Role of Computed Tomography Angiography in the Short-Term Follow-up of Aortic Coarctation Repair

ABSTRACT Introduction: Coarctation of the aorta (CoA) is a narrowing of the thoracic aorta that often manifests as discrete stenosis but may be tortuous or in long segment. The study aimed to evaluate pre and post-surgical aspects of pediatric patients submitted to CoA surgical correction and to identify possible predisposing factors for aortic recoarctation. Methods: Twenty-five patients were divided into groups according to presence (N=8) or absence (N=17) of recoarctation after surgical correction of CoA and evaluated according to clinical-demographic profile, vascular characteristics via computed angiotomography (CAT), and other pathological conditions. Results: Majority of males (64%), ≥ 15 days old (76%), ≥ 2.5 kg (80%). There was similarity between groups with and without recoarctation regarding sex (male: 87% vs. 53%; P=0.277), age (≥ 15 days: 62.5 vs. 82%; P=0.505), and weight (≥ 2.5 kg: 87.5 vs. 76.5; P=0,492). Altered values of aortic root/Valsalva diameter, proximal transverse arch, and distal isthmus, and normal values for aorta prevailed in preoperative CAT. Normal values for the aortic root/Valsalva sinus diameter were observed with and without recoarctation, the same for both groups regarding ascending and descending aorta in postoperative CAT. No significant difference for altered values of proximal transverse arch and alteration in distal isthmus was observed. Conclusion: No predictive risk for recoarctation was observed. CTA proved to be important in CoA diagnosis and management, since CoA is mainly related with altered diameter of aortic root/sinus of Valsalva and proximal and distal aortic arch/isthmus, however, it failed to show predictive risk for recoarctation.

Use of Ozonized Water in the Prevention of Surgical Site Infection in Children Undergoing Cardiovascular Surgery

ABSTRACT Introduction: Since the reduction of healthcare-associated infections has been a focus for quality patient care, this study aimed to evaluate the surgical site infection rate of children who underwent cardiovascular surgery after implementation of ozonized water system for hand and body hygiene allied to previously implemented preventive measures. Methods: Two uniformly comparable groups of pediatric patients underwent cardiovascular surgery. Group A (187) patients were operated prior to installation of ozonized water system (March 1 to August 31, 2019), and group B (214) patients were operated after installation of ozonized water system (October 1, 2019, to March 31, 2020). Ozonized water was used for professional hand hygiene and patient body hygiene. Results: There was statistical significance for surgical site infection reduction in group B (P=0.0289), with a relative risk of 0.560 (95% confidence interval = 0.298 to 0.920), inferring the risk of being diagnosed with surgical site infections in group B was 44% less than in group A. There was no statistical significance regarding mechanical ventilation time (P=0.1998) or mortality (P=0.4457). Conclusion: Ozonized water for professional hand hygiene and patient body hygiene was an adjuvant combined with traditional preventive methods to reduce the risk of surgical site infection, although no impact on hospital stay or mortality was observed.

Tetralogy of Fallot with an Anomalous Course of the Brachiocephalic Vein

ABSTRACT Clinical data: Infant, 11-month-old, male, diagnosis of Tetralogy of Fallot with retrotracheoesophageal course of the brachiocephalic vein. Usual findings of Tetralogy of Fallot on physical examination. Technical description: Chest radiography showed slightly reduced pulmonary vascular markings and no cardiomegaly. Normal preoperative electrocardiogram with postoperative right bundle branch block. Usual findings of Tetralogy of Fallot on echocardiogram. Postoperative computed tomography angiography confirmed left brachiocephalic vein with anomalous retrotracheoesophageal course, configuring a U-shaped garland vein, in addition to postoperative findings of total correction of Tetralogy of Fallot. Operation: Complete surgical repair was performed with pulmonary valve commissurotomy and placement of bovine pericardial patch to solve right ventricular outflow tract obstruction, pulmonary trunk enlargement, and ventricular septal defect closure. Comments: Systemic venous drainage may show variations in patients with Tetralogy of Fallot. These abnormalities are usually of little clinical relevance, as they are asymptomatic. We presented a rare case of retrotracheoesophageal course of an anomalous left brachiocephalic vein with intraoperative diagnosis, confirmed by imaging during postoperative follow-up, without compromising clinical management or surgical approach.

Abernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis

ABSTRACT Clinical data: Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. Chest radiography: Cardiomegaly with prevalence of pulmonary vascular network. Electrocardiogram: Ectopic atrial rhythm with right ventricular overload and left anterosuperior divisional block. Echocardiogram: Single atrium with absent interatrial septum, atrioventricular connection with a single valve and two orifices, with increased pulmonary pressure and high Qp/Qs. Computed tomography: Absence of portal vein and intrahepatic segment of the inferior vena cava. Infrahepatic portion continuing with the azygos system at the level of the thoracic cavity, presence of mesenteric-caval communication associated with signs suggestive of hepatic peribiliary fibrosis. Diagnosis: Abernethy malformation is a rare condition and represents an extrahepatic portosystemic shunt that develops between the mesenteric-portal vasculature and the systemic veins. It may be associated with cardiac malformations and advance with pulmonary hypertension and even the need for liver transplantation. Persistent cyanosis after corrective surgery led to a deeper investigation and correct diagnosis of this malformation. Operation: Sternotomy with 68 minutes of cardiopulmonary bypass and nine minutes of total circulatory arrest. In the postoperative period, persistence of cyanosis was evident, even though there were no immediate complications. Patient was discharged on the 10th postoperative day. An abdominal computed tomography angiography confirmed the diagnosis of Abernethy type I malformation, and the patient was transferred for liver transplantation after congenital heart disease treatment.

Pediatric Heart Transplant: Initial Experience in a Tertiary Center in Brazil

ABSTRACT Introduction: Pediatric heart transplantation is the definitive therapy for children with end-stage heart failure. This paper describes our initial experience in pediatric heart transplantation in a tertiary center in Brazil Methods: This is a historical prospective descriptive cohort study based on a review of the medical records of children undergoing heart transplantation at Hospital de Base and Hospital da Criança e Maternidade de São José do Rio Preto. Variables were displayed as frequency, mean, or median. Statistical analysis and Kaplan-Meier actuarial curve were obtained with the aid of Microsoft® Excel® 2019 and STATSDirect version 3.3.5. Results: Between January 2010 and December 2020, ten children underwent bicaval orthotopic heart transplantation, 30% of which were under one year of age. Nine patients had end-stage heart failure (International Society for Heart and Lung Transplantation-Heart Failure D) and 50% of the recipients were transplanted under conditions of progressive clinical deterioration (Interagency Registry for Mechanically Assisted Circulatory Support ≤ 2). Forty percent of the recipients had a panel-reactive antibody > 20% on virtual crossmatch. In the postoperative period, 80% of patients required high dose of inotropic support (vasoactive-inotropic score > 10) for > 48 hours. The death-free survival rate at 131 months was 77.1±14.4%. Most patients (88.9%) in late follow-up had an episode of active cytomegalovirus infection. Cellular rejection, with or without clinical repercussion, was present in 44.4% of the patients. Conclusion: Pediatric heart transplantation produces acceptable and feasible outcomes as definitive therapy for children with end-stage heart failure.

Common Arterial Trunk with Interrupted Aortic Arch

Abstract Clinical data: Patient diagnosed with common arterial trunk, submitted to pulmonary artery banding in another center and lost to clinical follow-up. Referred to our center at four years old, extremely cyanotic. Chest radiography: Cardiomegaly; attenuated peripheral vascular markings. Electrocardiography: Right ventricular hypertrophy. Echocardiography: Common arterial trunk, but it was not possible to analyze all the structures. Computed tomography angiography: Van Praagh type A4 common arterial trunk. Extremely hypoplastic right and left pulmonary arteries. Diagnosis: Association of aortic arch interruption type A is uncommon and should be considered. Operation: Debanding of pulmonary arteries allowing for possible future complete repair.

