Cardiovascular Risk Factors in Children and Adolescents with Fontan Circulation

Abstract Background: Long-term outcomes of patients with Fontan circulation are uncertain regarding the prevalence and role of risk factors (RFs) such as increased body mass index (BMI), arterial hypertension, and hypercholesterolemia. Objectives: To describe the prevalence of RFs in patients with univentricular heart, with variable follow-up times. Methods: This mixed cohort study was performed with 66 patients, who underwent blood count, fasting blood glucose, C-reactive protein (CRP), and lipid profile tests; systolic/diastolic blood pressure (SBP/DBP) measurements; and anthropometric and sociodemographic data collection. Cardiovascular RFs among first-degree relatives and physical activity habits were also assessed. Prevalence was described using proportions, with a 95% confidence interval. Continuous variables (height, weight, age, SBP, DBP) were described as means and standard deviations (m±SD). Associations between RFs were assessed using chi-squared or Fisher's exact tests. Spearman's correlation was used for analyzing CRP and the presence of 2 or more RFs. The Shapiro-Wilk test was used to check for data normality. Statistical significance considered p<0.05. Results: In our population, 19.7% were overweight, mean SBP was 89.44±37.4, and mean DBP was 60.0±26.08. The most prevalent diseases in the interviewees' families were systemic arterial hypertension (30.3%), obesity (16.7%), and 2 or more cardiovascular RFs among first-degree relatives (13.8%). We observed a trend towards significance between the presence of 2 familial RFs and overweight, as well as a risk profile for cardiovascular disease. There was an association between the BMI percentile, the presence of 2 or more RFs (p<0.05), and CRP (p<0.01). Conclusions: Overweight is common in patients with univentricular heart, being related to more than 2 cardiovascular RFs among first-degree relatives; physical inactivity and changes in lipid profiles are also frequent.

Uso Atual de Ressonância Magnética Cardíaca Pediátrica no Brasil / Current Use of Pediatric Cardiac Magnetic Resonance Imaging in Brazil

Resumo Fundamento Dados sobre o uso de ressonância magnética cardíaca (RMC) em crianças no Brasil são escassos. Objetivos Buscamos oferecer informações sobre as práticas atuais de RMC pediátricas no Brasil. Métodos Um questionário foi enviado a médicos solicitantes de RMC de todo o país, cobrindo informações sobre si próprios, sobre seus serviços de RMC, contexto clínico dos pacientes e sobre os obstáculos para a realização de RMC em crianças. Para a análise estatística, um p < 0,05 bilateral foi considerado significativo. Resultados A pesquisa obteve 142 respostas. Foi relatado que a RMC está disponível para 79% dos respondentes, dos quais 52% raramente ou nunca a utilizam. As indicações mais comuns são cardiomiopatias (84%), pós-operatório de correção de tetralogia de Fallot (81%) e malformações do arco aórtico (53%). A complexidade do exame se correlacionou à relação RMC/cirurgia (Rho = 0,48, IC 95% = 0,32-0,62, p < 0,0001) e ao número de exames de RMC (Rho = 0,52, IC 95% = 0,38-0,64, p < 0,0001). A complexidade da RMC esteve associada à sua realização por cardiologistas pediátricos (RC 2,04, IC 95% 1,2-3,89, p < 0,01). Os principais obstáculos ao uso mais frequente de RMC foram o alto custo (65%), a necessidade de sedação (60%) e o número insuficiente de profissionais qualificados (55%). Conclusão A RMC pediátrica não é usada frequentemente no Brasil. A presença de um cardiologista pediátrico a frente dos exames esteve associado ao uso de RMC em pacientes mais complexos. O treinamento de especialistas em RMC pediátrica e a educação dos médicos solicitantes são passos importantes na direção de um uso mais abrangente de RMC no Brasil. (Arq Bras Cardiol. 2021; 116(2):305-312)

