Advocacy at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.

Recommendations for developing effective and safe paediatric and congenital heart disease services in low-income and middle-income countries: a public health framework.

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.

Sildenafil's Early, Late Impact on Ventricular Septal Repair: Older Children Using the Double Patch.

BACKGROUND: Delayed diagnosis in children with a ventricular septal defect (VSD) is common in low- and middle-income countries. Consequently, these children present with elevated pulmonary vascular resistance (PVR) and pulmonary arterial hypertension (PAH). The study investigators introduced the double-flap valve VSD patch closure technique (DFV) in 1996 to reduce early postoperative risk. Long-term results are presented in this report. METHODS: This was a retrospective single-institution study of patients who underwent DFV between May 1996 and July 2015. Beginning in 2005, all candidates for DFV received sildenafil preoperatively and postoperatively. Preoperative catheterization data and operative, postoperative, hospital, and follow-up data were analyzed. RESULTS: A total of 40 patients underwent the DFV procedure. Patients' demographics were comparable between the sildenafil and nonsildenafil groups. One of 39 patients (2.6%) was lost to follow-up. Early mortality was 2.5% (1 of 40), and late mortality was 2.6% (1 of 38). Sildenafil improved preoperative oxygen saturation, improved preoperative hemodynamics, and shortened postoperative ventilation time. In both groups, abnormal hemodynamic values improved with a 100% oxygen challenge. The median age at late follow-up was 26.3 years (interquartile range [25%, 75%], 20.9, 29.9 years), and the median time since operation was 19.2 years (interquartile range, 11.4, 22.7 years). Current discharge survival was 97.3%. A total of 18% of patients had severe PAH in late follow-up. Multivariate analysis revealed only a baseline PVR-to-systemic vascular resistance ratio of 0.8 or greater as a significant predictor of late severe PAH. CONCLUSIONS: Long-term follow-up demonstrated that 60% of the patients will achieve normal or nearly normal pulmonary artery pressures. Furthermore, the study demonstrated that sildenafil improves preoperative hemodynamics and postoperative management. Children with VSD, elevated PVR, and PAH should not be denied operation.

Perioperative complications in a paediatric cardiac surgery program with limited systemic resources.

BACKGROUND: The perioperative complications rate in paediatric cardiac surgery, as well as the failure-to-rescue impact, is less known in low- and middle-income countries. AIM: To evaluate perioperative complications rate, mortality related to complications, different patients' demographics, and procedural risk factors for perioperative complication and post-operative death. METHODS: Risk factors for perioperative complications and operative mortality were assessed in a retrospective single-centre study which included 296 consecutive children undergoing cardiac surgery. RESULTS: Overall mortality was 5.7%. Seventy-three patients (24.7%) developed 145 perioperative complications and had 17 operative mortalities (23.3%). There was a strong association between the number of perioperative complications and mortality - 8.1% among patients with only 1 perioperative complication, 35.3% - with 2 perioperative complications, and 42.1% - with 3 or more perioperative complications (p = 0.007). Risk factors of perioperative complications were younger age (odds ratio 0.76; (95% confidence interval 0.61, 0.93), previous cardiac surgery (odds ratio 3.5; confidence interval 1.33, 9.20), extracardiac structural anomalies (odds ratio 3.03; confidence interval 1.27, 7.26), concomitant diseases (odds ratio 3.23; confidence interval 1.34, 7.72), and cardiopulmonary bypass (odds ratio 6.33; confidence interval 2.45, 16.4), whereas the total number of perioperative complications per patient was the only predictor of operative death (odds ratio 1.89; confidence interval 1.06, 3.37). CONCLUSIONS: In a program with limited systemic resources, failure-to-rescue is a major contributor to operative mortality in paediatric cardiac surgery. Despite the comparable crude mortality, the operative mortality among patients with perioperative complications in our series was significantly higher than in the developed world. A number of initiatives are needed in order to improve failure-to-rescue rates in low- and middle-income countries.

Pediatric Cardiac Service Development Programs for Low- and Middle-Income Countries in Need of Improving or Initiating Local Services.

Pediatric cardiac services are deficient in most of the world. Various estimates are that between 80 and 90% of the world's children do not receive adequate cardiac care for their congenital or acquired heart disease. We began a modest effort in 1992 to assist in the development of pediatric cardiac services in low- and middle-Income countries (LMIC). Since then, we have provided services in 32 countries based on 3 distinctive development strategies, in order to meet the local needs for pediatric cardiac services. Our goal has always been to provide education, training and sufficient experience so that eventually we leave a site with a fully functional, independently operating pediatric cardiac service that is sustainable over time. The margin between success and failure is dependent upon a number of factors and we hope that this chapter will provide others with the tools for success.

Linking world bank development indicators and outcomes of congenital heart surgery in low-income and middle-income countries: retrospective analysis of quality improvement data.