COVID-19 in the perioperative period of cardiovascular surgery: the brazilian experience

Abstract Introduction: We investigated the clinical course and outcomes of patients submitted to cardiovascular surgery in Brazil and who had developed symptoms/signs of coronavirus disease 2019 (COVID-19) in the perioperative period. Methods: A retrospective multicenter study including 104 patients who were allocated in three groups according to time of positive real time reverse transcriptase-polymerase chain reaction (RT-PCR) for the SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2): group 1, patients who underwent cardiac surgery > 10 days after positive RT-PCR; group 2, patients with a positive RT-PCR within 10 days before or after surgery; group 3, patients who presented positive RT-PCR > 10 days after surgery. The primary outcome was mortality and secondary outcomes were postoperative complications, intensive care unit (ICU) length of stay, and postoperative days of hospitalization. Results: The three groups were similar with respect to age, the European System of Cardiac Operative Risk Evaluation score, and comorbidities, except hypertension. Postoperative complications and death were significantly higher in groups 2 and 3 than in group 1, and no significant difference between groups 2 and 3 was seen. Group 2 showed a high prevalence of surgery performed as an urgent procedure. Although no significant differences were observed in ICU length of stay, total postoperative hospitalization time was significantly higher in group 3 than in groups 1 and 2. Conclusion: COVID-19 affecting the postoperative period of patients who underwent cardiovascular surgery is associated with a higher rate of morbidity and mortality. Delaying procedures in RT-PCR-positive patients may help reduce risks of perioperative complications and death.

Isolated Coarctation Repair in Neonates and Infants Through Left Thoracotomy: Short-Term Outcomes

Abstract Introduction: End-to-end anastomosis and extended end-to-end anastomosis are typically used as surgical approaches to coarctation of the aorta (CoAo) with access at the subclavian artery or an interposition graft. The objective of this study is to analyze the impact of surgical and anatomical characteristics and techniques on early outcomes after surgical treatment of CoAo without cardiopulmonary bypass through left thoracotomy. Methods: This is a quantitative, observational, and cross-sectional analysis of patients who underwent repair of CoAo between July 1, 2010 and December 31, 2017. Seventy-two patients were divided into three groups according to age: 34 in group A (≤ 30 days), 24 in group B (31 days to one year), and 14 in group C (≥ 1 year to 18 years). Results: Aortic arch hypoplasia was associated in 30.8% of the cases, followed by ventricular septal defect (13.2%). The preductal location was more frequent in group A (73.5%), ductal in group B (41.7%), and postductal in group C (71.4%). Long coarcted segment was predominant in groups A and C (61.8% and 71.4%, respectively) and localized in group B (58.3%). Extended end-to-end anastomosis technique was prevalent (68%), mainly in group A (91.2%). Mortality in 30 days was 1.4%. Conclusion: Most of the patients were children under one year of age, and extended end-to-end anastomosis was the most used technique, secondary to arch hypoplasia. Further, overall mortality was low in spite of moderate morbidity in the first 30 postoperative days.

Rare association between atrioventricular septal defect and partial anomalous pulmonary venous connection

Abstract Clinical data: Infant, 7 months, female, referred to our department at one month of age, suspecting of congenital heart disease for further investigation. Chest radiography: Demonstrates cardiomegaly and prominent pulmonary vascular markings. Electrocardiography: Shows right ventricular hypertrophy and left anterior fascicular block. Echocardiography: Evidenced common atrioventricular valve with two orifices and the left superior pulmonary vein draining on the brachiocephalic vein. Computed tomography angiography: This complementary imaging exam was performed to confirm the diagnosis. Diagnosis: The patient presented an association between AVSD and PAPVC, a rare combination. The clinical picture of heart failure was preponderant, characterized by need for diuretics and complementary exams findings, and early surgical treatment was indicated. Operation: The operation was performed through a median sternotomy with 123 minutes of cardiopulmonary bypass and 89 minutes of cross-clamping time. The patient had no postoperative complications, remaining 10 days hospitalized.

