Early and long-term outcomes of mitral valve replacement with mechanical valve in rheumatic heart disease.

Background: Rheumatic fever and rheumatic heart disease is endemic in India. Mitral valve replacement with mechanical valve is the commonest surgical procedure performed in rheumatic heart disease (RHD). However, there are no studies reporting the long-term outcomes of mechanical mitral valve replacement in rheumatic heart disease from India. Objective: The primary objective of the study was to look at the long-term survival following mechanical mitral valve replacement in RHD. The secondary objectives included follow up complications and event-free survival. Methods: For this study, 238 patients who underwent mitral valve replacement with TTK Chitra™ valve from 1st January 2006 to 31st December 2018 for RHD were included for analysis and reporting. The median follow-up period was 3371.50 days (9.3 years). Total follow-up was 2044 patient-years. Results: The mean age of the study population was 39.72 ± 10.48 years (range: 18-68 years). Out of 238 patients operated, 155 patients (65.12%) were alive and 69 patients (28.99%) were dead, and 14 patients (5.88%) were lost to follow-up. The operative mortality was 6 (2.52%) and the follow-up mortality was 63 (26.47%). The reasons for follow-up mortality were cardiac complications in 22 (34.9%) patients, valve-related complications in 18 (28.5%) patients, sudden unexplained death in 13 (20.6%) patients, and non-valve/ non-cardiac death in 10patients (15.8%). The one-year survival was 94.0%, five-year survival was 83.6%, ten-year survival was 70.6% and 15-year survival was 62.9%. During follow-up, valve-related events occurred in 123(52%) patients. The 15-year event-free survival was 33.0%. Conclusions: The long term outcome of mechanical valve replacement of the mitral valve in RHD patients was less than favorable. Both cardiac complications and mechanical valve related complications reduced their survival.

2023 World Heart Federation guidelines for the echocardiographic diagnosis of rheumatic heart disease.

Rheumatic heart disease (RHD) is an important and preventable cause of morbidity and mortality among children and young adults in low-income and middle-income countries, as well as among certain at-risk populations living in high-income countries. The 2012 World Heart Federation echocardiographic criteria provided a standardized approach for the identification of RHD and facilitated an improvement in early case detection. The 2012 criteria were used to define disease burden in numerous epidemiological studies, but researchers and clinicians have since highlighted limitations that have prompted a revision. In this updated version of the guidelines, we incorporate evidence from a scoping review, an expert panel and end-user feedback and present an approach for active case finding for RHD, including the use of screening and confirmatory criteria. These guidelines also introduce a new stage-based classification for RHD to identify the risk of disease progression. They describe the latest evidence and recommendations on population-based echocardiographic active case finding and risk stratification. Secondary antibiotic prophylaxis, echocardiography equipment and task sharing for RHD active case finding are also discussed. These World Heart Federation 2023 guidelines provide a concise and updated resource for clinical and research applications in RHD-endemic regions.

Simultaneous Double Balloon Dilatation for Supravalvar Pulmonary Obstruction After Arterial Switch Operation.

The optimal approach for supravalvar right ventricular outflow tract obstruction(RVOTO) after arterial switch operation(ASO) is unclear. The results of percutaneous balloon dilatation have been variable. We report the results of simultaneous double balloon dilation for RVOTO after ASO. Sixteen patients (1.3(0.7-3.8) years; 9.8(8.1-15.1) kgs underwent the procedure at 14(8-44.5) months after ASO. Salient technical features included placement of balloons over stiff guide-wires positioned in both branch pulmonary arteries to enable dilation of the distal-most main pulmonary artery (MPA) with high inflation pressures (~ 12-14 atmospheres) and short inflation-deflation cycles. Effective balloon size was based on the PA annulus or MPA distal to the narrowing. The final balloon: narrowest segment diameter ratio was 2.7. Following dilation, the right ventricle to systemic systolic pressure ratio decreased from 0.9 ± 0.18 to 0.52 ± 0.16 (p < 0.001) and mean RVOT gradient from 78 ± 18 to 34 ± 13.9 mmHg (p < 0.001). Narrowest diameter improved from 5.4 ± 2.2 to 9.2 ± 2.2 mm. There were no major complications. Two patients with inadequate relief (final RV-systemic ratios: 1.03 and 0.7) were referred for surgery. At median follow up of 9 months, IQR 7-22, range 5-73, others are free of re interventions with median RVOT gradient of 42, IQR 27-49, range 21-55 mmHg. The immediate and short-term follow up results of double balloon dilatation for supravalvar RVOTO is encouraging and may avoid the need for repeat surgery in the majority of patients. Further follow up is needed to determine the long-term durability of the results.

