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2.
Clin Cardiol ; 46(10): 1194-1201, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37489866

RESUMO

BACKGROUND: Health care resource utilization (HCRU) and costs are important metrics of health care burden, but they have rarely been explored in the setting of cardiac ion channelopathies. HYPOTHESIS: This study tested the hypothesis that attendance-related HCRUs and costs differed between patients with Brugada syndrome (BrS) and congenital long QT syndrome (LQTS). METHODS: This was a retrospective cohort study of consecutive BrS and LQTS patients at public hospitals or clinics in Hong Kong, China. HCRUs and costs (in USD) for Accident and Emergency (A&E), inpatient, general outpatient and specialist outpatient attendances were analyzed between 2001 and 2019 at the cohort level. Comparisons were made using incidence rate ratios (IRRs [95% confidence intervals]). RESULTS: Over the 19-year period, 516 BrS (median age of initial presentation: 51 [interquartile range: 38-61] years, 92% male) and 134 LQTS (median age of initial presentation: 21 [9-44] years, 32% male) patients were included. Compared to LQTS patients, BrS patients had lower total costs (2 008 126 [2 007 622-2 008 629] vs. 2 343 864 [2 342 828-2 344 900]; IRR: 0.857 [0.855-0.858]), higher costs for A&E attendances (83 113 [83 048-83 177] vs. 70 604 [70 487-70 721]; IRR: 1.177 [1.165-1.189]) and general outpatient services (2,176 [2,166-2,187] vs. 921 [908-935]; IRR: 2.363 [2.187-2.552]), but lower costs for inpatient stay (1 391 624 [1 391 359-1 391 889] vs. 1 713 742 [1 713 166-1 714 319]; IRR: 0.812 [0.810-0.814]) and lower costs for specialist outpatient services (531 213 [531 049-531 376] vs. 558 597 [558268-558926]; IRR: 0.951 [0.947-0.9550]). CONCLUSIONS: Overall, BrS patients consume 14% less health care resources compared to LQTS patients in terms of attendance costs. BrS patients require more A&E and general outpatient services, but less inpatient and specialist outpatient services than LQTS patients.


Assuntos
Síndrome de Brugada , Síndrome do QT Longo , Humanos , Masculino , Adulto , Pessoa de Meia-Idade , Feminino , Estudos Retrospectivos , Síndrome do QT Longo/diagnóstico , Síndrome do QT Longo/epidemiologia , Síndrome do QT Longo/terapia , Aceitação pelo Paciente de Cuidados de Saúde , Arritmias Cardíacas/complicações , Custos de Cuidados de Saúde
3.
Curr Probl Cardiol ; 48(2): 101513, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36414041

RESUMO

Understanding health care resource utilisation and its associated costs are important for identifying areas of improvement regarding resource allocations. However, there is limited research exploring this issue in the setting of Brugada syndrome (BrS).This was a retrospective territory-wide study of BrS patients from Hong Kong. Healthcare resource utilisation for accident and emergency (A&E), inpatient and specialist outpatient attendances were analyzed over a 19-year period, with their associated costs presented in US dollars. A total of 507 BrS patients with a mean presentation age of 49.9 ± 16.3 years old were included. Of these, 384 patients displayed spontaneous type 1 electrocardiographic (ECG) Brugada pattern and 77 patients had presented with ventricular tachycardia/ventricular fibrillation (VT/VF). At the individual patient level, the median annualized costs were $110 (52-224) at the (A&E) setting, $6812 (1982-32414) at the inpatient setting and $557 (326-1001) for specialist outpatient attendances. Patients with initial VT/VF presentation had overall greater costs in inpatient ($20161 [9147-189215] vs $5290 [1613-24937],P < 0.0001) and specialist outpatient setting ($776 [438-1076] vs $542 [293-972],P = 0.015) compared to those who did not present VT. In addition, patients without Type 1 ECG pattern had greater median costs in the specialist outpatient setting ($7036 [3136-14378] vs $4895 [2409-10554],p=0.019). There is a greater health care demand in the inpatient and specialist outpatient settings for BrS patients. The most expensive attendance type was inpatient setting stay at $6812 per year. The total median annualized cost of BrS patients without VT/VF presentation was 78% lower compared to patients with VT/VF presentation.


