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Clinical Data: A nine-month-old female infant diagnosed with transposition of the great arteries with symptoms of heart failure associated with cyanosis and difficulty in gaining weight was referred to our center with late diagnosis (at nine months of age)., Chest Radiography: Cardiomegaly; attenuated peripheral vascular markings.Electrocardiography: Sinus rhythm with biventricular overload and aberrantly conducted supraventricular extra systoles., Echocardiography: Wide atrial septal defect, ventricular axis torsion with concordant atrioventricular connection and discordant ventriculoarterial connection., Computed Tomography Angiography: Concordant atrioventricular connection, right ventricle positioned superiorly and left ventricle positioned inferiorly; discordant ventriculoarterial connection with right ventricle connected to the aorta and left ventricle connected to pulmonary artery., Diagnosis: Crisscross heart is a rare congenital heart defect, accounting for 0.1% of congenital heart diseases. It consists of the 90º rotation of ventricles' axis in relation to their normal position; therefore, ventricles are positioned in the superior-inferior direction rather than anterior-posterior. Most cases have associated cardiac anomalies, and in this case, it is associated with transposition of the great arteries. The complexity and rarity of its occurrence make diagnosis and surgical treatment challenging., Operation: Modified Senning procedure using the pericardial sac in the construction of a tunnel from pulmonary veins to the right atrium. Cardiopulmonary bypass time of 147 minutes with nine minutes of total circulatory arrest.

Congenital heart disease (CHD) affects eight to ten out of every 1,000 births, resulting in approximately 23,057 new cases in Brazil in 2022. About one in four children with CHD requires surgery or other procedures in the first year of life, and it is expected that approximately 81% of these children with CHD will survive until at least 35 years of age. Professionals choosing to specialize in CHD surgery face numerous challenges, not only related to mastering surgical techniques and the complexity of the diseases but also to the lack of recognition by medical societies as a separate subspecialty. Furthermore, families face difficulties when access to services capable of providing treatment for these children. To address these challenges, it is essential to have specialized hospitals, qualified professionals, updated technologies, sustainable industry, appropriate financing, quality assessment systems, and knowledge generation. The path to excellence involves specialization across all involved parties. As we reflect on the importance of Pediatric Cardiovascular Surgery and Congenital Heart Diseases establishing themselves as a subspecialty of Cardiovascular Surgery, it is essential to look beyond our borders to countries like the United States of America and United Kingdom, where this evolution is already a reality. This autonomy has led to significant advancements in research, education, and patient care outcomes, establishing a care model. By following this path in Brazil, we not only align our practice with the highest international standards but also demonstrate our maturity and the ability to meet the specific needs of patients with CHD and those with acquired childhood heart disease.

Clinical Data: Female, seven years old, referred to our service complaining about congestive heart failure symptoms due to mitral valve regurgitation and atrial septal defect. Technical description: Echocardiographic findings compatible with Barlow's disease and atrial septal defect, ostium secundum type., Operation: She was submitted to mitral valvuloplasty with chordal shortening and prosthetic posterior ring (Gregori-Braile®) along with patch atrioseptoplasty., Comments: Mitral valve regurgitation is a rare congenital heart disease and Barlow's disease is probably rarer. Mitral valve repair is the treatment of choice.

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.

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.

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.

