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Article ; Online: Effect of chronic kidney disease in ischemic cardiomyopathy: Long-term follow-up - REVISION-DM2 trial.

Hueb, Thiago Ovanessian / Lima, Eduardo Gomes / Rocha, Mauricio S / Siqueira, Sergio F / Nishioka, Silvana Angelina Dório / Peixoto, Giselle L / Saccab, Marcos M / Garcia, Rosa Maria Rahmi / Ramires, José Antonio F / Kalil Filho, Roberto / Martinelli Filho, Martino

Medicine

2019 Volume 98, Issue 12, Page(s) e14692

Abstract: A strong association exists between chronic kidney disease (CKD) and coronary artery disease (CAD). The role of CKD in the long-term prognosis of CAD patients with versus those without CKD is unknown. This study investigated whether CKD affects ... ...

Abstract	A strong association exists between chronic kidney disease (CKD) and coronary artery disease (CAD). The role of CKD in the long-term prognosis of CAD patients with versus those without CKD is unknown. This study investigated whether CKD affects ventricular function.From January 2009 to January 2010, 918 consecutive patients were selected from an outpatient database. Patients had undergone percutaneous, surgical, or clinical treatment and were followed until May 2015.In patients with preserved renal function (n = 405), 73 events (18%) occurred, but 108 events (21.1%) occurred among those with CKD (n = 513) (P < .001). Regarding left ventricular ejection fraction (LVEF) <50%, we found 84 events (21.5%) in CKD patients and 12 (11.8%) in those with preserved renal function (P < .001). The presence of LVEF <50% brought about a modification effect. Death occurred in 22 (5.4%) patients with preserved renal function and in 73 (14.2%) with CKD (P < .001). In subjects with LVEF <50%, 66 deaths (16.9%) occurred in CKD patients and 7 (6.9%) in those with preserved renal function (P = .001). No differences were found in CKD strata regarding events or overall death among those with preserved LVEF. In a multivariate model, creatinine clearance remained an independent predictor of death (P < .001).We found no deleterious effects of CKD in patients with CAD when ventricular function was preserved. However, there was a worse prognosis in patients with CKD and ventricular dysfunction.Resgistry number is ISRCTN17786790 at https://doi.org/10.1186/ISRCTN17786790.
MeSH term(s)	Aged ; Cardiovascular Agents/therapeutic use ; Coronary Artery Disease/epidemiology ; Coronary Artery Disease/mortality ; Coronary Artery Disease/physiopathology ; Coronary Artery Disease/therapy ; Follow-Up Studies ; Heart Function Tests ; Humans ; Kaplan-Meier Estimate ; Kidney Function Tests ; Middle Aged ; Percutaneous Coronary Intervention/methods ; Renal Insufficiency, Chronic/epidemiology ; Renal Insufficiency, Chronic/physiopathology ; Reoperation/statistics & numerical data ; Smoking/epidemiology ; Socioeconomic Factors
Chemical Substances	Cardiovascular Agents
Language	English
Publishing date	2019-03-21
Publishing country	United States
Document type	Journal Article ; Observational Study
ZDB-ID	80184-7
ISSN	1536-5964 ; 0025-7974
ISSN (online)	1536-5964
ISSN	0025-7974
DOI	10.1097/MD.0000000000014692
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Article: Impact of diabetes mellitus on ischemic cardiomyopathy. Five-year follow-up. REVISION-DM trial.

Hueb, Thiago / Rocha, Mauricio S / Siqueira, Sergio F / Nishioka, Silvana Angelina D Orio / Peixoto, Giselle L / Saccab, Marcos M / Lima, Eduardo Gomes / Garcia, Rosa Maria Rahmi / Ramires, José Antonio F / Kalil Filho, Roberto / Martinelli Filho, Martino

Diabetology & metabolic syndrome

2018 Volume 10, Page(s) 19

Abstract: Background: Patients with ischemic cardiomyopathy and severe left ventricular dysfunction have a worse survival prognosis than patients with preserved ventricular function. The role of diabetes in the long-term prognosis of this patient group is unknown. ...

