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Aims To determine the impact of a quality improvement (QI) initiative in the area of paediatric echocardiography (echo) in a low- to middle-income country (LMIC). Care for patients with congenital heart disease is challenging, especially in LMICs. Collaborative learning through QI projects is imperative to ensure improvement in delivery processes leading to better patient outcomes. Methods and results This QI initiative was taken by a team consisting of physicians and sonographers. Problems were identified, a key driver diagram (KDD) was created, and simple process re-engineering was done using interventions based on the KDD. Metrics (five process and one outcome) were assessed to determine the effectiveness of the QI project. The process metrics assessed were comprehensiveness of exam, timeliness of reporting, diagnostic accuracy and error, and sedation adverse event rates of transthoracic echocardiograms, while a novel comprehensive echo laboratory (lab) quality score was developed as an outcome metric. Data were collected quarterly and analysed in the post-implementation phase. Significant improvement was seen in comprehensive mean score (20.4–29.7), timeliness (40–95%), and diagnostic accuracy rate (91–100%), while a decrease was seen in the diagnostic error rate (7.5–3.5%) and the sedation adverse event rate (6.8–0%), pre- vs. post-implementation. The overall quality outcome score improved from 7 to 19 and the echo lab was able to achieve adequate quality. Conclusion This QI initiative produced improvement in all the processes, and the overall quality of the echo lab without any substantial increase in resources or cost.

Background Evidence suggests improvement in nursing staff satisfaction, competence, and retention after implementation of evidence-based mentorship programmes. When guided by a framework of compassion, mentoring as a caring action can not only build healthy, transformative relationships but a similar behavior is reciprocated to patients which subsequently can drive patient experience of care. However, examples of on-job mentorship programs for nurses in low- and middle-income countries (LMIC) are limited. Objective The objective of the study was to develop an on-job nursing mentorship programme using a compassionate framework aimed at improving nurses’ experience and thus enhancing patient experience in a tertiary care hospital in Pakistan. Methods Designed as an intervention development study, it was completed between January 2018–December 2019. The programme was developed by a team composed of service and nursing leadership, director patient experience of care and a compassion specialist using a theory of change model. The package followed a series of steps, a) identification of a framework, b) creation of working group c) needs assessment and d) multiple meetings to frame the model followed by implementing the preconditions for roll-out of the programme with the frontline staff. Results The eventual outcome was improving the patient’s experience of compassion while the intermediate outcome was to have nurses demonstrate compassionate care. The pre-conditions were identified as: recruitment of staff with appropriate skills for pediatric care, provision of compassionate experience to the frontline nurses by addressing their specific pain points, development of competent head nurses as supervisors and creation of a compassionate culture. To ensure the pre-conditions, various interventions were planned with some implemented through the course of the study while others are in the process of being rolled out. These involved, inclusion of pediatric compassion specific module during orientation of new hires, creation of space to talk about compassionate skills with staff, provision of trainings and mentorship to create competent head nurses, and creating a culture that promoted and recognized compassionate care values. Conclusion The approach helped to delineate feasible pathways for an on-job compassionate mentorship programme enhancing routine supervisors' role as facilitators of compassionate care.

This JACC Focus Seminar provides an overview of and highlights recently published research on cardiomyopathies and nutritional heart disease that have a higher prevalence in tropical regions. The development of tropical cardiomyopathies and nutritional cardiovascular disease (CVD) is complicated by high rates of poverty, fragmented health care systems, and suboptimal access to health care because of socioeconomic inequalities, leading to the fact that children, adolescents, and young adults are disproportionally affected. Such tropical cardiomyopathies and nutritional CVD that have not been prevalent in high-income countries in the past decades are now reemerging. When treating migrants or refugees, it is important for attending physicians to consider the burden of endemic diseases in the countries of origin and the likelihood that such patients might be affected. In this review, the authors propose an approach for adequate diagnostic work-up leading to appropriate care for those with suspected or confirmed tropical cardiomyopathies and nutritional CVD.

Early postoperative catheterizations (EPOCs) within 6 weeks after a congenital heart surgical procedure can treat residual lesions and provide important clinical information. However, EPOCs are often assumed to impose additional risk on a vulnerable patient population. This study aimed to describe the EPOC population, evaluate procedural safety, compare EPOC patients with procedure-matched non-EPOC patients, and determine risk factors for poor outcomes using data from the Congenital Cardiac Catheterization Project on Outcomes registry.In a retrospective cohort, demographic, clinical, and procedural characteristics were analyzed for diagnostic and interventional catheterizations performed in 13 participating institutions from January 2014 to December 2017, excluding patients after heart transplant. The primary outcome was a high-severity adverse event (AE). Three distinct analyses included (1) describing the full cohort and EPOC patients, (2) comparing EPOC patients with and without a high-severity AE, and (3) comparing EPOC patients with controls matched on case type.This study included 17,776 catheterizations, with 1399 EPOCs. The high-severity AE rate was 6.4% overall, 8.9% in the EPOC cohort, and 8.4% in matched controls (P = .74). The association between EPOC status and high-severity AE was not significant in a multivariable model (P = .17). In EPOCs with a high-severity AE, median procedure duration was 30 minutes longer (P.001), and median time from surgical procedure to catheterization was 3 days longer (P = .05).EPOC was not associated with additional risk. Individual patient characteristics of size and hemodynamic vulnerability may serve as informative predictors. Timely catheterization may preempt further clinical deterioration, and intraprocedure duration optimization may correlate with improved outcomes.