The global burden of paediatric and congenital heart disease (PCHD) is substantial. We propose a novel public health framework with recommendations for developing effective and safe PCHD services in low-income and middle-income countries (LMICs). This framework was created by the Global Initiative for Children's Surgery Cardiac Surgery working group in collaboration with a group of international rexperts in providing paediatric and congenital cardiac care to patients with CHD and rheumatic heart disease (RHD) in LMICs. Effective and safe PCHD care is inaccessible to many, and there is no consensus on the best approaches to provide meaningful access in resource-limited settings, where it is often needed the most. Considering the high inequity in access to care for CHD and RHD, we aimed to create an actionable framework for health practitioners, policy makers and patients that supports treatment and prevention. It was formulated based on rigorous evaluation of available guidelines and standards of care and builds on a consensus process about the competencies needed at each step of the care continuum. We recommend a tier-based framework for PCHD care integrated within existing health systems. Each level of care is expected to meet minimum benchmarks and ensure high-quality and family centred care. We propose that cardiac surgery capabilities should only be developed at the more advanced levels on hospitals that have an established foundation of cardiology and cardiac surgery services, including screening, diagnostics, inpatient and outpatient care, postoperative care and cardiac catheterisation. This approach requires a quality control system and close collaboration between the different levels of care to facilitate the journey and care of every child with heart disease. This effort was designed to guide readers and leaders in taking action, strengthening capacity, evaluating impact, advancing policy and engaging in partnerships to guide facilities providing PCHD care in LMICs., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

Objective: As COVID-19 continues to affect the global population, it is crucial to study the impact of the disease in vulnerable populations. This study of a diverse, international cohort aims to provide timely, experiential data on the course of disease in paediatric patients with congenital heart disease (CHD)., Methods: Data were collected by capitalising on two pre-existing CHD registries, the International Quality Improvement Collaborative for Congenital Heart Disease: Improving Care in Low- and Middle-Income Countries and the Congenital Cardiac Catheterization Project on Outcomes. 35 participating sites reported data for all patients under 18 years of age with diagnosed CHD and known COVID-19 illness during 2020 identified at their institution. Patients were classified as low, moderate or high risk for moderate or severe COVID-19 illness based on patient anatomy, physiology and genetic syndrome using current published guidelines. Association of risk factors with hospitalisation and intensive care unit (ICU) level care were assessed., Results: The study included 339 COVID-19 cases in paediatric patients with CHD from 35 sites worldwide. Of these cases, 84 patients (25%) required hospitalisation, and 40 (12%) required ICU care. Age <1 year, recent cardiac intervention, anatomical complexity, clinical cardiac status and overall risk were all significantly associated with need for hospitalisation and ICU admission. A multivariable model for ICU admission including clinical cardiac status and recent cardiac intervention produced a c-statistic of 0.86., Conclusions: These observational data suggest risk factors for hospitalisation related to COVID-19 in paediatric CHD include age, lower functional cardiac status and recent cardiac interventions. There is a need for further data to identify factors relevant to the care of patients with CHD who contract COVID-19 illness., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

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.

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.

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.

Background: Coronary artery fistula (CAF) is a direct connection of one or more coronary arteries to cardiac chambers or a large vessel; it may be associated with congenital heart disease., Objective: To establish CAF pathway patterns from echocardiographic data and to correlate them with clinical aspects and congenital heart disease., Methods: A total of 7.183 medical records of children under the age of five years with cardiac disease submitted to color Doppler echocardiography and Spearman's Correlation test were used to associate signs and symptoms and cardiopathy to CAF with a significance level of 5%., Results: Twenty six children (0.0036%) presented CAF: from the right coronary artery (RCA) to the right ventricle (RV) 26.92%, from the left coronary artery (LCA) to the RV 23.08%, from the anterior interventricular branch (AIVB) to RV 23.08%, RCA to right atrium (RA) 11.54%, LCA for pulmonary trunk (PT) 7.69% or AIVB for PT 7.69%. In 57.69% of the patients, there was a positively correlated symptomatology to CAF with p=0.445 related to dyspnea or cyanosis (53.84%); in 96.15%, congenital heart disease associated with CAF, mainly interventricular communication (IVC) or interatrial communication (IAC) in 34.62% positively correlated to CAF with p=0.295. CAF pathway was represented in three dimensions by software modeling, texturing and animation Cinema 4D R19., Conclusion: CAF is an uncommon anatomical entity that presents a clinical picture compatible with dyspnea and cyanosis, and this is associated with congenital heart disease, mainly with IVC or IAC. According to echocardiographic analyzes, fistulas in RCA, LCA, or AIVB represent about one-third of the patients, with a priority pathway for right heart chambers.