Assuntos
Síndrome de Brugada , Humanos , Adulto , Pessoa de Meia-Idade , Idoso , Síndrome de Brugada/epidemiologia , Síndrome de Brugada/terapia , Estudos Retrospectivos , Hong Kong/epidemiologia , Fibrilação Ventricular/complicações , Arritmias Cardíacas , Eletrocardiografia , Aceitação pelo Paciente de Cuidados de Saúde
6.
J Cardiovasc Electrophysiol ; 33(8): 1874-1879, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35695789

RESUMO

BACKGROUND: Fever is a potential side effect of the Covid-19 vaccination. Patients with Brugada syndrome (BrS) have an increased risk of life-threatening arrhythmias when experiencing fever. Prompt treatment with antipyretic drugs is suggested in these patients. AIM OF THE STUDY: To evaluate the incidence and management of fever within 48 h from Covid-19 vaccination among BrS patients. METHODS: One hundred sixty-three consecutive patients were enrolled in a prospective registry involving five European hospitals with a dedicated inherited disease ambulatory. RESULTS: The mean age was 50 ± 14 years and 121 (75%) patients were male. Prevalence of Brugada electrocardiogram (ECG) pattern type-1, -2, and -3 was 32%, 44%, and 24%, respectively. Twenty-eight (17%) patients had an implantable cardioverter-defibrillator (ICD). Fever occurred in 32 (19%) BrS patients after 16 ± 10 h from vaccination, with a peak of body temperature of 37.9° ± 0.5°. Patients with fever were younger (39 ± 13 vs. 48 ± 13 years, p = .04). No additional differences in terms of sex and cardiovascular risk factors were found between patients with fever and not. Twenty-seven (84%) out of 32 patients experienced mild fever and five (16%) moderate fever. Pharmacological treatment with antipyretic drugs was required in 18 (56%) out of 32 patients and was associated with the resolution of symptoms. No patient required hospital admission and no arrhythmic episode was recorded in patients with ICD within 48 h after vaccination. No induced type 1 BrS ECG pattern and new ECG features were found among patients with moderate fever. CONCLUSION: Fever is a common side effect in BrS patients after the Covid-19 vaccination. Careful evaluation of body temperature and prompt treatment with antipyretic drugs may be needed.


Assuntos
Antipiréticos , Síndrome de Brugada , Vacinas contra COVID-19 , COVID-19 , Desfibriladores Implantáveis , Adulto , Antipiréticos/efeitos adversos , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/epidemiologia , Síndrome de Brugada/terapia , COVID-19/epidemiologia , COVID-19/prevenção & controle , Vacinas contra COVID-19/efeitos adversos , Eletrocardiografia , Feminino , Febre/induzido quimicamente , Febre/diagnóstico , Febre/epidemiologia , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Vacinação/efeitos adversos
7.
Gen Hosp Psychiatry ; 78: 14-27, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35763920

RESUMO

OBJECTIVE: The Food and Drug Administration (FDA) warned about lamotrigine's arrhythmogenicity based on in vitro data. This systematic review investigates lamotrigine's effect on cardiac conduction and risk of sudden cardiac death (SCD) in individuals with and without cardiovascular disease. METHODS: We searched Web of Science and PubMed from inception through August 2021. We included studies measuring electrocardiogram (ECG) changes, laboratory abnormalities, or SCD among patients taking lamotrigine. Studies examining sudden unexpected death in epilepsy were excluded for scope. Two reviewers assessed articles and extracted data. We used the Effective Public Healthcare Panacea Project tool to evaluate confidence in evidence. RESULTS: Eight randomized controlled trials, 9 nonrandomized observational studies, and 24 case reports were identified, with >3054 total participants, >1606 of whom used lamotrigine. One randomized trial of older patients found an average QRS increase of 3.5 +/- 13.1 ms. Fifteen studies reported no changes in ECG parameters. Case reports documented QRS widening (13), Brugada syndrome (6), QTc prolongation (1) and SCD (2), though many ingested toxic quantities of lamotrigine and/or other medications. CONCLUSIONS: Evidence is insufficient to support the breadth of the FDA warning concerning lamotrigine's cardiac risk. Lamotrigine at therapeutic doses may be associated with modest, non-dangerous QRS widening.