Abstract	Background: Patients with ischemic cardiomyopathy and severe left ventricular dysfunction have a worse survival prognosis than patients with preserved ventricular function. The role of diabetes in the long-term prognosis of this patient group is unknown. This study investigated whether the presence of diabetes has a long-term impact on left ventricular function. Methods: Patients with coronary artery disease who underwent coronary artery bypass graft surgery, percutaneous coronary intervention, or medical therapy alone were included. All patients had multivessel disease and left ventricular ejection fraction measurements. Overall mortality, nonfatal myocardial infarction, stroke, and additional interventions were investigated. Results: From January 2009 to January 2010, 918 consecutive patients were selected and followed until May 2015. They were separated into 4 groups: G1, 266 patients with diabetes and ventricular dysfunction; G2, 213 patients with diabetes without ventricular dysfunction; G3, 213 patients without diabetes and ventricular dysfunction; and G4, 226 patients without diabetes but with ventricular dysfunction. Groups 1, 2, 3, and 4, respectively, had a mortality rate of 21.6, 6.1, 4.2, and 10.6% ( Conclusion: In this sample, regardless of the treatment previously received patients with or without diabetes and preserved ventricular function experienced similar outcomes. However, patients with ventricular dysfunction had a worse prognosis compared with those with normal ventricular function; patients with diabetes had greater mortality than patients without diabetes.
Language	English
Publishing date	2018-03-15
Publishing country	England
Document type	Journal Article
ZDB-ID	2518786-7
ISSN	1758-5996
ISSN	1758-5996
DOI	10.1186/s13098-018-0320-y
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Article ; Online: Predictors of death in chronic Chagas cardiomyopathy patients with pacemaker.

Peixoto, Giselle de Lima / Martinelli Filho, Martino / Siqueira, Sérgio Freitas de / Nishioka, Silvana Angelina D'Ório / Pedrosa, Anísio Alexandre Andrade / Teixeira, Ricardo Alkmim / Costa, Roberto / Kalil Filho, Roberto / Ramires, José Antônio Franchini

International journal of cardiology

2018 Volume 250, Page(s) 260–265

Abstract: Background: Chronic Chagas cardiomyopathy (CCC) is the most serious and frequent manifestation of Chagas disease. Conduction abnormalities and bradycardia requiring pacemaker are common. The aim of this study was to determine the rate and predictors of ... ...

Abstract	Background: Chronic Chagas cardiomyopathy (CCC) is the most serious and frequent manifestation of Chagas disease. Conduction abnormalities and bradycardia requiring pacemaker are common. The aim of this study was to determine the rate and predictors of death in CCC patients with pacemaker. Methods: In this single-center prospective cohort study we assessed the outcome of 396 CCC patients with pacemaker, followed-up for at least 24months. All patients underwent a clinical and device assessment, 12-lead electrocardiography and echocardiography. Results: During the median follow-up of 1.9years (Interquartile range 1.6-2.4), there were 65 (16.4%) deaths, yielding an annual mortality rate of 8.6%. The major cause was sudden death (33.8%), followed by heart failure (HF), 32.3%. All the investigated variables were examined as potential predictors of death. The final multivariate logistic regression model included five independent variables: advanced HF functional class (OR [odds ratio] 6.71; 95% confidence interval [95% CI] 1.95-23.2; P=0.003), renal disease (OR 5.71; 95% CI 1.80-18.0; P=0.003), QRS ≥150ms (OR 2.80; 95% CI 1.08-7.27; P=0.034), left atrial enlargement (OR 2.75; 95% CI 1.09-6.95; P=0.032) and left ventricular ejection fraction ≤43% (OR 2.31; 95% CI 1.07-4.97; P=0.032). The model had good discrimination, confirmed by bootstrap validation (optimism-adjusted c-statistic of 0.78) and the calibration curve showed a proper calibration (slope=0.972). Conclusions: CCC patients with pacemaker have a high annual mortality rate despite that the pacemaker related variables were not predictors of death. The independent predictors of death can help us to identify the poor prognosis patients.
MeSH term(s)	Aged ; Chagas Cardiomyopathy/diagnosis ; Chagas Cardiomyopathy/mortality ; Chronic Disease ; Cohort Studies ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Mortality/trends ; Pacemaker, Artificial/trends ; Predictive Value of Tests ; Prospective Studies
Language	English
Publishing date	2018-01-01
Publishing country	Netherlands
Document type	Journal Article
ZDB-ID	779519-1
ISSN	1874-1754 ; 0167-5273
ISSN (online)	1874-1754
ISSN	0167-5273
DOI	10.1016/j.ijcard.2017.10.031
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Article ; Online: A cohort study of cardiac resynchronization therapy in patients with chronic Chagas cardiomyopathy.