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.

Background: Aiming at fostering local development of cardiology and cardiovascular surgery centers in developing countries, the nonprofit organization Children's HeartLink (CHL) encourages centers to participate in the International Quality Improvement Collaborative Database for Congenital Heart Disease (IQIC). The definition of parameters and data to evaluate patient treatment provides an opportunity to improve quality of care, reducing morbidity and mortality. The objective of the study was to analyze the outcomes of the partnership between CHL and IQIC database with a single pediatric cardiology and cardiovascular surgery center for seven years providing continuous follow-up to guide actions aiming at morbidity and mortality reduction in patients with pediatric and congenital heart diseases., Methods: Data were collected from January 2011 to December 2017 independently and with external audits and included preoperative information (demographic data, nutritional status, chromosomal abnormalities), Risk Adjustment for Congenital Heart Surgery (RACHS-1) score, and postoperative information such as infections or complications within the first 30 days or until hospital discharge and/or death., Results: In the preoperative period, there was a trend toward an increase in the number of newborn patients. The postoperative period showed significant surgical procedure variations between groups for RACHS-1 risk category ( P = .003), prevalence of risk categories 2 and 3, and an increase in risk categories 4, 5, and 6, mainly in the last two years. Decreases in surgical site infection ( P = .03), bacterial sepsis, and other infections (both P < .001) were observed. At the 30-day postoperative follow-up, there was a decrease of in-hospital ( P = .16) and 30-day ( P = .14) mortality., Conclusion: The partnership between CHL and this seven-year analysis of IQIC database demonstrated structural and human flaws, whose resolution led to significant decrease in infection and reduction in mortality despite an increase in the complexity of our pediatric and congenital heart disease population.

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.

Background: The nursing profession faces continuous transformations demonstrating the importance of professional continuing education to extend knowledge following technological development without impairing quality of care. Nursing assistants and technicians account for nearly 80% of nursing professionals in Brazil and are responsible for uninterrupted patient care. Extensive knowledge improvement is needed to achieve excellence in nursing care. The objective was to develop and evaluate a continuing education program for nursing technicians at a pediatric cardiac intensive care unit (PCICU) using a virtual learning environment entitled EDUCATE., Methods: From July to September 2015, a total of 24 nursing technicians working at the PCICU at a children's hospital located in the northwestern region of São Paulo state (Brazil) fully participated in the continuing education program developed in a virtual learning environment using Wix platform, allowing access to video classes and pre- and post-training theoretical evaluation questionnaires outside the work environment. The evaluation tools recorded participants' knowledge evolution, technological difficulties, educational, and overall rating., Results: Knowledge development was descriptively presented as positive in more than 66.7%. Content and training were considered "excellent" by most participants and 90% showed an interest in the use of technological resources. Technical difficulties were found and quickly resolved by 40% of participants including Internet access, login, and lack of technical expertise., Conclusion: The continuing education program using a virtual learning environment positively contributed to the improvement in theoretical knowledge of nursing technicians in PCICU.

Background: Little has been published regarding surgery for transposition of the great arteries (TGA) in the developing world., Objectives: This study sought to identify patient characteristics, surgical interventions, institutional characteristics, risk factors for mortality, and outcomes among patients undergoing surgery for TGA in this setting., Methods: Developing world congenital heart surgical programs submitted de-identified data to a novel international collaborative database as part of a quality improvement project. We conducted a retrospective cohort study that included all cases of TGA with intact ventricular septum and TGA with ventricular septal defect performed from 2010 to 2013. Demographic, surgical, and institutional characteristics and their associations with in-hospital mortality were identified., Results: There were 778 TGA operations performed at 26 centers, 480 (62%) for TGA with intact ventricular septum and 298 (38%) for TGA with ventricular septal defect. Most (80%) were single-stage arterial switch operations, but 20% were atrial baffling procedures (atrial switch operation) or 2-stage repairs (pulmonary artery band followed by arterial switch operation). Age at operation was >30 days in one-half of the cases and did not vary significantly with operation type. Survival was 85% and did not significantly vary with age at operation or operation type. Preceding septostomy was infrequently reported (16%) and was not associated with surgical mortality. Mortality was associated with lower World Health Organization weight/body mass index-for-age percentile and lower institutional volume of TGA repair., Conclusions: Surgical repair of TGA performed in the developing world is associated with an early survival of 85%. Type of surgical repair and age at operation varied considerably, but no associations with mortality were identified. In contrast, poor nutrition and small surgical volume were most strongly associated with mortality. Multicenter collaborative quality improvement efforts may benefit patients with TGA in the developing world., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Introduction: 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.