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.

Evaluation of TTK Chitra heart valve prosthesis in pediatric patients.

This report highlights the outcome of valve replacement using TTK Chitra heart mechanical valve in a subgroup of pediatric patients This cohort of 27 pediatric patients with implantations during January 2006 to December 2018 was followed up prospectively. The cohort consisted of 12 aortic valve replacement (AVR), 14 mitral valve replacement (MVR), and 1 double valve replacement (DVR) patients. Total follow-up was 254 patient-years (AVR = 107, MVR = 136, DVR = 11) being 90% complete. The results show that the survival rates and event-free rates were satisfactory. Despite many reservations due to the high risk involved, the long-term benefits of having a durable valve replacement seem to outweigh the risks and offer acceptable long-term survival.

Rescue right ventricular outflow tract stenting for refractory hypoxic spells.

BACKGROUND: While right ventricular outflow tract stenting (RVOTS) has become an acceptable alternative to palliative surgery in Tetralogy of Fallot (TOF) and similar physiologies, its utility for relief of refractory hypoxic spells is unclear. METHODS: Patients who underwent RVOTS for emergency relief of refractory hypoxic spells were identified. Specific modifications to enable expeditious RVOTS included use of stent delivery systems (guiding catheter or long sheath) upfront to minimize catheter exchanges; using coronary wires to cross RVOT initially; stabilizing the catheter with a wire in the aorta while crossing RVOT with a second wire. RESULTS: From 2015 to 2022, 11 patients underwent RVOTS for hypoxic spells refractory to medical management. Their median age was 27 days (IQR 8.5-442.5); weight 3.27 kg (2.7-8.96); 9 males. Median pulmonary annulus Z score was -4.13 (IQR-4.85 to -0.86). Thirteen stents with median diameter 5 (4-6.5) mm and length 19 (16-19.75) mm were implanted, fluoroscopy time:13.6 (11-26.3) min; procedure time (60, 30-70 min). All were ventilated. Oxygen saturations improved from 45% (40-60) to 90% (84-92); (p < 0.0001) with no major complications. Postprocedure ventilation was needed for 21 (20-49) hours and 4 required diuretic infusion for pulmonary over-circulation. Four needed re-stenting 13 days to 5 months later. At median follow-up of 7 (4-17) months; 2 died from unrelated causes, 3 underwent surgery (two correction and one aorto-pulmonary shunt) and 6 await surgery. CONCLUSION: RVOTS enables safe, expeditious and effective short-term palliation for refractory hypoxic spells. Specific technical modifications facilitate safety, ease and swiftness.

Prenatal diagnosis lowers neonatal cardiac care costs in resource-limited settings.

BACKGROUND: Prenatal diagnosis of critical CHDs and planned peripartum care is an emerging concept in resource-limited settings. OBJECTIVE: To report the impact of prenatal diagnosis and planned peripartum care on costs of neonatal cardiac care in a resource-limited setting. METHODS: Prospective study (October 2019 to October 2020). Consecutive neonates undergoing surgery or catheter-based interventions included. Patients were divided into prenatal (prenatal diagnosis) and post-natal (diagnosis after birth) groups. Costs of cardiac care (total, direct, and indirect) and health expenses to income ratio were compared between study groups; factors impacting costs were analysed. RESULTS: A total of 105 neonates were included, including 33 in prenatal group. Seventy-seven neonates (73.3%) underwent surgical procedures while the rest needed catheter-based interventions. Total costs were 16.2% lower in the prenatal group (p = 0.008). Direct costs were significantly lower in the prenatal group (18%; p = 0.02), especially in neonates undergoing surgery (20.4% lower; p = 0.001). Health expenses to income ratio was also significantly lower in the prenatal group (2.04 (1.03-2.66) versus post-natal:2.58 (1.55-5.63), p = 0.01);, particularly in patients undergoing surgery (prenatal: 1.58 (1.03-2.66) vs. post-natal: 2.99 (1.91-6.02); p = 0.002). Prenatal diagnosis emerged as the only modifiable factor impacting costs on multivariate analysis. CONCLUSION: Prenatal diagnosis and planned peripartum care of critical CHD is feasible in resource-limited settings and is associated with significantly lower costs of neonatal cardiac care. The dual benefit of improved clinical outcomes and lower costs of cardiac care should encourage policymakers in resource-limited settings towards developing more prenatal cardiac services.

Culture-negative sepsis after pediatric cardiac surgery: Incidence and outcomes.

