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Arrhythmogenic cardiomyopathy (ACM) epitomises a genetic anomaly hallmarked by a relentless fibro-fatty transmogrification of cardiac myocytes. Initially typified as a right ventricular-centric disease, contemporary observations elucidate a frequent occurrence of biventricular and left-dominant presentations. The diagnostic labyrinth of ACM emerges from its clinical and imaging properties, often indistinguishable from other cardiomyopathies. Precision in diagnosis, however, is paramount and unlocks the potential for early therapeutic interventions and vital cascade screening for at-risk individuals. Adherence to the criteria established by the 2010 task force remains the cornerstone of ACM diagnosis, demanding a multifaceted assessment incorporating electrophysiological, imaging, genetic, and histological data. Reflecting the evolution of our understanding, these criteria have undergone several revisions to encapsulate the expanding spectrum of ACM phenotypes. This review seeks to crystallise the genetic foundation of ACM, delineate its clinical and radiographic manifestations, and offer an analytical perspective on the current diagnostic criteria. By synthesising these elements, we aim to furnish practitioners with a strategic, evidence-based algorithm to accurately diagnose ACM, thereby optimising patient management and mitigating the intricate challenges of this multifaceted disorder.
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AIMS: The optimum timing of cardiac resynchronization therapy (CRT) implantation is unknown. We explored long-term outcomes after CRT in relation to the time interval from a first heart failure hospitalization (HFH) to device implantation. METHODS AND RESULTS: A database covering the population of England (56.3 million in 2019) was used to quantify clinical outcomes after CRT implantation in relation to first HFHs. From 2010 to 2019, 64 968 patients [age: 71.4 ± 11.7 years; 48 606 (74.8%) male] underwent CRT implantation, 57% in the absence of a previous HFH, 12.9% during the first HFH, and 30.1% after ≥1 HFH. Over 4.54 (2.80-6.71) years [median (interquartile range); 272 989 person-years], the time in years from the first HFH to CRT implantation was associated with a higher risk of total mortality [hazard ratio (HR); 95% confidence intervals (95% CI)] (1.15; 95% CI 1.14-1.16, HFH (HR: 1.26; 95% CI 1.24-1.28), and the combined endpoint of total mortality or HFH (HR: 1.19; 95% CI 1.27-1.20) than CRT in patients with no previous HFHs, after co-variate adjustment. Total mortality (HR: 1.67), HFH (HR: 2.63), and total mortality or HFH (HR: 1.92) (all P < 0.001) were highest in patients undergoing CRT ≥2 years after the first HFH. CONCLUSION: In this study of a healthcare system covering an entire nation, delays from a first HFH to CRT implantation were associated with progressively worse long-term clinical outcomes. The best clinical outcomes were observed in patients with no previous HFH and in those undergoing CRT implantation during the first HFH. CONDENSED ABSTRACT: The optimum timing of CRT implantation is unknown. In this study of 64 968 consecutive patients, delays from a first heart failure hospitalization (HFH) to CRT implantation were associated with progressively worse long-term clinical outcomes. Each year from a first HFH to CRT implantation was associated with a 21% higher risk of total mortality and a 34% higher risk of HFH. The best outcomes after CRT were observed in patients with no previous HFHs and in those undergoing implantation during their first HFH.
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Terapia de Ressincronização Cardíaca , Insuficiência Cardíaca , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Feminino , Terapia de Ressincronização Cardíaca/efeitos adversos , Terapia de Ressincronização Cardíaca/métodos , Resultado do Tratamento , Dispositivos de Terapia de Ressincronização Cardíaca , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/terapia , InglaterraRESUMO
AIMS: The past decade has seen an increased delivery of cardiac resynchronization therapy (CRT) for patients with heart failure (HF). We explored whether clinical outcomes after CRT have changed from the perspective of an entire public healthcare system. METHODS AND RESULTS: A national database covering the population of England (56.3 million in 2019) was used to explore clinical outcomes after CRT from 2010 to 2019. A total of 64 698 consecutive patients (age 71.4 ± 11.7 years; 74.8% male) underwent CRT-defibrillation [n = 32 313 (49.7%)] or CRT-pacing [n = 32 655 (50.3%)] implantation. From 2010-2011 to 2018-2019, there was a 76% increase in CRT implantations. During the same period, the proportion of patients with hypertension (59.6-73.4%), diabetes (26.5-30.8%), and chronic kidney disease (8.62-22.5%) increased, as did the Charlson comorbidity index (CCI ≥ 3 from 20.0% to 25.1%) (all P < 0.001). Total mortality decreased at 30 days (1.43-1.09%) and 1 year (9.51-8.13%) after implantation (both P < 0.001). At 2 years, total mortality [hazard ratio (HR): 0.72; 95% confidence interval (CI) 0.69-0.76] and total mortality or HF hospitalization (HR: 0.59; 95% CI 0.57-0.62) decreased from 2010-2011 to 2018-2019, after correction for age, race, sex, device type (CRT-defibrillation or pacing), comorbidities (hypertension, diabetes, chronic kidney disease, and myocardial infarction), or the CCI (HR: 0.81; 95% CI 0.77-0.85). CONCLUSIONS: From the perspective of an entire public health system, survival has improved and HF hospitalizations have decreased after CRT implantation over the past decade. This prognostic improvement has occurred despite an increasing comorbidity burden.
