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Combined small cell lung cancer (cSCLC) is relatively unusual. We report a case of cSCLC in a 78-year-old man with no prior medical history who presented for evaluation of right upper lobe (RUL) lung mass. A CT scan showed a 3.0 × 2.5 × 2.3 cm RUL lung mass with mildly prominent mediastinal and hilar lymphadenopathy. A right thoracotomy with right upper lobectomy and lymphadenectomy was performed. Histological examination and immunohistochemical stains confirmed the diagnosis of combined small cell lung carcinoma (SCLC) with adenocarcinoma (AC) and squamous cell carcinoma (SCC) components.While there are available guidelines for treating SCLC, the optimal treatment for cSCLC which will improve prognosis has not been adequately determined. We report a very rare category of primary lung malignant neoplasm to represent our institution's experience in diagnosing and managing this type of rare case.

Colorectal cancer (CRC) is the third leading cause of cancer-related deaths worldwide. The tumor suppressor gene MT-CO1, and Kristen Rat Sarcoma Virus (KRAS), an oncogene are primarily responsible for controlling cell apoptosis, cell cycle arrest, and cell proliferation, and any irregularities in these genes could lead to cancer. This study aims to examine the expression of KRAS and MT-CO1 in CRC biopsy specimens and investigate their relationship with one another in CRC patients residing in the Erbil city of Kurdistan Region, Iraq. The study involved categorizing 42 sets of colorectal cancer tissues and their corresponding controls based on their types and patients' clinical characteristics. The expression of KRAS and MT-CO1 in the samples was assessed using Reverse Transcriptase-Polymerase Chain Reaction (RT-PCR), with statistical significance set at p<0.05. The expression of KRAS was found to be significantly higher in CRC compared to the control (n=42, p=0.0001). On the other hand, the expression of MT-CO1 did not exhibit significant differences compared to the control group with a p-value of 0.12. Furthermore, the Chi-square and correlation analysis results depicted that MT-CO1 expression negatively correlates with KRAS expression (p= 0.0001, r= -0.047) in CRC tissues. In conclusion, the variation in the expression of KRAS and MT-CO1, and their correlations could potentially serve as a good indicator in the detection and prognosis of CRC, which might lead to better translational research on the same. However, for a better understanding of the underlying mechanisms, further analysis is required.

Introduction: Timely identification of at-risk neonates (ARNs) in the community is essential to reduce mortality in low-resource settings. Tools such as American Academy of Pediatrics pulse oximetry (POx) and WHO Young Infants Clinical Signs (WHOS) have high specificity but low sensitivity to identify ARNs. Our aim was assessing the value of POx and WHOS independently, in combination and with machine learning (ML) from clinical features, to detect ARNs in a low/middle-income country., Methods: This prospective cohort study was conducted in a periurban community in Pakistan. Eligible live births were screened using WHOS and POx along with clinical information regarding pregnancy and delivery. The enrolled neonates were followed for 4 weeks of life to assess the vital status. The predictive value to identify ARNs, of POx, WHOS and an ML model using maternal and neonatal clinical features, was assessed., Results: Of 1336 neonates, 68 (5%) had adverse outcomes, that is, sepsis (n=40, 59%), critical congenital heart disease (n=2, 3%), severe persistent pulmonary hypertension (n=1), hospitalisation (n=8, 12%) and death (n=17, 25%) assessed at 4 weeks of life. Specificity of POx and WHOS to independently identify ARNs was 99%, with sensitivity of 19% and 63%,respectively. Combining both improved sensitivity to 70%, keeping specificity at 98%. An ML model using clinical variables had 44% specificity and 76% sensitivity. A staged assessment, where WHOS, POx and ML are sequentially used for triage, increased sensitivity to 85%, keeping specificity 75%. Using ML (when WHOS and POx negative) for community follow-up detected the majority of ARNs., Conclusion: Classic screening, combined with ML, can help maximise identifying ARNs and could be embedded in low-resource clinical settings, thereby improving outcome. Sequential use of classic assessment and clinical ML identifies the most ARNs in the community, still optimising follow-up clinical care., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY. Published by BMJ.)

