ABSTRACT Introduction: Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome. Methods: Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute’s Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal. Results: Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality. Conclusion: The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon’s technical expertise and well-structured hospital facilities. Introduction CHDs (CHDs pathology development factors utero septum syndrome Methods metaaggregation meta aggregation Institutes Institute s or JBIQARI JBI QARI JBI-QARI appraisal Results 6 1 included Discussion pressure However rate mortality Conclusion welldescribed well described literature however surgeons surgeon wellstructured structured facilities

ABSTRACT Introduction: Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome. Methods: Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute’s Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal. Results: Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality. Conclusion: The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon’s technical expertise and well-structured hospital facilities.

ABSTRACT Introduction: Congenitally corrected transposition of the great arteries (CCTGA) is a rare anomaly. Current data available regarding adult cases is derived from small series, information simultaneously presented in pediatric publications, and one classical multicenter study. This review, not aimed to exhaust the subject, has the purpose to examine the literature addressing presentation, diagnostic methodology, and management of afflicted adult patients. Methods: A comprehensive search was undertaken in three major databases (PubMed, Cochrane, SciELO), using the keywords “congenitally corrected transposition of the great arteries” and “adults”. Relevant articles in English, Spanish, and Portuguese were extracted and critically appraised in this review. Steps for study selection were: (1) identification of titles of records through databases searching, (2) removal of duplicates, (3) screening and selection of abstracts, (4) final inclusion in the study. Results: Four hundred sixty-five publications on CCTGA in adult patients were retrieved, and 166 were excluded; 299 studies were used for this review including 76 full-text articles, 70 studies related to general aspects of the subject, and, due to the small number of publications, 153 case reports. Sixty-one articles referring to combined experiences in pediatric and adult patients and judged to be relevant, but retrieved from another sources, were also included. Conclusion: Albeit clinical presentation and diagnostic criteria have been well stablished, there seems to be room for discussion related to clinical and surgical management of CCTGA in adults. Considering the rarity of the disease, well designed multicenter studies may provide answers. Introduction (CCTGA anomaly series subject methodology Methods PubMed, PubMed (PubMed Cochrane SciELO, SciELO , SciELO) congenitally adults . “adults” English Spanish 1 (1 searching 2 (2 duplicates 3 (3 abstracts 4 (4 Results sixtyfive sixty five 16 excluded 29 7 fulltext full text 15 reports Sixtyone Sixty relevant sources included Conclusion stablished disease answers “adults (

Abstract Isolated left ventricular apical hypoplasia is a rare cardiomyopathy, with a broad range of clinical presentations. Since this entity was already described in association with osteomuscular diseases, mutation in the Lamin A/C gene has been regarded as a possible cause of this disease. This study describes the case of an asymptomatic teenager with isolated left ventricular apical hypoplasia and arthrogriposis but with no mutations in the entire Lamin A/C gene.

Abstract Introduction: Major adverse events (MAE) are unexpected but undesirably frequent after pediatric congenital heart surgery and contribute to poorer outcomes. The aim of this study was to test the predictive value of a ratio between central venous oxygen saturation and arterial lactate (ScvO2/lactate) for MAE after pediatric congenital heart surgery in a Brazilian university hospital. Methods: We conducted a retrospective observational study in a tertiary care university hospital, including 194 infants and children submitted to surgery for congenital heart disease. The predictive value of ScvO2, lactate, and ScvO2/lactate ratio were assessed by the area under the receiver operating characteristics curve (AUC), sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). Results: The incidence of MAE was 16% — cardiac arrest/death, unplanned reoperation, and low cardiac output syndrome were the most common events. Overall, ScvO2/lactate ratio discriminated patients with and without MAE very well (AUC 0.842), performing better than either variable alone, with sensitivity of 48%, specificity of 94%, PPV of 60%, and NPV of 91%. Conclusion: A ScvO2/lactate ratio > 5 can accurately identify patients at low risk of MAE after pediatric congenital heart surgery, with very good specificity and NPV, but poor sensitivity and PPV.

