Flutter Atrial na Síndrome do PRKAG2: Características Clínicas e Eletrofisiológicas / Atrial Flutter in PRKAG2 Syndrome: Clinical and Electrophysiological Characteristics

Resumo Fundamento A síndrome do PRKAG2 é uma rara doença genética autossômico dominante, fenocópia da miocardiopatia hipertrófica, caracterizada pelo acúmulo intracelular de glicogênio. Manifestações clínicas incluem pré-excitação ventricular, hipertrofia ventricular, distúrbio de condução cardíaca e arritmias atriais. Objetivo Comparar características clínicas e eletrofisiológicas observadas em pacientes com flutter atrial, com e sem síndrome do PRKAG2. Métodos Estudo observacional, comparativo de pacientes com flutter atrial: grupo A, cinco pacientes de família com síndrome do PRKAG2; e grupo B, 25 pacientes sem fenótipo da síndrome. O nível de significância foi de 5%. Resultados Todos os pacientes do grupo A apresentaram pré-excitação ventricular e bloqueio de ramo direito; quatro tinham marca-passo (80%). Pacientes do grupo A tinham menor idade (39±5,4 vs. 58,6±17,6 anos, p=0,021), e maior espessura de septo interventricular (mediana=18 vs. 10 mm; p<0,001) e parede posterior (mediana=14 vs. 10 mm; p=0,001). Quatro do grupo A foram submetidos a estudo eletrofisiológico, sendo observada via acessória fascículo-ventricular; em três foi realizada ablação do flutter atrial. Todos os do grupo B foram submetidos à ablação do flutter atrial, sem evidência de via acessória. Observado maior prevalência no grupo B de hipertensão arterial, diabetes mellitus, doença coronariana e apneia do sono, sem diferença estatisticamente significante. Conclusão Portadores da síndrome do PRKAG2 apresentaram flutter atrial em idade mais precoce, e menos comorbidades, quando comparados a pacientes com flutter atrial sem fenótipo da mutação. Importante suspeitar de miocardiopatia geneticamente determinada, como síndrome do PRKAG2, em jovens com flutter atrial, especialmente na presença de pré-excitação ventricular e hipertrofia ventricular familiar.

Abstract Background PRKAG2 syndrome is a rare autosomal dominant disease, a phenocopy of hypertrophic cardiomyopathy characterized by intracellular glycogen accumulation. Clinical manifestations include ventricular preexcitation, cardiac conduction disorder, ventricular hypertrophy, and atrial arrhythmias. Objective To compare the clinical and electrophysiological characteristics observed in patients with atrial flutter, with and without PRKAG2 syndrome. Methods An observational study comparing patients with atrial flutter: group A consisted of five patients with PRKAG2 syndrome from a family, and group B consisted of 25 patients without phenotype of PRKAG2 syndrome. The level of significance was 5%. Results All patients in group A had ventricular preexcitation and right branch block, and four had pacemakers (80%). Patients in group A were younger (39±5.4 vs 58.6±17.6 years, p=0.021), had greater interventricular septum (median=18 vs 10 mm; p<0.001) and posterior wall thickness (median=14 vs 10 mm; p=0.001). In group A, four patients were submitted to an electrophysiological study, showing a fasciculoventricular pathway, and atrial flutter ablation was performed in tree. All patients in group B were submitted to ablation of atrial flutter, with no evidence of accessory pathway. Group B had a higher prevalence of hypertension, diabetes mellitus, coronary artery disease and sleep apnea, with no statistically significant difference. Conclusion Patients with PRKAG2 syndrome presented atrial flutter at an earlier age and had fewer comorbidities when compared to patients with atrial flutter without mutation phenotype. The occurrence of atrial flutter in young individuals, especially in the presence of ventricular preexcitation and familial ventricular hypertrophy, should raise the suspicion of PRKAG2 syndrome.

Atrial Flutter in PRKAG2 Syndrome: Clinical and Electrophysiological Characteristics. / Flutter Atrial na Síndrome do PRKAG2: Características Clínicas e Eletrofisiológicas.