Assuntos
Anticonvulsivantes , Síndrome de Brugada , Síndrome de Brugada/induzido quimicamente , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/etiologia , Eletrocardiografia , Humanos , Lamotrigina/efeitos adversos , Medição de Risco
8.
J Oral Maxillofac Surg ; 79(11): 2269.e1-2269.e11, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34453905

RESUMO

Brugada syndrome (BrS) carries the risk of major dysrhythmias increased further by exposure to pro-dysrhythmic factors related to oral surgical procedures such as local anesthetics, anxiety, and postoperative pain. Such risk can be handled by updated multidisciplinary management. In 2020, 3 male BrS patients needed oral surgical treatments at the Complex Unit of Odontostomatology of Aldo Moro University of Bari, Italy. Multidisciplinary individual risk assessment involved cardiologic hazard ratio stratification, event-free survival stratification, and 5-year average risk of ventricular dysrhythmias and sudden cardiac death for Brugada patients; American Society of Anesthesiologists physical status risk class; expected complexity and duration of the procedure; and anxiety score measured by the Modified Dental Anxiety Scale. The authors administered conscious sedation by intravenous diazepam to both a patient susceptible to vasovagal syncope needing tooth extraction with concomitant cystectomy (longer-lasting procedure) and to another who needed routine tooth extraction (brief procedure) but had a moderate dental anxiety score; the last 1 received local anesthesia alone due to his low anxiety, low susceptibility to vasovagal syncope, and need for routine tooth extraction. After positioning external biphasic defibrillator pads, 12-leads continuous electrocardiogram, and peripheral venous access, extractions were performed with local anesthesia by lidocaine 2% with epinephrine 1:100,000; acetaminophen was suggested for postoperative analgesia. No electrocardiographic changes occurred in the perioperative period. The current multidisciplinary individual risk assessment allowed us to detect each BrS patient's risk factors for major dysrhythmias and to adapt oral surgical and anesthesiologic protocols for safe targeted treatment.


Assuntos
Síndrome de Brugada , Arritmias Cardíacas , Morte Súbita Cardíaca , Eletrocardiografia , Humanos , Masculino , Medição de Risco , Extração Dentária
9.
J Am Heart Assoc ; 10(16): e021144, 2021 08 17.
Artigo em Inglês | MEDLINE | ID: mdl-34387130

RESUMO

Background Optimal management of asymptomatic Brugada syndrome (BrS) with spontaneous type I electrocardiographic pattern is uncertain. Methods and Results We developed an individual-level simulation comprising 2 000 000 average-risk individuals with asymptomatic BrS and spontaneous type I electrocardiographic pattern. We compared (1) observation, (2) electrophysiologic study (EPS)-guided implantable cardioverter-defibrillator (ICD), and (3) upfront ICD, each using either subcutaneous or transvenous ICD, resulting in 6 strategies tested. The primary outcome was quality-adjusted life years (QALYs), with cardiac deaths (arrest or procedural-related) as a secondary outcome. We varied BrS diagnosis age and underlying arrest rate. We assessed cost-effectiveness at $100 000/QALY. Compared with observation, EPS-guided subcutaneous ICD resulted in 0.35 QALY gain/individual and 4130 cardiac deaths avoided/100 000 individuals, and EPS-guided transvenous ICD resulted in 0.26 QALY gain and 3390 cardiac deaths avoided. Compared with observation, upfront ICD reduced cardiac deaths by a greater margin (subcutaneous ICD, 8950; transvenous ICD, 6050), but only subcutaneous ICD improved QALYs (subcutaneous ICD, 0.25 QALY gain; transvenous ICD, 0.01 QALY loss), and complications were higher. ICD-based strategies were more effective at younger ages and higher arrest rates (eg, using subcutaneous devices, upfront ICD was the most effective strategy at ages 20-39.4 years and arrest rates >1.37%/year; EPS-guided ICD was the most effective strategy at ages 39.5-51.3 years and arrest rates 0.47%-1.37%/year, and observation was the most effective strategy at ages >51.3 years and arrest rates <0.47%/year). EPS-guided subcutaneous ICD was cost-effective ($80 508/QALY). Conclusions Device-based approaches (with or without EPS risk stratification) can be more effective than observation among selected patients with asymptomatic BrS. BrS management should be tailored to patient characteristics.