Resumo Fundamento A avaliação da artéria coronária após a cirurgia de Jatene ainda é um desafio clínico. Objetivo Correlacionar alterações anatômicas identificadas por tomografia computadorizada cardíaca (TCC) com alterações fisiológicas detectadas na avaliação clínica para diagnosticar obstrução coronária no pós-operatório tardio de pacientes submetidos à cirurgia de Jatene. Métodos Este estudo incluiu 61 pacientes consecutivos com idade média de 9,4 anos que foram submetidos à cirurgia de Jatene. Os pacientes realizaram ecocardiografia, eletrocardiografia, teste cardiopulmonar do exercício, e tomografia computadorizada cardíaca para avaliação da capacidade funcional e anatomia da artéria coronária. Resultados A tomografia computadorizada cardíaca revelou que somente 3,3% dos pacientes apresentaram estenose da artéria coronária. Esses pacientes eram assintomáticos, e não foram detectados sinais de isquemia miocárdicas pelos exames realizados. Conclusão A incidência de anormalidades da artéria coronária é de 3,3% no seguimento tardio de nossa coorte de pacientes submetidos à cirurgia de Jatene. Não existe uma diretriz clara sobre o porquê, quando, e como esses pacientes deveriam ser rastreados, ou o que propor quando pacientes assintomáticos forem diagnosticados com obstrução coronária.

Abstract Background Coronary artery evaluation remains after arterial switch operation a clinical challenge. Objective This study aims to correlate anatomical changes diagnosed by cardiac computed tomography (CCT) with physiological alterations on clinical evaluation to diagnose coronary obstruction in late ASO patients. Methods This study included 61 consecutive patients with mean age of 9.4 years who underwent ASO. The patients were submitted to echocardiography, electrocardiography, cardiopulmonary exercise test, and cardiac computed tomography to evaluate functional capacity and coronary artery anatomy. Results Cardiac computed tomography revealed that only 3.3% of the patients had coronary stenosis. These patients were asymptomatic, and no signs of myocardial ischemia were detected by the tests. Conclusion The incidence of coronary abnormalities in late ASO patients was 3.3% in our cohort. There is no clear guideline as to why, when, and how these patients should be screened or what to propose when a coronary obstruction is diagnosed in asymptomatic patients.

RESUMO Objetivos: validar o conteúdo e aparência da cartilha “A volta para casa após a cirurgia cardíaca da criança” e avaliar a aprendizagem cognitiva de familiares em seu uso. Métodos: estudo metodológico e quase-experimental do tipo antes e depois, com entrevista semiestruturada, pré-teste e leitura da cartilha no hospital; pós-teste e validação ocorreu após alta hospitalar. Utilizou-se estatística não paramétrica Wilcoxon. Resultados: participaram 19 familiares de crianças cardiopatas. A média de acertos aumentou 14 pontos percentuais do pré para o pós-teste; maioria dos erros sobre medicamentos. Verificou-se diferença significante (p<0,0001) na comparação entre conhecimento cognitivo prévio ao uso da cartilha e aprendizagem adquirida no uso desta. Todos os itens da cartilha receberam avaliação positiva, exceto o tamanho da letra. Conclusões: a cartilha contribui para o familiar apreender as necessidades de atenção à criança após cirurgia cardíaca, podendo ser usada para evitar ocorrências indesejadas e possibilitar um cuidado seguro no domicílio.

ABSTRACT Objectives: to validate the content and appearance of the booklet “Going home after a child’s cardiac surgery” and assess family members’ cognitive learning regarding its use. Methods: a methodological and quasi-experimental study of before and after type, with semi-structured interview, pre-test and reading of the booklet in a hospital; post-test and validation occurred after hospital discharge. Wilcoxon non-parametric statistics were used. Results: nineteen family members of children with heart disease participated. The average of correct answers increased 14 percentage points from pre- to post-test; most were medication errors. There was a significant difference (p <0.0001) in the comparison between cognitive knowledge prior to using the booklet and learning acquired from its use. All items received a positive rating, except font size. Conclusions: the booklet helps family members to understand a child’s needs after cardiac surgery, and can be used to prevent unwanted occurrences and enable safe care at home.

