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.

Performance of a Risk Analytic Tool (Index of Tissue Oxygen Delivery "IDO2") in Pediatric Cardiac Intensive Care Unit of a Developing Country.

Objective: To determine the performance of a commercially available risk analytic tool (IDO2) to estimate the risk for SVO2 < 40% in patients admitted in cardiac intensive care unit (CICU). Methods: Medical and T3 records of all patients (aged 1 day to 12 years, weight >2 kg) who received care in the CICU between October 1st, 2019 and October 1st, 2020, had SvO2 lab(s) drawn during CICU course and whose data was transmitted to T3, were included. The average IDO2 Index was computed in the 30-min period immediately prior to each SvO2 measurement and used as a predictor score for SvO2 < 40%. Results: A total of 69 CICU admissions from 65 patients, median age 9.3 months (interquartile range 20.8) were identified. Surgical and medical patients were 61 (88%) and 8 (12%) respectively; 4 (5.7%) patients had single ventricle physiology. Tetralogy of Fallot n = 23 (33.3%) and ventricular septal defects 17 (24.6%) were major cardiac diagnosis. Sixty-one (89.9%) of the admissions were successfully discharged from the hospital. Of the 187-total included SvO2 labs, 17 (9%) were <40%. The AUC of estimating SvO2 < 40% IDO2 was 0.87 [confidence interval (CI): 0.79-0.94]. Average IDO2 above 75 had the highest absolute risk (42.11, CI: 20.25-66.50) and highest RR (4.63, CI: 2.31-9.28, p-value < 0.0001) of SvO2 < 40%. Conclusion: IDO2 performed well in estimating low SvO2 (<40%) in pediatric patients presenting to a CICU in a low resource setting. Future work is needed to determine the effect of this risk analytic tool on clinical outcomes in such a setting.

Reduction of cardiac iron overload by optimising iron chelation therapy in transfusion dependent thalassaemia using cardiac T2* MRI: a quality improvement project from Pakistan.

OBJECTIVES: Cardiac T2* MRI (T2*CMR), for accurate estimation of myocardial siderosis, was introduced as part of a QI collaborative to optimise chelation therapy in order to improve cardiac morbidity in transfusion dependent thalassaemia (TDT) patients. We report the impact of this QI initiative from two thalassaemia centres from this collaborative. DESIGN AND SETTING: A key driver based quality initiative was implemented to improve chelation in TDT patients registered at these two centres in Karachi, Pakistan. Protocol optimisation and compliance to treatment through training, communication and feedback were used as the drivers for QI intervention. Preintervention variables (demographics, chelation history, T2*CMR, echocardiography and holters) were collected from January 2015 to December 2016) and compared with variables in the post implementation phase (January to December 2019). A standardised adverse event severity for chelators and its management was devised for safe drug therapy as well as ensuring compliance to the regimen. Preintervention and postintervention variables were compared using non-parametric test. P value<0.05 was statistically significant. RESULTS: 100 patients with TDT, median age 17 (9-34) years, were included. An increase or stabilisation of T2*CMR was documented in 82% patients in the postintervention phase especially in patients with severe myocardial iron overload (5.5 vs 5.3 ms, p <0.01). Significantly fewer patients had abnormal echocardiographic findings (3.5% vs 26%, p <0.05) in the postintervention versus preintervention period. CONCLUSION: This QI initiative improved the chelation therapy leading to improved cardiac status in TDT patients at the participating centres.

Exploring the influence of socio-cultural factors and environmental resources on the health related quality of life of children and adolescents after congenital heart disease surgery: parental perspectives from a low middle income country.

BACKGROUND: Health related quality of life (HRQOL) is an important indicator of long-term well-being, influenced by environmental factors such as family, culture, societal norms and available resources. This study aimed to explore parental perspectives on the influence of socio-cultural factors and environmental resources on the HRQOL of children and adolescents after congenital heart disease (CHD) surgery. METHODS: Using a descriptive, qualitative design, semi-structured interviews of children/adolescents who had CHD surgery in this low-middle income country (LMIC) were collected between July to December 2017. There were 20 families enrolled, which included 18 parent dyads (mother and father) and two single mothers, making a total of 38 participants. Initial inductive analysis was further refined using the Social Ecological Model as an analytic lens. RESULTS: At the intrapersonal level, unrealistic expectations of surgery, residual CHD symptoms and difficulty maintaining educational progress were of great concern. There were low levels of health literacy and understanding about CHD among family and friends, however, strong kinship ties were an important resource at the interpersonal level. These families lived in poverty and mothers often carried the sole burden of care for their sick children. At the institutional level, there were unclear expectations of the child's needs at school, and parents had poor access to psychological, family-planning and genetic counselling, and poor access to CHD education resources. At a sociocultural level, religion and trust in God were important coping factors, however, CHD was a gendered experience with particular concerns around scarring and the marriageability of girls. Parents noted the deficit of antenatal and specialist CHD services and felt the consequence of a lack of a universal health care system at the public policy level. CONCLUSION: Socio-ecological factors have the potential to explain the issues and challenges that children living in LMIC experience with CHD after surgery. The study findings will help to inform future interventions to be implemented in countries like Pakistan.