BACKGROUND: PRKAG2 syndrome is a rare autosomal dominant disease, a phenocopy of hypertrophic cardiomyopathy characterized by intracellular glycogen accumulation. Clinical manifestations include ventricular preexcitation, cardiac conduction disorder, ventricular hypertrophy, and atrial arrhythmias. OBJECTIVE: To compare the clinical and electrophysiological characteristics observed in patients with atrial flutter, with and without PRKAG2 syndrome. METHODS: An observational study comparing patients with atrial flutter: group A consisted of five patients with PRKAG2 syndrome from a family, and group B consisted of 25 patients without phenotype of PRKAG2 syndrome. The level of significance was 5%. RESULTS: All patients in group A had ventricular preexcitation and right branch block, and four had pacemakers (80%). Patients in group A were younger (39±5.4 vs 58.6±17.6 years, p=0.021), had greater interventricular septum (median=18 vs 10 mm; p<0.001) and posterior wall thickness (median=14 vs 10 mm; p=0.001). In group A, four patients were submitted to an electrophysiological study, showing a fasciculoventricular pathway, and atrial flutter ablation was performed in tree. All patients in group B were submitted to ablation of atrial flutter, with no evidence of accessory pathway. Group B had a higher prevalence of hypertension, diabetes mellitus, coronary artery disease and sleep apnea, with no statistically significant difference. CONCLUSION: Patients with PRKAG2 syndrome presented atrial flutter at an earlier age and had fewer comorbidities when compared to patients with atrial flutter without mutation phenotype. The occurrence of atrial flutter in young individuals, especially in the presence of ventricular preexcitation and familial ventricular hypertrophy, should raise the suspicion of PRKAG2 syndrome.

FUNDAMENTO: A síndrome do PRKAG2 é uma rara doença genética autossômico dominante, fenocópia da miocardiopatia hipertrófica, caracterizada pelo acúmulo intracelular de glicogênio. Manifestações clínicas incluem pré-excitação ventricular, hipertrofia ventricular, distúrbio de condução cardíaca e arritmias atriais. OBJETIVO: Comparar características clínicas e eletrofisiológicas observadas em pacientes com flutter atrial, com e sem síndrome do PRKAG2. MÉTODOS: Estudo observacional, comparativo de pacientes com flutter atrial: grupo A, cinco pacientes de família com síndrome do PRKAG2; e grupo B, 25 pacientes sem fenótipo da síndrome. O nível de significância foi de 5%. RESULTADOS: Todos os pacientes do grupo A apresentaram pré-excitação ventricular e bloqueio de ramo direito; quatro tinham marca-passo (80%). Pacientes do grupo A tinham menor idade (39±5,4 vs. 58,6±17,6 anos, p=0,021), e maior espessura de septo interventricular (mediana=18 vs. 10 mm; p<0,001) e parede posterior (mediana=14 vs. 10 mm; p=0,001). Quatro do grupo A foram submetidos a estudo eletrofisiológico, sendo observada via acessória fascículo-ventricular; em três foi realizada ablação do flutter atrial. Todos os do grupo B foram submetidos à ablação do flutter atrial, sem evidência de via acessória. Observado maior prevalência no grupo B de hipertensão arterial, diabetes mellitus, doença coronariana e apneia do sono, sem diferença estatisticamente significante. CONCLUSÃO: Portadores da síndrome do PRKAG2 apresentaram flutter atrial em idade mais precoce, e menos comorbidades, quando comparados a pacientes com flutter atrial sem fenótipo da mutação. Importante suspeitar de miocardiopatia geneticamente determinada, como síndrome do PRKAG2, em jovens com flutter atrial, especialmente na presença de pré-excitação ventricular e hipertrofia ventricular familiar.

Prevalence and prognostic value of ventricular dyssynchrony in Chagas cardiomyopathy.

