ABSTRACT 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. (CHD 1000 1 000 1,00 births 23057 23 057 23,05 2022 life 81 3 age challenges Furthermore hospitals professionals technologies industry financing systems generation parties Kingdom reality research education outcomes model 100 00 1,0 2305 2 05 23,0 202 8 10 0 1, 230 23, 20

ABSTRACT Introduction: Risk Adjustment for Congenital Heart Surgery 1 (RACHS-1) score is a simple model that can be easily applied and has been widely used for mortality comparison among pediatric cardiovascular services. It is based on the categorization of several surgical palliative or corrective procedures, which have similar mortality in the treatment of congenital heart disease. Objective: To analyze the in-hospital mortality in pediatric patients (<18 years) submitted to cardiac surgery for congenital heart disease based on RACHS-1 score, during a 12-year period. Methods: A retrospective date analysis was performed from January 2003 to December 2014. The survey was divided in two periods of six years long each, to check for any improvement in the results. We evaluated the numbers of procedures performed, complexity of surgery and hospital mortality. Results: Three thousand and two hundred and one surgeries were performed. Of these, 3071 were able to be classified according to the score RACHS-1. Among the patients, 51.7% were male and 47.5% were younger than one year of age. The most common RACHS-1 category was 3 (35.5%). The mortality was 1.8%, 5.5%, 14.9%, 32.5% and 68.6% for category 1, 2, 3, 4 and 6, respectively. There was a significant increase in the number of surgeries (48%) and a significant reduction in the mortality in the last period analysed (13.3% in period I and 10.4% in period II; P=0.014). Conclusion: RACHS-1 score was a useful score for mortality risk in our service, although we are aware that other factors have an impact on the total mortality.

ABSTRACT The perspective of the integrated health system has a network of care with multiple integration dimensions among subsystems as nuclear representation, relating the clinical aspects and governance to the representations and collective values. The normative integration aims to ensure coherence between the system of representations and values of society simultaneously with the interfaces of clinical and functional integration. It builds a bridge with governance, which allows, through their skills, management of all system components, encouraging cooperation, communication and information, in order to ensure the population under their responsibility to access excellence services, exceeding their expectations. The integration of care consists of a durable coordination of clinical practices for those who suffer from health problems in order to ensure continuity and full range of the required professional services and organizations, coordinated in time and space, in accordance with the available knowledge. It is possible to establish the type of health equipment for each level of care for patients with congenital heart diseases. This strategy intends to offer timely care in appropriate moments and places, efficiently, operating cooperatively an interdependently, with ongoing exchange of its resources. Thus, situational integration establishes the system connection with the assessment environment that proposes to carry out value judgment, guided by an objective worldview, about an intervention or any of its components, in order to objectify the decision making.

ResumoIntrodução:Cardiopatia congênita é uma anormalidade na estrutura ou função cardiocirculatória, ocorrente desde o nascimento, mesmo que diagnosticada posteriormente. Pode resultar em morte intraútero, na infância ou na idade adulta. Foi responsável por 6% dos óbitos infantis, no Brasil, em 2007.Objetivo:Estimar a subnotificação na prevalência das cardiopatias congênitas no Brasil e seus subtipos.Métodos:Os cálculos das prevalências foram realizados aplicando-se coeficientes, atribuindo-lhes função de taxas para cálculos dos agravos. O estudo faz aproximação entre a literatura e os registros governamentais. Adotou-se estimativa de 9:1000 nascimentos e taxas de prevalências para subtipos, aplicadas aos nascimentos de 2010. As estimativas de nascimentos com cardiopatia congênita foram comparadas com as notificações ao Ministério da Saúde. Foram estudados por métodos descritivos com uso de taxas e coeficientes, representados em tabelas.Resultados:A incidência, no Brasil, é de 25.757 novos casos/ano, distribuídos em: Norte 2.758; Nordeste 7.570; Sudeste 10.112; Sul 3.329; e Centro-Oeste 1.987. Em 2010, foram notificados ao SINASC/MS 1.377 casos de nascidos com cardiopatias congênitas, o que representa 5,3% do estimado para Brasil. No mesmo período, os subtipos mais frequentes foram: comunicação interventricular (7.498); comunicação interatrial (4.693); persistência do canal arterial (2.490); estenose pulmonar (1.431); tetralogia de Fallot (973); coarctação da aorta (973); transposição das grandes artérias (887); e estenose aórtica 630. A prevalência de cardiopatias congênitas, para o ano de 2009, foi 675.495 crianças e adolescentes e 552.092 adultos.Conclusão:Há, no Brasil, subnotificação na prevalência das cardiopatias congênitas, sinalizando para a necessidade de adequações na metodologia de seu registro.