Assuntos
Síndrome de Brugada/terapia , Técnicas de Apoio para a Decisão , Desfibriladores Implantáveis , Cardioversão Elétrica/instrumentação , Adulto , Doenças Assintomáticas , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/economia , Síndrome de Brugada/mortalidade , Pesquisa Comparativa da Efetividade , Análise Custo-Benefício , Desfibriladores Implantáveis/economia , Cardioversão Elétrica/efeitos adversos , Cardioversão Elétrica/economia , Cardioversão Elétrica/mortalidade , Eletrocardiografia , Custos de Cuidados de Saúde , Humanos , Pessoa de Meia-Idade , Modelos Econômicos , Anos de Vida Ajustados por Qualidade de Vida , Recuperação de Função Fisiológica , Fatores de Tempo , Resultado do Tratamento
10.
J Clin Ultrasound ; 49(6): 573-579, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33599988

RESUMO

PURPOSE: The time interval between the onset of the P-wave on electrocardiogram (ECG) and peak A' velocity of the lateral left atrial wall assessed by tissue Doppler imaging (PA-TDI interval) determine total atrial conduction time (TACT) which reflects atrial remodeling and arrhythmic substrate. In this retrospective study, we aimed to assess TACT in patients with atrioventricular nodal reentrant tachycardia (AVNRT) with and without drug-induced type 1 Brugada electrocardiogram ECG pattern (DI-Type 1 BrP) and control subjects. METHODS: Study population consisted of 62 consecutive patients (46 women; mean age 44 ± 12 years) undergoing electrophysiological study and ablation for symptomatic, drug-resistant AVNRT, and 42 age-matched and sex-matched control subjects. All patients and control subjects underwent ajmaline challenge test and tissue Doppler imaging. RESULTS: A DI-Type 1 BrP was uncovered in 24 of 62 patients with AVNRT (38.7%). PA-TDI interval was similar among AVNRT patients with and without DI-Type 1 BrP (124 ± 12 ms vs 119 ± 14 ms, respectively, P = .32), but significantly longer in patients with AVNRT with as well as without DI-Type 1 BrP than in control subjects (124 ± 12 ms and 119 ± 14 ms vs 105 ± 11 ms, respectively, P < .001). CONCLUSION: The TACT assessed by PA-TDI interval is longer in patients with AVNRT with and without DI-Type 1 BrP than in age-matched and sex-matched healthy control subjects.


Assuntos
Remodelamento Atrial , Síndrome de Brugada/induzido quimicamente , Síndrome de Brugada/complicações , Taquicardia por Reentrada no Nó Atrioventricular/complicações , Taquicardia por Reentrada no Nó Atrioventricular/patologia , Adulto , Estudos de Casos e Controles , Ablação por Cateter , Eletrocardiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taquicardia por Reentrada no Nó Atrioventricular/fisiopatologia , Taquicardia por Reentrada no Nó Atrioventricular/terapia
11.
Circ Genom Precis Med ; 13(4): e002797, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32490690

RESUMO

BACKGROUND: Brugada syndrome (BrS) is an oligogenic arrhythmic disease with increased risk of sudden cardiac arrest. Several BrS or ECG traits-related single-nucleotide polymorphisms (SNPs) were identified through previous genome-wide association studies in white patients. We aimed to validate these SNPs in BrS patients in the Taiwanese population, assessing the cumulative effect of risk alleles and the BrS-polygenic risk score in predicting cardiac events. METHODS: We genotyped 190 unrelated BrS patients using the TWB Array, and Taiwan Biobank was used as controls. SNPs not included in the array were imputed by IMPUTE2. Cox proportional hazards model was used to evaluate the associations between each particular SNP, the collective BrS-polygenic risk score, and clinical outcomes. RESULTS: Of the 88 previously reported SNPs, 22 were validated in Taiwanese BrS patients (P<0.05). Of the 22 SNPs, 2 (rs10428132 and rs9388451) were linked with susceptibility to BrS, 10 were SNPs previously reaching genome-wide significance, and 10 were SNPs associated with ECG traits. For the 3 most commonly reported SNPs, disease risk increased consistently with the number of risk alleles (odds ratio, 3.54; Ptrend=1.38×10-9 for 5 risk alleles versus 1). Similar patterns were observed in both SCN5A mutation+ (odds ratio, 3.66; Ptrend=0.049) and SCN5A mutation- (odds ratio, 3.75; Ptrend=8.54×10-9) subgroups. Furthermore, BrS patients without SCN5A mutations had more risk alleles than BrS patients with SCN5A mutations regardless of the range of polygenic risk scores. Three SNPs (rs4687718, rs7784776, and rs2968863) showed significant associations with the composite outcome (sudden cardiac arrest plus syncope, hazard ratio, 2.13, 1.48, and 0.41; P=0.02, 0.006, and 0.008, respectively). CONCLUSIONS: Our findings suggested that some SNPs associated with BrS or ECG traits exist across multiple populations. The cumulative risk of the BrS-related SNPs is similar to that in white BrS patients, but it appears to correlate with the absence of SCN5A mutations.