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Terapia de Ressincronização Cardíaca , Desfibriladores Implantáveis , Diabetes Mellitus , Insuficiência Cardíaca , Hipertensão , Insuficiência Renal Crônica , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Feminino , Terapia de Ressincronização Cardíaca/efeitos adversos , Resultado do Tratamento , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/terapia , Insuficiência Cardíaca/etiologia , Prognóstico , Hipertensão/diagnóstico , Hipertensão/epidemiologia , Hipertensão/terapia , Insuficiência Renal Crônica/terapiaRESUMO
There has been a progressive evolution in the management of patients with chronic heart failure and reduced ejection fraction (HFrEF), including cardiac resynchronisation therapy (CRT) in those that fulfil pre-defined criteria. However, there exists a significant proportion with refractory symptoms in whom CRT devices are not clinically indicated or ineffective. Cardiac contractility modulation (CCM) is a novel therapy that incorporates administration of non-excitatory electrical impulses to the interventricular septum during the absolute refractory period. Implantation is analogous to a traditional transvenous pacemaker system, but with the use of two right ventricular leads. Mechanistic studies have shown augmentation of left ventricular contractility and beneficial global effects on reverse remodeling, primarily through alterations in calcium handling. This appears to occur without increasing myocardial oxygen consumption. Data from clinical trials have shown translational improvements in functional capacity and quality of life, though long-term outcome data are lacking. This review explores the rationale, evidence base and limitations of this nascent technology.
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Insuficiência Cardíaca , Disfunção Ventricular Esquerda , Insuficiência Cardíaca/terapia , Humanos , Contração Miocárdica , Qualidade de Vida , Volume Sistólico , Resultado do TratamentoRESUMO
BACKGROUND: Observational studies investigating risk factors in coronavirus disease 2019 (COVID-19) have not considered the confounding effects of advanced care planning, such that a valid picture of risk for elderly, frail and multi-morbid patients is unknown. We aimed to report ceiling of care and cardiopulmonary resuscitation (CPR) decisions and their association with demographic and clinical characteristics as well as outcomes during the COVID-19 pandemic. METHODS: Retrospective, observational study conducted between 5th March and 7th May 2020 of all hospitalised patients with COVID-19. Ceiling of care and CPR decisions were documented using the Recommended Summary Plan for Emergency Care and Treatment (ReSPECT) process. Unadjusted and multivariable regression analyses were used to determine factors associated with ceiling of care decisions and death during hospitalisation. RESULTS: A total of 485 patients were included, of whom 409 (84·3%) had a documented ceiling of care; level one for 208 (50·9%), level two for 75 (18·3%) and level three for 126 (30·8%). CPR decisions were documented for 451 (93·0%) of whom 336 (74·5%) were 'not for resuscitation'. Advanced age, frailty, White-European ethnicity, a diagnosis of any co-morbidity and receipt of cardiovascular medications were associated with ceiling of care decisions. In a multivariable model only advanced age (odds 0·89, 0·86-0·93 p < 0·001), frailty (odds 0·48, 0·38-0·60, p < 0·001) and the cumulative number of co-morbidities (odds 0·72, 0·52-1·0, p = 0·048) were independently associated. Death during hospitalisation was independently associated with age, frailty and requirement for level two or three care. CONCLUSION: Ceiling of care decisions were made for the majority of patients during the COVID-19 pandemic, broadly in line with known predictors of poor outcomes in COVID-19, but with a focus on co-morbidities suggesting ICU admission might not be a reliable end-point for observational studies where advanced care planning is routine.