Paediatric and congenital heart disease (PCHD) is common but remains forgotten on the global health agenda. Congenital heart disease is the most frequent major congenital anomaly, affecting approximately 1 in every 100 live births. In high-income countries, most children now live into adulthood, whereas in low- and middle-income countries, over 90% of patients do not get the care they need. Rheumatic heart disease is the most common acquired cardiovascular disease in children and adolescents. While almost completely eradicated in high-income countries, over 30-40 million people live with rheumatic heart disease in low- and middle-income countries. Challenges exist in the care for PCHD and, increasingly, adult congenital heart disease (ACHD) worldwide. In this review, we summarize the current status of PCHD and ACHD care through the health systems lens of workforce, infrastructure, financing, service delivery, information management and technology, and governance. We further highlight gaps in knowledge and opportunities moving forward to improve access to care for all those living with PCHD or ACHD worldwide., (© 2023 The Author(s).)

Background: Safety events and technical success (TS) have been previously reported for aortic and pulmonary valvuloplasty, but a composite performance measure as a novel, patient-centered strategy has neither been developed nor been studied. This study aims to refine a procedural performance (PP) variable, a composite of TS and procedural safety, for isolated, standard-risk aortic and pulmonary valvuloplasty., Methods: A multicenter review was performed using data from the Congenital Cardiac Catheterization Project on Outcomes registry. Data were collected for all cases of isolated balloon aortic and pulmonary valvuloplasty from 2014 through 2017. Patients were excluded if they were aged <1 month, were inpatient at the time of the procedure, or had significant comorbidities, such as Williams or Noonan syndrome. Criteria for TS were developed and categorized (optimal, satisfactory, and unsatisfactory) by expert consensus based on previous outcome research. Adverse events (AE) were categorized by severity (level 1-5) using established criteria. Level 4 and 5 severity AE were considered high-severity AE. Using criteria of TS and AE severity, PP was divided into 3 composite outcome classes. Factors correlating with class III (suboptimal) PP were analyzed., Results: There were 169 cases of aortic and 270 cases of pulmonary valvuloplasty in the cohorts. In the aortic valvuloplasty cohort, a suboptimal PP (class III) occurred in 14% of cases, mostly due to high-severity AE (7%). No significant correlation between patient or case characteristics and PP was demonstrated. In the pulmonary valvuloplasty cohort, class III PP occurred in 9% of cases, predominantly due to residual valve gradient, which correlated with lower weight ( P = .02)., Conclusions: We designed a composite variable of PP consisting of TS and safety as a comprehensive measure of outcome. Incorporating both TS and AE may better reflect patient outcome than each metric measured separately. PP indices may identify areas for further investigation and quality improvement., (© 2023 The Author(s).)

Introduction: Medical colleges globally have student organizations that serve to enable students' involvement in research. However, details of their approach and activities are seldom published to serve as learning for student organizations in other settings. The Student Research Forum (SRF), a student organization based at a private medical school in Pakistan aims to facilitate students in acquiring research skills. Following the observation of a downward trajectory of student initiative and interest, SRF leadership restructured the organization and improve its impact. This study describes the development and implementation evaluation of the interventions., Methodology: The operational framework was revised using the Theory of Change by the core group. Major interventions included enhanced social media and outreach coordination, research workshops, journal clubs, and mentorship to increase research output, mentorship opportunities, and knowledge of medical research; ultimately improving quality in research. The outcomes generated over the course of the study's duration from July 2019 to September 2021 were analyzed using the process metrics of reach, adoption, and efficacy., Results: As a result of the interventions, SRF expanded its reach by conducting a total of 41 events during the duration of the study, facilitated by social media growth on each of SRF's online platforms, with a 300% increase in followers on Facebook, and a nationwide network of 91 student ambassadors. An annual workshop series taught research skills to more than 3800 participants. Students leading their own events, SRF featuring international speakers, and the abstracts submitted to SRF's annual conference, along with the conference's reach of 10,000 students, are seen as improvements in the ToC-informed interventions' adoption. The efficacy of the interventions manifested as the REACH program allocated 56 research projects to vetted applicants., Conclusion: The applied interventions have accelerated SRF's progress towards achieving its long-term outcome of increased quality in research as translated by increased research output quantity, mentorship, and knowledge of medical research. Further evaluation is required to assess the success of the ToC. As SRF continues to grow, a continued analysis of the implementation outcomes is imperative to gauge its effectiveness., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2023 Haroon et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Background: No published data are available on the patient, procedural characteristics, and outcomes of congenital heart disease (CHD) cardiac catheterization performed in low- and middle-income countries (LMICs)., Objectives: The objective of this study was to describe procedural characteristics and patient outcomes of CHD cardiac catheterizations in LMICs., Methods: Cases performed between January 2019 and December 2020 from 15 centers in the International Quality Improvement Collaborative Congenital Heart Disease Catheterization Registry (IQIC-CHDCR) data were included. The Procedural Risk in Congenital Cardiac Catheterization (PREDIC3T) classification was used to stratify risk. Outcomes of interest included mortality, severe adverse events (SAEs), and procedural efficacy. Procedural efficacy, based on technical and safety endpoints, was categorized into optimal, adequate, and inadequate for 5 common interventional procedures., Results: There were 3,287 cases, of which 60% (n = 1,973) were interventional cases. Most of the cases (66%) were in patients between the ages of 1 to 18 years with a median patient age of 4 years. PREDIC3T risk class 1 and 2 were most common in 37% and 38% of cases, respectively. SAEs occurred in 2.8% while the death was reported within <72 hours post catheterization 1%. The majority of device implantation procedures patent ductus arteriosus (67%) and atrial septal defect (60%) had optimal procedure efficacy outcomes., Conclusions: This study demonstrates that congenital cardiac catheterization is safely performed in LMICs. Future work addressing predictors of SAEs and adverse procedural outcomes may help future quality improvement initiatives., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2023 The Authors.)

