Ductal Stenting in Low-Resource Environments.

Duct-dependent pulmonary circulation has traditionally been addressed by the Blalock-Taussig-Thomas shunts (BTTS). Recently, catheter-based alternatives such as ductal stenting have emerged as a particularly advantageous option, especially in resource-constrained settings. This article delves into the nuances of ductal stenting within low-resource environments, highlighting its relative ease of application, reduced morbidity, and cost-effectiveness as key factors in its favor. Comparisons in mortality between the two procedures are however likely to be confounded by selection biases. Ductal stenting appears to be particularly beneficial for palliating older infants and children with cyanotic congenital heart disease and diminished pulmonary blood flow who present late. Additionally, it serves as a valuable tool for left ventricular training in late-presenting transposition with an intact ventricular septum. A meticulous pre-procedure echocardiographic assessment of anatomy plays a pivotal role in planning access and hardware, with additional imaging seldom required for this purpose. The adaptation of adult coronary hardware has significantly enhanced the technical feasibility of ductal stenting. However, challenges such as low birth weight and sepsis specifically impact the performance of ductal stenting and patient recovery in low-resource environments. There is potential for systematic application of quality improvement processes to optimize immediate and long-term outcomes of ductal stenting. There is also a need to prospectively examine the application of ductal stenting in low-resource environments through multi-center registries.

Prostaglandins for adult liver transplanted recipients.

BACKGROUND: Prostaglandins are naturally occurring lipids that are synthesised from arachidonic acid. Multiple studies have evaluated the benefits of prostaglandins in reducing ischaemia reperfusion injury after liver transplantation. New studies have been published since the previous review, and hence it was important to update the evidence for this intervention. OBJECTIVES: To evaluate the benefits and harms of prostaglandins in adults undergoing liver transplantation compared with placebo or standard care. SEARCH METHODS: We used standard, extensive Cochrane search methods. The latest search date was 27 December 2022. SELECTION CRITERIA: We included randomised clinical trials evaluating prostaglandins initiated in the perioperative period compared with placebo or standard care for adults undergoing liver transplantation. We included trials irrespective of reported outcomes. DATA COLLECTION AND ANALYSIS: We used standard Cochrane methods. Our primary outcomes were 1. all-cause mortality, 2. serious adverse events, and 3. health-related quality of life. Our secondary outcomes were 4. liver retransplantation, 5. early allograft dysfunction, 6. primary non-function of the allograft, 7. acute kidney failure, 8. length of hospital stay, and 9. adverse events considered non-serious. We used GRADE to assess certainty of evidence. MAIN RESULTS: We included 11 randomised clinical trials with 771 adult liver transplant recipients (mean age 47.31 years, male 61.48%), of whom 378 people were randomised to receive prostaglandins and 393 people were randomised to either placebo (272 participants) or standard care (121 participants). All trials were published between 1993 and 2016. Ten trials were conducted in high- and upper-middle-income countries. Prostaglandins may reduce all-cause mortality up to one month (risk ratio (RR) 0.86, 95% confidence interval (CI) 0.61 to 1.23; risk difference (RD) 21 fewer per 1000, 95% CI 63 fewer to 36 more; 11 trials, 771 participants; low-certainty evidence). Prostaglandins may result in little to no difference in serious adverse events (RR 0.92, 95% CI 0.60 to 1.40; RD 81 fewer per 1000, 95% CI 148 fewer to 18 more; 6 trials, 568 participants; low-certainty evidence). None of the included trials reported health-related quality of life. Prostaglandins may result in little to no difference in liver retransplantation (RR 0.98, 95% CI 0.49 to 1.96; RD 1 fewer per 1000, 95% CI 33 fewer to 62 more; 6 trials, 468 participants; low-certainty evidence); early allograft dysfunction (RR 0.62, 95% CI 0.33 to 1.18; RD 137 fewer per 1000, 95% CI 241 fewer to 47 more; 1 trial, 99 participants; low-certainty evidence); primary non-function of the allograft (RR 0.58, 95% CI 0.26 to 1.32; RD 23 fewer per 1000, 95% CI 40 fewer to 16 more; 7 trials, 624 participants; low-certainty evidence); and length of hospital stay (mean difference (MD) -1.15 days, 95% CI -5.44 to 3.14; 4 trials, 369 participants; low-certainty evidence). Prostaglandins may result in a large reduction in the development of acute kidney failure requiring dialysis (RR 0.42, 95% CI 0.24 to 0.73; RD 100 fewer per 1000, 95% CI 132 fewer to 49 fewer; 5 trials, 477 participants; low-certainty evidence). The evidence is very uncertain about the effect of prostaglandins on adverse events considered non-serious (RR 1.19, 95% CI 0.42 to 3.36; RD 225 fewer per 1000, 95% CI 294 fewer to 65 fewer; 4 trials, 329 participants; very low-certainty evidence). Two trials reported receiving funding; one of these was with vested interests. We found one registered ongoing trial. AUTHORS' CONCLUSIONS: Eleven trials evaluated prostaglandins in adult liver transplanted recipients. Based on low-certainty evidence, prostaglandins may reduce all-cause mortality up to one month; may cause little to no difference in serious adverse events, liver retransplantation, early allograft dysfunction, primary non-function of the allograft, and length of hospital stay; and may have a large reduction in the development of acute kidney injury requiring dialysis. We do not know the effect of prostaglandins on adverse events considered non-serious. We lack adequately powered, high-quality trials evaluating the effects of prostaglandins for people undergoing liver transplantation.

