Challenges of Congenital Heart Surgery in Brazil: It is Time to Designate Pediatric Congenital Heart Surgery Subspecialty.

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.

Extracorporeal membrane oxygenation support in children with severe coronavirus disease-2019: A case series.

Background: The coronavirus disease-2019 (COVID-19) pandemic has predominantly affected the adult population, but with a significantly lower prevalence in children. Most pediatric patients with COVID-19 have mild course; however, a small number progressed to acute respiratory distress syndrome, hypoxemia, despite optimized conventional therapies. Thus, this study aimed to report a series of six cases of children with severe acute respiratory syndrome coronavirus 2 infection who were supported by extracorporeal membrane oxygenation (ECMO) due to refractory hypoxemic respiratory failure. Methods: This observational, retrospective, and descriptive study reported a series of cases. Data were retrospectively collected from the medical records of patients who were admitted to the Pediatric Cardiologic Intensive Care of Hospital Dr. Carlos Alberto Studart Gomes and Hospital Regional da Unimed, between March 1, 2020, and June 30, 2021. Sociodemographic, clinical, and laboratory data were analyzed. Findings: The median age was 1.8 years (range: 0.4-14.5 years), 66.7% were males, and weight varied from 13 to 110 kg. The mean time between the onset of symptoms and cannulation, ECMO duration, and ventilation time were 15 days (range: 6-24 days)], 11 days (range: 6-19 days), and 20.5 days (range: 14-33 days), respectively. Five (83.3%) children were successfully decannulated and four survived with hospital discharge. One child died on ECMO support due to multiple organ dysfunction syndromes after 13 days and another one died 3 days after decannulation due to extensive hemorrhagic stroke. Our case series revealed a 33.3% in-hospital mortality rate. ECMO appears as a viable intervention in selected patients who failed conventional therapies in the pediatric population. Funding: This observational study received no funding.

Analysis of Surgical Mortality for Congenital Heart Defects Using RACHS-1 Risk Score in a Brazilian Single Center.

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.

Proposal of an Integrated Health Care Network System for Patients with Congenital Heart Defects.

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.

Criss-cross heart: report of two cases, anatomic and surgical description and literature review.

Criss-cross heart is an extremely rare anomaly, characterized by an abnormal rotation of the ventricular mass along its major axis. It may be associated with any malformation of the heart segments and connections. Due to the complex structural changes and rarity of the anomaly, the rotation of ventricular axis is often misdiagnosed. In this paper, two cases of criss-cross heart are reported, with emphasis on diagnostic and surgical techniques used to corrected the main defects. A literature review on the subject is also presented which, although sparse, emphasized on the morphologic, diagnostic and surgical aspects of the anomaly.

Pulmonary artery banding: a simple procedure? A critical analysis at a tertiary center.

OBJECTIVE: Although pulmonary artery banding (PAB) seems to be a technically simple procedure it presents several peculiarities and is related to a significant morbidity and mortality. The aim of this study is to analyze the experience of a tertiary hospital on the PAB by assessing and correlating many aspects related to the procedure. METHODS: Between January 2000 and December 2008, 61 patients undergone PAB due to congenital heart disease with increased pulmonary blood flow at Messejana Heart Hospital were assessed as for mortality, complications, stay in mechanical ventilation and need for intensive care unit (ICU), use of vasoactive drugs, difficulties in the adjustment on the banding and reoperations. Some statistical analyzes were performed to compare the subgroups. RESULTS: In 46.8% of the patients the intended pressoric adjustment was not achieved and in 6.5% it was necessary another surgery to readjust the banding. The mean time of mechanical ventilation was 14.1+/-49.6 days and ICU 14.16+/-10.92 days. In 82.6% of the patients vasoactives drugs were administrated for 10.3+/-12.79 days. Severe complications were noted in 49.15% of patients and cardiac insufficiency was the most common one with an incidence of 44%. The mortality rate was 8.2% and it was not influenced by weight or associated procedures with the PAB neither if univentricular or biventricular heart disease. CONCLUSION: The PAB can be performed with acceptable mortality rates compatible with the ones of the world literature. Nevertheless, the adjustment of the banding is difficult to be assessed during the surgery by making the procedure complex and justifying the high incidence of complications and long stay in ICU. It wasn't found any specific risk factor significant to mortality neither uni- or biventricular heart disease.