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Planejamento Antecipado de Cuidados , COVID-19/terapia , Tomada de Decisão Clínica , Adulto , Idoso , Idoso de 80 Anos ou mais , Reanimação Cardiopulmonar , Feminino , Humanos , Cuidados para Prolongar a Vida , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
BACKGROUND: Cardiac resynchronisation therapy (CRT) confers symptomatic and survival benefits in chronic heart failure with reduced ejection fraction (HFrEF). There remains a paucity of data on long-term performance of left ventricular (LV) leads, particularly with newer quadripolar lead designs. METHODS: This single-centre study utilised an electronic, outpatient HFrEF database to identify CRT recipients (2008-2014). The primary endpoint was temporal trend in LV pacing thresholds during follow-up. Secondary outcomes were complications relating to acute or chronic lead failure and device-related infections. RESULTS: Two hundred eighty patients were included, with mean (±SD) age of 74.2 years (±9.0) and median follow-up of 7.6 years (interquartile range 4-9). Mean LV threshold was 1.37 V (±0.73) at implant and remained stable over the study period. No differences were observed based upon lead manufacturer. Compared to non-quadripolar leads (n = 216), those of quadripolar designs (n = 64) had a lower threshold at 6 months (1.20 vs 1.37 V; P = .04) and at the end of the study period (1.32 vs 1.46 V; P = .04). Patients with HFrEF of ischaemic aetiology had higher thresholds at implant (1.46 vs 1.34 V; P = .05), and this persisted until the end of follow-up (1.49 vs 1.34 V; P = .03). There was low incidence of acute (0.71%; 2/280) and chronic lead failure (1.79%; 5/280), with four cases (1.43%) of device infection. CONCLUSIONS: LV leads in the context of CRT have excellent chronic stability and low rates of adverse events. Those with newer quadripolar lead designs have lower thresholds at initial follow-up and in the longer term.
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Dispositivos de Terapia de Ressincronização Cardíaca , Insuficiência Cardíaca/terapia , Idoso , Eletrodos Implantados , Desenho de Equipamento , Análise de Falha de Equipamento , Feminino , Seguimentos , Humanos , Masculino , Estudos Retrospectivos , Volume SistólicoRESUMO
BACKGROUND: Ivabradine is licensed as add-on therapy in patients with severe left ventricular systolic dysfunction (LVSD), normal sinus rhythm, and suboptimal heart rate (HR) control, but effects are not fully established. This study sought to assess the impact of ivabradine therapy on hemodynamic and functional outcome measures in all patients with LVSD. METHODS: MEDLINE (1996-2017), Embase (1996-2017), Cochrane Central Register of Controlled Trials (CENTRAL), Cochrane Database of Systematic Reviews, ClinicalTrials.gov , and ISI Web of Science were searched for randomized clinical trials (RCTs) comparing standard medical therapy (SMT) plus ivabradine to SMT alone for patients with LVSD of any severity. Each trial was assessed using the Cochrane Collaborations Risk of Bias tool. RESULTS: Eight RCTs with 17,823 patients were included. Add-on use of ivabradine reduced resting HR (mean difference [MD] 10.3 bpm; p < 0.001), improved ejection fraction (EF) (MD 3.6%, p < 0.001), and preserved systolic blood pressure (MD 3.4 mmHg; p = 0.09). Stratified analyses according to severity of LVSD did not influence conferred benefits on HR and EF. Small improvements were noted in exercise tolerance (standardized MD 5.9 s; p = 0.004) and peak oxygen consumption (MD 2.9 ml/kg/min; p = 0.02). DISCUSSION: Adjunct therapy with ivabradine in patients with LVSD results in a favorable hemodynamic profile and correlates with improved functional capacity. Benefits appear to be broadly preserved irrespective of baseline EF. This was a meta-analysis of RCTs, though limited by exclusion of post hoc analyses, lack of access to patient level data, and inter-study variability in some baseline characteristics. Further, large-scale RCTs are warranted to evaluate effectiveness of ivabradine in cohorts with non-severe LVSD.
