Tertiary prevention and treatment of rheumatic heart disease: a National Heart, Lung, and Blood Institute working group summary.

Although entirely preventable, rheumatic heart disease (RHD), a disease of poverty and social disadvantage resulting in high morbidity and mortality, remains an ever-present burden in low-income and middle-income countries (LMICs) and rural, remote, marginalised and disenfranchised populations within high-income countries. In late 2021, the National Heart, Lung, and Blood Institute convened a workshop to explore the current state of science, to identify basic science and clinical research priorities to support RHD eradication efforts worldwide. This was done through the inclusion of multidisciplinary global experts, including cardiovascular and non-cardiovascular specialists as well as health policy and health economics experts, many of whom also represented or closely worked with patient-family organisations and local governments. This report summarises findings from one of the four working groups, the Tertiary Prevention Working Group, that was charged with assessing the management of late complications of RHD, including surgical interventions for patients with RHD. Due to the high prevalence of RHD in LMICs, particular emphasis was made on gaining a better understanding of needs in the field from the perspectives of the patient, community, provider, health system and policy-maker. We outline priorities to support the development, and implementation of accessible, affordable and sustainable interventions in low-resource settings to manage RHD and related complications. These priorities and other interventions need to be adapted to and driven by local contexts and integrated into health systems to best meet the needs of local communities.

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.

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.

The global unmet need of congenital cardiac care: a quantitative analysis of the global burden of disease.

BACKGROUND: CHDs are one of the most frequent congenital malformations, affecting one in hundred live births. In total, 70% will require treatment in the first year of life, but over 90% of cases in low- and middle-income countries receive no treatment or suboptimal treatment. As a result, CHDs are responsible for 66% of preventable deaths due to congenital malformations in low- and middle-income countries. This study examines the unmet need of congenital cardiac care around the world based on the global burden of disease. MATERIALS AND METHODS: CHD morbidity and mortality data for 2006, 2011, and 2016 were collected from the Institute for Health Metrics and Evaluation Global Burden of Disease Results Tool and analysed longitudinally to assess trends in excess morbidity and mortality. RESULTS: Between 2006 and 2016, a 20.7% reduction in excess disability-adjusted life years and 20.6% reduction in excess deaths due to CHDs were observed for children under 15. In 2016, excess global morbidity and mortality due to CHDs remained high with 14,788,418.7 disability-adjusted life years and 171,761.8 paediatric deaths, respectively. In total, 90.2% of disability-adjusted life years and 91.2% of deaths were considered excess. CONCLUSION: This study illustrates the unmet need of congenital cardiac care around the world. Progress has been made to reduce morbidity and mortality due to CHDs but remains high and largely treatable around the world. Limited academic attention for global paediatric cardiac care magnifies the lack of progress in this area.

Cardiac surgical missions: what works, what does not, where we need to go from here.

PURPOSE OF REVIEW: Although overall neonatal and infant mortality continues to decrease around the world, years of life lost to congenital heart disease (CHD) rank among the top 10 leading causes for that age group in Latin America, Central Asia, Africa and the Middle East. Short-term surgical missions (STSMs) continue to be the only resource available in some areas of the world. RECENT FINDINGS: There is a need for better communications between charity organizations and the supported institutions. We observe a raising concern with the limited published information on the results of the STSMs and a call for patient safety, the use of international databases and accountability.Funding and human resources in low-income and middle-income countries (LMIC) are a persistent problem and a shift toward long-term collaboration rather than short-term visits opens the path to sustainability.The cost-effectiveness of STSMs treating congenital heart disease in LMIC seems to be very high whereas the management of adults with CHD and rheumatic valvulopathies appear as a growing concern. SUMMARY: Pediatric cardiac surgery STSMs continue to deserve full support and funding, long-term commitments, accountability and coordinated international funding are needed to achieve global sustainable coverage.

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.

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: 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.

Establishing Sustainable Cardiothoracic Surgery Programs in Underserved Countries.

Congenital heart disease is the most common birth defect worldwide, and accounts for a high proportion of the world's infant mortality. About 9 of every 10 babies born each year are born in areas without adequate access to heart surgery; overcoming this problem will necessitate addressing the worldwide shortage of an estimated 3,700 pediatric cardiac surgeons. Establishing sustainable heart surgery programs requires more than an investment of money: political, social, and cultural issues unique to each environment need to be addressed. Organizations desiring to help develop cardiac surgical centers need to focus on communication and bidirectional education, and to make a long-term commitment to each site. By identifying and addressing obstacles, success rates are high.

Cost-effectiveness of Humanitarian Pediatric Cardiac Surgery Programs in Low- and Middle-Income Countries.