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 more of the operation.

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 within up to three months, but for the group of patients below normal developmental pattern of the return occurred within 12 months.

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.

Objective: To evaluate if the findings of multislice computed tomography (MSCT) are associated with clinical and laboratory tests routinely used in the late follow-up of children undergoing surgical treatment of total anomalous pulmonary venous connection (TAPVC)., Methods: From January 2002 to December 2007, 12 patients operated due to CATVP were evaluated with history, physical examination, chest X-ray, electrocardiogram, echocardiography and MSCT. Specific changes observed in each one of these tests were identified and compared with MSCT qualitative findings., Results: Eleven patients were in functional class I (NYHA), three had nonspecific murmurs, and three were below the 15th percentile of weight and height. Two had pulmonary field abnormalities and three had a slight increase of the cardiac area in the X-ray examination. In the electrocardiogram, one patient had right ventricular overload and one had junctional rhythm. All echocardiograms were within the normal range, except for one patient with stenosis between the superior vena cava and right atrium. MSCT was completely normal in four patients, three had compression of the pulmonary veins and four had significant caliber reduction, which correlated with the other findings. Thus, MSCT showed a sensitivity of 87.5%, specificity of 0.75%, positive predictive value of 87.5%, negative predictive value of 75% and accuracy of 83.3% to demonstrate anatomic changes compared to changes in the physical examination or other additional tests., Conclusion: MSTC may provide valuable information and complement the diagnosis of possible anatomical and functional changes in the late follow-up of patients undergoing surgical repair of TAPVC.

Congenital mitral valve malformations are rare, but are well known and described entities. Mitral valve malformations involve mitral valve apparatuses (leaflets and annulus) and subvalvar apparatuses (chordae and papillary muscle). Case reports of accessory mitral leaflets were already described, but were usually an appendix of the normal valve. We describe here a case report and present the images of a trileaflet mitral valve sustained by three papillary muscles in a young girl with subaortic stenosis., (© 2011 Copyright the Authors. Journal Compilation © 2011 Wiley Periodicals, Inc.)

Bacterial endocarditis is a severe infectious disease. of which treatment is traditionally carried out in hospitalized patients through intravenous medication. The possibility of at-home or ambulatory treatment. for stringently selected cases. is attractive from the social as well as from the economic point of view. We report 6 patients with a diagnosis of bacterial endocarditis caused by Streptococcus. treated partially or completely on an outpatient basis. All of them evolved without complications and presented complete resolution of the infection.

The professional activity that the cardiovascular surgeon performs is much more than a simple gesture to mechanically operate the patient's heart. There is in every act of intraoperative most notions of physiology and physics than we generally realize. This paper discusses, in the light of mathematics, on the dynamics of fluids, ie blood, focused on invasive measurements of blood pressure, the effect of vessel size on its internal resistance and the flow passing through it in conversion of various units of measurements of pressure and resistance, blood viscosity and its relationship to the vessel, hemodilution, differences in laminar and turbulent flow, velocity and blood pressure and wall tension after a stenosis and the origin of poststenotic aneurysm. This study is not to enable the reader to the knowledge of all physics, but to show it as a useful tool in explaining phenomena known in the routine of cardiovascular surgery.