Surgical treatment for congenital heart defects in down syndrome patients

Abstract Objective: To analyze data related to surgical treatment in patients with congenital heart defects (CHD) and Down syndrome (DS) based on information from International Quality Improvement Collaborative Database for Congenital Heart Disease (IQIC). Methods: Between July 1, 2010 and December 31, 2017, 139 patients with CHD and DS underwent surgery at Hospital de Base and Hospital da Criança e Maternidade de São José do Rio Preto (FUNFARME)/Faculdade de Medicina de São José do Rio Preto - SP (FAMERP). A quantitative, observational and cross-sectional study was performed in which the pre, intra and postoperative data were analyzed in an IQIC database. The data included gender, age, prematurity, weight, preoperative procedures, diagnosis, associated cardiac and non-cardiac anomalies, Risk Adjustment for Congenital Heart Surgery (RACHS-1), type of surgery, cardiopulmonary bypass (CPB), perfusion time, aortic clamping time and CPB temperature, bacterial sepsis, surgical site infection and other infections, length of stay in intensive care unit (ICU), length of hospital stay and in-hospital mortality. Results: The most prevalent procedures were complete atrioventricular septal defect repair (58 - 39.45%), followed by closure of ventricular septal defect (36 - 24.49%). The RACHS-1 categories 1, 2, 3 and 4 were distributed as 22 (15%); 49 (33.3%); 72 (49%) and 4 (2.7%), respectively. There were no procedures classified as categories 5 or 6. Bacterial sepsis occurred in 10.2% of cases, surgical site infection in 6.1%, other infections in 14.3%. The median length of ICU stay was 5 days and the median length of hospital stay was 11 days. In-hospital mortality was 6.8%. Conclusion: Surgical treatment in patients with CHD and DS usually does not require highly complex surgical procedures, but are affected by infectious complications, resulting in a longer ICU and hospital length of stay with considerable mortality.

Factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of repair of complete atrioventricular septal defect / Fatores associados à insuficiência da valva atrioventricular esquerda nos primeiros 30 dias após correção de defeito de septo atrioventricular total

AbstractIntroduction:Left atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defects.Objective:To determine factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of surgical repair of complete atrioventricular septal defect.Methods:We assessed the results of 53 consecutive patients 3 years-old and younger presenting with complete atrioventricular septal defect that were operated on at our practice between 2002 and 2010. The following variables were considered: age, weight, absence of Down syndrome, grade of preoperative atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. Median age was 6.7 months; median weight was 5.3 Kg; 86.8% had Down syndrome. At the time of preoperative evaluation, there were 26 cases with moderate or severe left atrioventricular valve regurgitation (49.1%). Abnormalities on the left atrioventricular valve were found in 11.3%; annuloplasty was performed in 34% of the patients.Results:At the time of postoperative evaluation, there were 21 cases with moderate or severe left atrioventricular valve regurgitation (39.6%). After performing a multivariate analysis, the only significant factor associated with moderate or severe left atrioventricular valve regurgitation was the absence of Down syndrome (P=0.03).Conclusion:Absence of Down syndrome was associated with moderate or severe postoperative left atrioventricular valve regurgitation after surgical repair of complete atrioventricular septal defect at our practice.

ResumoIntrodução:A insuficiência da valva atrioventricular esquerda é a lesão residual mais preocupante após o tratamento cirúrgico do defeito de septo atrioventricular.Objetivo:Determinar fatores associados à insuficiência da valva atrioventricular esquerda de grau moderado ou importante nos primeiros 30 dias após correção de defeito de defeito de septo atrioventricular total.Métodos:Avaliamos os resultados em 53 pacientes consecutivos menores de 3 anos com defeito de septo atrioventricular total, operados em nosso serviço entre 2002 e 2010. Avaliamos as seguintes variáveis: idade, peso, ausência de síndrome de Down, grau de insuficiência da valva atrioventricular esquerda antes da correção, anormalidades na valva atrioventricular e uso de anuloplastia. A mediana da idade foi de 6,7 meses e a do peso de 5,3 Kg; 86,8% tinham síndrome de Down. Antes da operação, 26 apresentavam insuficiência da valva atrioventricular esquerda pelo menos moderada (49,1%). Anormalidades na valva atroventricular foram encontradas em 11,3% dos casos; anuloplastia foi realizada em 34% dos pacientes.Resultados:Após a correção, houve 21 casos com insuficiência moderada ou grave da valva atrioventricular esquerda (39,6%). Após realização de análise multivariada, o único fator associado com esses graus de insuficiência foi a ausência da síndrome de Down (P=0,03).Conclusão:Ausência de síndrome de Down esteve associada com insuficiência moderada ou grave da valva atrioventricular esquerda após correção cirúrgica de defeito de septo atrioventricular total em nosso serviço.

Factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of repair of incomplete atrioventricular septal defect / Fatores associados à insuficiência moderada ou grave da valva atrioventricular esquerda nos primeiros 30 dias de correção de defeito de septo atrioventricular incompleto

Abstract Introduction: Left atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defect. Objective: To determine factors associated with moderate or greater left atrioventricular valve regurgitation within 30 days of surgical repair of incomplete atrioventricular septal defect. Methods: We assessed the results of 51 consecutive patients 14 years-old and younger presenting with incomplete atrioventricular septal defect that were operated on at our practice between 2002 and 2010. The following variables were considered: age, weight, absence of Down syndrome, grade of preoperative left atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. The median age was 4.1 years; the median weight was 13.4 Kg; 37.2% had Down syndrome. At the time of preoperative evaluation, there were 23 cases with moderate or greater left atrioventricular valve regurgitation (45.1%). Abnormalities on the left atrioventricular valve were found in 17.6%; annuloplasty was performed in 21.6%. Results: At the time of postoperative evaluation, there were 12 cases with moderate or greater left atrioventricular valve regurgitation (23.5%). The variation between pre- and postoperative grades of left atrioventricular valve regurgitation of patients with atrioventricular valve malformation did not reach significance (P=0.26), unlike patients without such abnormalities (P=0.016). During univariate analysis, only absence of Down syndrome was statistically significant (P=0.02). However, after a multivariate analysis, none of the factors reached significance. Conclusion: None of the factors studied was determinant of a moderate or greater left atrioventricular valve regurgitation within the first 30 days of repair of incomplete atrioventricular septal defect in the sample. Patients without abnormalities on the left atrioventricular valve benefit ...

Resumo Introdução: A insuficiência da valva atrioventricular esquerda é a lesão residual mais preocupante após o tratamento cirúrgico do defeito de septo atrioventricular. Objetivo: Determinar fatores associados à insuficiência da valva atrioventricular esquerda de grau moderado ou importante nos primeiros 30 dias após correção de defeito de defeito de septo atrioventricular. Métodos: Avaliamos os resultados em 51 pacientes consecutivos menores de 14 anos com defeito de septo atrioventricular incompleto, operados em nosso serviço entre 2002 e 2010. Avaliamos as seguintes variáveis: idade, peso, ausência de síndrome de Down, grau de insuficiência da valva atrioventricular esquerda antes da correção, anormalidades na valva atrioventricular e uso de anuloplastia. A mediana da idade foi de 4,1 anos e a do peso de 13,4 Kg; 37,2% tinham síndrome de Down; antes da operação, 23 apresentavam insuficiência da valva atrioventricular esquerda pelo menos moderada (45,1%); anormalidades na valva atrioventricular foram encontradas em 17,6% dos casos; anuloplastia foi realizada em 21,6% dos pacientes. Resultados: Após a correção cirúrgica, 12 casos apresentaram insuficiência da valva atrioventricular esquerda pelo menos moderada (23,5%). A variância entre os graus de insuficiência da valva atrioventricular esquerda pré e pós-operatória nos pacientes com anormalidades na valva atrioventricular não teve significância estatística (P=0,26), ao contrário daqueles sem tais anormalidades (P=0,016). Pela análise univariada, apenas a ausência de síndrome de Down teve significância estatística (P=0,02). Porém, após análise multivariada, nenhum dos fatores teve significância. Conclusão: Nenhum dos fatores estudados foi determinante de insuficiência da valva atrioventricular esquerda de grau moderado ou importante nos primeiros 30 dias após a correção de defeito de septo atrioventricular incompleto na população avaliada. Pacientes sem anormalidades na valva atrioventricular ...