BACKGROUND: Chagas cardiomyopathy is one important cause of heart failure in Latin America. Ventricular dyssynchrony may be a factor of decompensation in the course of this disease, but there are no data on its prevalence and its main prognostic implications yet. OBJECTIVE: Describe prevalence and prognostic value of ventricular dyssynchrony in Chagas cardiomyopathy. METHODS: 56 patients with Chagas cardiomyopathy were consecutively selected by two positive serologies and an ejection fraction < 45% in the echocardiogram. The echocardiogram evaluated the presence of intraventricular dyssynchrony using 3 criteria and interventricular dyssynchrony using 1 criterion. Patients were followed for 21 ± 14 months and cardiac events were defined as the combination of death and hospitalization. RESULTS: The average age of the population was 56 ± 10 years, 50% males. Mean ejection fraction was 30 ± 8% and 87% presented functional class I/II (NYHA). The prevalence of interventricular dyssynchrony was 34% (95% CI: 22%-48%) and intraventricular dyssynchrony had a prevalence of 85% (95% CI: 75%-93%). The prevalence of intraventricular dyssynchrony was similar among patients with QRS duration < 0.12 s or > 0.12 s (85% and 89%, respectively, p = 0.66). Twenty events were recorded. The incidence of combined events was similar in patients with or without intraventricular dyssynchrony (35% versus 38%, p = 0.9) and those with or without interventricular dyssynchrony (39% versus 34%, p = 0.73). CONCLUSION: Patients with Chagas cardiomyopathy have high intraventricular and moderate interventricular prevalence of dyssynchrony. The high prevalence is independent from the QRS width. The ventricular dyssynchrony does not have any prognostic value in patients with Chagas cardiomyopathy.

Prevalência e valor prognóstico da dissincronia ventricular na cardiomiopatia chagásica / Prevalence and prognostic value of ventricular dyssynchrony in chagas cardiomyopathy / Prevalencia y valor pronóstico de la disincronía ventricular en la miocardiopatía chagásic

FUNDAMENTO: A cardiomiopatia chagásica é uma causa importante de insuficiência cardíaca na América Latina. A dissincronia ventricular pode ser um fator de descompensação no curso da doença, mas não há dados sobre sua prevalência e principais implicações prognósticas, ainda. OBJETIVO: Descrever a prevalência e valores prognósticos da dissincronia ventricular na cardiomiopatia chagásica. MÉTODOS: 56 pacientes com cardiomiopatia chagásica foram selecionados consecutivamente através de dois testes sorológicos positivos e uma fração de ejeção (FE) < 45 por cento no ecocardiograma. O ecocardiograma avaliou a presença de dissincronia intraventricular utilizando três critérios e dissincronia interventricular utilizando 1 critério. Os pacientes foram seguidos por 21 ± 14 meses e os eventos cardíacos foram definidos como a combinação de morte e hospitalização. RESULTADOS: A média da idade da população era de 56 ± 10 anos, sendo 50 por cento do sexo masculino. A FE média era de 30 ± 8 por cento e 87 por cento dos pacientes apresentavam classe funcional I/II (NYHA). A prevalência de dissincronia interventricular era de 34 por cento (IC95 por cento: 22 por cento-48 por cento) e a dissincronia intraventricular tinha uma prevalência de 85 por cento (IC95 por cento: 75 por cento-93 por cento). A prevalência de dissincronia intraventricular era similar em pacientes com duração do QRS < 0,12 s ou > 0,12 s (85 por cento e 89 por cento, respectivamente, p = 0,66). Vinte eventos foram registrados. A incidência de eventos combinados foi similar em pacientes com ou sem dissincronia intraventricular (35 por cento versus 38 por cento, p = 0,9) e aqueles com ou sem dissincronia interventricular (39 por cento versus 34 por cento, p = 0,73). CONCLUSÃO: Pacientes com cardiomiopatia chagásica apresentam alta prevalência de dissincronia intraventricular e moderada prevalência de dissincronia interventricular. A alta prevalência independe da largura do QRS. A dissincronia ventricular não tem qualquer valor prognóstico em pacientes com cardiomiopatia chagásica.