OBJECTIVE: Many low-income and middle-income countries (LMICs) struggle to provide the health services investment required for life-saving congenital heart disease (CHD) surgery. We explored associations between risk-adjusted CHD surgical mortality from 17 LMICs and global development indices to identify patterns that might inform investment strategies. DESIGN: Retrospective analysis: country-specific standardised mortality ratios were graphed against global development indices reflective of wealth and healthcare investment. Spearman correlation coefficients were calculated. SETTING AND PARTICIPANTS: The International Quality Improvement Collaborative (IQIC) keeps a volunteer registry of outcomes of CHD surgery programmes in low-resource settings. Inclusion in the IQIC is voluntary enrolment by hospital sites. Patients in the registry underwent congenital heart surgery. Sites that actively participated in IQIC in 2013, 2014 or 2015 and passed a 10% data audit were asked for permission to share data for this study. 31 sites in 17 countries are included. OUTCOME MEASURES: In-hospital mortality: standardised mortality ratios were calculated. Risk adjustment for in-hospital mortality uses the Risk Adjustment for Congenital Heart Surgery method, a model including surgical risk category, age group, prematurity, presence of a major non-cardiac structural anomaly and multiple congenital heart procedures during admission. RESULTS: The IQIC registry includes 24 917 congenital heart surgeries performed in children<18 years of age. The overall in-hospital mortality rate was 5.0%. Country-level congenital heart surgery standardised mortality ratios were negatively correlated with gross domestic product (GDP) per capita (r=-0.34, p=0.18), and health expenditure per capita (r=-0.23, p=0.37) and positively correlated with under-five mortality (r=0.60, p=0.01) and undernourishment (r=0.39, p=0.17). Countries with lower development had wider variation in mortality. GDP per capita is a driver of the association between some other measures and mortality. CONCLUSIONS: Results display a moderate relationship among wealth, healthcare investment and malnutrition, with significant variation, including superior results in many countries with low GDP per capita. These findings provide context and optimism for investment in CHD procedures in low-resource settings.

Bidirectional Glenn for residual outflow obstruction in Tetralogy of Fallot.

BACKGROUND: Residual right ventricular outflow obstruction during Tetralogy of Fallot repair necessitates peri-operative revision often requiring trans-annular patch with its negative sequels. Bidirectional Glenn shunt in this setting reduces trans-pulmonary gradient to avoid revision. METHODS: Bidirectional Glenn shunt was added during Tetralogy repair in patients with significant residual obstruction. A total of 53 patients between January, 2011 and June, 2018 were included. Final follow-up was conducted in July, 2018. RESULTS: Mean age at operation was 5.63±3.1 years. Right to left ventricular pressure ratio reduced significantly (0.91±0.09 versus 0.68±0.05; p<0.001) after bidirectional Glenn, avoiding revision in all cases. Glenn pressures at ICU admission decreased significantly by the time of ICU discharge (16.7±3.02 versus 13.5±2.19; p<0.001). Pleural drainage ≥ 7 days was seen in 14 (26.4%) patients. No side effects related to bidirectional Glenn-like facial swelling or veno-venous collaterals were noted. Mortality was 3.7%. Discharge echocardiography showed a mean trans-pulmonary gradient of 32.11±5.62 mmHg that decreased significantly to 25.64±5 (p<0.001) at the time of follow-up. Pulmonary insufficiency was none to mild in 45 (88.2%) and moderate in 6 (11.8%). Mean follow-up was 36.12±25.15 months (range 0.5-90). There was no interim intervention or death. At follow-up, all the patients were in NYHA functional class 1 with no increase in severity of pulmonary insufficiency. CONCLUSION: Supplementary bidirectional Glenn shunt significantly reduced residual right ventricular outflow obstruction during Tetralogy of Fallot repair avoiding revision with satisfactory early and mid-term results.

Results of international assistance for a paediatric heart surgery programme in a single Ukrainian centre.

BACKGROUND: Surgery for CHD has been slow to develop in parts of the former Soviet Union. The impact of an 8-year surgical assistance programme between an emerging centre and a multi-disciplinary international team that comprised healthcare professionals from developed cardiac programmes is analysed and presented.Material and methodsThe international paediatric assistance programme included five main components - intermittent clinical visits to the site annually, medical education, biomedical engineering support, nurse empowerment, and team-based practice development. Data were analysed from visiting teams and local databases before and since commencement of assistance in 2007 (era A: 2000-2007; era B: 2008-2015). The following variables were compared between periods: annual case volume, operative mortality, case complexity based on Risk Adjustment for Congenital Heart Surgery (RACHS-1), and RACHS-adjusted standardised mortality ratio. RESULTS: A total of 154 RACHS-classifiable operations were performed during era A, with a mean annual case volume by local surgeons of 19.3 at 95% confidence interval 14.3-24.2, with an operative mortality of 4.6% and a standardised mortality ratio of 2.1. In era B, surgical volume increased to a mean of 103.1 annual cases (95% confidence interval 69.1-137.2, p<0.0001). There was a non-significant (p=0.84) increase in operative mortality (5.7%), but a decrease in standardised mortality ratio (1.2) owing to an increase in case complexity. In era B, the proportion of local surgeon-led surgeries during visits from the international team increased from 0% (0/27) in 2008 to 98% (58/59) in the final year of analysis. CONCLUSIONS: The model of assistance described in this report led to improved adjusted mortality, increased case volume, complexity, and independent operating skills.