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., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Children born small for gestational age (SGA) may experience more long-term neurodevelopmental issues than those born appropriate for gestational age (AGA). This study aimed to assess differences in the neurodevelopment of children born SGA or AGA within a periurban community in Pakistan., Methods: This was a prospective cohort study in which study participants were followed from the pilot Doppler cohort study conducted in 2018. This pilot study aimed to develop a pregnancy risk stratification model using machine learning on fetal Dopplers. This project identified 119 newborns who were born SGA (2.4±0.4 kg) based on International Fetal and Newborn Growth Consortium standards. We assessed 180 children (90 SGA and 90 AGA) between 2 and 4 years of age (76% of follow-up rate) using the Malawi Developmental Assessment Tool (MDAT)., Findings: Multivariable linear regression analysis comparing the absolute scores of MDAT showed significantly lower fine motor scores (β: -0.98; 95% CI -1.90 to -0.06) among SGAs, whereas comparing the z-scores using multivariable logistic regression, SGA children had three times higher odds of overall z-scores ≤-2 (OR: 3.78; 95% CI 1.20 to 11.89) as compared with AGA children., Interpretation: SGA exposure is associated with poor performance on overall MDAT, mainly due to changes in the fine motor domain in young children. The scores on the other domains (gross motor, language and social) were also lower among SGAs; however, none of these reached statistical significance. There is a need to design follow-up studies to assess the impact of SGA on child's neurodevelopmental trajectory and school performance., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY. Published by BMJ.)

Competing Interests: Competing interests: None declared.

Background: A study from a tertiary care center in Pakistan demonstrated that a leadership development intervention led to improved family experience of care outcomes. The objective of the current paper is to assess the implementation of this intervention and identify barriers and facilitators to inform sustainability and scalability., Methods: A working group designed the intervention using a theory-of-change model to strengthen leadership development to achieve greater employee engagement. The interventions included: i) purpose and vision through purpose-driven leadership skills trainings; ii) engaging managers via on-the-job mentorship programme for managers, iii) employee voice i.e., facilitation of upward communication to hear the employees using Facebook group and subsequently inviting them to lead quality improvement (QI) projects; and iv) demonstrating integrity by streamlining actions taken based on routine patient experience data. Implementation outcomes included acceptability, adoption, fidelity across degree & quality of execution and facilitators & barriers to the implementation. Data analyzed included project documentation records and posts on the Facebook group. Analysis indicated acceptability and adoption of the intervention by the employees as178 applications for different QI projects were received. Leadership sessions were delivered to 455 (75%) of the employees and social media communication was effective to engage employees. However, mentorship package was not rolled out nor the streamlined processes for action on patient experience data achieved the desired fidelity. Only 6 QI projects were sustained for at least a year out of the 18 approved by the working group. Facilitators included leadership involvement, real-time recognition and feedback and value-creation through participation by national and international celebrities. Challenges identified were the short length of the intervention and incentives not being institutionalized. The authors conclude that leadership development through short training sessions and on-going communications facilitated by social media were the key processes that helped achieve the outcomes. However, a long-term strategy is needed for individual managerial behaviours to sustain., (© 2022. The Author(s).)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Abbas, Hussain, Shahbaz, Siddiqui and Hasan.)