Adult congenital heart diseases (ACHD) have distinct health care needs that require life-long care. Limited data is available from low-middle income countries (LMIC). This descriptive study conducted in Pakistan, aimed to assess patients and health care professionals understanding of the needs for ACHD care and the perceived barriers to care.A telephone survey was conducted of ACHD patients. An e mail survey was sent to the paediatric and adult cardiologists of five institutions (3 public and 2 private) that provide ACHD services in Pakistan. Descriptive statistics (frequencies, mean ± SD, median) were used for data analysis.A total of 128 ACHD patients were surveyed, 65 (51%) were females with a mean age of 29.4±10.4 years. Atrial septal defect repair was the most common surgical procedure. Mean age at surgery was 25.6±10.49 years, and a surgical follow-up period of 3.8±2.3 years. Majority (n=3, 60%) of the health care professionals (HCPs) responded that 75-100% of the ACHD surgical patients would need lifelong care, yet 10-25% return to their cardiology clinics. Most of the surveyed ACHD patients (89%, n=114) demonstrated a lack of understanding of life-long care after surgery due to not being communicated by their HCPs. Cost and travelling issues were the barriers highlighted by HCPs. Both ACHD patients (96%, n=122) and HCP (100%, n=5) underscored their interest in life long care.Majority of ACHD patients in Pakistan did not know that life-long follow-up is needed. Education regarding lifelong care for ACHD patients was identified as a means to alleviate the knowledge gap.

ObjectivesCardiac 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 settingA 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 valueResults100 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 ConclusionThis QI initiative improved the chelation therapy leading to improved cardiac status in TDT patients at the participating centres.

Challenges and special aspects related to the management and prognosis of pulmonary hypertension (PH) in middle- to low-income regions (MLIRs) range from late presentation to comorbidities, lack of resources and expertise, cost, and rare options of lung transplantation. Expert consensus recommendations addressing the specific challenges for prevention and therapy of PH in MLIRs with limited resources have been lacking. To date, 6 MLIR-PH registries containing mostly adult patients with PH exist. Importantly, the global prevalence of PH is much higher in MLIRs compared with high-income regions: group 2 PH (left heart disease), pulmonary arterial hypertension associated with unrepaired congenital heart disease, human immunodeficiency virus, or schistosomiasis are highly prevalent. This consensus statement provides selective, tailored modifications to the current PH guidelines to address the specific challenges faced in MLIRs, resulting in the first pragmatic and cost-effective consensus recommendations for PH care providers, patients, and their families.

The patient experience team at a private tertiary care hospital used the Theory of Change to design a family-centered developmental monitoring intervention, building on an ongoing initiative. The design entailed (i) a monitoring form: Survey of Well-Being of Young Children (SWYC) being an easy parent-report measure; (ii) family support intervention: the Care for Child Development module to enhance parent-child interactions; (iii) timing: utilizing wait time to also enhance families' experience; (iv) the service providers: psychology trainees as volunteers; and (v) reinforcement: by the pediatrician in the regular consultation health visit capitalizing on the established rapport with families. All families with children under 5 years 5 months 31 days of age in selected acute, complex, and developmental care clinics were eligible. Feedback from stakeholders indicated that the monitoring process was useful and imparted important information for parents and pediatricians, while the trainees felt the experience to be significant for their own learning. The authors conclude that the designed intervention model for a family-centric approach was acceptable and feasible. Key recommendations have been presented for further scale-up.

Background: With the dearth of trained care providers to diagnose congenital heart disease (CHD) and a surge in machine learning (ML) models, this review aims to estimate the diagnostic accuracy of such models for detecting CHD.Methods: A comprehensive literature search in the PubMed, CINAHL, Wiley Cochrane Library, and Web of Science databases was performed. Studies that reported the diagnostic ability of ML for the detection of CHD compared to the reference standard were included. Risk of bias assessment was performed using Quality Assessment for Diagnostic Accuracy Studies-2 tool. The sensitivity and specificity results from the studies were used to generate the hierarchical Summary ROC (HSROC) curve.Results: We included 16 studies (1217 participants) that used ML algorithm to diagnose CHD. Neural networks were used in seven studies with overall sensitivity of 90.9% (95% CI 85.2–94.5%) and specificity was 92.7% (95% CI 86.4–96.2%). Other ML models included ensemble methods, deep learning and clustering techniques but did not have sufficient number of studies for a meta-analysis. Majority (n=11, 69%) of studies had a high risk of patient selection bias, unclear bias on index test (n=9, 56%) and flow and timing (n=12, 75%) while low risk of bias was reported for the reference standard (n=10, 62%).Conclusion: ML models such as neural networks have the potential to diagnose CHD accurately without the need for trained personnel. The heterogeneity of the diagnostic modalities used to train these models and the heterogeneity of the CHD diagnoses included between the studies is a major limitation.

Background: A major contributor to under-five mortality is the death of children in the 1st month of life. Intrapartum complications are one of the major causes of perinatal mortality. Fetal cardiotocograph (CTGs) can be used as a monitoring tool to identify high-risk women during labor. Aim: The objective of this study was to study the precision of machine learning algorithm techniques on CTG data in identifying high-risk fetuses. Methods: CTG data of 2126 pregnant women were obtained from the University of California Irvine Machine Learning Repository. Ten different machine learning classification models were trained using CTG data. Sensitivity, precision, and F1 score for each class and overall accuracy of each model were obtained to predict normal, suspect, and pathological fetal states. Model with best performance on specified metrics was then identified. Results: Determined by obstetricians' interpretation of CTGs as gold standard, 70% of them were normal, 20% were suspect, and 10% had a pathological fetal state. On training data, the classification models generated by XGBoost, decision tree, and random forest had high precision (>96%) to predict the suspect and pathological state of the fetus based on the CTG tracings. However, on testing data, XGBoost model had the highest precision to predict a pathological fetal state (>92%). Conclusion: The classification model developed using XGBoost technique had the highest prediction accuracy for an adverse fetal outcome. Lay health-care workers in low- and middle-income countries can use this model to triage pregnant women in remote areas for early referral and further management.