Health-related quality of life in adult CHD surgical patients in a low middle-income country: a mixed-methods study.

BACKGROUND AND OBJECTIVES: This mixed-methods study aimed to assess health-related quality of life in young adults with CHD following surgery in a low middle-income country, Pakistan. Despite the knowledge that geographic, cultural and socio-economic factors may shape the way health and illness is experienced and managed and consequently determine a person's health-related quality of life, few health-related quality of life studies are conducted in low middle-income countries. This deficit is pronounced in CHD, so there is little guidance for patient care. METHODS: The study utilised concurrent, mixed methods. Adults with CHD (n = 59) completed health-related quality of life surveys (PedsQLTM 4.0 Generic Core Scale, PedsQLTM Cognitive Functioning Scale and PedsQLTM 3.0 Cardiac Module). Semi-structured interview data were collected from a nested sub-sample of 17 participants and analysed using qualitative content analysis, guided by the revised Wilson-Cleary model of health-related quality of life. RESULTS: The lowest health-related quality of life domain was emotional with the mean score (71.61 ± 20.6), followed by physical (78.81 ± 21.18) and heart problem (79.41 ± 18.05). There was no statistical difference in general or cardiac-specific health-related quality of life between mild, moderate or complex CHD. Qualitative findings suggested low health-related quality of life arose from a reduced capacity to contribute to family life including family income and gender. A sense of reduced marriageability and fear of dependency were important socio-cultural considerations. CONCLUSIONS: CHD surgical patients in this low-income country experience poor health-related quality of life, and contributing factors differ to those reported for high-income countries. Socio-cultural understandings should underpin assessment, management and care-partnering with young adults with CHD following surgical correction.

2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT.

The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term "pulmonary hypertension" and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.

Ruptured saphenous venous graft pseudoaneurysm presenting as a pulsatile chest mass.

A 72-year-old man with prior history of coronary artery bypass grafting and sternal wire infection presented with non-ST-segment elevation myocardial infarction. His coronary angiogram revealed stenosis of the distal left main coronary artery (LMCA) and a pseudoaneurysm of saphenous venous graft (SVG) to right posterior descending artery. Patient developed ventricular fibrillation during admission, and postcardiopulmonary resuscitation, a pulsatile chest mass was observed which was diagnosed with computed tomography as a chest wall collection resulting from rupture of the pseudo-aneurysm. He underwent percutaneous coronary intervention of the LMCA with drug-eluting stents followed by successful coil embolization of the SVG pseudoaneurysm. Patient had an uneventful recovery postprocedure.

Health-related quality of life in surgical children and adolescents with congenital heart disease compared with their age-matched healthy sibling: a cross-sectional study from a lower middle-income country, Pakistan.

OBJECTIVE: Inconsistencies have been reported in health-related quality of life (HRQOL) in postoperative congenital heart disease (CHD). Despite the need for lifelong care due for residual symptoms, only a few studies have explored cardiac-related HRQOL but none in lower middle-income countries (LMIC). This study therefore addresses the gap by exploring HRQOL and its associated predictors in postoperative CHD in Pakistan. OUTCOME MEASURES: General and cardiac-related HRQOL, associated predictors. METHODS: This cross-sectional study recruited patients with CHD and age-matched healthy siblings as controls (n=129 each) at a single centre in Pakistan. Patients and their siblings completed HQROL surveys (PedsQL 4.0 Generic Core, PedsQL Cognitive Functioning). Patients only completed PedsQL 3.0 Cardiac module. Generalised linear models identified predictors. RESULTS: The sample mean age was 8.84±3.87 years and 70% were below the poverty line for an LMIC. The majority (68%) had their first surgery after 1 year of age and were interviewed at a mean 4.08±1.91 years postoperatively.Patients with CHD had lower HRQOL in all domains compared with their age-matched siblings, with the biggest differences for total HRQOL (effect size, d=-1.35). Patients with complex CHD had lower HRQOL compared with simple to moderate CHDs in cardiac-related HRQOL. The lowest scores were for treatment problems (effect size, d=-0.91). HRQOL was worse for patients who were on cardiac medications, had complex CHD, longer cardiopulmonary bypass time, re-operations and were female. CONCLUSIONS: HRQOL issues persist in postoperative patients with CHD in LMIC, Pakistan. Solutions are needed to address poor HRQOL and lifelong concerns of patients and their parents.