BACKGROUND: Chagas cardiomyopathy is one important cause of heart failure in Latin America. Ventricular dyssynchrony may be a factor of decompensation in the course of this disease, but there are no data on its prevalence and its main prognostic implications yet. OBJECTIVE: Describe prevalence and prognostic value of ventricular dyssynchrony in Chagas cardiomyopathy. METHODS: 56 patients with Chagas cardiomyopathy were consecutively selected by two positive serologies and an ejection fraction < 45 percent in the echocardiogram. The echocardiogram evaluated the presence of intraventricular dyssynchrony using 3 criteria and interventricular dyssynchrony using 1 criterion. Patients were followed for 21 ± 14 months and cardiac events were defined as the combination of death and hospitalization. RESULTS: The average age of the population was 56 ± 10 years, 50 percent males. Mean ejection fraction was 30 ± 8 percent and 87 percent presented functional class I/II (NYHA). The prevalence of interventricular dyssynchrony was 34 percent (95 percent CI: 22 percent-48 percent) and intraventricular dyssynchrony had a prevalence of 85 percent (95 percent CI: 75 percent-93 percent). The prevalence of intraventricular dyssynchrony was similar among patients with QRS duration < 0.12 s or > 0.12 s (85 percent and 89 percent, respectively, p = 0.66). Twenty events were recorded. The incidence of combined events was similar in patients with or without intraventricular dyssynchrony (35 percent versus 38 percent, p = 0.9) and those with or without interventricular dyssynchrony (39 percent versus 34 percent, p = 0.73). CONCLUSION: Patients with Chagas cardiomyopathy have high intraventricular and moderate interventricular prevalence of dyssynchrony. The high prevalence is independent from the QRS width. The ventricular dyssynchrony does not have any prognostic value in patients with Chagas cardiomyopathy.

FUNDAMENTO: La miocardiopatía chagásica es una causa importante de insuficiencia cardíaca en América Latina. La disincronía ventricular puede ser un factor de descompensación en el curso de la enfermedad, pero todavía no hay datos sobre su prevalencia e implicaciones pronósticas importantes. OBJETIVO:Describir la prevalencia y valores pronósticos de la disincronía ventricular en la miocardiopatía chagásica. MÉTODOS:56 pacientes con cardiomiopatía chagásica se seleccionaron de forma consecutiva en dos pruebas serológicas positivas y una fracción de eyección (FE) < 45 por ciento en el ecocardiograma. El ecocardiograma evaluó la presencia de disincronía intraventricular usando tres criterios y disincronía interventricular con un criterio. Los pacientes fueron seguidos durante 21 ± 14 meses, y los eventos cardíacos se definen como la combinación de muerte y hospitalización. RESULTADOS:La media de edad de la población fue de 56 ± 10 años, y el 50 por ciento hombres era del sexo masculino. La fracción de eyección (FE) media fue de 30 ± 8 por ciento y el 87 por ciento de los pacientes tenía clase funcional I/II (NYHA). La prevalencia de disincronía interventricular fue del 34 por ciento (IC 95 por ciento: 22 por ciento-48 por ciento) y la disincronía intraventricular tenía una prevalencia del 85 por ciento (IC 95 por ciento: 75 por ciento-93 por ciento). La prevalencia de disincronía intraventricular era similar en pacientes con duración del QRS < 0,12 s ó > 0,12 s (85 por ciento y 89 por ciento respectivamente, p = 0,66). Veinte eventos se registraron. La incidencia de eventos combinados fue similar en pacientes con o sin la disincronía intraventricular (35 por ciento versus 38 por ciento, p = 0,9) y aquellos con o sin la disincronía interventricular (39 por ciento versus 34 por ciento, p = 0,73). CONCLUSIÓN:Pacientes con miocardiopatía chagásica tienen alta prevalencia de disincronía intraventricular y moderada prevalencia de disincronía interventricular. La alta prevalencia independiente de la anchura del QRS. La disincronía ventricular no tiene algún valor pronóstico en pacientes con miocardiopatía chagásica.

Perfuração tardia de ventrículo direito em portador de cardioversor desfibrilador implantável / Delayed right ventricular perforation in patient with implantable cardioverter - defibrillator / Perforación tardía de VD en portador de DCI

Descrevemos o caso de uma paciente de 62 anos que retornou para avaliação nove meses após implante de cardioversor desfibrilador implantável (CDI) com sinais de perfuração tardia do ventrículo direito. São discutidos os sinais clínicos que permitem o diagnóstico dessa apresentação tardia, assim como as condutas e a frequência dessa complicação na literatura.

We describe the case of a 62-year-old patient who returned for evaluation nine months after receiving an implantable cardioverter-defibrillator (ICD) with signs of delayed right ventricular (RV) perforation. The clinical signs that allowed the diagnosis of this late presentation to be achieved are discussed herein, as well as the conduct and the frequency of this complication in the literature.