Background: The purpose of the study was to design and test the feasibility of a recent guideline of family-centered psychosocial support as an approach to developmental monitoring in a pediatric outpatient setting.Methods: The patient experience team at a private tertiary care hospital leveraged an on-going patient and family-centric initiative in the service to implement the intervention. The intervention delivery model was designed using the theory of change model and entailed the following i) the service providers: paediatricians but with support from psychology trainee volunteers to address time constraints; ii) monitoring form: Survey of Well-Being of Young Children (SWYC) being feasible and designed for regular developmental monitoring as parent report; iii) family support intervention: the Care for Child Development module to enhance parent-child interactions given team’s successful experience with inpatient children; iv) timing: utilize wait time to also enhance families’ experience and v) reinforcement: reinforced by the paediatrician in the consultation visit to capitalize on the established rapport and relationship with families. All families with children under 5 years 5 months of age in selected clinics (acute care, complex care, developmental issues) were eligible. The study was evaluated for acceptability and implementation feasibility. Families were interviewed about their experience; trainees provided a written narrative while physicians provided feedback on email. These were thematically analyzed using an inductive approach.Results: A total 182 families were administered the SWYC with 54% children detected for further review on cognitive milestones and 76% on social-emotional milestones. Forty-eight families were interviewed regarding their experience with the intervention. They reported the monitoring process to be useful and important for them as parents indicating acceptability. Paediatricians and trainees found the intervention to be important for parents and children’s health also requesting further understanding about child’s functioning. The trainees further expressed the experience to be significant for their own learning.Conclusion: The authors conclude that the intervention model for a family-centric approach to monitoring was acceptable to the families and the service providers. The intervention when implemented using a robust behaviour change framework can enhance feasibility.

Aims Congenital heart disease (CHD) is the most common congenital malformation. Despite the worldwide burden to patient wellbeing and health system resource utilization, tracking of long-term outcomes is lacking, limiting the delivery and measurement of high-value care. To begin transitioning to value-based healthcare in CHD, the International Consortium for Health Outcomes Measurement aligned an international collaborative of CHD experts, patient representatives, and other stakeholders to construct a standard set of outcomes and risk-adjustment variables that are meaningful to patients. Methods and results The primary aim was to identify a minimum standard set of outcomes to be used by health systems worldwide. The methodological process included four key steps: (i) develop a working group representative of all CHD stakeholders; (ii) conduct extensive literature reviews to identify scope, outcomes of interest, tools used to measure outcomes, and case-mix adjustment variables; (iii) create the outcome set using a series of multi-round Delphi processes; and (iv) disseminate set worldwide. The Working Group established a 15-item outcome set, incorporating physical, mental, social, and overall health outcomes accompanied by tools for measurement and case-mix adjustment variables. Patients with any CHD diagnoses of all ages are included. Following an open review process, over 80% of patients and providers surveyed agreed with the set in its final form. Conclusion This is the first international development of a stakeholder-informed standard set of outcomes for CHD. It can serve as a first step for a lifespan outcomes measurement approach to guide benchmarking and improvement among health systems.

Universal primary education is critical for individual academic growth and overall adult productivity of nations. Estimates indicate that 25% of 59 million primary age out of school children drop out and early grade failure is one of the factors. An objective and feasible screening measure to identify at-risk children in the early grades can help to design appropriate interventions. The objective of this study was to use a Machine Learning algorithm to evaluate the power of Electroencephalogram (EEG) data collected at age 4 in predicting academic achievement at age 8 among rural children in Pakistan. Demographic and EEG data from 96 children of a cohort along with their academic achievement in grade 1–2 measured using an academic achievement test of Math and language at the age of 7–8 years was used to develop the machine learning algorithm. K- Nearest Neighbor (KNN) classifier was used on different model combinations of EEG, sociodemographic and home environment variables. KNN model was evaluated using 5 Stratified Folds based on the sensitivity and specificity. In the current dataset, 55% and 74% failed in the mathematics and language test respectively. On testing data across each fold, the mean sensitivity and specificity was calculated. Sensitivity was similar when EEG variables were combined with sociodemographic, and home environment (Math = 58.7%, Language = 66.3%) variables but specificity improved (Math = 43.4% to 50.6% and Language = 32% to 60%). The model requires further validation for EEG to be used as a screening measure with adequate sensitivity and specificity to identify children in their preschool age who may be at high risk of failure in early grades.

The current study conducted in Pakistan aimed to test if parental stimulation practices modify the effect of general child health status on early developmental risks in hospitalized children. Development was assessed using the Survey of Well-being of Young Children. Child health status was a global rating on a Likert scale. Parental engagement was categorized based on the number of activities with their children (low ≤ 3, high > 3). A total of 231 children were assessed. Children with poor health status were reported to be 1.9 (95% Confidence Intervals [CI] 1.4–2.8, p = 0.000) times at risk of developmental delay by parents who had lower engagement and about 3 times (3.63 for mothers CI 1.79–7.37, p = 0.003; 2.96 for fathers CI 1.17–7.49, p = 0.027) significantly at risk of behaviour–emotional concerns by parents with higher engagement. The authors conclude that parental engagement and developmental screening can be incorporated as part of in-patient paediatric assessment. publishedVersion

Social media can complement organizational communication strategy which is integral to employee engagement. However, successful case studies which can allow replication are limited. The objective of the study is to describe the design, implementation, and evaluation of a social media-based communication strategy in a tertiary care hospital in Pakistan. The leadership of the pediatric service line developed an intervention plan to engage the employees with the newly reframed vision to improve patient and family experience. An online communication platform—Facebook page—was created for all employees of the pediatric service line. The strategy to influence employees was based on Cialdini's six principles of persuasion. Implementation of the strategy between October 2017 and December 2019 was evaluated for reach, discussion themes, and outcomes using the framework by Murdough (2009). Quantitative indicators included total posts, mean comments, and reactions per post. Posts were qualitatively analyzed with an emergent approach for insights into the discussion. The analysis revealed a total of 9,085 posts, with mean reactions per post of 8.4, mean comments of 7.2, and active viewership by 90% members on average. In terms of post types, photos were the highest (4,779), while videos were the lowest (1,163). Qualitative analysis indicated 54% of the posts were of the theme “inspirational and thought provoking,” while the greatest engagement was generated on the theme “challenges and solution.” The authors conclude that the strategy was successfully implemented to maintain active membership, engage employees in meaningful conversations, and have them express intent to execute quality improvement projects.