Abstract Background Data on the use of cardiac magnetic resonance imaging (CMR) on children in Brazil is lacking. Objectives This study sought to provide information on current pediatric CMR practices in Brazil. Methods A questionnaire was sent out to referring physicians around the country. It covered information on the respondents, their CMR practices, the clinical context of the patients, and barriers to CMR use among children. For statistical analysis, two-sided p < 0.05 was considered significant. Results The survey received 142 replies. CMR was reported to be available to 79% of the respondents, of whom, 52% rarely or never use CMR. The most common indications were found to be cardiomyopathies (84%), status of post-tetralogy of Fallot repair (81%), and aortic arch malformations (53%). Exam complexity correlated with CMR-to-surgery ratio (Rho = 0.48, 95% CI = 0.32-0.62, p < 0.0001) and with the number of CMR exams (Rho = 0.52, 95% CI = 0.38-0.64, p < 0.0001). Further, a high CMR complexity score was associated with pediatric cardiologists conducting the exams (OR 2.14, 95% CI 1.2-3.89, p < 0.01). The main barriers to a more frequent use of CMR were its high cost (65%), the need for sedation (60%), and an insufficient number of qualified professionals (55%). Conclusion Pediatric CMR is not used frequently in Brazil. The presence of a pediatric cardiologist who can perform CMR exams is associated with CMR use on more complex patients. Training pediatric CMR specialists and educating referring providers are important steps toward a broader use of CMR in Brazil. (Arq Bras Cardiol. 2021; 116(2):305-312)

Clinical and Epidemiological Profiles of Patients Admitted to a Pediatric Cardiac Intensive Care Unit

Abstract Background: Congenital and acquired heart diseases are important causes of morbidity and mortality in children. In critical congenital heart defects, when treatment is not adequate, clinical manifestations may lead to death in the neonatal period. Objective: To establish the clinical and epidemiological profile of patients admitted to the pediatric cardiac intensive care unit (UTI) in a tertiary hospital. Methods: This was a cross-sectional study conducted from January 2013 to December 2014, based on analysis of patients' medical records. The study sample was composed of 307 children and adolescents with congenial and acquired heart diseases. The score Risk Adjustement for Congenital Heart Surgery 1 (RACHS-1) was used for categorization of the various surgical procedures. Descriptive statistics were calculated using the Satistical Package for Social Sciences (SPSS). Categorical variables were compared using the Pearson's chi-square test, considering a level of significance of 5%. Results: There was a predominance of patients aged between 28 days and one year (44%). Congenital heart diseases (91.9%) prevailed over acquired heart diseases (8.1%). Extracorporeal circulation was used in 138 patients who underwent surgical procedures, lasting from 12 to 261 minutes. Most patients (88.9%) were discharged from the ICU and 11.1% died. Using the score RACHS-1, corrective cardiac surgery was performed in 75.8% and paliative surgery in 24.2% of the patients. Conclusions: Patients aged between 28 days to one year, with cyanotic congenital heart disease, undergoing cardiac surgery with extracorporeal circulation duration longer than 120 minutes are at a higher risk of death.

Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation / Resultados da Conversão da Cirurgia de Fontan-Kreutzer para Cavo-Pulmonar Total Devido Falência da Circulação Univentricular

Abstract Background: The Fontan-Kreutzer procedure (FK) was widely performed in the past, but in the long-term generated many complications resulting in univentricular circulation failure. The conversion to total cavopulmonary connection (TCPC) is one of the options for treatment. Objective: To evaluate the results of conversion from FK to TCPC. Methods: A retrospective review of medical records for patients who underwent the conversion of FK to TCPC in the period of 1985 to 2016. Significance p < 0,05. Results: Fontan-type operations were performed in 420 patients during this period: TCPC was performed in 320, lateral tunnel technique in 82, and FK in 18. Ten cases from the FK group were elected to conversion to TCPC. All patients submitted to Fontan Conversion were included in this study. In nine patients the indication was due to uncontrolled arrhythmia and in one, due to protein-losing enteropathy. Death was observed in the first two cases. The average intensive care unit (ICU) length of stay (LOS) was 13 days, and the average hospital LOS was 37 days. A functional class by New York Heart Association (NYHA) improvement was observed in 80% of the patients in NYHA I or II. Fifty-seven percent of conversions due to arrhythmias had improvement of arrhythmias; four cases are cured. Conclusions: The conversion is a complex procedure and requires an experienced tertiary hospital to be performed. The conversion has improved the NYHA functional class despite an unsatisfactory resolution of the arrhythmia.