Describimos un caso de una mujer de 62 años que regresó para evaluación nueve meses después de implantación de un desfibrilador cardiaco implantable (DCI) con signos de perforación tardía del ventrículo derecho. Se discuten los signos clínicos que permitan el diagnóstico de esta presentación tardía, así como el comportamiento y la frecuencia de esta complicación en la literatura.

Delayed right ventricular perforation in patient with implantable cardioverter - defibrillator.

We describe the case of a 62-year-old patient who returned for evaluation nine months after receiving an implantable cardioverter-defibrillator (ICD) with signs of delayed right ventricular (RV) perforation. The clinical signs that allowed the diagnosis of this late presentation to be achieved are discussed herein, as well as the conduct and the frequency of this complication in the literature.

Atrial fibrillation ablation in Brazil: results of the registry of the Brazilian Society of Cardiac Arrhythmias.

BACKGROUND: Aiming to define the profile of curative atrial fibrillation (AF) ablation in Brazil, the Brazilian Cardiac Arrhythmia Society [Sociedade Brasileira de Arritmias Cardíacas] (SOBRAC) created the Brazilian Registry of AF Ablation [Registro Brasileiro de Ablação da FA]. OBJECTIVE: To describe the results of this registry. METHODS: A questionnaire was sent to SOBRAC members asking about data on patients submitted to AF ablation between September 2005 and November, 2006. RESULTS: A total of 29 groups from 13 states completed the forms. Of these, 22 (76%) had performed AF ablations. Between 1998 and 2001, 7 groups (32%) initiated AF ablations and between 2002 and 2006, 15 groups began to perform them (68%). From 1998 to 2006, 2,374 patients were submitted to ablation, 755 (32%) of them during the registry period. Most (70%) were males and 89% presented with paroxysmal or persistent AF. Ancillary imaging methods (intracardiac echocardiography and electroanatomic mapping) were used by 9 groups (41%). During an average five-month follow-up period, total success was 82% and success without use of antiarrhythmic agents was 57%. Nevertheless, 35% of the patients required two or more procedures. There were 111 complications (14.7%) and 2 deaths (0.26%). CONCLUSION: Curative AF ablation has been increasing significantly in our country, with success rates comparable to international indexes, but often more than one procedure is necessary. Despite promising results, AF ablation still results in significant morbidity. Supplementary imaging methods have been used more and more in an effort to increase efficacy and safety of the procedure. These findings should be considered by public and private funding agencies.

Ablação da fibrilação atrial no Brasil: resultados do registro da Sociedade Brasileira de Arritmias Cardíacas / Atrial fibrillation ablation in Brazil: results of the registry of the Brazilian Society of Cardiac Arrhythmias

FUNDAMENTO: Buscando delinear o perfil da ablação curativa de fibrilação atrial (FA) no Brasil, a Sociedade Brasileira de Arritmias Cardíacas (SOBRAC) idealizou o Registro Brasileiro de Ablação da FA. OBJETIVO: Descrever os resultados desse registro. MÉTODOS: Foi enviado um formulário aos sócios da SOBRAC, inquirindo sobre os dados de pacientes submetidos a ablação de FA entre setembro de 2005 e novembro de 2006. RESULTADOS: No total, 29 grupos, de 13 Estados, responderam ao formulário. Desses, 22 (76 por cento) realizaram ablações de FA. Entre 1998 e 2001, 7 grupos (32 por cento) iniciaram ablações de FA e entre 2002 e 2006, 15 grupos (68 por cento). De 1998 a 2006, 2.374 pacientes foram submetidos a ablação, sendo 755 (32 por cento) no período do registro. A maioria (70 por cento) era do sexo masculino e 89 por cento apresentavam FA paroxística ou persistente. Métodos auxiliares de imagem (ecocardiografia intracardíaca e mapeamento eletroanatômico) foram utilizados por 9 grupos (41 por cento). Durante seguimento médio de cinco meses, o sucesso total foi de 82 por cento e o sucesso sem uso de antiarrítmicos foi de 57 por cento. Contudo, 35 por cento dos pacientes necessitaram de dois ou mais procedimentos. Houve 111 complicações (14,7 por cento) e 2 óbitos (0,26 por cento). CONCLUSÃO: A ablação curativa de FA vem crescendo significativamente em nosso País, com taxas de sucesso comparáveis às internacionais, mas comumente há necessidade de mais de um procedimento. Apesar dos resultados promissores, a ablação de FA ainda acarreta morbidade significativa. Métodos auxiliares de imagem têm sido cada vez mais utilizados, visando a aumentar a eficácia e a segurança do procedimento. Esses achados devem ser considerados pelos órgãos pagadores públicos e privados.