Assuntos
Síndrome de Brugada/genética , Estudo de Associação Genômica Ampla , Adulto , Alelos , Povo Asiático/genética , Síndrome de Brugada/patologia , Estudos de Casos e Controles , Eletrocardiografia , Feminino , Predisposição Genética para Doença , Humanos , Masculino , Pessoa de Meia-Idade , Canal de Sódio Disparado por Voltagem NAV1.5/genética , Razão de Chances , Polimorfismo de Nucleotídeo Único , Modelos de Riscos Proporcionais , Sistema de Registros , Medição de Risco , Análise de Sequência de DNA , Taiwan
13.
Am Heart J ; 220: 213-223, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31864099

RESUMO

BACKGROUND: Cardiogenic syncope in Brugada syndrome (BrS) increases the risk of major events. Nevertheless, clinical differentiation between cardiogenic and vasovagal syncope can be challenging. We characterized the long-term incidence of major events in a large cohort of BrS patients who presented with syncope. METHODS: From a total of 474 patients, syncope was the initial manifestation in 135 (28.5%) individuals (43.9 ±â€¯13.9 years, 71.1% male). The syncope was classified prospectively as cardiogenic, vasovagal, or undefined if unclear characteristics were present. Clinical, electrocardiographic, genetic, and electrophysiologic features were analyzed. Cardiogenic syncope, sustained ventricular arrhythmias, and sudden death were considered major events in follow-up. RESULTS: In 66 patients (48.9%), the syncope was cardiogenic; in 51 (37.8%), vasovagal and in 18 (13.3%); undefined. The electrophysiology study (EPS) inducibility was more frequent in patients with cardiogenic syncope and absent in all patients with undefined syncope (28 [53.8%] vs 5 [12.2%] vs 0 [0%]; P < .01). During follow-up (7.7 ±â€¯5.6 years), only patients with cardiogenic syncope presented major events (16 [11.9%]). Among patients with inducible EPS, 7 (21.2%) presented major events (P = .04). The negative predictive value of the EPS for major events was 92.4%. The incidence rate of major events was 2.6% person-year. Parameters associated with major events included cardiogenic syncope (hazard ratio [HR] 6.3; 95% CI 1.1-10.4; P = .05), spontaneous type 1 electrocardiogram (HR 3.7; 95% CI 1.3-10.5; P = .01), and inducible EPS (HR 2.8; 95% CI 1.1-8.8; P = .05). CONCLUSIONS: An accurate syncope classification is crucial in BrS patients for risk stratification. In patients with syncope of unclear characteristics, the EPS may be helpful to prevent unnecessary implantable cardioverter defibrillators.


Assuntos
Síndrome de Brugada/complicações , Síncope/etiologia , Adulto , Arritmias Cardíacas/etiologia , Síndrome de Brugada/fisiopatologia , Morte Súbita Cardíaca/etiologia , Desfibriladores Implantáveis , Eletrocardiografia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Canal de Sódio Disparado por Voltagem NAV1.5/genética , Valor Preditivo dos Testes , Prevalência , Síncope/classificação , Síncope/epidemiologia , Síncope/fisiopatologia , Síncope Vasovagal/epidemiologia , Síncope Vasovagal/etiologia , Síncope Vasovagal/fisiopatologia , Teste da Mesa Inclinada
14.
Europace ; 21(12): 1900-1910, 2019 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-31647530