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Hemodinâmica/efeitos dos fármacos , Ivabradina/farmacologia , Disfunção Ventricular Esquerda , Pressão Sanguínea/efeitos dos fármacos , Fármacos Cardiovasculares/farmacologia , Tolerância ao Exercício/efeitos dos fármacos , Humanos , Volume Sistólico/efeitos dos fármacos , Resultado do Tratamento , Disfunção Ventricular Esquerda/tratamento farmacológico , Disfunção Ventricular Esquerda/fisiopatologiaRESUMO
Tapasin is an integral component of the peptide-loading complex (PLC) important for efficient peptide loading onto MHC class I molecules. We investigated the function of the tapasin-related protein, TAPBPR. Like tapasin, TAPBPR is widely expressed, IFN-γ-inducible, and binds to MHC class I coupled with ß2-microglobulin in the endoplasmic reticulum. In contrast to tapasin, TAPBPR does not bind ERp57 or calreticulin and is not an integral component of the PLC. ß2-microglobulin is essential for the association between TAPBPR and MHC class I. However, the association between TAPBPR and MHC class I occurs in the absence of a functional PLC, suggesting peptide is not required. Expression of TAPBPR decreases the rate of MHC class I maturation through the secretory pathway and prolongs the association of MHC class I on the PLC. The TAPBPR:MHC class I complex trafficks through the Golgi apparatus, demonstrating a function of TAPBPR beyond the endoplasmic reticulum/cis-Golgi. The identification of TAPBPR as an additional component of the MHC class I antigen-presentation pathway demonstrates that mechanisms controlling MHC class I expression remain incompletely understood.
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Apresentação de Antígeno/imunologia , Antígenos de Histocompatibilidade Classe I/imunologia , Imunoglobulinas/metabolismo , Proteínas de Membrana/metabolismo , Apresentação de Antígeno/efeitos dos fármacos , Calnexina/metabolismo , Calreticulina/metabolismo , Retículo Endoplasmático/efeitos dos fármacos , Retículo Endoplasmático/metabolismo , Complexo de Golgi/efeitos dos fármacos , Complexo de Golgi/metabolismo , Células HEK293 , Antígenos HLA-A/metabolismo , Células HeLa , Humanos , Interferon gama/farmacologia , Cinética , Proteínas de Membrana Transportadoras/metabolismo , Peptídeos/imunologia , Ligação Proteica/efeitos dos fármacos , Isomerases de Dissulfetos de Proteínas/metabolismo , Multimerização Proteica/efeitos dos fármacos , Transporte Proteico/efeitos dos fármacos , Microglobulina beta-2/metabolismoRESUMO
We present the case of a patient with Alström syndrome who was found to have evidence of a prothrombotic state on autopsy after sudden cardiac death. To the best of our knowledge, this case of persistent prothrombotic milieu is the first described in a patient with Alström syndrome.
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OBJECTIVE: To determine the diagnostic yield of a 'high' N-terminal pro-brain natriuretic peptide (NT-proBNP) in patients with suspected heart failure (HF) referred from primary to secondary care. METHODS: In this retrospective study, cardiac diagnoses were quantified in consecutive patients with an NT-proBNP>400 ng/L referred from primary care centres to a specialist HF service. RESULTS: Among 654 consecutive patients (age: 78.5±9.72 years; 45.9% men; left ventricular ejection fraction (LVEF): 55.4±12.5% (mean±SD)), the primary diagnoses were: valvular disease (39.4%), HF (29.2%; 13.3% with LVEF<40%) and atrial fibrillation (AF; 17.3%). In terms of primary or secondary diagnoses, 68% of patients had valve disease, 46.9% had AF and 29.2% had HF. A cardiac diagnosis was made in 85.9%. In multivariable analyses, NT-proBNP predicted HF with LVEF<40% (OR: 10.2, 95% CI: 5.63 to 18.3) and HF with any LVEF (OR: 6.13, 95% CI: 3.79 to 9.93). In canonical linear discriminant analyses, NT-proBNP correctly identified 54.5% of patients with HF. The remainder were misclassified as valvular disease, AF or no cardiac diagnosis. CONCLUSION: Among patients with an NT-proBNP>400 ng/L referred through a primary care HF pathway, most patients had valve disease or AF rather than HF. NT-proBNP cannot discriminate among HF, valve disease and AF. On this basis, NT-proBNP may be best employed in detecting cardiac disease in general rather than HF per se.