Ethics in humanitarian efforts: giving due credit to the local team.

BACKGROUND: It has become increasingly apparent that only the truly effective humanitarian work emphasises empowering local practitioners. One problem, though, is that we are often seen as the "experts" who have come to "save" the children. This perception may adversely affect the confidence in the country's own providers. METHODS: Non-profit organisations performing paediatric heart surgery in developing countries were identified from two sources: the CTSnet "volunteerism" web page and an Internet search using the term "Pediatric Heart Surgery Medical Mission." The website of each organisation was reviewed, seeking a "purpose" or "mission" statement or summary of the organisation's work. A separate Internet search of news articles was performed. The top five articles were analysed for each organisation, and the findings are then analysed using the Principlist and Utilitarian ethical systems. RESULTS: A total of 10 separate non-profit organisations were identified. The websites of eight (80%) placed significant emphasis on the educational aspects of their work and/or on interaction with local professionals. However, of 43 news articles reviewed, reporters mentioned education of, or interaction with, local professionals in only 14 (33%), and four out of 10 organisations studied had no mention of the local providers in any article. CONCLUSIONS: Although non-profit organisations emphasise the teaching and programme-building aspects of their efforts, media reports largely focus on simpler and more emotional stories such as patient successes or large donations. Acknowledgement of the clinical and financial contributions of the host countries is both a duty following from the principle of justice and an important factor in long-term programme building.

Ethics in humanitarian efforts: when should resources be allocated to paediatric heart surgery?

BACKGROUND: In countries with ample resources, no debate exists as to whether heart surgery should be provided. However, where funding is limited, what responsibility exists to care for children with congenital heart defects? If children have a "right" to surgical treatment, to whom is the "duty" to provide it assigned? These questions are subjected to ethical analysis. METHODS: Examination is initially based on the four principles of medical ethics: autonomy, beneficence, non-maleficence, and justice. Consideration of beneficence and justice is expanded using a consequentialist approach. RESULTS: Social structures, including governments, exist to foster the common good. Society, whether by means of government funding or otherwise, has the responsibility, according to the means available, to assure health care for all based on the principles of beneficence, non-maleficence, and justice. In wealthy countries, adequate resources exist to fund appropriate treatment; hence it should be provided to all based on distributive justice. In resource-limited countries, however, decisions regarding provision of care for expensive or complex health problems must be made with consideration for broader effects on the general public. Preliminary data from cost-effectiveness analysis indicate that many surgical interventions, including cardiac surgery, may be resource-efficient. Given that information, utilitarian ethical analysis supports dedication of resources to congenital heart surgery in many low-income countries. In the poorest countries, where access to drinking water and basic nutrition is problematic, it will often be more appropriate to focus on these issues first. CONCLUSION: Ethical analysis supports dedication of resources to congenital heart surgery in all but the poorest countries.

Potential Deleterious Interactions between Certain Chemical Compounds and a Thermoplastic Polyurethane Heat Exchanger Membrane Oxygenator.

Extracorporeal membrane oxygenation (ECMO) has become a powerful tool in the race to reverse failure to rescue events. Rapid implementation set the stage for the advent of the 30-day wet-priming storage as a standard practice. A recent alert regarding methylene blue (MB) unidirectional leach from patient's circulation through the oxygenator thermoplastic polyurethane (TPU) heat-exchanger membrane into the heater-cooler unit (HCU) water bath led us to believe that despite reassurances, the reverse process might be possible. To that effect, we performed a pilot in vitro experiment. We tested three adult ECMO sets (Adult Quadrox iD Oxygenator, Getinge, Doral, FL) probing for the transfer of MB between the water bath of a Sarns Dual Heater Cooler (Terumo Corporation, Ann Arbor, MI) and the circuit stored wet-primed for 30 days. In each test, 1,500 mg of reconstituted MB (HiMedia, Mumbai, India) were added to the 7.5 L of water in the HCU, circulated for 6 hours on which the water lines were disconnected and the setup was stored for 30 days. The primed circuit was tested for MB transfer at days 0, 13, and 30 by means of optical density (OD) at 665 nm and 26.5°C. Transference of MB from the HCU water bath into the ECMO circuit could be detected as early as day 13 after setup, achieving significant values by day 30 (median OD .019 (.014-.021). Expected OD if no diffusion present: 0. The complete separation of water interfaces between the patient's circuit and the HCU water bath may prove to be more dogma than fact when certain chemical substances are used in conjunction with TPU membrane oxygenators. Whether the transfer of substances is due to chemical processes or molecular weight needs further evaluation. Meanwhile, the use of chemicals for the cleaning of the HCU should be mindful of potential noxious effects.