Background Evidence suggests improvement in nursing staff satisfaction, competence, and retention after implementation of evidence-based mentorship programmes. When guided by a framework of compassion, mentoring as a caring action can not only build healthy, transformative relationships and energize the workplace environments but a similar behavior is reciprocated to patients which subsequently can drive patient experience of care. However, examples of on-job mentorship programs for nurses in low- and middle-income countries (LMIC) are limited owing to human and financial constraints. Objective The current study describes the development of a feasible an on-job nursing mentorship program using a compassionate framework aimed at improving nurses’ experience and thus enhancing patient experience from a tertiary care hospital in Pakistan. Methods The intervention package was built by a team composed of service and nursing leadership, director patient experience of care and a compassion specialist using a theory of change model. The package followed a series of steps that were based on the principles of implementation science, a) identification of a framework, b) creating a working group and c) multiple meetings to frame the model followed by implementing the preconditions for roll-out of the programme. Results The intermediate outcome was to have nurses demonstrate skills of compassion with the eventual outcome of improving the patient's experience of compassion. The pre-conditions were identified as: recruitment of staff with appropriate skills for pediatric care, precision of compassionate experience definition, creation of a compassionate culture and development of competent head nurses as supervisor. In order to ensure the pre-conditions, various interventions were planned and some were implemented through the course of designing. These involved, creation of space to talk about compassionate skills with staff, provide trainings and on-job coaching, revising career ladder of supervisors, inclusion of pediatric compassion specific nursing education service orientation program, recognition of efforts and promotion of compassionate values in numerous communication platforms, arrangement of training workshops, creation of checklists and development of dashboards. Conclusion The approach helped to delineate feasible pathways for an on-job compassionate mentorship programme through strengthening routine supervision leading to improved patient experience of care in the pediatric service.

Background Keeping in view the developmental origin of health and disease hypothesis, the aim of this study was to assess differences in cardiac and vascular structure and function in children exposed to preeclampsia in utero compared with those of normotensive mothers. The hypothesis under investigation was that children exposed to preeclampsia would have altered cardiac and vascular structure and function compared with the unexposed group. Methods This was a retrospective cohort study that included children 2 to 10 years of age born to mothers with and without exposure to preeclampsia in utero (n = 80 in each group). Myocardial morphology and function using echocardiography and carotid intima-media thickness and pulse-wave velocity were determined. Multivariate linear regression was used to compare preeclampsia-exposed and nonexposed groups. Subgroup analysis to assess differences between early- and late-onset preeclampsia was also performed. Results Forty-one percent of mothers (n = 33) had early-onset preeclampsia. Children in the exposed group had a significantly higher prevalence of stage 1 systolic and diastolic hypertension (22% [n = 18] and 35% [n = 18], respectively) compared with the unexposed group (9% [n = 7] and 19% [n = 15], respectively; P = .01). Children in the exposed group also had higher pulse-wave velocity compared with those in the unexposed group (0.42 ± 0.1 vs 0.39 ± 0.1, P = .03). Subgroup analysis revealed that changes in blood pressure and pulse-wave velocity were determined primarily by early-onset preeclampsia. There was no significant difference in cardiac morphology or systolic and diastolic function between the exposed and unexposed groups. Conclusion In utero exposure to preeclampsia has an effect on vascular function in children aged 2 to 10 years, related primarily to early-onset disease. Routine blood pressure screening should be recommended for such children.

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.

Challenges and special aspects related to the management and prognosis of pulmonary hypertension (PH) in middle- to low-income regions (MLIRs) range from late presentation to comorbidities, lack of resources and expertise, cost, and rare options of lung transplantation. Expert consensus recommendations addressing the specific challenges for prevention and therapy of PH in MLIRs with limited resources have been lacking. To date, 6 MLIR-PH registries containing mostly adult patients with PH exist. Importantly, the global prevalence of PH is much higher in MLIRs compared with high-income regions: group 2 PH (left heart disease), pulmonary arterial hypertension associated with unrepaired congenital heart disease, human immunodeficiency virus, or schistosomiasis are highly prevalent. This consensus statement provides selective, tailored modifications to the current PH guidelines to address the specific challenges faced in MLIRs, resulting in the first pragmatic and cost-effective consensus recommendations for PH care providers, patients, and their families. ispartof: JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY vol:75 issue:19 pages:2463-2477 ispartof: location:United States status: published

ObjectiveInconsistencies 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 measuresGeneral and cardiac-related HRQOL, associated predictors.MethodsThis 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.ResultsThe 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.ConclusionsHRQOL 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.