Background: Significant proportion of congenital heart surgery (CHS) children exhibit sepsis, but have negative blood culture and defined "culture negative sepsis (CNS)." Aims and Objectives: Retrospective analysis of CNS patients undergoing CHS. Material and Methods: 437 consecutive CHS children grouped as controls (antibiotic prophylaxis), CNS, and culture positive sepsis (CPS). Results: Incidences of CNS and CPS were 16% and 7%. Median mechanical ventilation (MV) in hours among CPS, CNS, and control was 116 (45-271), 63 (23-112), and 18 (6-28) (P < 0.001), respectively. Multivariable linear regression identified CPS (median ratio: 3.1 [2.3-4.1], P < 0.001), CNS (median ratio: 5.6 [3.7-8.4], P < 0.001), and weight (kg) (median ratio: 0.98 [0.96-0.99], P = 0.009) as associations of MV. Intensive care unit (ICU) stay (hours) was 192 (120-288) in CNS, 288 (156-444) in CPS, and 72 (48-120) in controls (P < 0.0001). Multivariable linear regression showed CNS (median ratio: 2.4 [2.0-2.9], P< 0.001) CPS (median ratio: 3.3 [2.5-4.4], P < 0.001), and weight (median ratio: 0.98 [0.97-0.99], P ≤ 0.001) prolonging ICU stay. Mortality was 10.7%, 2.9% and 1.2% in CPS, CNS, and control (P = 0.03). Multivariable regression identified CPS an independent predictor of mortality with odds ratio 8.6 (1.7-44.9; P = 0.010). 11.26% patients in CNS and 79.3% in CPS received antibiotics for more than 10 days. Conclusion: Incidence of CNS was 16%; duration of MV and ICU stay and mortality was significantly less in CNS than CPS patients.

Impact of COVID-19 pandemic on pediatric cardiac services in India.

BACKGROUND: COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India. AIMS: The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. METHODS: We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. RESULTS: The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). CONCLUSIONS: The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic.

Outcome of COVID-19-positive children with heart disease and grown-ups with congenital heart disease: A multicentric study from India.

BACKGROUND: Outcome data of children with heart disease who acquired COVID-19 infection are limited. AIMS: We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. MATERIALS AND METHODS: The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group. RESULTS: From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3-96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients (n = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% (n = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period (P < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9-41,605, P = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1-814.7, P = 0.046). CONCLUSIONS: Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes.

Transaxillary Approach for Surgical Repair of Simple Congenital Cardiac Lesions: Pitfalls, and Complications.

BACKGROUND: In the treatment of simple congenital cardiac lesions, percutaneous and cosmetic surgical approaches have steadily gained prominence. Surgically, right vertical axillary approach is sparsely used despite superior cosmesis and less pain and blood product usage. Knowledge of potential pitfalls could lead to its more widespread acceptance. METHODS: We retrospectively analyzed perioperative outcomes of 104 consecutive patients who underwent surgery by this technique between mid-2016 and December 2019, including ostium secundum (67), sinus venosus (34), coronary sinus (1), and ostium primum (1) atrial septal defects and hemianomalous pulmonary venous connection (1). Perioperative variables, surgical times, complications, and follow-up data were analyzed. RESULTS: Patient weight ranged from 6.8 to 41 kg. Incision length ranged from 4 to 6 cm. There was no mortality. All cannulation was central. Difficulty in cannulation (inferior vena cava) was seen in two patients. Morbidity included pneumothorax in 2 (1.9%) patients and subcutaneous emphysema necessitating prolonged intercostal drain retention in 20 (19.2%) patients. Surgical time increased linearly (r = 0.567; P < .001) with increasing patient weight but cardiopulmonary bypass (CPB) time remained unaffected. No chest deformities or paresthesia were noted on follow-up. Scar size decreased in some patients. CONCLUSIONS: Right vertical axillary approach can be safely employed to treat simple congenital cardiac lesions with adequate awareness of potential pitfalls. Increasing patient weight increases the surgical time but does not affect CPB times. Incidence of pneumothorax and subcutaneous emphysema is similar to other thoracotomy approaches. It is cosmetically superior.

Conversion of prior univentricular repairs to septated circulation: Case selection, challenges, and outcomes.