Martinelli Filho, Martino / de Lima Peixoto, Giselle / de Siqueira, Sérgio Freitas / Martins, Sérgio Augusto Mezzalira / Nishioka, Silvana Angelina D'ório / Pedrosa, Anísio Alexandre Andrade / Teixeira, Ricardo Alkmim / Dos Santos, Johnny Xavier / Costa, Roberto / Kalil Filho, Roberto / Ramires, José Antônio Franchini

Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology

2018 Volume 20, Issue 11, Page(s) 1813–1818

Abstract: Aims: Cardiac resynchronization therapy (CRT) is an established procedure for patients with heart failure. However, trials evaluating its efficacy did not include patients with chronic Chagas cardiomyopathy (CCC). We aimed to assess the role of CRT in a ...

Abstract	Aims: Cardiac resynchronization therapy (CRT) is an established procedure for patients with heart failure. However, trials evaluating its efficacy did not include patients with chronic Chagas cardiomyopathy (CCC). We aimed to assess the role of CRT in a cohort of patients with CCC. Methods and results: This retrospective study compared the outcomes of CCC patients who underwent CRT with those of dilated (DCM) and ischaemic cardiomyopathies (ICM). The primary endpoint was all-cause mortality and the secondary endpoints were the rate of non-advanced New York Heart Association (NYHA) class 12 months after CRT and echocardiographic changes evaluated at least 6 months after CRT. There were 115 patients in the CCC group, 177 with DCM, and 134 with ICM. The annual mortality rates were 25.4%, 10.4%, and 11.3%, respectively (P < 0.001). Multivariate analysis adjusted for potential confounders showed that the CCC group had a two-fold [hazard ratio 2.34 (1.47-3.71), P < 0.001] higher risk of death compared to the DCM group. The rate of non-advanced NYHA class 12 months after CRT was significantly higher in non-CCC groups than in the CCC group (DCM 74.0% vs. ICM 73.9% vs. 56.5%, P < 0.001). Chronic Chagas cardiomyopathy and ICM patients had no improvement in the echocardiographic evaluation, but patients in the DCM group had an increase in left ventricular ejection fraction and a decrease in left ventricular end-diastolic diameter. Conclusion: This study showed that CCC patients submitted to CRT have worse prognosis compared to patients with DCM and ICM who undergo CRT. Studies comparing CCC patients with and without CRT are warranted.
MeSH term(s)	Brazil/epidemiology ; Cardiac Resynchronization Therapy/adverse effects ; Cardiac Resynchronization Therapy/methods ; Cardiomyopathy, Dilated/diagnosis ; Cardiomyopathy, Dilated/mortality ; Cardiomyopathy, Dilated/physiopathology ; Cardiomyopathy, Dilated/therapy ; Chagas Cardiomyopathy/diagnosis ; Chagas Cardiomyopathy/mortality ; Chagas Cardiomyopathy/physiopathology ; Chagas Cardiomyopathy/therapy ; Defibrillators, Implantable ; Echocardiography/methods ; Female ; Heart Failure/etiology ; Heart Failure/mortality ; Heart Failure/therapy ; Humans ; Male ; Middle Aged ; Prognosis ; Stroke Volume
Language	English
Publishing date	2018-03-05
Publishing country	England
Document type	Journal Article
ZDB-ID	1449879-0
ISSN	1532-2092 ; 1099-5129
ISSN (online)	1532-2092
ISSN	1099-5129
DOI	10.1093/europace/eux375
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Article ; Online: Global and regional ventricular repolarization study by body surface potential mapping in patients with left bundle-branch block and heart failure undergoing cardiac resynchronization therapy.

Douglas, Roberto A G / Samesima, Nelson / Filho, Martino M / Pedrosa, Anísio A / Nishioka, Silvana A D / Pastore, Carlos A

Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc

2012 Volume 17, Issue 2, Page(s) 123–129

Abstract: Background: The controversial effects promoted by cardiac resynchronization therapy (CRT) on the ventricular repolarization (VR) have motivated VR evaluation by body surface potential mapping (BSPM) in CRT patients.: Methods: Fifty-two CRT patients, ... ...