RESUMO

AIMS: Brugada syndrome (BrS) represents a major cause of sudden cardiac death in young individuals. The risk stratification to forecast future life-threatening events is still controversial. Non-invasive assessment of late potentials (LPs) has been proposed as a risk stratification tool. However, their nature in BrS is still undetermined. The purpose of this study is to assess the electrophysiological determinants of non-invasive LPs. METHODS AND RESULTS: Two hundred and fifty consecutive patients with (Group 1, n = 96) and without (Group 2, n = 154) BrS-related symptoms were prospectively enrolled in the registry. Signal-averaged electrocardiogram (SAECG) was performed in all subjects before undergoing epicardial mapping. Group 1 patients exhibited larger arrhythmogenic substrates (AS; 5.8 ± 2.8 vs. 2.6 ± 2.1 cm2, P < 0.001) with more delayed potentials (220.4 ± 46.0 vs. 186.7 ± 42.3 ms, P < 0.001). Late potentials were present in 82/96 (85.4%) Group 1 and in 31/154 (20.1%) Group 2 individuals (P < 0.001). Patients exhibiting LPs had more frequently a spontaneous Type 1 pattern (30.1% vs. 10.9%, P < 0.001), SCN5A mutation (34.5% vs. 21.2%, P = 0.02), and exhibited a larger AS with longer potentials (5.8 ± 2.7 vs. 2.2 ± 1.7 cm2; 231.2 ± 37.3 vs. 213.8 ± 39.0 ms; P < 0.001, respectively). Arrhythmogenic substrate dimension was the strongest predictor of the presence of LPs (odds ratio 1.9; P < 0.001). An AS area of at least 3.5 cm2 identified patients with LPs (area under the curve 0.88, 95% confidence interval 0.843-0.931; P < 0.001) with a sensitivity of 86%, specificity 88%, positive predictive value 85%, and negative predictive value 89%. CONCLUSION: The results of this study support the role of the epicardial AS as an electrophysiological determinant of non-invasive LPs, which may serve as a tool in the non-invasive assessment of the BrS substrate, as SAECG-LPs could be considered an expression of the abnormal epicardial electrical activity.ClinicalTrials.gov number (NCT02641431; NCT03106701).


Assuntos
Potenciais de Ação , Síndrome de Brugada/fisiopatologia , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis , Eletrocardiografia/métodos , Mapeamento Epicárdico/métodos , Processamento de Sinais Assistido por Computador , Taquicardia Ventricular/fisiopatologia , Fibrilação Ventricular/fisiopatologia , Adolescente , Adulto , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/terapia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Medição de Risco , Taquicardia Ventricular/terapia , Fibrilação Ventricular/terapia , Adulto Jovem
15.
J Perioper Pract ; 29(5): 140-146, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30212282

RESUMO

Brugada syndrome, an autosomal dominant genetic disorder, is characterised by abnormal electrocardiogram findings and increased risk of ventricular tachyarrhythmias and sudden cardiac death. Our report describes the multi-disciplinary perioperative management of a 28-year-old patient presenting to the Duke Transplant Center with a familial sodium channel gene SCN51 mutation concerning Brugada syndrome. We discuss the preparatory work-up, medication review and appropriate post-surgical follow-up for patients undergoing liver transplant surgery with cardiac monitoring.


Assuntos
Síndrome de Brugada/genética , Predisposição Genética para Doença , Transplante de Fígado , Medição de Risco , Adulto , Feminino , Humanos , Equipe de Assistência ao Paciente , Assistência Perioperatória , Cuidados Pós-Operatórios
16.
Adv Exp Med Biol ; 1065: 181-190, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30051385

RESUMO

INTRODUCTION: Cardiac events in patients with Brugada syndrome (BS) typically occur at rest and mainly during sleep, suggesting that changes in autonomic modulation play an important role in the arrhythmogenesis of the disease. Moreover, sex differences in clinical manifestations of BS have been reported, identifying male patients with worse prognosis. The aim of our work was to assess and compare, according to sex, autonomic response to exercise in a clinical series including 105 BS patients. METHOD: Standard 12-lead electrocardiogram recordings were collected during a physical stress test divided into four phases: warm-up, incremental exercise, active recovery, and passive recovery. Spectral non-stationary heart rate variability indicators were extracted by means of a smoothed pseudo Wigner-Ville distribution approach that adapts frequency bands to respiratory information. These indicators were then averaged in non-overlapped windows of 1 min for each patient to compare groups at each minute of the physical stress test. RESULTS: From the last minute of warm-up and until the third minute of incremental exercise, asymptomatic male patients presented significantly greater low-frequency (LF) values ([Formula: see text]: p = 0.015;[Formula: see text]: p = 0.024; [Formula: see text]: p = 0.011; [Formula: see text]: p = 0.002) than asymptomatic females. Conversely, asymptomatic women showed increased vagal modulation during the first minutes of incremental exercise ([Formula: see text]: p = 0.031; [Formula: see text]: p = 0.001). However, no significant differences were observed between symptomatic male and female patients. CONCLUSION: As previously reported in healthy subjects, enhanced parasympathetic and decreased sympathetic tones appear to be not only greater in women but also defensive during cardiac stress. Based on the results, asymptomatic patients presented same-sex tendencies. However, we observed that symptomatic males developed a more female-like autonomic modulation, probably related to a more protective autonomic response to exercise. These results could be a step forward toward the understanding of the autonomic function in BS along with a potential impact on risk stratification.