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.

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.

Pregnancy and early post-natal outcomes of fetuses with functionally univentricular heart in a low-and-middle-income country.

BACKGROUND: Care of children with functionally univentricular hearts is resource-intensive. OBJECTIVES: To analyse pregnancy and early post-natal outcomes of fetuses with functionally univentricular hearts in the setting of a low-middle-income country. METHODS: A retrospective study was conducted during the period of January 2008-October 2019. Study variables analysed included gestational age at diagnosis, maternal and fetal comorbidities and cardiac diagnosis including morphologic type of single ventricle. Outcomes analysed included pregnancy outcomes, type of post-natal care and survival status on the last follow-up. RESULTS: A total of 504 fetuses were included. Mean maternal age was 27.5 ± 4.8 years and mean gestational age at diagnosis was 25.6 ± 5.7 weeks. Pregnancy outcomes included non-continued pregnancies (54%), live births (42.7%) and loss to follow-up (3.3%). Gestational age at diagnosis was the only factor that impacted pregnancy outcomes (non-continued pregnancies 22.5 ± 3.5 vs. live births 29.7 ± 5.7 weeks; p < 0.001). Of the 215 live births, intention-to-treat was reported in 119 (55.3%) cases; of these 103 (86.6%) underwent cardiac procedures. Seventy-nine patients (36.7%) opted for comfort care. On follow-up (median 10 (1-120) months), 106 patients (21%) were alive. Parental choice of intention-to-.treat or comfort care was the only factor that impacted survival on follow-up. CONCLUSIONS: Prenatal diagnosis of functionally univentricular hearts was associated with overall low survival status on follow-up due to parental decisions on not to continue pregnancy or non-intention-to-treat after birth. Early detection of these complex defects by improved prenatal screening can enhance parental options and reduce resource impact in low-and-middle-income countries.

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.

Early weight trends after congenital heart surgery and their determinants.