Resumo Fundamento: O procedimento de Fontan-Kreutzer (FK) foi amplamente realizado no passado, mas a longo prazo gerou muitas complicações, resultando em falha na circulação univentricular. A conversão para conexão cavopulmonar total (CCPT) é uma das opções de tratamento. Objetivo: Avaliar os resultados da conversão de FK para CCPT. Métodos: Revisão retrospectiva de prontuários de pacientes submetidos à conversão de FK para CCPT no período de 1985 a 2016. Significância p < 0,05. Resultados: Operações do tipo Fontan foram realizadas em 420 pacientes durante este período: CCPT foi realizada em 320, técnica de túnel lateral em 82 e FK em 18. Dez casos do grupo FK foram eleitos para conversão em CCPT. Todos os pacientes submetidos à conversão de Fontan foram incluídos neste estudo. Em nove pacientes, a indicação deveu-se a arritmia não controlada e em um devido à enteropatia perdedora de proteínas. A morte foi observada nos dois primeiros casos. O tempo médio de internação na unidade de terapia intensiva (UTI) foi de 13 dias e o tempo médio de internação hospitalar foi de 37 dias. Uma classe funcional pela melhora da New York Heart Association (NYHA) foi observada em 80% dos pacientes em NYHA I ou II. Cinquenta e sete por cento das conversões devido a arritmias tiveram melhora das arritmias; quatro casos foram curados. Conclusões: A conversão é um procedimento complexo e requer que um hospital terciário experiente seja realizado. A conversão melhorou a classe funcional da NYHA, apesar de uma resolução insatisfatória da arritmia.

Use of an Extracellular Matrix Patch for Sternal Wound Dehiscence after Cardiac Surgery in a Neonate.

Management of sternal wound dehiscence in newborns after cardiac operations can be a slow and lengthy process, during which the risk of progression to deep sternal wound infection and mediastinitis remains a concern. We report the case of a neonate born with single-ventricle physiology who underwent a Damus-Kaye-Stansel procedure as first-stage palliation toward creating Fontan circulation. The postoperative period was characterized by sterile wound dehiscence of the subcutaneous layers. We used a CorMatrix extracellular matrix patch as an adjunct to repair the wound defect. After 7 weeks, the wound had healed with excellent results. To our knowledge, this is the first report of extracellular matrix patch implantation for sternal wound reconstruction in a neonate.

Cardiopulmonary Resuscitation in Infants and Children With Cardiac Disease: A Scientific Statement From the American Heart Association.

Cardiac arrest occurs at a higher rate in children with heart disease than in healthy children. Pediatric basic life support and advanced life support guidelines focus on delivering high-quality resuscitation in children with normal hearts. The complexity and variability in pediatric heart disease pose unique challenges during resuscitation. A writing group appointed by the American Heart Association reviewed the literature addressing resuscitation in children with heart disease. MEDLINE and Google Scholar databases were searched from 1966 to 2015, cross-referencing pediatric heart disease with pertinent resuscitation search terms. The American College of Cardiology/American Heart Association classification of recommendations and levels of evidence for practice guidelines were used. The recommendations in this statement concur with the critical components of the 2015 American Heart Association pediatric basic life support and pediatric advanced life support guidelines and are meant to serve as a resuscitation supplement. This statement is meant for caregivers of children with heart disease in the prehospital and in-hospital settings. Understanding the anatomy and physiology of the high-risk pediatric cardiac population will promote early recognition and treatment of decompensation to prevent cardiac arrest, increase survival from cardiac arrest by providing high-quality resuscitations, and improve outcomes with postresuscitation care.