Abstract	Background: The controversial effects promoted by cardiac resynchronization therapy (CRT) on the ventricular repolarization (VR) have motivated VR evaluation by body surface potential mapping (BSPM) in CRT patients. Methods: Fifty-two CRT patients, mean age 58.8 ± 12.3 years, 31 male, LVEF 27.5 ± 9.2, NYHA III-IV heart failure with QRS181.5 ± 14.2 ms, underwent 87-lead BSPM in sinus rhythm (BASELINE) and biventricular pacing (BIV). Measurements of mean and corrected QT intervals and dispersion, mean and corrected T peak end intervals and their dispersion, and JT intervals characterized global and regional (RV, Intermediate, and LV regions) ventricular repolarization response. Results: Global QTm (P < 0.001) and QTc(m) (P < 0.05) were decreased in BIV; QTm was similar across regions in both modes (P = ns); QTc(m) values were lower in RV/LV than in Intermediate region in BASELINE and BIV (P < 0.001); only RV/Septum showed a significant difference (P < 0.01) in the BIV mode. QTD values both of BASELINE (P < 0.01) and BIV (P < 0.001) were greater in the Intermediate than in the LV region. CRT effect significantly reduced global/regional QTm and QTc(m) values. QTD was globally decreased in RV/LV (Intermediate: P = ns). BIV mode significantly reduced global T peak end mean and corrected intervals and their dispersion. JT values were not significant. Conclusions: Ventricular repolarization parameters QTm, QTc(m), and QTD global/regional values, as assessed by BSPM, were reduced in patients under CRT with severe HF and LBBB. Greater recovery impairment in the Intermediate region was detected by the smaller variation of its dispersion.
MeSH term(s)	Analysis of Variance ; Body Surface Potential Mapping ; Bundle-Branch Block/physiopathology ; Bundle-Branch Block/therapy ; Cardiac Resynchronization Therapy/methods ; Female ; Heart Failure/physiopathology ; Heart Failure/therapy ; Humans ; Male ; Middle Aged ; Treatment Outcome
Language	English
Publishing date	2012-05-18
Publishing country	United States
Document type	Journal Article
ZDB-ID	1325530-7
ISSN	1542-474X ; 1082-720X
ISSN (online)	1542-474X
ISSN	1082-720X
DOI	10.1111/j.1542-474X.2012.00500.x
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Zs.A 4953: Show issues

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Article ; Online: Brazilian Guidelines for Cardiac Implantable Electronic Devices - 2023.

Teixeira, Ricardo Alkmim / Fagundes, Alexsandro Alves / Baggio Junior, José Mário / Oliveira, Júlio César de / Medeiros, Paulo de Tarso Jorge / Valdigem, Bruno Pereira / Teno, Luiz Antônio Castilho / Silva, Rodrigo Tavares / Melo, Celso Salgado de / Elias Neto, Jorge / Moraes Júnior, Antonio Vitor / Pedrosa, Anisio Alexandre Andrade / Porto, Fernando Mello / Brito Júnior, Hélio Lima de / Souza, Thiago Gonçalves Schroder E / Mateos, José Carlos Pachón / Moraes, Luis Gustavo Belo de / Forno, Alexander Romeno Janner Dal / D'Avila, Andre Luiz Buchele /

Cavaco, Diogo Alberto de Magalhães / Kuniyoshi, Ricardo Ryoshim / Pimentel, Mauricio / Camanho, Luiz Eduardo Montenegro / Saad, Eduardo Benchimol / Zimerman, Leandro Ioschpe / Oliveira, Eduardo Bartholomay / Scanavacca, Mauricio Ibrahim / Martinelli Filho, Martino / Lima, Carlos Eduardo Batista de / Peixoto, Giselle de Lima / Darrieux, Francisco Carlos da Costa / Duarte, Jussara de Oliveira Pinheiro / Galvão Filho, Silas Dos Santos / Costa, Eduardo Rodrigues Bento / Mateo, Enrique Indalécio Pachón / Melo, Sissy Lara De / Rodrigues, Thiago da Rocha / Rocha, Eduardo Arrais / Hachul, Denise Tessariol / Lorga Filho, Adalberto Menezes / Nishioka, Silvana Angelina D'Orio / Gadelha, Eduardo Barreto / Costa, Roberto / Andrade, Veridiana Silva de / Torres, Gustavo Gomes / Oliveira Neto, Nestor Rodrigues de / Lucchese, Fernando Antonio / Murad, Henrique / Wanderley Neto, José / Brofman, Paulo Roberto Slud / Almeida, Rui M S / Leal, João Carlos Ferreira