Introduction: Equipping young medical trainees with fundamental research skills can be a promising strategy to address the need for professionals who can understand and responsibly communicate evolving scientific evidence during a pandemic. Despite an ardent interest to partake in research, most educational institutions in Pakistan and other low-middle income countries have not yet adopted a comprehensive strategy for research skills education. The authors aimed to design and assess the feasibility of implementing the first nation-wide virtual research workshop for medical students in Pakistan. Methods: The course "Beginners Guide to Research," designed as a nation-wide virtual research workshop series, was conducted for medical students across Pakistan in June 2020. Four interactive live workshops took place online on alternate days from June 22nd, 2020, to June 27th, 2020, each lasting 1-2 h. Outcomes included: (i) reach, (ii) efficacy as indexed by pre-post change in score pertaining to knowledge and application of research and (iii) self-rated perceptions about understanding of research on a Likert scale. Results: 3,862 participants enrolled from 41 cities and 123 institutions. Enrolled participants belonged to the following provinces: Sindh ( n = 1,852, 48.0%), Punjab ( n = 1,767, 45.8%), Khyber Pakhtunkhwa ( n = 109, 2.8%), Azad Jammu and Kashmir ( n = 84, 2.2%) Balochistan ( n = 42, 1.1%). We also saw a few registrations from international students ( n = 8, 0.2%). Mean (SD) age of enrolled medical students was 21.1 (2.1) years, 2,453 (63.5%) participants were female and 2,394 (62.0%) were from private-sector medical colleges. Two thousand ninety-three participants participants filled out all four pre-test and post-test forms. The total median knowledge score improved from 39.7 to 60.3% with the highest improvements in concepts of research bioethics and literature search ( p < 0.001) with greater change for females compared to males (+20.6 vs. +16.2%, p < 0.001) and private institutions compared to public ones (+16.2 vs. +22.1%, p < 0.001). Conclusion: The overwhelming enrollment and significant improvement in learning outcomes (>50% of baseline) indicate feasibility of a medical student-led research course during a pandemic, highlighting its role in catering to the research needs in the LMICs., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Noorali, Inam, Shahbaz, Rauf, Aamir, Khalid, Abbas, Saeed, Musharraf, Merchant, Hasan, Rasheed, Jehan, Tariq and Haider.)

Along with inadequate access to high-quality care, competing health priorities, fragile health systems, and conflicts, there is an associated delay in evidence generation and research from LMICs. Lack of basic epidemiologic understanding of the disease burden in these regions poses a significant knowledge gap as solutions can only be developed and sustained if the scope of the problem is accurately defined. Congenital heart disease (CHD), for example, is the most common birth defect in children. The prevalence of CHD from 1990 to 2017 has progressively increased by 18.7% and more than 90% of children with CHD are born in Low and Middle-Income Countries (LMICs). If diagnosed and managed in a timely manner, as in high-income countries (HICs), most children lead a healthy life and achieve adulthood. However, children with CHD in LMICs have limited care available with subsequent impact on survival. The large disparity in global health research focus on this complex disease makes it a solid paradigm to shape the debate. Despite many challenges, an essential aspect of improving research in LMICs is the realization and ownership of the problem around paucity of local evidence by patients, health care providers, academic centers, and governments in these countries. We have created a theory of change model to address these challenges at a micro- (individual patient or physician or institutions delivering health care) and a macro- (government and health ministries) level, presenting suggested solutions for these complex problems. All stakeholders in the society, from government bodies, health ministries, and systems, to frontline healthcare workers and patients, need to be invested in addressing the local health problems and significantly increase data to define and improve the gaps in care in LMICs. Moreover, interventions can be designed for a more collaborative and effective HIC-LMIC and LMIC-LMIC partnership to increase resources, capacity building, and representation for long-term productivity., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Hasan, Rasheed, Wahid, Kumar and Zuhlke.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Rasheed, Mughis, Elahi and Hasan.)