Importance: Endorsement of global humanitarian interventions is based on either proven cost-effectiveness or perceived public health benefits. The cost-effectiveness and long-term benefits of global humanitarian pediatric cardiac surgery are unknown, and funding for this intervention is insufficient. Objectives: To determine the cost-effectiveness of the intervention (multiple 2-week-long humanitarian pediatric cardiac surgery program assistance trips to various low- and middle-income countries [LMICs]) and to produce a measure of the long-lasting effects of global humanitarian programs. Design, Setting, and Participants: International, multicenter cost-effectiveness analysis of a cohort of children (aged <16 years) undergoing surgical treatment of congenital heart disease during 2015 in LMICs, including China, Macedonia, Honduras, Iran, Iraq, Libya, Nigeria, Pakistan, Russia, and Ukraine. The study also assessed estimated improvement in the United Nations Human Development Indicators (life expectancy, years of schooling, and gross national income) for each individual survivor, as a proxy for long-term benefits of the intervention. Main Outcomes and Measures: The primary outcome was cost-effectiveness of the intervention. The secondary outcomes were potential gains in life expectancy, years of schooling, and gross national income per capita for each survivor. Results: During 2015, 446 patients (192 [43%] female; mean [SD] age, 3.7 [5.4] years) were served in 10 LMICs at an overall cost of $3 210 873. Of them, 424 were children. The cost-effectiveness of the intervention was $171 per disability-adjusted life-year averted. Each survivor in the cohort (390 of 424) potentially gained 39.9 disability-adjusted life-years averted, 3.5 years of schooling, and $159 533 in gross national income per capita during his or her extended lifetime at purchasing power parity and 3% discounting. Conclusions and Relevance: Humanitarian pediatric cardiac surgery in LMICs is highly cost-effective. It also leaves behind a lasting humanitarian footprint by potentially improving individual development indices.

Use of extracorporeal membrane oxygenation for adults in cardiac arrest (E-CPR): a meta-analysis of observational studies.

Published data on the use of extracorporeal membrane oxygenation (ECMO) as a supportive measure during or immediately after cardiopulmonary resuscitation (CPR) in adults (older than 18 years) shows mixed results. To assess the clinical outcomes of the use of ECMO in this modality and to look for predictors of mortality, we performed a meta-analysis (MA) of individual patients collected from observational studies. An electronic PubMed search restricted to English-language publications between 1990 and 2007, using a consensus restrictive criterion, retrieved 141 titles. After full text evaluation, 11 clinical series and nine case reports were considered appropriate and included in our MA. Data on 135 individually identified adult patients (male:female = 1.6:1) were collected. Median age for the group was 56 years (range 18-83), and the median ECMO run was 54 hours (range 0-3881). Overall survival to hospital discharge was 40% (54 of 135). The most common diagnosis leading to cardiac arrest was acute myocardial infarction (46 of 135 patients). Compared with the youngest group (17-41 years), odds ratio (OR) for mortality was higher for age group 41-56 years (OR 2.9 95%; CL, 1.6-8.2) and those older than 67 years (OR 3.4%; 95% CL, 1.2-9.7). Duration of ECMO support measured in days was also a predictor of mortality, with significant better outcome for those supported between 0.875 and 2.3 days (OR 0.2; 95% CL, 0.07-0.6). There was a negative trend in survival when manual CPR lasted >30 minutes without prompt ECMO initiation (OR 1.9; 95% CL, 0.9-4.2). This work confirms the expectations for a better survival when E-CPR is used in younger patients, for shorter periods of time and after expeditious implementation during or immediately after manual CPR. Neurologic sequelae and other major complications, although suspected to be high, are poorly described in the reviewed literature.

Dissecting multidisciplinary cardiac surgery rounds.

BACKGROUND: Multidisciplinary rounds in the critical care environment have demonstrated increased communication, a reduction in medical errors, a shorter hospital stay, and consequently, economic savings. We attempt to assess the cost of this intervention, and to review the time utilization of professionals participating in the process. METHODS: We analyzed video-recorded weekly multidisciplinary teaching rounds on cardiac patients in a pediatric intensive care unit (n = 22). Rounding time was categorized as presentation or discussion and was measured in minutes. The cost of a round was calculated by multiplying the hourly salary of all healthcare professionals present by the time spent rounding and measured in US dollars. RESULTS: Median rounding time per patient was 15 minutes (range, 5 to 29). Patient presentation took between 2 and 8 minutes (median 4), or 26% of the rounding time. Time needed for discussion, including teaching and planning, varied between 2 and 25 minutes (median 10.5). Median number of participants was 13.5 (range, 11 and 16). Mean cost in salaries per patient rounded was $140.87 (95% confidence interval: $106.80 to $174.90). CONCLUSIONS: Multidisciplinary rounds are a low-cost medical intervention with proven benefits. Available tools and rounding cultural changes should be adopted to shorten data retrieval and presentation time to the benefit of discussion and teaching. Current billing requirements for rounding multidisciplinary teams do not reflect the realities of their time use.

Berlin heart as a bridge to recovery for a failing Fontan.