AbstractIntroduction:Congenital heart disease is an abnormality in the structure or cardiocirculatory function, occurring from birth, even if diagnosed later. It can result in intrauterine death in childhood or in adulthood. Accounted for 6% of infant deaths in Brazil in 2007.Objective:To estimate underreporting in the prevalence of congenital heart disease in Brazil and its subtypes.Methods:The calculations of prevalence were performed by applying coefficients, giving them function rates for calculations of health problems. The study makes an approach between the literature and the governmental registries. It was adopted an estimate of 9: 1000 births and prevalence rates for subtypes applied to births of 2010. Estimates of births with congenital heart disease were compared with the reports to the Ministry of Health and were studied by descriptive methods with the use of rates and coefficients represented in tables.Results:The incidence in Brazil is 25,757 new cases/year, distributed in: North 2,758; Northeast 7,570; Southeast 10,112; South 3,329; and Midwest 1,987. In 2010, were reported to System of Live Birth Information of Ministry of Health 1,377 cases of babies with congenital heart disease, representing 5.3% of the estimated for Brazil. In the same period, the most common subtypes were: ventricular septal defect (7,498); atrial septal defect (4,693); persistent ductus arteriosus (2,490); pulmonary stenosis (1,431); tetralogy of Fallot (973); coarctation of the aorta (973); transposition of the great arteries (887); and aortic stenosis 630. The prevalence of congenital heart disease, for the year of 2009, was 675,495 children and adolescents and 552,092 adults.Conclusion:In Brazil, there is underreporting in the prevalence of congenital heart disease, signaling the need for adjustments in the methodology of registration.

Este artigo discute os vários momentos da formulação da Política Nacional de Atenção Cardiovascular de Alta Complexidade, tendo como foco a atenção cardiovascular pediátrica. Processo iniciado pela necessidade de atender demandas locais, passa a ter dimensões nacionais pela constatação do déficit de atendimento aos portadores de cardiopatias congênitas (CC). Em 2002, a deficiência de procedimentos no Brasil era de 65%. Demonstrar-se-á a participação de vários agentes, da sociedade civil e do Poder Público, na formulação da política, fazendo-se uso de alguns autores na discussão para fundamentar cada momento do processo. Em 15 de junho de 2004, por meio da Portaria Nº 1169/GM, que instituía a Política Nacional de Atenção Cardiovascular de Alta Complexidade e, com a Portaria Nº 210 SAS/MS, na mesma data, a cirurgia cardiovascular pediátrica estava regulamentada. Destaca-se a importância da participação da sociedade civil na elaboração de políticas públicas sociais, sendo imperativa, para o aprofundamento das questões na área social, a intervenção de agentes que vivenciam as dificuldades.

The authors discuss the different moments of the National Policy of High-complexity Cardiovascular Care, focusing on the attention to Cardiovascular Pediatrics. This process begun with the attempt to meet the local needs, but, very soon, became a national matter due to the verification of a significant shortage in the attention given to patients with congenital cardiovascular diseases. In 2002 the shortage of procedures in these cases was around 65%. The participation of different agents from the civil society and the Government will be demonstrated regarding the formulation of the politics directed to the attention to cardiovascular pediatrics. Some authors are cited during the discussion to base each moment of the process. The National Policy of High-complexity Cardiovascular Care was regulated on June 15th, 2004, through the Ministerial Directive Nº1169/GM as well as the Pediatric Cardiovascular Surgery, on the same date, through the Ministerial Directive Nº 210 SAS/MS. The importance of the civil society participation in the elaboration of the public social politics is emphasized. The intervention of agents who experience the day-to-day difficulties is of the utmost importance to the better knowledge of questions involving the social area