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., (© 2021. The Author(s).)

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., (Copyright © 2021 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Rasheed, Hookmani, Waleed, Fatima, Siddiqui, Khurram and Hasan.)

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., Competing Interests: The funder, CSIMAC provided support in the form of salaries for authors [MR] and the salaries of the authors [SA, HS, AS, MN] were supported by Ephlux Private Limited. This commercial affiliation does not alter our adherence to PLOS ONE policies on sharing data and materials.

Objectives: To describe the development of a quality collaborative for congenital cardiac catheterization centers in low and middle-income countries (LMICs) including pilot study data and a novel procedural efficacy measure., Background: Absence of congenital cardiac catheterization registries in LMICs led to the development of the International Quality Improvement Collaborative Congenital Heart Disease Catheterization Registry (IQIC-CHDCR). As a foundation for this initiative, the IQIC is a collaboration of pediatric cardiac surgical programs from LMICs. Participation in IQIC has been associated with improved patient outcomes., Methods: A web-based registry was designed through a collaborative process. A pilot study was conducted from October through December 2017 at seven existing IQIC sites. Demographic, hemodynamic, and adverse event data were obtained and a novel tool to assess procedural efficacy was applied to five specific procedures. Procedural efficacy was categorized using ideal, adequate, and inadequate., Results: A total of 429 cases were entered. Twenty-five adverse events were reported. The five procedures for which procedural efficacy was measured represented 48% of cases (n = 208) and 71% had complete data for analysis (n = 146). Procedure efficacy was ideal most frequently in patent ductus arteriosus (95%) and atrial septal defect (90%) device closure, and inadequate most frequently in coarctation procedures (100%), and aortic and pulmonary valvuloplasties (50%)., Conclusions: The IQIC-CHDCR has designed a feasible collaborative to capture catheterization data in LMICs. The novel tool for procedural efficacy will provide valuable means to identify areas for quality improvement. This pilot study and lessons learned culminated in the full launch of the IQIC-CHDCR., (© 2020 Wiley Periodicals, Inc.)

Objective: 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., Methods: 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., Results: 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., Conclusions: 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.

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., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

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.

Objectives: The aim of this study was to establish multidisciplinary care for patients with transfusion-dependent thalassaemia (TDT) by creating a TDT quality improvement (QI) collaborative in a resource-constrained setting. This study presents our initial experience of creating this collaborative, the baseline characteristics of the participants, the proposed QI interventions and the outcome metrics of the collaborative., Design and Setting: TDT QI collaborative is a database comprising patients with TDT from four centres in Karachi, Pakistan. Study variables included symptoms of cardiac or endocrine dysfunction, physical examination including anthropometry and Tanner staging, chelation therapy, results of echocardiography, T2* cardiac MRI (CMR) and serum ferritin. The main outcome of this collaborative was improvement in TDT-related morbidity and mortality. Interventions addressing the key drivers of outcome were designed and implemented., Results: At the time of reporting, the total number of patients in this database was 295. Most patients reported cardiac symptoms corresponding to New York Heart Association class 2. Approximately half (52%, n=153) of the patients demonstrated severe myocardial iron overload (T2* <10 ms). Majority of the patients (58%, n=175) were not on adequate chelation therapy. There was no difference in echocardiographic measures of systolic and diastolic left ventricle among the different spectrums of iron overloaded myocardium., Conclusion: Using T2* CMR and endocrine testing, we have identified significant burden of iron siderosis in our patients with TDT. Lack of adequate iron load assessment and standardised management was observed. Interventions designed to target these key drivers of outcome are the unique part of this QI-based TDT registry., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