Arquivos brasileiros de cardiologia

2023 Volume 120, Issue 1, Page(s) e20220892

Title translation	Diretriz Brasileira de Dispositivos Cardíacos Eletrônicos Implantáveis – 2023.
MeSH term(s)	Humans ; Brazil ; Pacemaker, Artificial ; Defibrillators, Implantable ; Heart ; Cardiac Resynchronization Therapy Devices
Language	Portuguese
Publishing date	2023-01-23
Publishing country	Brazil
Document type	Journal Article
ZDB-ID	730261-7
ISSN	1678-4170 ; 0066-782X
ISSN (online)	1678-4170
ISSN	0066-782X
DOI	10.36660/abc.20220892
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Article ; Online: "Hyper-response" evaluated by 3D echocardiography after cardiac resynchronization therapy.

Hotta, Viviane Tiemi / Vieira, Marcelo Luiz Campos / Rassi, Daniela do Carmo / Nishioka, Silvana Angelina D'orio / Martinelli Filho, Martino / Mathias, Wilson

Arquivos brasileiros de cardiologia

2011 Volume 96, Issue 6, Page(s) e119–22

Abstract: Cardiac resynchronization therapy consists of a promising treatment for patients with severe heart failure, but about 30% of patients do not exhibit clinical improvement with this procedure. However, approximately 10% of patients undergoing this therapy ... ...

Abstract	Cardiac resynchronization therapy consists of a promising treatment for patients with severe heart failure, but about 30% of patients do not exhibit clinical improvement with this procedure. However, approximately 10% of patients undergoing this therapy may have hyperresponsiveness, and three-dimensional echocardiography can provide an interesting option for the selection and evaluation of such patients.
MeSH term(s)	Cardiac Resynchronization Therapy/adverse effects ; Echocardiography, Three-Dimensional ; Female ; Heart Failure/diagnostic imaging ; Heart Failure/therapy ; Humans ; Middle Aged ; Recovery of Function
Language	Spanish
Publishing date	2011-07-25
Publishing country	Brazil
Document type	Case Reports ; Journal Article ; Video-Audio Media
ZDB-ID	730261-7
ISSN	1678-4170 ; 0066-782X
ISSN (online)	1678-4170
ISSN	0066-782X
DOI	10.1590/s0066-782x2011000600019
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Article ; Online: Evidence for cardiac safety and antiarrhythmic potential of chloroquine in systemic lupus erythematosus.

Teixeira, Ricardo Alkmim / Borba, Eduardo F / Pedrosa, Anísio / Nishioka, Silvana / Viana, Vilma S T / Ramires, José A / Kalil-Filho, Roberto / Bonfá, Eloísa / Martinelli Filho, Martino

2014 Volume 16, Issue 6, Page(s) 887–892

Abstract: Aims: To perform a comprehensive evaluation of heart rhythm disorders and the influence of disease/therapy factors in a large systemic lupus erythematosus (SLE) cohort.: Methods and results: Three hundred and seventeen consecutive patients of an ... ...