Comparison of enteral versus intravenous potassium supplementation in hypokalaemia in paediatric patients in intensive care post cardiac surgery: open-label randomised equivalence trial (EIPS).

OBJECTIVES: The primary objective was to compare the efficacy of enteral potassium replacement (EPR) and intravenous potassium replacement (IVPR) as first-line therapy. Secondary objectives included comparison of adverse effects and number of doses required to resolve the episode of hypokalaemia. TRIAL DESIGN: The EIPS trial is designed as a randomised, equivalence trial between two treatment arms. STUDY SETTING: The study was conducted at the paediatric cardiac intensive care unit (PCICU) at Aga Khan University Hospital, Karachi. PARTICIPANTS: 41 patients (aged 1 month to 15 years) who were admitted to PCICU post cardiac surgery were recruited (23 IVPR arm and 18 EPR arm). INTERVENTION: Intervention arms were block randomised on alternate weeks for IVPR and EPR. OUTCOME MEASURE: Change in serum potassium levels in (mmol/L) and percentage change after each event of potassium replacement by the intravenous or enteral route. RESULTS: Both groups (41 patients) had similar baseline characteristics. Mean age was 4.7 (SD±4) years while the most common surgical procedure was ventricular septal defect repair (12 patients, 29.3%). No mortality was observed in either arm. Four episodes of vomiting and one arrhythmia were seen in the EPR group. After adjusting for age, potassium level at the beginning of the episode, average urine output, inotropic score and diuretic dose, it was found that there was no statistically significant difference in change in potassium levels after EPR and IVPR: 0.86 mmol/L (±0.8) and 0.82 mmol/L (±0.7) respectively (p=0.86, 95% CI -0.08 to 1.10), or percentage change in potassium level after enteral and intravenous replacement: 26% (±30) and 24% (±20) (95% CI -3.42 to 4.03, p=0.87). CONCLUSION: EPR may be an equally efficacious alternative first-line therapy in treating hypokalaemia after surgery in selective patients with congenital heart disease. ETHICS AND DISSEMINATION: This study has been approved by Ethics Review Committee at AKU. TRIAL REGISTRATION NUMBER: NCT02015962.

Burden of Cardiac Siderosis in a Thalassemia-Major Endemic Population: A Preliminary Report From Pakistan.

OBJECTIVE: To describe the initial experience and demographics of T2* cardiac magnetic resonance-based myocardial-iron quantification of transfusion-dependent thalassemia-major (TM) patients from Pakistan and the correlation with serum ferritin. METHODS: Eligible TM patients presenting between April 2014 and April 2015 to Aga Khan University Hospital, Pakistan, for T2*CMR were included. The severity of myocardial-iron deposition was defined as follows: normal T2*>20 ms, mild-moderate T2*10 to 20 ms, and severe T2*<10 ms. Cardiac symptoms were classified using the NYHA functional classification. Echocardiographic systolic and diastolic functions were performed. Continuous variables were presented as the median (minimum-maximum value). Correlation was measured using the Spearman rank correlation. Multivariate logistic regression was used to determine factors associated with the NYHA functional class. RESULTS: A total of 83 patients (49 male and 34 female) with TM, age 19 (5 to 45) years at presentation for T2*CMR, were reviewed. At presentation, 70% of the patients were classified as NYHA class II or worse. T2*<20 ms was observed in 62.6% of the patients, with 47% showing severe iron deposition (T2<10 ms). No correlation of T2*<20 ms (r=-0.157, P=0.302) and T2*<10 ms (r=-0.128, P=0.464) was observed with serum ferritin. On multivariate analysis, lower T2* values correlated with a worsening NYHA functional class. CONCLUSIONS: There is a high prevalence of severe myocardial iron load in Pakistani TM patients. Serum ferritin did not correlate with T2* values. Lower T2* was the only clinical factor associated with the NYHA functional class.

On-table Extubation after Open Heart Surgery in Children: An Experience from a Tertiary Care Hospital in a Developing Country.