OBJECTIVES: Complex congenital heart defects that present earlier in life are sometimes channelled in the single ventricle pathway, because of anatomical or logistic challenges involved in biventricular correction. Given the long-term functional and survival advantage, and with the surgeons' improved understanding of the cardiac anatomy, we have consciously explored the feasibility of a biventricular repair in these patients when they presented later for Fontan completion. We present a single institution's 10-year experience in achieving biventricular septation of prior univentricular repairs, the technical and physiological challenges and the surgical outcomes. METHODS: Between June 2010 and December 2019, 246 patients were channelized in the single ventricle pathway, of which 32 patients were identified as potential biventricular candidates at the time of evaluation for Fontan palliation, considering their anatomic feasibility. The surgical technique involves routing of the left ventricle to the aorta across the ventricular septal defect, ensuring an adequate sized right ventricular cavity, establishing right ventricle-pulmonary artery continuity and taking down the Glenn shunt with rerouting of the superior vena cava to the right atrium. This is a retrospective study where we reviewed the unique physiological and surgical characteristics of this subset of patients and analysed their surgical outcomes and complications. RESULTS: Biventricular conversion was achieved in all cases except in 3 patients, who had the Glenn shunt retained leading to a one and a half ventricle repair. The average age of the patients was 4.9 years of whom 18 were male. The average cardiopulmonary bypass time was 371 min with an average cross clamp time of 162 min. There was one mortality in a patient with corrected transposition of great arteries (c-TGA) with extensive arterio-venous malformations (AVMs). At a median follow-up of 60 months, all patients remained symptom free except two with NYHA II symptoms, one being treated for branch pulmonary artery stenosis with balloon dilatation and the other with multiple AVMs who needed coil closure. One patient with branch pulmonary artery (PA) stenosis required balloon dilatation and stent placement. CONCLUSION: The possibility of achieving the surgical goal in this unique subset of patients evolves with the progressive experience of the congenital heart surgeon. Case selection is a crucial aspect in achieving the desired outcome, and this 'borderline' substrate is often recognized at the time of evaluation for the Fontan completion. A comprehensive preoperative imaging and planning helps in achieving the surgical septation and reconnection to achieve the desired physiological circulation. Though technically challenging, the surgery has excellent short- and mid-term outcomes as evidenced by our 10-year experience.

Contemporary Diagnosis and Management of Rheumatic Heart Disease: Implications for Closing the Gap: A Scientific Statement From the American Heart Association.

The global burden of rheumatic heart disease continues to be significant although it is largely limited to poor and marginalized populations. In most endemic regions, affected patients present with heart failure. This statement will seek to examine the current state-of-the-art recommendations and to identify gaps in diagnosis and treatment globally that can inform strategies for reducing disease burden. Echocardiography screening based on World Heart Federation echocardiographic criteria holds promise to identify patients earlier, when prophylaxis is more likely to be effective; however, several important questions need to be answered before this can translate into public policy. Population-based registries effectively enable optimal care and secondary penicillin prophylaxis within available resources. Benzathine penicillin injections remain the cornerstone of secondary prevention. Challenges with penicillin procurement and concern with adverse reactions in patients with advanced disease remain important issues. Heart failure management, prevention, early diagnosis and treatment of endocarditis, oral anticoagulation for atrial fibrillation, and prosthetic valves are vital therapeutic adjuncts. Management of health of women with unoperated and operated rheumatic heart disease before, during, and after pregnancy is a significant challenge that requires a multidisciplinary team effort. Patients with isolated mitral stenosis often benefit from percutaneous balloon mitral valvuloplasty. Timely heart valve surgery can mitigate the progression to heart failure, disability, and death. Valve repair is preferable over replacement for rheumatic mitral regurgitation but is not available to the vast majority of patients in endemic regions. This body of work forms a foundation on which a companion document on advocacy for rheumatic heart disease has been developed. Ultimately, the combination of expanded treatment options, research, and advocacy built on existing knowledge and science provides the best opportunity to address the burden of rheumatic heart disease.

Neonatal cardiac surgery in low resource settings: implications of birth weight.

OBJECTIVE: We sought to evaluate the association between low birth weight (LBW) and outcomes following neonatal cardiac surgery in a low-income and middle-income country setting where LBW prevalence is high and its impact on surgical outcomes is undefined. DESIGN: Single-centre retrospective cohort study. SETTING: Referral paediatric hospital in Southern India PATIENTS: All neonatal cardiac surgical cases between January 2011 and December 2018. LBW was defined as <2.5 kg. MAIN OUTCOME MEASURES: Patient demographics, corrective versus palliative surgery and postoperative outcomes were collected from the institutional database which undergoes regular audit as part of International Quality Improvement Collaborative for Congenital Heart Disease. In-hospital mortality was the primary outcome measure. RESULTS: Of 569 neonatal cardiac operations, 123 (21.6%) had LBW (mean: 2.2±0.3 kg); 18.7% <2 kg and 21.1% were preterm (<37 weeks). Surgery type (corrective vs palliative) or non-cardiac anomalies were not associated with birth weight. Birth weight did not correlate with ICU length of stay (LOS) and mechanical ventilation but was lower in those with postoperative sepsis. Overall in-hospital mortality was 7.0%; LBW neonates had higher mortality (OR 2.16, 95% CI 1.09 to 4.29, p=0.025). Multivariable analyses revealed birth weight (OR per 100 g decrease in weight: 1.12; 95% CI 1.03 to 1.22), age at surgery (OR per day increase in age of 0.93; 95% CI 0.87 to 0.99) and palliative intervention (OR 4.46 (95% CI 1.91 to 10.44) as independent predictors of in-hospital mortality. CONCLUSION: LBW adversely impacts in-hospital mortality outcomes following neonatal cardiac surgery in a resource-limited setting without increase in ICU or hospital LOS.