RESUMEN Objetivos: validar el contenido y apariencia del cuadernillo “El regreso a casa después de la cirugía cardíaca del niño” y evaluar el aprendizaje cognitivo de los familiares en su uso. Métodos: estudio metodológico y cuasi experimental del tipo antes y después, con entrevista semiestructurada, pretest y lectura del cuadernillo en el hospital; la prueba posterior y la validación se produjeron después del alta hospitalaria. Se utilizaron estadísticas no paramétricas de Wilcoxon. Resultados: participaron 19 familiares de niños con cardiopatías. El promedio de respuestas correctas aumentó 14 puntos porcentuales desde el preprueba hasta el posprueba; la mayoría de los errores de medicación. Hubo una diferencia significativa (p<0,0001) en la comparación entre el conocimiento cognitivo previo al uso del cuadernillo y el aprendizaje adquirido al usarlo. Todos los elementos del folleto recibieron una evaluación positiva, excepto el tamaño de fuente. Conclusiones: el folleto ayuda al familiar a comprender las necesidades de cuidado del niño después de la cirugía cardíaca, lo que puede usarse para prevenir incidentes no deseados y permitir un cuidado seguro en el hogar.

Abstract Objective: Description of adult congenital heart disease (CHD) outpatient characteristics has not been reported and several aspects regarding these patients require attention. We describe the 12-year experience of a Brazilian unit. Methods: The main characteristics of 1168 patients were reviewed annotating for each patient age, gender, city of residence, main diagnosis, functional class at last examination, defect complexity and in-hospital referral pattern. Results: Increasing workload was documented. Among the CHD patients, 663 (57%) were between 14 and 30 years old and 920 (79%) lived in the referral region. Referrals were made by hospital cardiologists for 611 (52%) patients, while 519 (45%) were referred by pediatric cardiologists. Regarding CHD severity, 637 (55%) had a defect of mild complexity. Of the patients analyzed, 616 (53%) had undergone an intervention, mainly atrial septal defect (ASD) closure, correction of tetralogy of Fallot, ventricular septal defect (VSD) closure and relief of coarctation of the aorta (CoAo). The main diagnosis of the 552 (47%) patients not submitted to an intervention were ASD, VSD, aortic stenosis, complex CHD and pulmonary stenosis. Regarding functional class, 1016 (87%) were in class I and 280 (24%) were lost to follow-up. Seventy-three patients had died, mainly due to cardiac death. Conclusion: In a unit were complex pediatric congenital heart surgery started twenty years ago, an increasing adult CHD workload was documented. Referral came predominantly from cities around the unit, most patients had low complexity defects and were in functional class I, a significant loss of follow-up was documented, and the death of patients was mainly due to the heart defect.

Abstract This article describes our proposal for routine anesthesia, intraoperative medical management, cerebral and physiological monitoring during pediatric cardiac surgery with cardiopulmonary bypass that intend to provide appropriate anesthesia (analgesia, hypnosis), neuroprotection, adequate cerebral and systemic oxygen supply, and preventing against drugs neurotoxicity. A concise retrospective data is presented.