IntroductionRheumatic heart diseases (RHDs) contribute significant morbidity and mortality globally. To reduce the burden of RHD, timely initiation of secondary prophylaxis is important. The objectives of this study are to determine the frequency of subclinical RHD and to train a deep learning (DL) algorithm using waveform data from the digital auscultatory stethoscope (DAS) in predicting subclinical RHD.Methods and analysisWe aim to recruit 1700 children from a group of schools serving the underprivileged over a 12-month period in Karachi (Pakistan). All consenting students within the age of 5–15 years with no underlying congenital heart disease will be eligible for the study. We will gather information regarding sociodemographics, anthropometric data, history of symptoms or diagnosis of rheumatic fever, phonocardiogram (PCG) and electrocardiography (ECG) data obtained from DAS. Handheld echocardiogram will be performed on each study participant to assess the presence of a mitral regurgitation (MR) jet (>1.5 cm), or the presence of aortic regurgitation (AR) in any view. If any of these findings are present, a confirmatory standard echocardiogram using the World Heart Federation (WHF) will be performed to confirm the diagnosis of subclinical RHD. The auscultatory data from digital stethoscope will be used to train the deep neural network for the automatic identification of patients with subclinical RHD. The proposed neural network will be trained in a supervised manner using labels from standard echocardiogram of the participants. Once trained, the neural network will be able to automatically classify the DAS data in one of the three major categories—patient with definite RHD, patient with borderline RHD and normal subject. The significance of the results will be confirmed by standard statistical methods for hypothesis testing.Ethics and disseminationEthics approval has been taken from the Aga Khan University, Pakistan. Findings will be disseminated through scientific publications and to collaborators.Article focusThis study focuses on determining the frequency of subclinical RHD in school-going children in Karachi, Pakistan and developing a DL algorithm to screen for this condition using a digital stethoscope.

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.

Supplemental Digital Content is available in the text., Background: The use of cardiac computed tomography angiography (CCTA) as a complementary diagnostic modality to echocardiography in patients with congenital heart diseases (CHDs) is expanding in low- and middle-income countries. The adoption of As Low As Reasonably Achievable techniques is not widespread, resulting in significant unintended radiation exposure, especially in children. Simple quality improvement measures geared toward reducing radiation dose can have a impact on patient safety in resource-limited centers in low- and middle-income countries. Objectives: To determine how a quality improvement initiative can reduce radiation exposure during CCTA in patients with CHD. Methods: We designed a key driver -based quality initiative to reduce radiation dose during CCTA for CHD using protocol optimization, communication, and training and implementation as the drivers for intervention. Preintervention variables (radiation exposure, scanning protocols, and image quality) were collected from September 2012 to July 2016 and compared with variables in the postimplementation phase (February 2017 to July 2017). We compared quantitative and categorical variables using the chi-square test. Linear regression analysis was used to evaluate the effect of various factors on radiation dose. Results: We documented a reduction in the effective dose in the postintervention versus preintervention phase (mean, 2.0 versus 21 mSv, P < 0.0001, respectively). Linear regression showed that the optimal organizational levels are associated with the same reduction in radiation. This finding shows that the time factor translates a combination of organizational and technical factors that contributed to the reduction in radiations. Conclusions: Our project showed a reduction in CCTA-associated radiation exposure.

CONTEXT: In the last few decades, data acquisition and processing has seen tremendous amount of growth, thus sparking interest in machine learning (ML) within the health care system. OBJECTIVE: Our aim for this review is to provide an evidence map of the current available evidence on ML in pediatrics and adolescent medicine and provide insight for future research. DATA SOURCES: A literature search was conducted by using Medline, the Cochrane Library, the Cumulative Index to Nursing and Allied Health Literature Plus, Web of Science Library, and EBSCO Dentistry & Oral Science Source. STUDY SELECTION: Articles in which an ML model was assessed for the diagnosis, prediction, or management of any condition in children and adolescents (0–18 years) were included. DATA EXTRACTION: Data were extracted for year of publication, geographical location, age range, number of participants, disease or condition under investigation, study methodology, reference standard, type, category, and performance of ML algorithms. RESULTS: The review included 363 studies, with subspecialties such as psychiatry, neonatology, and neurology having the most literature. A majority of the studies were from high-income (82%; n = 296) and upper middle-income countries (15%; n = 56), whereas only 3% (n = 11) were from low middle-income countries. Neural networks and ensemble methods were most commonly tested in the 1990s, whereas deep learning and clustering emerged rapidly in the current decade. LIMITATIONS: Only studies conducted in the English language could be used in this review. CONCLUSIONS: The interest in ML has been growing across various subspecialties and countries, suggesting a potential role in health service delivery for children and adolescents in the years to come.

IntroductionPre-eclampsia is a common disorder associated with serious maternal and fetal complications. It is associated with abnormal placentation, which significantly reduces flow, resulting in a relative hypoxic state. These pathophysiological changes lead to subtle macrovascular and cardiac structural and functional changes in the fetus. This can predispose the child with maternal history of pre-eclampsia to risk of premature cardiovascular disease.Methods and analysisThe children will be identified from a cohort of women with pre-eclampsia. The study will be conducted at The Aga Khan University Hospital, Karachi. Inclusion criteria will be children who are between 2 and 5 years of age and have a maternal history of pre-eclampsia. The child’s current weight, height and blood pressure will be recorded. A two-dimensional functional echocardiogram and vascular assessment will be performed to evaluate alterations in cardiac function as well as macrovascular remodelling in these children. Data will be presented as mean±SD, median (IQR) or percentages as appropriate. Independent t-test or Mann-Whitney U test will be used for testing of continuous variables (based on the assumption of normality). A pEthics and disseminationEthical approval has been obtained from AKUH Ethics Review Committee. Findings will be disseminated through scientific publications and project summaries for the participants.