Impact of transport on arrival status and outcomes in newborns with heart disease: a low-middle-income country perspective.

OBJECTIVES: We sought to systematically study determinants of "clinical status at arrival after transport" of neonates with CHD and its impact on clinical outcomes in a low- and middle-income country environment. METHODS AND RESULTS: Consecutive neonates with CHD (n = 138) transported (median distance 138 km; 5-425 km) to a paediatric cardiac programme in Southern India were studied prospectively. Among 138 neonatal transports, 134 were in ambulances. Four neonates were transported by family in private vehicles; 60% with duct-dependent circulation (n = 57) were transported without prostaglandin E1. Clinical status at arrival after transport was assessed using California modification of TRIPS Score (Ca-TRIPS), evidence of end-organ injury and metabolic insult.Upon arrival, 42% had end-organ injury, 24% had metabolic insult and 36% had Ca-TRIPS Score >25. Prior to surgery or catheter intervention, prolonged ICU stay (>48 hours), prolonged ventilation (>48 hours), blood stream sepsis, and death occurred in 48, 15, 19, and 3.6%, respectively. Ca-TRIPS Score >25 was significantly associated with mortality (p = 0.005), sepsis (p = 0.035), and prolonged ventilation (p < 0.001); end-organ injury with prolonged ICU stay (p = 0.031) and ventilation (p = 0.045); metabolic insult with mortality (p = 0.012) and sepsis (p = 0.015).Fifteen babies needed only medical management, 10 received comfort care (due to severe end-organ injury in 3), 107 underwent cardiac surgery (n = 83) or catheter intervention (n = 24), with a mortality of 6.5%. Clinical status at arrival after transport did not impact post-procedure outcomes. CONCLUSION: Neonates with CHD often arrive in suboptimal status after transport in low- and middle-income countries resulting in adverse clinical outcomes. Robust transport systems need to be integrated in plans to develop newborn heart surgery in low- and middle-income countries.

Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication.

JUSTIFICATION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. OBJECTIVES: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Prenatal diagnosis and planned peri-partum care as a strategy to improve pre-operative status in neonates with critical CHDs in low-resource settings: a prospective study.

BACKGROUND: Prenatal diagnosis and planned peri-partum care is an unexplored concept for care of neonates with critical CHDs in low-middle-income countries. OBJECTIVE: To report the impact of prenatal diagnosis on pre-operative status in neonates with critical CHD. METHODS: Prospective observational study (January 2017-June 2018) in tertiary paediatric cardiac facility in Kerala, India. Neonates (<28 days) with critical CHDs needing cardiac interventions were included. Pre-term infants (<35 weeks) and those without intention to treat were excluded. Patients were grouped into those with prenatal diagnosis and diagnosis after birth. Main outcome measure was pre-operative clinical status. RESULTS: Total 119 neonates included; 39 (32.8%) had prenatal diagnosis. Eighty infants (67%) underwent surgery while 32 (27%) needed catheter-based interventions. Pre-operative status was significantly better in prenatal group; California modification of transport risk index of physiological stability (Ca-TRIPS) score: median 6 (0-42) versus 8 (0-64); p < 0.001; pre-operative assessment of cardiac and haemodynamic status (PRACHS) score: median 1 (0-4) versus 3 (0-10), p < 0.001. Age at cardiac procedure was earlier in prenatal group (median 5 (1-26) versus 7 (1-43) days; p = 0.02). Mortality occurred in 12 patients (10%), with 3 post-operative deaths (2.5%). Pre-operative mortality was higher in postnatal group (10% versus 2.6%; p = 0.2) of which seven (6%) died due to suboptimal pre-operative status precluding surgery. CONCLUSION: Prenatal diagnosis and planned peri-partum care had a significant impact on the pre-operative status in neonates with critical CHD in a low-resource setting.