Abstract Objective: ASSIST is the first Brazilian initiative in building a collaborative quality improvement program in pediatric cardiology and congenital heart disease. The purposes of this manuscript are: (a) to describe the development of the ASSIST project, including the historical, philosophical, organizational, and infrastructural components that will facilitate collaborative quality improvement in congenital heart disease care; (b) to report past and ongoing challenges faced; and (c) to report the first preliminary data analysis. Methods: A total of 614 operations were prospectively included in a comprehensive online database between September 2014 and December 2015 in two participating centers. Risk Adjustment for Congenital Heart Surgery (RACHS) 1 and Aristotle Basic Complexity (ABC) scores were obtained. Descriptive statistics were provided, and the predictive values of the two scores for mortality were calculated by multivariate logistic regression models. Results: Many barriers and challenges were faced and overcome. Overall mortality was 13.4%. Independent predictors of in-hospital death were: RACHS-1 categories (3, 4, and 5/6), ABC level 4, and age group (≤ 30 days, and 30 days - 1 year). Conclusion: The ASSIST project was successfully created over a solid base of collaborative work. The main challenges faced, and overcome, were lack of institutional support, funding, computational infrastructure, dedicated staff, and trust. RACHS-1 and ABC scores performed well in our case mix. Our preliminary outcome analysis shows opportunities for improvement.

ResumoDurante as últimas décadas, os avanços verificados no diagnóstico e tratamento das cardiopatias congênitas têm permitido que muitos indivíduos cheguem à idade adulta. Devido principalmente à grande diversidade diagnóstica e também às comorbidades habitualmente presentes nesse grupo etário, esses pacientes necessitam ser atendidos numa unidade multidisciplinar, se o objetivo for proporcionar uma assistência adequada. Neste trabalho revisamos, com base na experiência dos autores e na literatura internacional, as condições estruturais que devem estar disponíveis para esses pacientes. Procuramos ressaltar aspectos como as características da unidade, o critério usualmente adotado para transferência desses pacientes da unidade pediátrica, a composição das equipes médica e paramédica levando em consideração os problemas específicos dos pacientes e também o modelo de assistência ambulatorial e hospitalar. Enfatizamos, ainda, a importância do armazenamento dos dados dos pacientes, a necessidade fundamental de apoio institucional e a importância de oferecer treinamento profissional. A relevância da pesquisa clínica é também abordada, particularmente a importância da confecção de estudos multicêntricos, como uma metodologia apropriada para essa heterogênea população de pacientes.

AbstractDuring the last decades, advances in diagnosis and treatment of congenital heart disease have allowed many individuals to reach adulthood. Due mainly to the great diagnostic diversity and to the co-morbidities usually present in this age group, these patients demand assistance in a multidisciplinary facility if an adequate attention is aimed. In this paper we reviewed, based in the international literature and also on the authors’ experience, the structural conditions that should be available for these patients. We highlighted aspects like the facility characteristics, the criteria usually adopted for patient transfer from the paediatric setting, the composition of the medical and para- medical staff taking into account the specific problems, and also the model of outpatient and in-hospital assistance. We also emphasized the importance of patient data storage, the fundamental necessity of institutional support and also the compromise to offer professional training. The crucial relevance of clinical research is also approached, particularly the development of multicenter studies as an appropriate methodology for this heterogeneous patient population.

INTRODUÇÃO: Os autores relatam a experiência inicial da oclusão da comunicação interatrial ostium secundum (CIA) com a utilização da prótese Memopart® (Shanghai Shape Memory Alloy Co Ltd, Shanghai, China). MÉTODOS: Estudo prospectivo observacional, no qual uma série de pacientes portadores de defeitos com significativa repercussão hemodinâmica e características anatômicas favoráveis ao implante foi submetida à oclusão percutânea de CIA. O procedimento foi realizado por via femoral percutânea, pela técnica habitual. O período de seguimento foi de 10,3 ± 5 meses, com controles clínicos e ecocardiográficos 24 horas (ou antes da alta hospitalar), 1, 3, 6 e 12 meses após o implante. RESULTADOS: No período de fevereiro de 2012 a abril de 2013, foram submetidos à oclusão percutânea de CIA 21 pacientes, sendo 16 do sexo feminino, com idade média de 33,1 ± 18,7 anos. O diâmetro médio do defeito foi de 19,04 ± 6,25 mm e o tamanho da prótese foi de 21,42 ± 6,73 mm (8 a 34 mm). O implante foi realizado com êxito em todos os casos, verificando-se oclusão total no controle antes da alta hospitalar. No seguimento, todos os pacientes estiveram assintomáticos e comprovou-se a persistência da oclusão total do defeito. Não houve mortalidade e nem outras complicações na série. CONCLUSÕES: A oclusão percutânea da CIA utilizando-se prótese Memopart® é um procedimento eficaz e seguro, dentro dos limites desta investigação. O implante da prótese é simples e apresenta alto índice de oclusão imediata, inclusive de defeitos de grandes dimensões.