Background Beta thalassaemia is a common inherited blood disorder. The need for frequent blood transfusions in this condition poses a difficult problem to healthcare systems. The most common cause of morbidity and mortality is cardiac dysfunction from iron overload. The use of iron chelation therapy has reduced the severity of systemic iron overload but specific, non-toxic treatment is required for removal of iron from the myocardium. Objectives To assess the effects of calcium channel blockers combined with standard iron chelation therapy in people with transfusion-dependent beta thalassaemia on the amount of iron deposited in the myocardium, on parameters of heart function, and on the incidence of severe heart failure or arrhythmias and related morbidity and mortality. Search methods We searched the Cochrane Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched ongoing trials databases, and the reference lists of relevant articles and reviews. Date of last search: 24 February 2018. Selection criteria We included randomised controlled trials of calcium channel blockers combined with standard chelation therapy compared with standard chelation therapy alone or combined with placebo in people with transfusion-dependent beta thalassaemia. Data collection and analysis Two authors independently applied the inclusion criteria for the selection of trials. Two authors assessed the risk of bias of trials and extracted data and a third author verified these assessments. The authors used the GRADE system to assess the quality of the evidence. Main results Two randomised controlled trials (n = 74) were included in the review; there were 35 participants in the amlodipine arms and 39 in the control arms. The mean age of participants was 24.4 years with a standard deviation of 8.5 years. There was comparable participation from both genders. Overall, the risk of bias in included trials was low. The quality of the evidence ranged across outcomes from low to high, but the evidence for most outcomes was judged to be low quality. Cardiac iron assessment, as measured by heart T2*, did not significantly improve in the amlodipine groups compared to the control groups at six or 12 months (low-quality evidence). However, myocardial iron concentration decreased significantly in the amlodipine groups compared to the control groups at both six months, mean difference -0.23 mg/g (95% confidence interval -0.07 to -0.39), and 12 months, mean difference -0.25 mg/g (95% confidence interval -0.44 to -0.05) (low-quality evidence). There were no significant differences between treatment and control groups in serum ferritin (low-quality evidence), liver T2* (low-quality evidence), liver iron content (low-quality evidence) and left ventricular ejection fraction (low-quality evidence). There were no serious adverse events reported in either trial; however, one trial (n = 59) reported mild adverse events, with no statistically significant difference between groups (low-quality evidence). Authors' conclusions The available evidence does not clearly suggest that the use of calcium channel blockers is associated with a reduction in myocardial iron in people with transfusion-dependent beta thalassaemia, although a potential for this was seen. There is a need for more long-term, multicentre trials to assess the efficacy and safety of calcium channel blockers for myocardial iron overload, especially in younger children. Future trials should be designed to compare commonly used iron chelation drugs with the addition of calcium channel blockers to investigate the potential interplay of these treatments. In addition, the role of baseline myocardial iron content in affecting the response to calcium channel blockers should be investigated. An analysis of the cost-effectiveness of the treatment is also required.

Cardiovascular, respiratory, and related disorders (CVRDs) are the leading causes of adult death worldwide, and substantial inequalities in care of patients with CVRDs exist between countries of high income and countries of low and middle income. Based on current trends, the UN Sustainable Development Goal to reduce premature mortality due to CVRDs by a third by 2030 will be challenging for many countries of low and middle income. We did systematic literature reviews of effectiveness and cost-effectiveness to identify priority interventions. We summarise the key findings and present a costed essential package of interventions to reduce risk of and manage CVRDs. On a population level, we recommend tobacco taxation, bans on trans fats, and compulsory reduction of salt in manufactured food products. We suggest primary health services be strengthened through the establishment of locally endorsed guidelines and ensured availability of essential medications. The policy interventions and health service delivery package we suggest could serve as the cornerstone for the management of CVRDs, and afford substantial financial risk protection for vulnerable households. We estimate that full implementation of the essential package would cost an additional US$21 per person in the average low-income country and $24 in the average lower-middle-income country. The essential package we describe could be a starting place for low-income and middle-income countries developing universal health coverage packages. Interventions could be rolled out as disease burden demands and budgets allow. Our outlined interventions provide a pathway for countries attempting to convert the UN Sustainable Development Goal commitments into tangible action.

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

Objective After complete surgical repair the majority of tetralogy of Fallot (ToF), patients stay ≤2 days in the Cardiac Intensive Care Unit (CICU) while some may stay longer. We undertook this study to investigate the factors associated with shorter vs. longer length of stay in the CICU to help manage resources effectively. Design Patients who underwent ToF repair at Aga Khan University, Pakistan, between July 2006 and December 2011 were studied in a case-control design. Clinical parameters were compared between short stay group (SSG) (≤2 days) and long stay group (LSG) (>2 days). Odds ratios were calculated, and regression was performed. Results Ninety-eight patients (LSG 65, SSG 33) were included. Patients with lower preoperative saturation were 2.67 times more likely to be in the LSG group (P = .02). At 4 hours postoperatively, patients with a higher inotropic score (odds ratio [OR] = 3.03, confidence interval [CI] = 1.19–7.7, P = .02), higher central venous pressure (OR = 3.04, CI = 1.27–7.32, P = .013), and significant tachycardia at 4 hours (OR = 3.5, CI = 1.19–10.3. P = .02) were at risk for having a prolonged CICU stay. On multivariate analysis, significant postoperative tachycardia at 4 hours (z-score ≥3) was highly specific (sensitivity = 38.5%, specificity = 84.9%) for predicting the chances of being in the LSG. Other predictors included preop O2 saturation ≤82.5% (sensitivity = 61.1%, specificity = 63.0%) and CVP ≥10 mm Hg at 4 hours (sensitivity = 55.4%, specificity = 71.9%). Conclusion Patients who end up staying longer in the CICU have features that are distinctive in the immediate postoperative period, and this can help clinicians in identifying patients who may need more support.