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Insuficiência Cardíaca , Doenças das Valvas Cardíacas , Masculino , Humanos , Idoso , Idoso de 80 Anos ou mais , Feminino , Volume Sistólico , Estudos Retrospectivos , Função Ventricular Esquerda , Encaminhamento e ConsultaRESUMO
Introduction: Sarcoidosis is a systemic inflammatory disorder associated with ventricular arrhythmias (VAs) and sudden death in the context of cardiac involvement. Guidelines advocate implantable cardioverter-defibrillator (ICD) implantation in specific subcohorts, but there is a paucity of data on outcomes. Methods and Results: A systematic review was performed to assess outcomes in patients with definite or probable cardiac sarcoidosis (CS) treated with ICD. Observational studies were identified from multiple databases from inception to 21st May 2021. Outcomes of interest included appropriate and inappropriate ICD therapies in addition to all-cause mortality. Study quality was assessed individually using the Newcastle Ottawa Scale (NOS).Eight studies were identified comprising 530 patients, with follow-up period of 24-66 months (weighted average 40 months). Mean age was 53.9 years with ejection fraction of 41.3%. Overall incidence of appropriate therapy was 38.1% during follow-up. Left ventricular systolic dysfunction (LVSD) with ejection fraction <40% was a predictor of appropriate therapy in the majority of studies, as were sustained VAs during electrophysiological testing (EP) in one study. There was no interaction with device indication (i.e. primary or secondary). Where documented, inappropriate therapy was primarily driven by atrial arrhythmias. All-cause mortality was 6.0% over a median follow-up period of 42 months. Only three studies achieved good quality in the comparability domain of NOS. Conclusions: Appropriate ICD therapy in patients with CS is commonly associated with LVSD, which can act as a surrogate for scar burden. The utility of EP testing in this setting remains unclear.
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Among primary prevention implantable cardioverter-defibrillator (ICD) recipients, 75% do not experience any appropriate ICD therapies during their lifetime, and nearly 25% have improvements in their left ventricular ejection fraction (LVEF) during the lifespan of their first generator. The practice guidelines concerning this subgroup's clinical need for generator replacement (GR) remain unclear. We conducted a proportional meta-analysis to determine the incidence and predictors of ICD therapies after GR and compared this to the immediate and long-term complications. A systematic review of existing literature on ICD GR was performed. Selected studies were critically appraised using the Newcastle-Ottawa scale. Outcomes data were analyzed by random-effects modeling using R (R Foundation for Statistical Computing, Vienna, Austria), and covariate analyses were conducted using the restricted maximum likelihood function. A total of 31,640 patients across 20 studies were included in the meta-analysis with a median (range) follow-up of 2.9 (1.2-8.1) years. The incidences of total therapies, appropriate shocks, and anti-tachycardia pacing post-GR were approximately 8, 4, and 5 per 100 patient-years, respectively, corresponding to 22%, 12%, and 12% of patients of the total cohort, with a high level of heterogeneity across the studies. Greater anti-arrhythmic drug use and previous shocks were associated with ICD therapies post-GR. The all-cause mortality was approximately 6 per 100 patient-years, corresponding to 17% of the cohort. Diabetes mellitus, atrial fibrillation, ischemic cardiomyopathy, and the use of digoxin were predictors of all-cause mortality in the univariate analysis; however, none of these were found to be significant predictors in the multivariate analysis. The incidences of inappropriate shocks and other procedural complications were 2 and 2 per 100 patient-years, respectively, which corresponded to 6% and 4% of the entire cohort. Patients undergoing ICD GR continue to require therapy in a significant proportion of cases without any correlation with an improvement in LVEF. Further prospective studies are necessary to risk-stratify ICD patients undergoing GR.