BACKGROUND: Aiming to define the profile of curative atrial fibrillation (AF) ablation in Brazil, the Brazilian Cardiac Arrhythmia Society [Sociedade Brasileira de Arritmias Cardíacas] (SOBRAC) created the Brazilian Registry of AF Ablation [Registro Brasileiro de Ablação da FA]. OBJECTIVE: To describe the results of this registry. METHODS: A questionnaire was sent to SOBRAC members asking about data on patients submitted to AF ablation between September 2005 and November, 2006. RESULTS: A total of 29 groups from 13 states completed the forms. Of these, 22 (76 percent) had performed AF ablations. Between 1998 and 2001, 7 groups (32 percent) initiated AF ablations and between 2002 and 2006, 15 groups began to perform them (68 percent). From 1998 to 2006, 2,374 patients were submitted to ablation, 755 (32 percent) of them during the registry period. Most (70 percent) were males and 89 percent presented with paroxysmal or persistent AF. Ancillary imaging methods (intracardiac echocardiography and electroanatomic mapping) were used by 9 groups (41 percent). During an average five-month follow-up period, total success was 82 percent and success without use of antiarrhythmic agents was 57 percent. Nevertheless, 35 percent of the patients required two or more procedures. There were 111 complications (14.7 percent) and 2 deaths (0.26 percent). CONCLUSION: Curative AF ablation has been increasing significantly in our country, with success rates comparable to international indexes, but often more than one procedure is necessary. Despite promising results, AF ablation still results in significant morbidity. Supplementary imaging methods have been used more and more in an effort to increase efficacy and safety of the procedure. These findings should be considered by public and private funding agencies.

Comparaçäo entre uso de duplo balão unifoil e baläo bifoil no sucesso da valvoplastia mitral por cateter balão / Comparison between the use of unifoil double balloon and bifoil balloon in successful mitral valvuloplasty by balloon catheter

PURPOSE--To compare immediate and long term results balloon mitral valvuloplasty (BMV) using double balloon or bifoil balloon. METHODS--One hundred and thirteen consecutive cases of BMV used aleatory double balloon (group DB--55 cases) or bifoil balloon (group BF--16 cases). Patients were similar regarding to age, sex, valvopaty etiology, functional class and echocardiographic score. Seventy one (63 per cent ) patients achieved 12 months follow-up. RESULTS--In group DB there were 2 (4 per cent ) insuccess, 2 (4 per cent ) cardiac tamponade and 2 (4 per cent ) deaths, 91 per cent patients had immediate criteria of success. Mitral valve area (MVA) increased from 0.8 to 1.69cm2 and mitral gradient (G) by echodopplercardiographic (ECHO) decreased from 17.9 to 4.8mmHg. Three (5 per cent ) patients developed severe mitral regurgitation (MR) and needed surgical intervention. At follow-up 2 (4 per cent ) developed mitral restenosis. MVA estimated by ECHO study after one year follow-up was inferior to 1.15cm2 in 15 (32 per cent ) cases, between 1.5 and 2.0cm2 in 17 (37 per cent ) and superior to 2.0cm2 in 14 (31 per cent ). In group BF there were 2 (12.5 per cent ) insuccess, 4 (25 per cent ) developed severe MR occurring 1 death immediate post-operative mitral valve replacement. Among 14 (87 per cent ) success cases, MVA increased from 0.8 to 1.89cm2 and G decreased from 18 to 6.4mmHg. Lately 2 (12.5 per cent ) needed surgical intervention because significative MR. At 12th month follow-up the ECHO study showed that in one (10 per cent ) case MVA was < 1.5cm2, and in 3 (27 per cent ) cases was between 1.5 and 2.0cm2. CONCLUSION--There were similar good results in both groups, however group DB had more restenosis, cardiac tamponade and vascular complications and group BF had more severe MR