BACKGROUND: The authors report their initial experience with the Memopart™ device (Shanghai Shape Memory Alloy Co Ltd, Shanghai, China) for the occlusion of secundum atrial septal defect (ASD). METHODS: This was a prospective observational study of a series of patients undergoing percutaneous occlusion of ASD with right ventricle volume overload and favorable anatomic characteristics. The procedure was performed by percutaneous femoral approach. The mean follow-up was 10.3 ± 5 months, with clinical and 24-hour echocardiographic evaluations (or before hospital discharge), 1, 3, 6, and 12 months after implantation. RESULTS: From February/2012 to April/2013, 21 patients, 16 females, mean age 33.1 ± 18.7 years, were submitted to percutaneous occlusion of an ASD. The average diameter of the defect was 19.04 ± 6.25 mm and the device size was 21.42 ± 6.73 mm (8 to 34 mm). Total occlusion of the defect was observed in all cases before hospital discharge. During follow-up, all patients were asymptomatic and without residual shunt. There was no deaths or any other complications in the series. CONCLUSIONS: The percutaneous closure of ASD using a Memopart™ device is an effective and safe procedure within the limits of this investigation. The device is user-friendly and has a high rate of immediate occlusion, even in large defects.

OBJETIVO: Relatar a experiência da instituição com o tratamento cirúrgico de adultos com cardiopatia congênita devido ao crescente aumento no número desses pacientes e consequentes necessidades de maior discussão do tema. MÉTODOS: Análise retrospectiva dos pacientes operados, com análise de dados demográficos, fatores de risco e resultados. RESULTADOS: Cento e noventa e um pacientes, com idade entre 16 e 74 anos, foram operados. Cirurgia primária foi realizada em 171 pacientes, 93 (55%) com comunicação interatrial. Dentre 20 (12%) reoperações, substituição de valva pulmonar ocorreu em seis casos. Os tempos médios de unidade de terapia intensiva e hospitalar foram 2,7 e 8,5 dias, respectivamente, maiores nas reoperações (P=0,001). Os tempos médios de circulação extracorpórea e pinçamento aórtico foram 68,6 e 44,7 minutos, respectivamente, maiores nas reoperações (P<0,0001 e P=0,0003, respectivamente). Mortalidade hospitalar foi 4,2%, sem relação com reoperação. Sexo masculino, classe funcional III-IV e idade avançada foram fatores preditivos de risco. Complicações importantes foram mais frequentes nas reoperações (P<0,003), principalmente fibrilação e flutter atrial. Entre 183 pacientes com alta hospitalar, 149 (82%) foram seguidos e a fibrilação e flutter atrial foram bastante prevalentes. O valor médio da classe funcional mudou significativamente após a cirurgia (1,66 para 1,11; P<0,0001). A estimativa de sobrevida geral foi de 96,2% em seis anos. CONCLUSÃO: Cirurgia em adultos com cardiopatia congênita pode ser realizada com baixa mortalidade e melhora funcional na maioria dos pacientes. Complicações imediatas e tardias são frequentes. Estudos multicêntricos são importantes para melhor caracterização dessa população de indivíduos no país.