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., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: The objective of this study was to assess differences in myocardial systolic and diastolic function and vascular function in children 2-5 years of age born to diabetic as compared to non-diabetic mothers., Methods: This study was a retrospective cohort conducted in 2016 at The Aga Khan University Hospital, Karachi, Pakistan. It included children between 2 and 5 years of age born to mothers with and without exposure to diabetes in utero (n = 68 in each group) and who were appropriate for gestational age. Myocardial morphology and function using echocardiogram and carotid intima media thickness (cIMT) and pulse wave velocity was performed to evaluate cardiac function as well as macrovascular remodelling in these children. Multiple linear regression was used to compare the groups., Results: There was no significant difference in cardiac morphology, myocardial systolic and diastolic function, and macrovascular assessment between the exposed and unexposed groups of AGA children. Subgroup analysis demonstrated a significantly decreased mitral E/A ratio in children whose mothers were on medications as compared to those on dietary control (median [IQR] = 1.7 [1.6-1.9] and 1.56 [1.4-1.7], respectively, p = 0.02), and a higher cIMT in children whose mothers were on medication as compared to controls (0.48 [0.44-0.52] and 0.46 [0.44-0.50], respectively, p = 0.03)., Conclusion: In utero exposure to uncontrolled maternal diabetes has an effect on the cardiovascular structure and function in children aged 2-5 years. However, future work requires long-term follow-up from fetal to adult life to assess these changes over the life course.

Background: As survival improves in the congenital heart disease (CHD) population, health-related quality of life (HRQOL) outcomes become increasingly important. While surgery improves survival, poor HRQOL occurs postoperatively and cardiac-related HRQOL outcomes are rarely reported., Objective: To conduct a systematic review and meta-analyses of general and cardiac-related HRQOL in CHD surgical children and young adults., Method: Medline, CINAHL and EMBASE were searched. Quantitative designs with a minimum of 80% CHD surgical patients and mean age ≤18 years compared with healthy controls were included in the review. Data were analysed in RevMan V.5.3 using a random effects model., Outcome Measures: General and cardiac-related HRQOL., Results: Studies (n=20) were conducted in high-income countries and included 3808 patients plus 2951 parental reports of patients. HRQOL was worse in postoperative patients with CHD versus healthy controls in all domains with the largest difference seen for physical function (standard mean difference (SMD) of -0.56, 95% CI -0.82 to -0.30). Cardiac-related HRQOL was worse in complex compared with simple CHD with the largest SMD (-0.60, 95% CI -0.80 to -0.40) for symptoms. Heterogeneity ranged from 0% to 90%., Conclusions: CHD surgical patients have substantially worse HRQOL compared with age-matched healthy controls. Strategies should focus on improving HRQOL in this subgroup. Results may not be applicable to low/middle-income countries given the dearth of relevant research., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2019. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Introduction: Pre-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 Analysis: The 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 p<0.05 will be used to determine statistical significance., Ethics and Dissemination: Ethical approval has been obtained from AKUH Ethics Review Committee. Findings will be disseminated through scientific publications and project summaries for the participants., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2018. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Reduced 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 Analysis: This 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 Dissemination: Ethics 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., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: Exercise stress echocardiography (ESE) can unmask ventricular dysfunction in asymptomatic patients with congenital heart disease (CHD), but its acquisition and interpretation is often challenging, and the method has not been validated in CHD. This study aimed to evaluate the feasibility of ESE using Doppler imaging and to assess myocardial response to exercise in patients with biventricular (BiV) and univentricular (UniV) circulation after CHD repair., Methods: In this single-center prospective study, we recruited 55 participants (17 females), median age 14 years (8-22 years). Our analysis categorized participants in these three groups: with structurally normal hearts as controls (n=21), with BiV circulation (n=20) and with UniV circulation (n=14). We acquired ESE images of the systemic ventricle including pulsed-wave flow and spectral tissue Doppler imaging (TDI) of lateral free wall before and immediately after standard, symptom-limited exercise tests on an electronically braked cycle ergometer., Results: During ESE we obtained inflow E-wave and TDI systolic (S') and early diastolic (E') velocities in 93% to 100% of participants at rest and in 90% to 100% of participants post exercise. Feasibility to obtain Doppler imaging parameter was the same across study groups. The myocardial response to exercise was increase in heart rate (HR), S' and inflow E-wave velocity in all participants. Patients with BiV circulation had preserved ventricular function at rest. While patients with UniV circulation had low S', E', and E-wave velocities at rest in comparison to controls and to BiV group (all P<.001), both patients with BiV and UniV circulation showed significant increases in HR, S' velocity and inflow E-wave velocity post exercise, with magnitudes of these increases higher in controls than in the BiV and UniV group. The S' and E' velocities were strongly associated with lower percent predicted peak oxygen consumption VO 2 (r s =0.614 and r s =0.64, respectively, both P<.001)., Conclusion: ESE with Doppler imaging is a practical noninvasive diagnostic method and sufficiently robust for the assessment of morphologic LV/systemic ventricles under exercise in patients after biventricular and univentricular CHD repair. Although patients with BiV and UniV circulation had both preserved myocardial response to exercise, the magnitude of this response was the lowest in patients with UniV circulation., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: Relief of postoperative right ventricular outflow tract (RVOT) obstruction with transcatheter pulmonary valve replacement (TPVR) results in functional improvement in the short term which we investigated at baseline (BL), early follow-up (FU), and midterm FU after TPVR., Methods: Echocardiography and cardiopulmonary exercise testing were performed at BL and at early (median 6 months) and midterm FU (median 2.5years) after TPVR., Results: Patients with RVOT obstruction (n=22, median age 17years) were studied. The max RVOT Doppler gradient fell from BL to early FU (60±24 to 26±8mm Hg, P<.001). Left ventricular (LV) end-diastolic and stroke volume increased at early FU (both P<.001) without further change, whereas LV ejection fraction improved throughout FU (P<.001). LV end-systolic and diastolic eccentricity (leftward septal displacement) improved early (both P≤.003), and end-diastolic eccentricity improved further at midterm FU (P=.02). Furthermore, whereas mitral inflow A wave velocity increased (P=.003), the LV A' velocity declined early (P=.007) without further change at midterm. RV systolic and early diastolic function was impaired at BL. Whereas RV strain improved partially at early and midterm FU (P≤.02), RV E' velocity did not improve throughout FU. Mildly impaired LV strain at BL fully recovered by midterm FU (P≤.002). Peak oxygen uptake improved at early and midterm FU (all P≤.003)., Conclusions: Patients with RVOT obstruction had biventricular systolic and diastolic dysfunction at BL. Relieving RVOT obstruction with TPVR reduced adverse ventricular and compensatory atrioventricular interaction, resulting in progressive biventricular functional improvement and remodeling at early and midterm FU., (Published by Elsevier Inc.)