Abstract	Aims: To perform a comprehensive evaluation of heart rhythm disorders and the influence of disease/therapy factors in a large systemic lupus erythematosus (SLE) cohort. Methods and results: Three hundred and seventeen consecutive patients of an ongoing electronic database protocol were evaluated by resting electrocardiogram and 142 were randomly selected for 24 h Holter monitoring for arrhythmia and conduction disturbances. The mean age was 40.2 ± 12.1 years and disease duration was 11.4 ± 8.1 years. Chloroquine (CQ) therapy was identified in 69.7% with a mean use of 8.5 ± 6.7 years. Electrocardiogram abnormalities were detected in 66 patients (20.8%): prolonged QTc/QTd (14.2%); bundle-branch block (2.5%); and atrioventricular block (AVB) (1.6%). Age was associated with AVB (P = 0.029) and prolonged QTc/QTd (P = 0.039) whereas anti-Ro/SS-A and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores were not (P > 0.05). Chloroquine was negatively associated with AVB (P = 0.01) as was its longer use (6.1 ± 6.9 vs. 1.0 ± 2.5 years, P = 0.018). Time of CQ use was related with the absence of AVB [odds ratio (OR) = 0.103; 95% confidence interval (CI) = 0.011-0.934, P = 0.043] in multiple logistic regression. Holter monitoring revealed abnormalities in 121 patients (85.2%): supraventricular ectopies (63.4%) and tachyarrhythmia (18.3%); ventricular ectopies (45.8%). Atrial tachycardia/fibrillation (AT/AF) were associated with shorter CQ duration (7.05 ± 7.99 vs. 3.63 ± 5.02 years, P = 0.043) with a trend to less CQ use (P = 0.054), and older age (P < 0.001). Predictors of AT/AF in multiple logistic regression were age (OR = 1.115; 95% CI = 1.059-1.174, P < 0.001) and anti-Ro/SS-A (OR = 0.172; 95% CI = 0.047-0.629, P = 0.008). Conclusions: Chloroquine seems to play a protective role in the unexpected high rate of cardiac arrhythmias and conduction disturbances observed in SLE. Further studies are necessary to determine if this antiarrhythmic effect is due to the disease control or a direct effect of the drug.
MeSH term(s)	Adult ; Anti-Arrhythmia Agents/therapeutic use ; Antirheumatic Agents/therapeutic use ; Arrhythmias, Cardiac/diagnosis ; Arrhythmias, Cardiac/epidemiology ; Arrhythmias, Cardiac/prevention & control ; Brazil/epidemiology ; Cardiotonic Agents/therapeutic use ; Causality ; Chloroquine/therapeutic use ; Comorbidity ; Electrocardiography/drug effects ; Electrocardiography/statistics & numerical data ; Feasibility Studies ; Female ; Humans ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/drug therapy ; Lupus Erythematosus, Systemic/epidemiology ; Male ; Off-Label Use ; Prevalence ; Retrospective Studies ; Risk Assessment ; Treatment Outcome
Chemical Substances	Anti-Arrhythmia Agents ; Antirheumatic Agents ; Cardiotonic Agents ; Chloroquine (886U3H6UFF)
Language	English
Publishing date	2014-06
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1449879-0
ISSN	1532-2092 ; 1099-5129
ISSN (online)	1532-2092
ISSN	1099-5129
DOI	10.1093/europace/eut290
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Article: Distrofia miotônica e cardiopatia: comportamento dos eventos arrítmicos e dos distúrbios da condução.

Nishioka, Silvana Angelina D'Orio / Martinelli Filho, Martino / Marie, Suely / Zatz, Mayana / Costa, Roberto

Arquivos brasileiros de cardiologia

2005 Volume 84, Issue 4, Page(s) 330–336

Abstract: Objective: To study the prevalence and natural evolution of arrhythmic events and conduction disturbances in myotonic dystrophy; to correlate the genetic defect with cardiovascular findings; to assess cardiac mortality, frequency, and predictive factors ...