Avaliação do desempenho do PIM-2 entre pacientes cardiopatas cirúrgicos e correlação dos resultados com RACHS-1 / Assessment of PIM-2 performance among surgical patients with heart disease and correlation of results with RACHS-1

RESUMO Objetivo: Avaliar o desempenho do Pediatric Index of Mortality (PIM) 2 e do Escore de Risco Ajustado para Cirurgia Cardíaca Congênita (RACHS) no pós-operatório de cardiopatas congênitos. Métodos: Estudo transversal retrospectivo. Foram coletados dados de prontuário para gerar os escores e predições com as técnicas preconizadas, os dados demográficos e os desfechos. Para estatística, utilizaram-se o teste de Mann-Whitney, o teste de Hosmer-Lemeshow, a taxa de mortalidade padronizada, a área sobre a curva COR, qui quadrado, regressão de Poisson com variância robusta e teste de Spearman. Resultados: Foram avaliados 263 pacientes, e 72 foram a óbito (27,4%). Estes apresentaram valores de PIM-2 significativamente maiores que os sobreviventes (p < 0,001). Na classificação RACHS-1, a mortalidade foi progressivamente maior, de acordo com a complexidade do procedimento, com aumento de 3,24 vezes na comparação entre os grupos 6 e 2. A área abaixo da curva COR para o PIM-2 foi 0,81 (IC95% 0,75 - 0,87) e, para RACHS-1, de 0,70 (IC95% 0,63 - 0,77). A RACHS apresentou melhor poder de calibração na amostra analisada. Foi encontrada correlação significativamente positiva entre os resultados de ambos os escores (rs = 0,532; p < 0,001). Conclusão: A RACHS apresentou bom poder de calibração, e RACHS-1 e PIM-2 demonstraram bom desempenho quanto à capacidade de discriminação entre sobreviventes e não sobreviventes. Ainda, foi encontrada correlação positiva entre os resultados dos dois escores de risco.

ABSTRACT Objective: To assess the performance of the Pediatric Index of Mortality (PIM) 2 and the Risk Adjustment for Congenital Heart Surgery (RACHS) in the postoperative period of congenital heart disease patients. Methods: Retrospective cross-sectional study. Data were collected from patient records to generate the scores and predictions using recommended techniques, demographic data and outcomes. The Mann-Whitney test, Hosmer-Lemeshow test, standardized mortality rate, area under the receiver operating characteristic (ROC) curve, chi square test, Poisson regression with robust variance and Spearman's test were used for statistical analysis. Results: A total of 263 patients were evaluated, and 72 died (27.4%). These patients presented significantly higher PIM-2 values than survivors (p < 0.001). In the RACHS-1 classification, mortality was progressively higher according to the complexity of the procedure, with a 3.24-fold increase in the comparison between groups 6 and 2. The area under the ROC curve for PIM-2 was 0.81 (95%CI 0.75 - 0.87), while for RACHS-1, it was 0.70 (95%CI 0.63 - 0.77). The RACHS presented better calibration power in the sample analyzed. A significantly positive correlation was found between the results of both scores (rs = 0.532; p < 0.001). Conclusion: RACHS presented good calibration power, and RACHS-1 and PIM-2 demonstrated good performance with regard to their discriminating capacities between survivors and non-survivors. Moreover, a positive correlation was found between the results of the two risk scores.

The outcomes of superior cavopulmonary connection operation: a single center experience

Abstract Introduction: The superior cavopulmonary connection operation is one of the stages of the palliative surgical management for patients with functionally single ventricle. After surviving this stage, the patients are potential candidates for the final palliative procedure: the Fontan operation. Objectives: This study aimed to analyze the outcomes of superior cavopulmonary connection operations in our center and to identify factors affecting the survival and the progression to Fontan stage. Methods: The outcomes of 161 patients were retrospectively analyzed after undergoing superior cavopulmonary connection operation in our center between 2005 and 2015. Results: The early mortality rate was 2.5%. Five (3.1%) patients underwent takedown of the superior cavopulmonary connection. The rate of exclusion from the Fontan stage was 8.3%. Statistical analysis revealed that elevated mean pulmonary artery pressure preoperatively and the prior palliation with pulmonary artery banding were risk factors for both early mortality and takedown; however, the age, the morphology of the single ventricle and the type of operation were not considered risk factors. Conclusion: The superior cavopulmonary connection operation can be performed with low rate mortality and morbidity; however, the elevated mean pulmonary artery pressure preoperatively and the prior pulmonary artery banding are associated with poor outcomes.