Assuntos
Sistema Nervoso Autônomo/fisiopatologia , Síndrome de Brugada/diagnóstico , Eletrocardiografia , Teste de Esforço , Frequência Cardíaca , Coração/inervação , Potenciais de Ação , Adulto , Síndrome de Brugada/fisiopatologia , Feminino , Disparidades nos Níveis de Saúde , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Fatores Sexuais , Fatores de Tempo
17.
Eur Heart J ; 38(40): 3029-3034, 2017 Oct 21.
Artigo em Inglês | MEDLINE | ID: mdl-29020354

RESUMO

Brugada syndrome (BS) is an inherited disease characterized by a coved-type ST-segment elevation in the right precordial leads and increased risk of sudden cardiac death (SCD), in the absence of structural abnormalities. The cornerstone of BS diagnosis and definition, is its characteristic ECG pattern that can be present spontaneously or unmasked by drugs. Brugada syndrome was first described 25 years ago; paradoxically, in an era of great technological development, a new syndrome was described with a technology developed almost a century before. Great scientific knowledge has been gathered since the description of the syndrome. The better understanding of its pathophysiology and genetic basis has led to several modifications in its definition. Despite these facts, the essential, the description of the specific ECG pattern has remained almost unchanged since the initial report. In this article, we present the definition of the BS, the rationale behind it and our thoughts about its future.


Assuntos
Síndrome de Brugada/diagnóstico , Síndrome de Brugada/genética , Consenso , Morte Súbita Cardíaca/etiologia , Eletrocardiografia , Previsões , Humanos , Canais Iônicos/genética , Publicações/estatística & dados numéricos , Publicações/tendências
18.
Eur Heart J Cardiovasc Imaging ; 18(2): 186-194, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27025513

RESUMO

AIMS: Patients with unexplainable giant T-wave inversion in the precordial leads and apical wall thickness <15 mm have been reported. These patients cannot be diagnosed as apical hypertrophic cardiomyopathy (AHCM) according to the current criteria. The objective of this study was to evaluate the apical morphological features of this type of patients using cardiac magnetic resonance. METHODS AND RESULTS: Institutional ethics approval and written informed consent were obtained. A total of 60 subjects with unexplainable giant T-wave inversion and 76 healthy volunteers were prospectively enrolled in the study. The segmented left ventricular (LV) wall thickness was measured according to the American Heart Association 17-segmented model. The apical angle (apA) as well as the regional variations in LV wall thickness was analysed. Considerable variation in LV wall thickness in normals was observed with progressive thinning from the base to apex (male and female, P < 0.01). The apical thickness of subjects with giant T-wave inversion was 8.10 ± 1.67 mm in male, which is thicker than that of controls (4.14 ± 1.17 mm, P < 0.01). In female, the apical thickness was also significantly different from controls (5.85 ± 2.16 vs. 2.99 ± 0.65 mm, P < 0.01). Compared with normals, the apA decreased significantly in male (87.44 ± 13.86 vs.115.03 ± 9.90°, P < 0.01) and female (90.69 ± 8.84 vs. 110.07 ± 13.58°, P < 0.01) subjects, respectively. CONCLUSION: Although the absolute thickness of apical wall was below the current diagnostic criteria of AHCM, the apical morphological features of subjects with unexplainable giant T-wave inversion were significantly different from normals. Whether these subjects should be included into a preclinical scope of AHCM needs further investigations.


Assuntos
Síndrome de Brugada/diagnóstico por imagem , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Eletrocardiografia/métodos , Interpretação de Imagem Assistida por Computador , Imagem Cinética por Ressonância Magnética/métodos , Adulto , Análise de Variância , Doença do Sistema de Condução Cardíaco , Cardiomiopatia Hipertrófica/fisiopatologia , Estudos de Casos e Controles , Feminino , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Prognóstico , Estudos Prospectivos , Reprodutibilidade dos Testes , Índice de Gravidade de Doença , Estatísticas não Paramétricas
19.
Artigo em Inglês | MEDLINE | ID: mdl-27282320