OBJECTIVE: To report the institution experience with the surgical treatment of adults with congenital heart disease due to the increasing number of these patients and the need for a better discussion of the subject. METHODS: Retrospective analysis describing demographic data, risk factors and results. RESULTS: 191 patients between 16 and 74 years old were operated on. Primary correction was done in 171 cases, 93 (55%) for atrial septal defect repair. Among 20 (12%) reoperations, pulmonary valve replacement was done in six cases. The mean intensive care and hospital stay were 2.7 and 8.5 days respectively, significantly greater for the reoperated cases (P=0.001). The mean bypass and clamping times were 68.6 and 44.7 minutes respectively, greater for the reoperated cases (P<0.0001 and P=0.0003 respectively). Hospital mortality was 4.2% and male sex, functional class III-IV and older age at operation were predictive risk factors. Significant complications were more frequent in the reoperated cases (P<0.003), mainly atrial flutter and fibrillation. Among 183 patients discharged, 149 (82%) are being followed and atrial flutter and fibrillation are common. The mean functional class value improved significantly after operation (1.66 to 1.11; P<0.0001). The estimated survival was 96.2% in six years. CONCLUSION: Heart surgery in adults with congenital heart disease can be accomplished with low mortality and functional class improvement. Immediate and late complications are frequent. Multicenter studies are important to better characterize this patient population in the country.

FUNDAMENTO: Experiências de serviços em adultos com cardiopatias congênitas não têm sido relatadas no nosso meio. OBJETIVO: Descrever o perfil clínico básico de adultos com cardiopatias congênitas atendidos ambulatorialmente em centro terciário. MÉTODOS: Anotaram-se dados referentes a idade, sexo, procedência, diagnóstico principal e diagnósticos secundários de 413 pacientes atendidos durante sete anos. RESULTADOS: G1 (não tratados): 195 pacientes, 51% mulheres, 57% entre 14 e 30 anos, 80% residentes na região. As cardiopatias mais frequentes foram comunicação interventricular (CIV) (31%), comunicação interatrial (CIA) (29%) e estenose pulmonar (7%). Os diagnósticos secundários predominantes foram hipertensão arterial (9%) e arritmias (5%). G2 (tratados): 218 pacientes, 56% mulheres, 57% entre 14 e 30 anos, 81% residentes na região. As cardiopatias mais frequentemente tratadas foram CIA (36%), tetralogia de Fallot (14%), coarctação da aorta (12%) e CIV (11%). Sessenta e nove (32%) pacientes foram operados na idade adulta. Dezesseis (7%) foram submetidos a um cateterismo intervencionista. Os diagnósticos secundários predominantes foram hipertensão arterial (18%) e arritmias (8%). CONCLUSÃO: Na casuística, predominaram pacientes tratados invasivamente, residentes na região e a maioria com idade abaixo de 40 anos. Defeitos como CIA, CIV e estenose pulmonar predominaram no grupo não tratado, ao passo que, nos tratados, a maioria tinha sido submetida à correção de CIA, tetralogia de Fallot, coarctação da aorta e CIV. Hipertensão arterial e arritmias foram relevantes em ambos os grupos, sendo também registrada grande diversidade de outras comorbidades.

BACKGROUND: Service experiences for adults with congenital heart disease have not been reported in our country. OBJECTIVE: To describe the basic clinical profile of adults with congenital heart disease in an outpatient tertiary care center. METHODS: We compiled data on age, gender, place of residence, primary diagnosis, and secondary diagnoses of 413 patients treated for seven years. RESULTS: G1 (untreated): 195 patients, 51% women, 57% between 14 and 30 years, 80% living in the region. The most frequent heart diseases were ventricular septal defect (VSD) (31%), atrial septal defect (ASD) (29%), and pulmonary stenosis (7%). The predominant secondary diagnoses were hypertension (9%) and arrhythmias (5%). G2 (treated): 218 patients, 56% women, 57% between 14 and 30 years, 81% living in the region. The most frequently treated heart diseases were: ASD (36%), tetralogy of Fallot (14%), coarctation of the aorta (12%), and VSD (11%). Sixty-nine (32%) patients were operated on for congenital heart diseases in adulthood. Sixteen (7%) underwent an interventional catheterization. The predominant secondary diagnoses were hypertension (18%) and arrhythmias (8%). CONCLUSION: In the study, most patients were treated invasively, all of them were residents in the region, and most of them were under 40 years of age. Defects such as ASD, VSD, and pulmonary stenosis predominated in the untreated group, whereas in the treated group, most patients had undergone surgical correction of ASD, tetralogy of Fallot, aortic coarctation, and VSD. Hypertension and arrhythmias were relevant in both groups, and a large variety of other comorbidities were also observed.