Evolução pondero-estatural de crianças com cardiopatias congênitas submetidas a tratamento cirúrgico / Evolution of weight and height of children with congenital heart disease undergoing surgical treatment

Objetivo: Avaliar a evolução pôndero-estatural de crianças com cardiopatias congênitas submetidas a tratamento cirúrgico com intuito de determinar quando atingem o limiar de desenvolvimento normal e se há diferenças entre pacientes com padrão de desenvolvimento abaixo do patamar da normalidade no pré-operatório (z-score<-2 para o parâmetro analisado) em relação ao grupo total de cardiopatas. Métodos: Acompanhamento prospectivamente de 27 crianças submetidas à operação em cinco períodos: pré-operatório e em quatro subsequentes retornos ambulatoriais: 1º mês, 3º mês, 6º mês e 12º mês após a alta hospitalar. Os parâmetros antropométricos usados foram a média do z-score (Zm) do peso (ZmP/I), da altura (ZmA/I), prega cutânea subescapular (ZmPCS/I), perímetro braquial (ZmPB/I) e prega cutânea tricipital (ZmPCT/I). A avaliação da evolução dos parâmetros foi feita pela análise de variância e a comparação com a população geral normal pelo teste t não pareado, tanto no grupo total dos cardiopatas, quanto nos subgrupos com parâmetros pré-operatórios abaixo do patamar da normalidade (Zm<-2). Resultados: No grupo total não houve evolução significativa dos Zm de todos os parâmetros. O ZmP/I foi estatisticamente menor que da população normal até o 1º mês de seguimento (P=0,028); o ZmA/I, somente no pré operatório (P=0,044); o ZmPCS/I, no o 1º mês (P=0,015) e no 12º mês (P=0,038); o ZmPB/I e o ZmPCT/I sempre foram estatisticamente iguais ao da população geral. Nos pacientes com desenvolvimento abaixo do limiar da normalidade houve variação importante do ZmP/I (P=0,002), do ZmA/I (P=0,001) e ...

Objective: To evaluate the height and weight development of children with congenital heart disease undergoing surgery with the goal of determining when they reach the threshold of normal development and whether there are differences between patients with developmental pattern below the level of normality preoperatively (z-score<-2 for the analyzed parameter) in comparison to the total group of cardiac patients. Methods: We prospectively followed up 27 children undergoing operation into five time periods: preoperatively and at four subsequent outpatient appointments: 1st month, 3rd month, 6th month and 12th month after hospital discharge. The anthropometric parameters used were median z-score (MZ), weight (WAZ), height (HAZ), subscapular skinfold (SSFAZ), upper arm circumference (UAC) and triceps skinfold (TSFAZ). The evolution assessment of the parameters was performed by analysis of variance and comparison with the general normal population from unpaired t test, both in the total group of cardiac patients, and in subgroups with preoperative parameters below the normal level (Zm<-2). Results: In the total group there was no significant evolution of MZ of all parameters. WAZ was statistically lower than the normal population until the 1st month of follow-up (P=0.028); HAZ only preoperatively (P=0.044), SSFAZ in the first month (P=0.015) and at 12th month (P=0.038), UAC and TSFAZ were always statistically equal to the general population. In patients whose development was below the level of normality, there were important variation of WAZ (P=0.002), HAZ (P=0.001) and UAC (P=0.031) after the operation, and the WAZ was lower than the normal population until the 3rd month (P=0.015); HAZ and UAC, until the first month (P=0.024 and P=0.039 respectively), SSFAZ, up to the 12th month (P=0.005), the TSFAZ only preoperatively (P=0.011). Conclusion: The operation promoted the return to normalcy for those with heart disease in general ...

Information technology implementing globalization on strategies for quality care provided to children submitted to cardiac surgery: International Quality Improvement Collaborative Program - IQIC / A tecnologia da informação implementando a globalização nas estratégias de qualidade para o atendimento às crianças submetidas à cirurgia cardíaca: o Programa de Colaboração Internacional - IQIC

Introduction: Congenital heart diseases are the world's most common major birth defect, affecting one in every 120 children. Ninety percent of these children are born in areas where appropriate medical care is inadequate or unavailable. Objective: To share knowledge and experience between an international center of excellence in pediatric cardiac surgery and a related program in Brazil. Methods: The strategy used by the program was based on long-term technological and educational support models used in that center, contributing to the creation and implementation of new programs. The Telemedicine platform was used for real-time monthly broadcast of themes. A chat software was used for interaction between participating members and the group from the center of excellence. Results: Professionals specialized in care provided to the mentioned population had the opportunity to share to the knowledge conveyed. Conclusion: It was possible to observe that the technological resources that implement the globalization of human knowledge were effective in the dissemination and improvement of the team regarding the care provided to children with congenital heart diseases. .