Title translation	Myotonic dystrophy and heart disease: behavior of arrhythmic events and conduction disturbances.
Abstract	Objective: To study the prevalence and natural evolution of arrhythmic events and conduction disturbances in myotonic dystrophy; to correlate the genetic defect with cardiovascular findings; to assess cardiac mortality, frequency, and predictive factors of sudden death; to correlate the severity of the neuromuscular and cardiac involvement; and to define the role of the electrophysiological study (EPS), in myotonic dystrophy. Methods: Periodic clinical assessment and the following tests were performed in 83 consecutive patients with a mean follow-up of 42+/-30.63 months: complementary examinations, genetic tests, electrocardiography, echocardiography, and Holter; electrophysiological study was performed in 59 cases. Results: Atrial tachyarrhythmia was observed in 10 (12%) patients, NSVT in 14 (17%), first-degree AVB in 24 (29%), LBBB in 19 (23%), and RBBB in 13 (16%). Symptoms, an increase in the PR interval, QRS enlargement, LVEF < 60%, and age were predictive factors of death. Nine patients died (4 sudden deaths; 2 due to heart failure; 3 due to other causes). Electrophysiological study: H-V interval > 70 ms in 34% and > 100 ms in 11% (postprocainamide). Conclusion: The prevalence of arrhythmic events and conduction disturbances ranged from 50% to 80% after 6 years, and did not correlate with the genetic defect. Atrial flutter was the most common sustained arrhythmia. Cardiac involvement increased as the neuromuscular disease became aggravated, but progression of the cardiac involvement was more rapid than that of the neuromuscular disease. Overall mortality was low (11%) and sudden death occurred in half of the cases. The EPS identified a group at risk for pacemaker implantation.
MeSH term(s)	Adolescent ; Adult ; Brazil/epidemiology ; Child ; Death, Sudden, Cardiac/epidemiology ; Death, Sudden, Cardiac/etiology ; Echocardiography ; Electrocardiography ; Female ; Follow-Up Studies ; Heart Block/etiology ; Heart Diseases/epidemiology ; Heart Diseases/etiology ; Humans ; Male ; Middle Aged ; Myotonic Dystrophy/complications ; Prevalence ; Severity of Illness Index ; Tachycardia/epidemiology ; Tachycardia/etiology
Language	Portuguese
Publishing date	2005-05-02
Publishing country	Brazil
Document type	English Abstract ; Journal Article
ZDB-ID	730261-7
ISSN	0066-782X
ISSN	0066-782X
DOI	10.1590/s0066-782x2005000400011
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Article: Is isolated congenital heart block associated to neonatal lupus requiring pacemaker a distinct cardiac syndrome?

Cruz, Rosana B P / Viana, Vilma S T / Nishioka, Silvana A D / Martinelli-F, Martino / Bonfa, Eloisa

Pacing and clinical electrophysiology : PACE

2004 Volume 27, Issue 5, Page(s) 615–620

Abstract: Isolated congenital heart block (ICHB) is frequently associated with neonatal lupus syndrome (NLS). Therefore few data are available regarding the long-term cardiac outcome of newborns with ICHB and the pathogenic mechanisms are not yet defined. In order ...

Abstract	Isolated congenital heart block (ICHB) is frequently associated with neonatal lupus syndrome (NLS). Therefore few data are available regarding the long-term cardiac outcome of newborns with ICHB and the pathogenic mechanisms are not yet defined. In order to compare demographic features and cardiological outcome of patients with ICHB submitted to pacemaker (PM) implantation with and without NLS, forty ICHB patients were evaluated pre- and post-PM implantation, by clinical, electrocardiogram, Holter Monitoring, treadmill test, and electrophysiological study. According to the presence of antibodies to 52 and 60 kDa Ro/SSA and La/SSB proteins in mother's sera, it was found that 60% (24/40) of patients had ICHB associated to NLS (ICHB/NL+). Twenty-three of 24 ICHB/NL+ patients were asymptomatic, and 16 (67%) were female (P = 0.013). The frequency of syncope, mitral insufficiency (MI), and congestive heart failure (CHF) was similar pre-PM implantation in both ICHB/NL+ and ICHB/NL- groups (P > 0.05). After PM implantation, MI and CHF were only observed in ICHB/NL+ patients, although not statistically significant. Interestingly, 67% of ICHB/NL+ were noticed before one year of age while only one fourth of ICHB/NL- was diagnosed in this period (P = 0.024). Almost half (46%) of ICHB/NL+ patients required PMs in the first 24 months of life, whereas only one in the ICHB/NL- received a PM at the same age (P = 0.02). In ICHB patients requiring PM implantation, the antibody-mediated lesion seems to be associated with an earlier onset and a more severe heart disease, in spite of the uniform criteria for PM indication.
MeSH term(s)	Adolescent ; Adult ; Cardiac Pacing, Artificial ; Chi-Square Distribution ; Child ; Child, Preschool ; Electrocardiography/methods ; Electrophysiologic Techniques, Cardiac ; Exercise Test ; Female ; Heart Block/congenital ; Heart Block/therapy ; Humans ; Infant ; Lupus Erythematosus, Systemic/congenital ; Male ; Middle Aged ; Syndrome
Language	English
Publishing date	2004-05
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	424437-0
ISSN	1540-8159 ; 0147-8389
ISSN (online)	1540-8159
ISSN	0147-8389
DOI	10.1111/j.1540-8159.2004.00495.x
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