OBJETIVO: Avaliar se a aprotinina em altas doses hemostáticas pode reduzir o processo inflamatório após circulação extracorpórea (CEC) em crianças. MÉTODOS: Estudo prospectivo randomizado em crianças de 30 dias a 4 anos de idade, submetidas à correção de cardiopatia congênita acianogênica, com CEC e divididas em dois grupos, um denominado Controle (n=9) e o outro, Aprotinina (n=10). Neste, o fármaco foi administrado antes e durante a CEC. A resposta inflamatória sistêmica e disfunções hemostática e multiorgânicas foram analisadas por marcadores clínicos e bioquímicos. Foram consideradas significantes as diferenças com P<0,05. RESULTADOS: Os grupos foram semelhantes quanto às variáveis demográficas e intra-operatórias, exceto por maior hemodiluição no Grupo Aprotinina. Não houve benefício quanto aos tempos de ventilação pulmonar mecânica, permanência no CTIP e hospitalar, nem quanto ao uso de inotrópicos e função renal. A relação PaO2/FiO2 (pressão parcial de oxigênio arterial/fração inspirada de oxigênio) apresentou queda significativa com 24 h pós-operatório, no Grupo Controle. As perdas sanguíneas foram semelhantes nos dois grupos. No grupo Aprotinina surgiu leucopenia significativa, em CEC, seguida de leucocitose. Fator de necrose tumoral alfa (TNF-α), Interleucinas (IL)-6, IL-8, IL-10, proporção IL-6/IL-10 não apresentaram diferenças marcantes intergrupos. A proporção IL-6/IL-10 PO aumentou no grupo Controle. Não houve complicações com o uso da aprotinina. CONCLUSÃO: Nesta casuística, a Aprotinina em altas doses hemostáticas não minimizou as manifestações clínicas e os marcadores séricos de resposta inflamatória sistêmica.

OBJECTIVE: To assess if the hemostatic high-dose aprotinin is able to reduce the inflammatory process after cardiopulmonary bypass (CPB) in children. METHODS: A prospective randomized study was performed on children aged 30 days to 4 years who underwent correction of acyanogenic congenital heart disease with CPB and were divided into two groups: Control (n=9) and Aprotinin (n=10). In the Aprotinin Group the drug was administered before and during CPB and the systemic inflammatory response and hemostatic and multiorgan dysfunctions were assessed through clinical and biochemical markers. Differences were considered to be significant when P<0.05. RESULTS: The groups were similar regarding demographic and intraoperative variables, except for a greater hemodilution in the Aprotinin Group. The drug had no benefit regarding time of mechanical pulmonary ventilation, staying in the postoperative ICU and length of hospitalization, or regarding the use of inotropic drugs and renal function. The partial arterial oxygen pressure/ inspired oxygen fraction ratio (PaO2/FiO2) was significantly reduced 24 h after surgery in the Control Group. Blood loss was similar for both groups. Significant leukopenia was observed in the Aprotinin Group during CPB, followed by leukocytosis. Tumor necrosis factor alpha (TNF- α), interleukins (IL)-6, IL-8, IL-10, IL-6/IL-10 ratio did not differ significantly between groups. The postoperative IL-6/IL-10 ratio increased significantly in the Control Group. There were no complications with the use of aprotinin. CONCLUSION: In this study, hemostatic high-dose aprotinin did not minimize the clinical manifestations or serum markers of the inflammatory systemic response.