RESUMO

BACKGROUND: To evaluate electrocardiographic parameters which are related with atrial and ventricular arrhythmias measured from 12-lead surface electrocardiogram (ECG) in workers occupationally exposed to lead. METHODS: Sixty lead-exposed workers and 60 healthy controls were enrolled. Twelve-lead surface ECG was recorded and measurements of P wave durations (Pmax, Pmin) and P wave dispersion (PWD), QT durations and dispersion (QTd), corrected QT (QTc), Tp-e interval, and Tp-e/QT ratio were analyzed. RESULTS: The lead-exposed and control groups were similar with respect to baseline demographic, laboratory, and transthoracic echocardiographic indices. PWD (26.3 ± 9.7 vs 22.0 ± 9.0 ms, P = 0.014), Pmin (89.9 ± 13.8 vs 79.2 ± 10.1 ms, P < 0.001), and Pmax (116.2 ± 15.0 vs 101.2 ± 14.2 ms, P < 0.001), QT maximum (377.0 ± 27.6 vs 364.9 ± 28.5 ms, P = 0.02), QTd (38.4 ± 16.5 vs 30.5 ± 12.4 ms, P = 0.004), Tp-e interval (78.9 ± 16.5 vs 70.3 ± 14.5 ms, P = 0.003), and Tp-e/QT ratio (0.22 ± 0.04 vs 0.20 ± 0.04, P = 0.013) were significantly higher in lead-exposed workers. QT minimum and QTc values did not differ significantly. QT maximum, QTd, and Tp-e/QT ratio were correlated with urine lead level and Tp-e interval was correlated with both blood and urine lead levels. CONCLUSIONS: Lead-exposed workers have a higher risk for atrial and ventricular arrhythmias even without overt cardiac diseases compared with healthy subjects. These workers should be followed closely for adverse cardiovascular outcomes especially arrhythmias.


Assuntos
Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/fisiopatologia , Eletrocardiografia/estatística & dados numéricos , Intoxicação por Chumbo/fisiopatologia , Exposição Ocupacional , Adulto , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/fisiopatologia , Doença do Sistema de Condução Cardíaco , Estudos Transversais , Eletrocardiografia/métodos , Feminino , Sistema de Condução Cardíaco/fisiopatologia , Humanos , Intoxicação por Chumbo/diagnóstico , Masculino , Medição de Risco
20.
Europace ; 19(11): 1864-1873, 2017 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-27738063

RESUMO

AIMS: To investigate the clinical characteristics, prognoses, and presence of risk factors in young patients with Brugada syndrome (BS). METHODS AND RESULTS: A consecutive cohort of 128 young BS patients (≤25 years old at diagnosis) was analysed. Eighty-eight patients (69%) were asymptomatic, whereas 40 (31%) presented with clinical manifestations of BS. Markers of prognosis and risk were identified upon comparison of these two groups. A history of malignant syncope was strong predictors of ventricular arrhythmic events. Family history of sudden cardiac death (SCD) and mutations in the SCN5A gene did not associate with increased risk. Symptomatic patients presented with significantly abnormal baseline electrical characteristics when compared with the asymptomatic cohort, including spontaneous type I electrocardiograph (ECG) patterns, sinus node dysfunction (SND), first-degree atrioventricular (AV) block, and intra-ventricular conduction delay. The symptomatic group more frequently exhibited atrial arrhythmias. Electrophysiological studies resulted positive more frequently in symptomatic patients, but no risk association for future events could be determined. During the follow-up period (mean: 65 months), 10 arrhythmic events occurred in nine symptomatic patients (event rate: 4.5% per year). No events occurred in the asymptomatic group. Variables significantly associated with arrhythmic events during follow-up were presence of symptoms at diagnosis and spontaneous type I ECG. The presence of atrial arrhythmias and conduction abnormalities was also associated with the risk of arrhythmic events during follow-up. CONCLUSION: Symptomatic BS in the young age is a rare but malignant condition that can manifest with a spectrum of electrical abnormalities (i.e. SND, atrial tachycardias, AV block, and infra-nodal conduction delay) and result in the extreme cases in lethal arrhythmic events and SCD.


Assuntos
Síndrome de Brugada/complicações , Sistema de Condução Cardíaco/fisiopatologia , Potenciais de Ação , Adolescente , Adulto , Fatores Etários , Doenças Assintomáticas , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/fisiopatologia , Síndrome de Brugada/terapia , Criança , Pré-Escolar , Desfibriladores Implantáveis , Progressão da Doença , Intervalo Livre de Doença , Cardioversão Elétrica/instrumentação , Eletrocardiografia , Técnicas Eletrofisiológicas Cardíacas , Feminino , Predisposição Genética para Doença , Frequência Cardíaca , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Modelos de Riscos Proporcionais , Estudos Prospectivos , Sistema de Registros , Fatores de Risco , Fatores de Tempo , Adulto Jovem
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