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Background: PRAETORIAN is the first randomized controlled trial that demonstrated the noninferiority of subcutaneous ICD (S-ICD) in comparison with transvenous ICD (TV-ICD). We retrospectively reviewed electronic records of patients with ICD implanted over the past 6 years, with the primary objective to compare our real-world single tertiary center experience with the randomized data from the PRAETORIAN study. Methods: Seventy S-ICD patients were compared with 197 TV-ICD patients, from July 2014 to June 2020 retrospectively, over a median period of 1304 days (296-2451 days). Primary composite endpoints included inappropriate shocks and device-related malfunctions. Results: Patients with S-ICD implantation were younger than those who received TV-ICD (mean, 49.7 years vs 63.9 years, p < .001). About 31.4% of S-ICDs were implanted for secondary prevention, and 58.6% of S-ICD patients had ischemic cardiomyopathy (ICM) with a median left ventricular ejection fraction of 32.5% (range: 10-67%). S-ICDs and TV-ICD had statistically similar inappropriate shocks (4.3% vs 4.6%, p = .78), device-related complications (11.4% vs 9.1%, p = .93), and the overall primary endpoints (15.7% vs 13.7%, p = .68). The findings remained the same even after age and gender adjustments and time-dependent analysis. Conclusion: Although single-center experience with a small number of S-ICD patients, results of the PRAETORIAN study has been replicated in our real-world experience of S-ICD and TV-ICD implantations across diverse etiologies, indications, and age groups confirming the comparable performance of S-ICD and TV-ICD when implanted in selected patients.
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AIMS: We compared strategies in the treatment of decompensated severe aortic stenosis. The hypothesis was that undertaking urgent or emergency transcatheter aortic valve implantation (TAVI) directly in such patients is safer and more effective than urgent or emergency balloon aortic valvuloplasty (BAV) followed by elective TAVI or surgical aortic valve replacement (SAVR). METHODS: This was a single-centre retrospective study including all consecutive patients who underwent urgent or emergency BAV or TAVI for decompensated severe aortic stenosis between September 2014 and February 2018. Primary endpoints were 30-day and 1-year mortality. RESULTS: Fifty-two patients underwent urgent or emergency BAV and 87 underwent TAVI. Baseline characteristics of the two groups were well matched. Significant differences were noted between the two groups in 30-day all-cause mortality (88.5% BAV patients alive at 30 days, 97.7% TAVI patients; Pâ<â0.05) and 1-year all-cause mortality (44.2% BAV patients alive at 1 year, 88.5% TAVI patients; Pâ<â0.001). At 1 year, the estimated hazard ratio for patients undergoing BAV was 11.2 (95% confidence interval: 4.67-26.9; Pâ<â0.001) when adjusted for potential confounding variables. Patients in the BAV group who successfully underwent subsequent TAVI or SAVR all survived for 365 days, but there was no significant 1-year mortality difference compared with those who underwent urgent or emergency TAVI (100 vs. 88.5%; Pâ>â0.155). CONCLUSION: Our results suggest treatment of decompensated severe aortic stenosis with urgent or emergency TAVI may be associated with improved survival outcomes when compared with a strategy of performing BAV as a bridge to subsequent TAVI or SAVR.
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Assistência Ambulatorial/normas , Estenose da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia , Emergências , Tratamento de Emergência/normas , Guias de Prática Clínica como Assunto , Substituição da Valva Aórtica Transcateter/métodos , Idoso , Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/diagnóstico , Feminino , Humanos , Masculino , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: In many cases, the cause of exercise-induced cardiopulmonary arrest in young persons is thought to be fatal arrhythmia, and one of the causes is ischaemic heart disease. Left main coronary artery atresia (LMCAA) is an extremely rare disease in which there is a congenital defect of the left main coronary artery, causing heart failure and exercise-induced angina attacks at a young age. Thus, it is disease that should be differentiated when examining young persons with chest pain. CASE SUMMARY: A 16-year-old boy experienced sudden cardiopulmonary arrest during soccer practice, was brought to our hospital for emergency treatment after return of spontaneous circulation. Elective coronary angiography revealed findings indicating an osmium defect in the left coronary artery (LCA) and blood flow via collateral circulation from the right coronary artery. Contrast-enhanced coronary computed tomography (CT) angiography showed a defect in the LCA ostium and LMCAA was diagnosed in the patient. After coronary artery bypass grafting was performed, but the patient was discharged in an ambulatory state with a wearable cardiac defibrillator. Postoperative course has been favourable. DISCUSSION: Left main coronary artery atresia is an extremely rare disease in which there is a congenital defect of the left main trunk of the coronary artery and should be differentiated when encountering cases of heart failure or exercise-induced angina/arrhythmia attacks in young persons who are not at risk for atherosclerosis. Exercise electrocardiogram may show a false negative result, and therefore coronary CT is useful for diagnosis.