We report the temporary use of a Berlin Heart ventricular assist device (Berlin Heart AG, Berlin, Germany) for cardiac support of an 18-month-old girl with rapidly progressive ventricular failure after completion of a fenestrated Fontan. After 6 months of cardiac assistance with a single pneumatic pump, catheterization data showed improvement of the ventricular function and the ventricular assist device was successfully removed. A follow-up echocardiogram 6 months after hospital discharge demonstrated marked improvement of ventricular function.

Development of an observational tool on computer use during rounds.

We present an observational tool to capture computer usage patterns during rounds to inform designs of information and communication technology to support clinical discourse during rounds. The tool captures choreography and logistics of information exchanges supported by clinical information systems during rounds. We developed the tool as part of an ongoing video-recording study of communication to under-stand how, when, and why computers are used during multidisciplinary clinical rounds.

Levels of vasopressin in children undergoing cardiopulmonary bypass.

OBJECTIVES: It is accepted treatment to give vasopressin to adults in postcardiotomy shock, but such use in children is controversial. Cardiopulmonary bypass is presumed to attenuate the normal endogenous vasopressin response to shock. We hypothesized that levels of vasopressin in children are altered by bypass, and that children having low endogenous levels perioperatively are more likely to develop hypotension, or require vasopressors. METHODS: Serial levels of vasopressin were assessed prospectively in children undergoing bypass at a single center. RESULTS: Of 61 eligible patients, we enrolled 39 (63%). Their median age was 5 months. The mean level of vasopressin prior to bypass was 18.6 picograms per millilitre, with an interquartile range from 2.6 to 11.4. Levels of vasopressin peaked during bypass at 87.1, this being highly significant compared to baseline (p < 0.00005), remained high for 12 hours at a mean of 73.5, again significantly different from baseline (p = 0.002), were falling at 24 hours, with a mean of 28.1 (p = 0.04), and had returned to baseline by 48 hours, when the mean was 7.4 (p = 0.3). Age, gender, and the category for surgical risk had no influence on the levels of vasopressin. There was no statistically significant relationship between the measured levels and hypotension or the requirement for vasopressors, although a few persistently hypotensive patients had high levels subsequent to bypass. Higher levels correlated with higher levels of sodium in the serum (r(s) = 0.37, p < 0.00005), higher osmolality (r(s) = 0.37, p < 0.00005), and positive fluid balance (r(s) = 0.23, p < 0.008). Preoperative use of inhibitors of angiotensin converting enzyme, preoperative congestive cardiac failure, and longer periods of bypass predicted higher levels during the first eight postoperative hours. CONCLUSIONS: Children do not have deficient endogenous levels of vasopressin following bypass, and lower levels are not associated with hypotension. Any therapeutic efficacy of infusion of vasopressin for post-cardiotomy shock in children is likely due to reasons other than physiologic replacement.

Extracorporeal membrane oxygenation after cardiac arrest in children: what do we know?

The use of extracorporeal membrane oxygenation (ECMO) as a resuscitative measure during or after manual cardiopulmonary resuscitation (CPR) shows sharply contrasting results. To assess the added value of ECMO in this situation and looking for predictors of mortality we performed a meta-analysis of individual patients collected from observational studies. An electronic Pubmed search restricted to English language publications between 1990 and 2007 using a consensus restrictive criterion retrieved 462 titles. Of those, 93 abstracts were considered appropriate for full text evaluation with 37 articles being included in our meta-analysis. In addition, unpublished data on a series of 98 non-duplicated patients from the author of one of the included studies was added. Data on 288 individually identified patients with a median age of 0.50 years and a median weight was 4.5 kg and demonstrated an overall survival to hospital discharge of 39.6% (114/288). Neurological complications were common, affecting 27% of all patients (77/288) and 14% of those discharged alive (16/114). Other common complications were renal failure (25%) and sepsis (17%). Odds ratios for mortality were higher for the presence of: any complication (OR 3.9, 95% CL 2.3-6.4), neurological (OR 3.3, 95% CL 1.7-6.1), renal (OR 5.1, 95% CL 2.5-10.3) and when the implementation of ECMO took >30 min (OR 2.1, 95% CL 1.1-3.8). Neck vessels cannulation had a lower association with mortality (p<.001). Simple rate comparison between manual CPR alone and the use of emergency ECMO shows a difference on survival to discharge of 12-23%. Its effectiveness is higher when implemented in the first 30 min after arrest. Age and weight do not seem to influence mortality. The incidence of complications is high, particularly neurological and renal, having a strong influence on survival. The specific characteristics of the neurological complications and their long-term effects on survivors are poorly reported in the literature.

Transient dynamic subaortic stenosis in premature neonates after patent ductus arteriosus ligation.

We describe 2 premature infants with PDA that did not respond to medical therapy and required surgical ligation. Both infants developed transient dynamic subaortic obstruction that resolved without specific therapy. This may have occurred due to sudden changes in the left ventricular volume.

A computing platform to support communication and sense-making in intensive care.

We developed a conceptual design of a mobile computing platform to support multi-disciplinary rounds in intensive care units.