The choice of the most appropriate heart rhythm test should be guided by clinical evaluation and optimized following accurate characterization of underlying symptoms, ‘red flags’, risk factors, and consideration of cost-effectiveness of the different tests. This review provides evidence-based guidance for assessing suspected arrhythmia in patients who present with symptoms or in the context of screening, such as atrial fibrillation or advanced conduction disturbances following transcatheter aortic valve implantation in high-risk groups. This is intended to help clinicians choose the most appropriate diagnostic tool to facilitate the management of patients with suspected arrhythmias.

Abstract Aims To develop quality indicators (QIs) that may be used to evaluate the quality of care and outcomes for adults with atrial fibrillation (AF). Methods and results We followed the ESC methodology for QI development. This methodology involved (i) the identification of the domains of AF care for the diagnosis and management of AF (by a group of experts including members of the ESC Clinical Practice Guidelines Task Force for AF); (ii) the construction of candidate QIs (including a systematic review of the literature); and (iii) the selection of the final set of QIs (using a modified Delphi method). Six domains of care for the diagnosis and management of AF were identified: (i) Patient assessment (baseline and follow-up), (ii) Anticoagulation therapy, (iii) Rate control strategy, (iv) Rhythm control strategy, (v) Risk factor management, and (vi) Outcomes measures, including patient-reported outcome measures (PROMs). In total, 17 main and 17 secondary QIs, which covered all six domains of care for the diagnosis and management of AF, were selected. The outcome domain included measures on the consequences and treatment of AF, as well as PROMs. Conclusion This document defines six domains of AF care (patient assessment, anticoagulation, rate control, rhythm control, risk factor management, and outcomes), and provides 17 main and 17 secondary QIs for the diagnosis and management of AF. It is anticipated that implementation of these QIs will improve the quality of AF care.