Introdução: Doenças cardíacas congênitas são consideradas o maior defeito ao nascimento, afetando uma em cada 120 crianças. Noventa por cento destes recém-nascidos encontram-se em áreas onde os cuidados médicos são inadequados ou indisponíveis. Objetivo: Compartilhar o conhecimento e a experiência en tre um centro de excelência internacional em cirurgia cardíaca pediátrica e um programa correlato no Brasil. Métodos: Estratégia utilizada pelo programa foi baseada em modelos de suporte tecnológico e educacional a longo termo advinda daquele centro, contribuindo para criação e efetivação de novos programas. Foi utilizada plataforma de Telemedicina para transmissão mensal em tempo real dos temas. Um progra ma de chat foi utilizado para a interação entre os membros par ticipantes e o grupo do centro de excelência. Resultados: Os profissionais especializados no cuidado des sa população tiveram a oportunidade de participar do conheci mento transmitido. Conclusão: Pode-se observar que os recursos tecnológicos que implementam a globalização do conhecimento humano fo ram efetivos na disseminação e aprimoramento da equipe no que diz respeito ao cuidado às crianças com cardiopatias congênitas. .

Síndrome do QT longo congênito associado a bloqueio atrioventricular 2: 1 em lactente com síncope de repetição tratado como epilepsia / Congenital long QT syndrome associated with 2: 1 atrioventricular block in infant with repeated syncope treated as epilepsy

A Síndrome do QT longo (SQTL) congênito é uma doença dos canais iônicos cardíacos que está associada com síncope, taquiarritmias ventriculares e morte súbita cardíaca em pacientes com coração normal. Raramente, apresenta-se com Bloqueio Atrioventricular 2:1, apresentando mortalidade maior que 50% nos primeiros seis meses de vida, mesmo com tratamento adequado. O caso aqui descrito relata a evolução clínico de lactente com 6 meses de idade com SQTL congênito, síncope de repetição e BAV 2:1 inicialmente tratado como epilepsia, que após tratamento clínico adequado com mudança de estilo de vida, betabloqueadores e implante de marcapasso, não apresentou mais eventos cardíacos. No seguimento tardio (com 6 anos de vida), foi implantado Cardiodesfibrilador Implantável, sem terapias até a presente data.

Congenital long QT syndrome is a disease of cardiac ion channels that is associated withs yncope, cardiac sudden death and ventricular tachyarrhythmias in patients with normal hearts. Rarely,it presents associated with 2:1 atrioventricular block; in this case the mortality rate is higher than 50% in the first six months of life even with adequate treatment. The case described here reports on the clinical evolution of a six-month-old infant with congenital long QT syndrome, syncope of repetition and 2:1 atrioventricular block initially treated as epilepsy, that after the correct clinical treatment with changes in lifestyle, beta-blockers and pacemaker implantation, did not have further cardiac events. In the long term follow-up (six years of life), a defibrillator was implanted without the need of further therapies todate.

Síndrome do QT longo congênito associado a bloqueio atrioventricular 2: 1 em lactente com síncopes de repetição tratado como epilepsia / Congenital long QT syndrome associated with 2: 1 atrioventricular block in infant with repeated syncope treated

A síndrome do QT longo (SQTL) congênito é uma doença dos canais iônicos cardíacos que está associada com síncope, taquiarritmias ventriculares e morte súbita cardíaca em pacientes com coração normal. Raramente, apresenta-se com boqueio atrioventricular 2:1, apresentando mortalidade maior que 50% nos primeiros 6 meses de vida, mesmo com tratamento adequado. O caso aqui descrito relata a evolução clínico de lactente com 6 meses de idade com SQTL congênito, síncope de repetição e BAV 2:1 inicialmente tratado como eplepsia, que após tratamento clínico adequado com mudança de estilo de vida, betabloqueadores e implante de marca-passo, não apresentou mais eventos cardíacos. No seguimento tardio (com 6 anos de vida), foi implantado cardiodesfibrilador implantável, sem terapias até a presente data.