Intro: Thalassemia Major (TM) is characterized by recurrent blood transfusions leading to myocardial siderosis and complications including arrhythmias and heart failure. These can be reversible with adequate chelation. We explored the effect of improved chelation and subsequent cardiac T2* MRI (CMR) values on regional myocardial function assessed by 2D STE in setting of normal LVEF. Methods: Data was derived from TM patients recruited for the AMIT study (efficacy of amlodipine with chelation in reducing myocardial iron). All patients underwent echocardiographic imaging at baseline, 6 and 12 months. LSsys was extracted from basal, mid, and apical segments of the LV medial and lateral wall by 2D STE technique. A linear regression model was run to determine the effect of improved T2* in the setting of improved chelation on regional LSsys strain parameters. Results: Nineteen TM patients (11 males), median age 16 (8-21) yrs. All participants were transfused every 2-3 weeks. (median pre-transfusional Hb of 9.3 (8.3-10.8) mg/dL). T2* based chelation regimen was initiated at baseline and compliance to this regimen was gauged at regular clinical visits. 25 data points were available from interval 2D STE imaging for analysis. Medial basal LSsys (-17.3% ± 2.2) and lateral basal LSsys (-16.7% ± 4.4) strains were significantly lower than mean apical LSsys strains (-24.9% ± 4.0) (p Conclusion: 2D STE can help identify regional myocardial dysfunction prior to development of overt cardiac dysfunction. In patients with transfusion dependent TM, compliance to an optimized chelation regimen can help improve regional myocardial function prior to progression to systolic dysfunction and clinically symptomatic sideroblastic complications.

This study sought to investigate the effects of exercise on the right ventricle in patients with an obstructed right ventricular outflow tract (RVOT) conduit before and after transcatheter pulmonary valve replacement (TPVR).Conventionally, assessment of the right ventricle in congenital heart disease patients with dysfunctional RVOT conduits is performed at rest. However, this does not reflect dynamic exercise changes.Exercise stress echocardiography (ESE) before and 6 months after TPVR was performed. ESE protocol included measurement of rest and immediate post-exercise RVOT maximal instantaneous gradients (MIGs), right ventricular (RV) systolic pressure, 2-dimensional fractional area change, and global longitudinal strain (GLS).Twenty patients with RVOT conduit obstruction (median age, 18 years), the majority (n = 14) with tetralogy of Fallot, completed the study. Pre-TPVR, the median resting MIG across the RVOT was 53 mm Hg (23 to 95 mm Hg) and increased to 93 mm Hg (49 to 156 mm Hg; p0.001) with exercise. After TPVR, the median MIG at rest was 26 mm Hg (6 to 41 mm Hg, and after exercise, it was 45 mm Hg (9 to 102 mm Hg), both significantly lower than before TPVR (p ≤ 0.001), but there was still a substantial increase in gradient with exercise in many patients. The RV fractional area change, RV GLS, and left ventricular GLS, both at rest and after exercise, were significantly greater after TPVR than before. A greater pre-TPVR exercise-related increase in RV function was associated with improvement in peak Vo2 after TPVR (p = 0.01).In patients with obstructed RVOT conduits, TPVR resulted in significant improvement in conduit stenosis and RV function at both rest and at peak exercise and in exercise cardiopulmonary function. The ability to augment RV function at peak exercise before TPVR was associated with improved exercise capacity 6 months after TPVR.

Artificial intelligence (AI) has revolutionized healthcare in the past few decades (1). Initially being used solely as a medical decision support system, it is anticipated that AI has a potential role in personalized medicine, patient monitoring and improve health service delivery and management (2). AI has penetrated the health care domain rapidly in high income settings where an estimated USD 150 million could be saved with such applications in the next 5 years (3). However, there is limited literature available on its use in low resource settings.

Operating rooms (OR) are often major source of hospital's financial success. High OR utilization is the key in contributing to decreasing disease burden and increasing financial stability. In current era concerted efforts are being taking to create high operating room utilization prior to increasing

BackgroundThe importance of registries for collaborative quality improvement has been overlooked in low/middle-income countries (LMIC). Aga Khan University Hospital (AKUH) in Pakistan joined the Congenital Cardiac Catheterization Project on Outcomes-Quality Improvement (C3PO-QI) in March 2017 with the goal of leveraging international collaboration to improve patient care and institutional standards.MethodsThe C3PO-QI key driver-based approach was used, with certain modifications, for process re-engineering in AKUH’s congenital cardiac catheterisation laboratory (CCL) to reduce radiation exposure during cardiac catheterisation procedures (the primary outcome of C3PO- QI). Educating staff and standardising procedural documentation were the principal goals of the process re-engineering. Data survey was used to assess staff knowledge, attitude and practice before and after the initiative. Additionally, case demographics and outcomes were compared between AKUH and C3PO-QI centres.ResultsThere was an increase in appropriate recording of radiation surrogates (0%–100%, p=0.00) and in the percentage of cases that met the established benchmark of ‘Ideal documentation’ (35% vs 95%, p=0.001). There was also an increase in self-reported staff interest during the case (25% vs 75%, p=0.001). AKUH versus C3PO-QI data showed similar demographic characteristics. There was a slight over-representation of diagnostic cases (42% vs 32%) as compared with interventional (58% vs 68%) at AKUH. Furthermore, interventional procedures were predominately PDA and ASD device closures (n=19 and 15, respectively). The frequency of adverse events were the same between AKUH and collaborative sites.ConclusionCollaborative efforts between developed and LMIC CCL are significant in advancing system-level processes.