BACKGROUND: Early weight trends after cardiac surgery in infants from low- and middle-income countries where the majority are undernourished have not been defined. We studied the early post-operative weight trends to identify specific factors associated with early weight loss and poor weight gain after discharge following congenital heart surgery in consecutive infants undergoing cardiac surgery at a referral hospital in Southern India. METHODS: This was a prospective observational study. Weights of the babies were recorded at different time points during the hospital stay and at 1-month post-discharge. A comprehensive database of pre-operative, operative, and post-operative variables was created and entered into a multivariate logistic regression analysis model to identify factors associated with excessive early weight loss after cardiac surgery, and poor weight gain following hospital discharge. RESULTS: The study enrolled 192 infants (mean age 110.7 ± 99.9 days; weight z scores - 2.5 ± 1.5). There was a small but significant (p < 0.001) decline in weight in the hospital following surgery (1.6% decline (interquartile range -5.3 to +1.7)); however, there was substantial growth following discharge (26.7% increase (interquartile range 15.3-41.8)). The variables associated with post-operative weight loss were cumulative nil-per-oral duration and cardiopulmonary bypass time, while weight gain following discharge was only associated with age. CONCLUSION: Weight loss is almost universal early after congenital heart surgery and is associated with complex surgery and cumulative nil-per-oral duration. After discharge, weight gain is almost universal and not associated with any of the perioperative variables.

Accuracy of a New Echocardiographic Index to Predict Need for Trans-annular Patch in Tetralogy of Fallot.

The long-term outcome after repair of tetralogy of Fallot (TOF) is critically dependent pulmonary valve competence that is compromised by trans-annular patch (TAP). We compared a new echocardiographic index [pulmonary annulus index (PAI)] to conventional methods of predicting need for TAP in infants undergoing TOF repair. Consecutive infants undergoing TOF repair were prospectively studied. Pre-operative aortic and pulmonary annuli and main pulmonary artery (MPA) diameters were measured and z scores determined. PAI was a ratio of observed to expected pulmonary annulus (PA) diameter. TAP was based on intra-operative sizing by surgeons blinded to PAI values. Receiver operator curves (ROC) were generated for all PAI, MPA z scores and pulmonary annulus z scores. Of 84 infants (8.6 ± 2.6 months; 7.5 ± 1.3 kg), 36 needed TAP (43%). All the three indices viz. PAI, Pulmonary annulus and MPA z scores performed similarly in predicting need for TAP (ROC curves ~ 80%). Combining cut-offs of MPA z scores (> - 3.83) with either PAI (> 0.73) or PA z score (> - 1.83) predicted avoidance of TAP with ~ 90% accuracy. When both PAI and MPA z scores were below the cut-offs there was an 80% likelihood of TAP. Failure to predict TAP was associated with unicommisural pulmonary valves. PAI was equal to PA z scores in predicting need for TAP during repair of TOF. Combining either with MPA z scores was the most accurate method of prediction. Failure of prediction was mainly due to presence of a unicommissural pulmonary valve.

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.

Health-related quality of life in Indian children: A community-based cross-sectional survey.

BACKGROUND & OBJECTIVES: There are limited data on health-related quality of life (HRQOL) related to Indian children. The objective of this study was to construct a generic HRQOL reference for children aged 2-18 yr from a community setting. METHODS: The study was a community-based cross-sectional survey. A total of 719 children/adolescents in the age group of 2-18 yr were enrolled using stratified random cluster sampling. A total of 40 clusters (cluster size 18) were selected for the study. The data contained child self-report and parent proxy report from healthy children and their parents/caretakers. The Pediatric Quality of Life Inventory 4.0 (PedsQL4.0) Generic Core Scale was used to collect HRQOL data. Questionnaires were self-administered for parents and children aged 8-18 yr. In the age group of five to seven years, parents assisted the children in filling questionnaires. RESULTS: The mean HRQOL total scores from child self-report and parent proxy report were 87.50±11.10 and 90.10±9.50 respectively, for children aged 2-18 yr. Social functioning had the highest scores and emotional functioning had the lowest scores for the entire sample and subgroups. The mean values for HRQOL in the current study were significantly different from the reference study for both child (87.39 vs. 83.91, P<0.001) and parent proxy reports (90.03 vs. 82.29, P<0.001) when compared between children aged 2-16 yr. INTERPRETATION & CONCLUSIONS: The study provided reference values for HRQOL in healthy children and adolescents from Kerala, India, that appeared to be different from existing international reference. Similar studies need to be done in different parts of India to generate a country-specific HRQOL reference for Indian children.