BACKGROUND: Recent advances in various disciplines of medicine have significantly changed the courses following cardiac surgery in children. On-table extubation (OTE) after open heart surgery in children is evolving. OBJECTIVE: To assess the rate of postoperative complications in children extubated on table after open heart surgery. DESIGN: This is a retrospective, descriptive study. SETTING: Operating room (OR) then admitted to the pediatric intensive care unit (PICU). PATIENTS: All pediatric patients (between 0 and 18 years) undergoing open heart surgery between January 2011 and June 2013. INTERVENTION: On-table extubation. OUTCOME MEASURES: Rates of immediate postoperative complications, i.e., re-intubation, significant bleeding, low cardiac output syndrome, and arrhythmia in PICU, were assessed. Data are presented as frequencies and mean ± standard deviation. RESULTS: A total of 82 patients were included. Mean age at time of operation was 7.25 ± 6.6 years. Fifty-three percent (n = 44) were <5 years old and 64% (n = 53) were men. Ventricular septal defect (47%, n = 39) was the most common lesion, followed by atrial septal defect (36%, n = 30), and tetralogy of Fallot (15%, n = 12), which were repaired. Cardiopulmonary bypass and aortic cross clamp time were 72.3 ± 34.2 and 47.3 ± 27.8 minutes, respectively. The mean inotrope score was 2.66 ± 3.53. There was no mortality in the cohort, whereas 97.8% (n = 80) had no complications during PICU stay. One patient (1.1%) required re-intubation for respiratory failure and one patient (1.1%) had arrhythmia that was medically managed. The mean length of PICU stay was 1.77 ± 0.985 days. CONCLUSION: On-table extubation in children after open heart surgery was feasible and safe in selected group of patients. There was no major complication observed in the PICU.

Effect of L-type calcium channel blocker (amlodipine) on myocardial iron deposition in patients with thalassaemia with moderate-to-severe myocardial iron deposition: protocol for a randomised, controlled trial.

INTRODUCTION: Sideroblastic cardiomyopathy secondary to repeated blood transfusions is a feared complication in thalassaemia. Control of myocardial iron is thus becoming the cornerstone of thalassaemia management. Recent evidence suggests a role for L-type Ca(2+) channels in mediating iron uptake by the heart. Blocking the cellular iron uptake through these channels may add to the benefit of therapy to standard chelation in reducing myocardial iron. We aim to determine the efficacy of amlodipine (a calcium channel blocker) as an adjunct to standard aggressive chelation in retarding myocardial iron deposition in thalassaemics with or without cardiomyopathy. OUTCOMES: The primary outcome is to compare the efficacy of amlodipine+chelation (intervention) versus standard chelation (control) in retarding myocardial iron deposition. Secondary outcomes include the effect of amlodipine therapy on systolic and diastolic function, strain and strain rate and liver iron content. METHODS AND ANALYSIS: This is a single-centre, parallel-group, prospective randomised control trial. Twenty patients will be randomised in a 1:1 allocation ratio into the intervention and control arms. In addition to conventional echocardiography, MRI T2* values for assessment of cardiac and liver iron load will be obtained at baseline and at 6 and 12 months. Cardiac T2* will be reported as the geometric mean and per cent coefficient of variation, and an increase in cardiac T2* values from baseline will be used as an end point to compare the efficacy of therapy. A p Value of <0.05 will be considered significant. STUDY SETTING: Department of Pediatric and Child Health, Aga Khan University Hospital, Karachi, Pakistan. ETHICS AND DISSEMINATION: This study has been approved by the Ethics Review Committee and Clinical Trials Unit at The Aga Khan University with respect to scientific content and compliance with applicable research and human subjects regulations. Findings will be reported through scientific publications and research conferences and project summary papers for participants. TRIAL REGISTRATION NUMBER: ClinicalTrials.Gov. Registration no: NCT02065492.

Late pulmonary valve replacement in patients with pulmonary atresia and intact ventricular septum: a case-matched study.

BACKGROUND: Pulmonary valve replacement (PVR) is a common therapy for chronic pulmonary regurgitation. However, the use of this strategy is mostly based on the studies performed on patients with tetralogy of Fallot (TOF) and not in patients with pulmonary atresia/intact ventricular septum (PA/IVS). The aim of this study is to evaluate our experience with PVR in patients with PA/IVS and compare them with a matched cohort of TOF patients. METHODS: Between 1995 and 2009, 13 patients with PA/IVS underwent a late PVR. Matched TOF control subjects were identified for 12 of these patients. Before and after PVR echocardiographic, magnetic resonance imaging, exercise test, Holter, and electrocardiographic data were compared between groups. RESULTS: There was no mortality in either group. The PVR improved pulmonary regurgitant fraction and right ventricular volumes in all patients. Patients with PA/IVS had more significant tricuspid regurgitation (TR [at least moderate]) by echocardiography and magnetic resonance imaging before PVR (n = 11 [85%] versus n = 1 [8%]; p = 0.003) and had more tricuspid valve repairs than TOF patients (n = 9 [69%] versus n = 1 [8%]; p = 0.004). Repair was undertaken by a combination of techniques. Although TR was improved early postoperatively, only 2 of 9 patients (22%) were free from significant TR at most recent follow-up (median 2.5 years; range, 0.1 to 10.9). No patient underwent reoperation at latest follow-up. CONCLUSIONS: Patients with PA/IVS can undergo a late PVR with excellent results. Significant TR and repair are more commonly observed among patients with PA/IVS compared with TOF patients. Although tricuspid valve repair improves regurgitation early, TR tends to recur, suggesting the need for further refinement of current surgical techniques.