Objective. Congenital heart disease (CHD) has an incidence of ~0.8-1%. Outcome of previously diagnosed CHD patients awaiting surgery (either correction or palliation) in a developing country setting is unknown. We strive to determine the outcome of patients with CHD awaiting surgery who present to pediatric intensive care unit (PICU) setting with an acute illness. Design. Retrospective cross-sectional chart review. Setting. Pediatric intensive care unit of The Aga Khan University Hospital, Karachi, Pakistan. Patient. Medical records of infants (1-12 months) with CHD awaiting surgery presenting to the PICU with an acute illness between January 2009 and June 2012 were included. Newly diagnosed CHD patients, those not requiring PICU admission, and those transferred to another hospital were excluded. Results. A total of 34 infants met the inclusion criteria. Median age at presentation was 5 months. Seventy-four percent of the infants had CHD lesion characterized by increased pulmonary blood flow (shunt lesions). Though none of the patients met the strict criteria for sepsis or pneumonia, 74% were admitted with a diagnosis of pneumonia or sepsis. Only 15% of patient had congestive heart failure as an admitting diagnosis. Oxygen therapy was given to 94% of these patients. Fifty-nine percent of these patients expired during the admission, 95% of those expired had multiorgan dysfunction. Conclusion. Patients with CHD awaiting surgery and who admitted to the PICU with acute illness are at high risk for mortality. Stringent criteria to diagnose pneumonia or sepsis should be used in these patients.

IntroductionReduced health-related quality of life (HRQOL) has been reported in postoperative patients with congenital heart disease (CHD). However, there is a paucity of data from low-income and middle-income countries (LMIC). Differences in sociodemographics and sociocultural contexts may influence HRQOL. This protocol paper describes a study exploring HRQOL in surgical patients with CHD from a tertiary hospital in Pakistan. The study findings will assist development of strategies to improve HRQOL in a resource-constrained context.Methods and analysisThis prospective, concurrent triangulation, mixed-methods study aims to compare HRQOL of postsurgery patients with CHD with age-matched healthy siblings and to identify HRQOL predictors. A qualitative component aims to further understand HRQOL data by exploring the experiences related to CHD surgery for patients and parents. Participants include patients with CHD (a minimum of n~95) with at least 1-year postsurgery follow-up and no chromosomal abnormality, their parents and age-matched, healthy siblings. PedsQL 4.0 Generic Core Scales, PedsQL Cognitive Functioning Scale and PedsQL 3.0 Cardiac Module will measure HRQOL. Clinical/surgical data will be retrieved from patients’ medical files. Student’s t-test will be used to compare the difference in the means of HRQOL between CHD and siblings. Multiple regression will identify HRQOL predictors. A subsample of enrolled patients (n~20) and parents (n~20) from the quantitative arm will be engaged in semistructured qualitative interviews, which will be analysed using directed content analysis. Anticipated challenges include patient recruitment due to irregular follow-up compliance. Translation of data collection tools to the Urdu language and back-translation of interviews increases the study complexity.Ethics and disseminationEthics approval has been obtained from The Aga Khan University, Pakistan (3737-Ped-ERC-15). Study findings will be published in peer-reviewed journals and presented at national and international conferences.

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.

Background— Transcatheter pulmonary valve (TPV) replacement is an emerging therapy intended to restore pulmonary valve function in patients with right ventricular outflow tract conduit dysfunction; the impact of this technique on ventricular strain and synchrony is not known. Methods and Results— Cardiac magnetic resonance and ECG data acquired at 1 center as part of the US Melody TPV trial were analyzed. Biventricular strain and mechanical synchrony measurements were made based on short-axis and 4-chamber steady-state free precession images using feature tracking software. Post- versus pre-TPV replacement findings were compared for all patients (n=31) and subgroups with predominant pulmonary regurgitation (n=13) or stenosis (n=18). Most patients had tetralogy of Fallot (18/31). After TPV replacement, left ventricular (LV) circumferential strain increased for the whole cohort ( P P =0.01; pulmonary stenosis P =0.02). LV longitudinal strain increased for the whole cohort ( P =0.02) and pulmonary regurgitation subgroup ( P =0.05); circumferential right ventricular strain increased for the pulmonary stenosis group only ( P =0.05). LV longitudinal synchrony improved significantly in the pulmonary regurgitation group (maximum wall delay P =0.03; cross-correlation delay P =0.01). Electric measures of synchrony did not improve. Conclusions— In patients with right ventricular outflow tract conduit dysfunction, TPV replacement is associated with improved global LV strain, as well as improved right ventricular strain and LV synchrony in subgroups. Given the associations between strain and synchrony and clinical outcomes, these findings support potential long-term benefits of TPV replacement.

Background— The Melody valve is approved for percutaneous pulmonary valve replacement in dysfunctional right ventricular outflow tracts. The function of this valve when subjected to high-pressure loads in humans is unknown. The aim of this study was to describe the immediate and short-term results of Melody valves implanted in a high-pressure environment. Methods and Results— Definitions of a high-pressure system were established for Melody valves implanted in the systemic (ie, aortic or mitral position) and pulmonary (ie, right ventricular outflow tract conduit or tricuspid valve annulus) circulations. Implants in these environments were ascertained from databases of the 5 centers that participated in the US Investigational Device Exemption trial. Thirty implants met the inclusion criteria: 23 pulmonary circulation implants (all in the pulmonary position) systemic circulation implants (5 in the native aortic position, 1 in a left ventricle-to-descending aorta conduit, and 1 in the mitral annulus). All pulmonary circulation implants were performed percutaneously in the catheterization laboratory. A hybrid approach (surgical exposure for transcatheter implant) was used for 4 of the aortic implants. There were no procedure-related deaths. Three patients died of nonprocedure- and nonvalve-related causes. At 1 year, freedom from moderate to severe regurgitation was 100%, and freedom from mild regurgitation was 90%. Freedom from moderate to severe stenosis was 86% at 1 year. Conclusions— Short-term performance of the Melody valve in high-pressure environments is encouraging, with good valve function in all patients.