Transposition of Great Arteries with Intramural Coronary Artery: Experience with a Modified Surgical Technique

Abstract Objective: Transposition of the great arteries is a common congenital heart disease. Arterial switch is the gold standard operation for this complex heart disease. Arterial switch operation in the presence of intramural coronary artery is surgically the most demanding even for the most experienced hands. We are presenting our experience with a modified technique for intramural coronary arteries in arterial switch operation. Methods: This prospective study involves 450 patients undergoing arterial switch operation at our institute from April 2006 to December 2013 (7.6 years). Eighteen patients underwent arterial switch operation with intramural coronary artery. The coronary patterns and technique used are detailed in the text. Results: The overall mortality found in the subgroup of 18 patients having intramural coronary artery was 16% (n=3). Our first patient had an accidental injury to the left coronary artery and died in the operating room. A seven-day old newborn died from intractable ventricular arrhythmia fifteen hours after surgery. Another patient who had multiple ventricular septal defects with type B arch interruption died from residual apical ventricular septal defect and sepsis on the eleventh postoperative day. The remainder of the patients are doing well, showing a median follow-up duration of 1235.34±815.26 days (range 369 - 2730). Conclusion: Transposition of the great arteries with intramural coronary artery is demanding in a subset of patients undergoing arterial switch operation. We believe our technique of coronary button dissection in the presence of intramural coronary arteries using coronary shunt is simple and can be a good addition to the surgeons' armamentarium.

Primary Prevention of Sudden Cardiac Death in Adults with Transposition of the Great Arteries: A Review of Implantable Cardioverter-Defibrillator Placement.

Transposition of the great arteries encompasses a set of structural congenital cardiac lesions that has in common ventriculoarterial discordance. Primarily because of advances in medical and surgical care, an increasing number of children born with this anomaly are surviving into adulthood. Depending upon the subtype of lesion or the particular corrective surgery that the patient might have undergone, this group of adult congenital heart disease patients constitutes a relatively new population with unique medical sequelae. Among the more common and difficult to manage are cardiac arrhythmias and other sequelae that can lead to sudden cardiac death. To date, the question of whether implantable cardioverter-defibrillators should be placed in this cohort as a preventive measure to abort sudden death has largely gone unanswered. Therefore, we review the available literature surrounding this issue.

Older Age at Completion of Fontan Procedure Is Associated with Improved Percentage of Predicted Maximum Oxygen Uptake.

We tested the hypothesis that later completion of the Fontan procedure is associated with improved exercise capacity in the current period of staged single-ventricle palliation. We performed a retrospective study, in Fontan patients, of exercise stress test data from April 2003 through March 2011. Patients were included if they had received staged palliations in accordance with current surgical strategy, defined as the performance of a superior cavopulmonary connection at ≤1 year of age, followed in subsequent years by Fontan completion. Patients with a pacemaker or respiratory exchange ratio <1 were excluded. Early and late Fontan groups were created on the basis of whether Fontan completion had been performed at <4 or ≥ 4 years of age. The primary predictor variable was age at Fontan completion, and the primary marker of exercise performance was the percentage of predicted maximum oxygen consumption. During the study period, 55 patients were identified (mean age, 11.7 ± 2.8 yr). Older age at Fontan completion correlated positively with higher percentages of predicted maximum oxygen consumption (R=0.286, P=0.034). Patients in whom Fontan completion was performed at ≥4 years of age had higher percentages of predicted maximum oxygen consumption than did those in whom completion was at <4 years of age (84.4 ± 21.5 vs 72.9 ± 18.1; P=0.041). Later Fontan completion might be associated with improved exercise capacity in patients palliated in accordance with contemporary surgical strategy.