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Endocardial transvenous permanent pacemakers (TVPs) are a mainstay within cardiology and used to treat a range of bradyarrhythmias. However, their use is associated with potential complications both at the time of implantation and longer term. The concept of a leadless pacemaker, where a self-contained device is placed within the right ventricle, has obvious attractions. Two leadless systems have been developed, though only one is currently available. Results from clinical trials have been promising but a number of hurdles need to be circumvented before leadless devices can usurp TVPs. At present, use is restricted to specialist centres, for a limited indication and for patients in whom conventional implantation is contraindicated. This article provides a contemporary critique of design types, evidence base and existing limitations of this nascent technology.
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Desenho de Equipamento , Marca-Passo Artificial , Arritmias Cardíacas/terapia , HumanosRESUMO
Use of angiography for the assessment of coronary lesions is limited by its inability to provide information regarding the functional significance of stenoses. A number of studies have demonstrated the presence of ischaemia to be the most important determinant of the benefit associated with coronary revascularisation in stable coronary artery disease. Assessment of intra-coronary physiology can guide percutaneous coronary intervention, and is often used for angiographically borderline stenoses. There is now increasing evidence to suggest that more routine use can improve clinical outcomes. Fractional flow reserve (FFR) is the most established measure of intra-coronary physiology, but is currently under-utilised. The main drawback of FFR is the dependence on a pharmacological infusion to maintain hyperaemia. An alternative technique which measures flow at a specific point in the cardiac cycle (instantaneous wave-free ratio) has been developed which obviates the need for hyperaemia and may replace FFR as the default measure.
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Doença da Artéria Coronariana , Vasos Coronários , Técnicas de Diagnóstico Cardiovascular , Reserva Fracionada de Fluxo Miocárdico/fisiologia , Doença da Artéria Coronariana/diagnóstico , Doença da Artéria Coronariana/fisiopatologia , Doença da Artéria Coronariana/terapia , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/fisiologia , Vasos Coronários/fisiopatologia , Medicina Baseada em Evidências , Humanos , Modelos Cardiovasculares , Intervenção Coronária PercutâneaRESUMO
Global ventricular impairment is a frequent presentation in clinical practice, but dissection of causative mechanisms from clinical associations is challenging. We present the case of a 19-year-old man who presented with dilated cardiomyopathy as the first presentation of coeliac disease. The manifestation of iron deficiency anaemia prompted gastroenterology input and enabled accurate diagnosis. It is unclear whether coeliac disease was simply coexistent or directly implicated in pathophysiology. Mechanisms may relate to nutritional deficiencies or autoimmune myocarditis arising from cross-reactivity. We advocate early multidisciplinary involvement in such contexts to aid with management strategy. Despite adherence to a gluten-free diet, ventricular dysfunction persisted and he has been referred to a cardiac transplant centre.
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Cardiomiopatia Dilatada , Doença Celíaca , Adulto , Cardiomiopatia Dilatada/complicações , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/fisiopatologia , Doença Celíaca/complicações , Doença Celíaca/diagnóstico , Doença Celíaca/fisiopatologia , Humanos , Masculino , Adulto JovemRESUMO
Interventional cardiology has been revolutionised by transcatheter aortic valve implantation (TAVI), which has become established as the benchmark treatment for severe aortic stenosis in patients at high risk for surgical aortic valve replacement (AVR). Increased procedural familiarity and progression in device technology has enabled improvements to be made in complication rates, which have led to a commensurate expansion in the use of TAVI; it is now a viable alternative to AVR in patients at intermediate surgical risk, and has been used in cohorts such as those with bicuspid aortic valves or pure, severe aortic regurgitation. Given the rapid expansion in the use of TAVI, including cohorts of younger patients with fewer co-morbidities, attention must be paid to further reducing remaining complications, such as cardiac tamponade or stroke. To this end, novel techniques and devices have been devised and trialled, with varying levels of success. Furthermore, significant work has gone into refining the technique with exploration of alternative imaging modalities, as well as alternative access routes to provide greater options for patients with challenging vascular anatomy. Whilst significant progress has been made with TAVI, areas of uncertainty remain such as the management of concomitant coronary artery disease and the optimum post-procedure antiplatelet regimen. As such, research in this field continues apace, and is likely to continue as use of TAVI becomes more widespread. This review provides a summary of the existing evidence, as well as an overview of recent developments and contentious issues in the field of TAVI.