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.

Background: Rheumatic heart disease (RHD) is a global cause of early heart failure. Early RHD is characterized by valvar regurgitation, leading to ventricular distention and possible elaboration of amino-terminal pro-brain natriuretic peptide (NT-proBNP). We investigated the ability of NT-proBNP to distinguish cases of latent RHD detected by echocardiographic screening from the controls., Materials and Methods: Ugandan children (N = 44, 36% males, mean age: 12 ± 2 years) with latent RHD (cases) and siblings (controls) by echocardiography were enrolled. Cases and controls were matched for age and sex, and they had normal hemoglobin (mean: 12.8 mg/dL). Children with congenital heart disease, pregnancy, left ventricular dilation or ejection fraction (EF) below 55%, or other acute or known chronic health conditions were excluded. RHD cases were defined by the World Heart Federation (WHF) 2012 consensus guideline criteria as definite. Controls had no echocardiography (echo) evidence for RHD. At the time of echo, venous blood samples were drawn and stored as serum. NT-proBNP levels were measured using sandwich immunoassay. Paired t-tests were used to compare NT-proBNP concentrations including sex-specific analyses., Results: The mean NT-proBNP concentration in the cases was 105.74 ± 67.21 pg/mL while in the controls, it was 86.63 ± 55.77 pg/mL. The cases did not differ from the controls (P = 0.3). In sex-specific analyses, male cases differed significantly from the controls (158.78 ± 68.82 versus 76 ± 42.43, P = 0.008). Female cases did not differ from the controls (75.44 ± 45.03 versus 92.30 ± 62.35 respectively, P = 0.4)., Conclusion: Serum NT-proBNP did not distinguish between latent RHD cases and the controls. Sex and within-family exposures may confound this result. More investigation into biomarker-based RHD detection is warranted.

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., (© 2015 Wiley Periodicals, Inc.)

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 O(2) 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., (© 2015 Wiley Periodicals, Inc.)

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., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Objectives: 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)., Background: 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., Methods: 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)., Results: 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; p < 0.001) 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)., Conclusions: 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., (Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

A 35 days old neonate with d-loop transposition of great arteries, underwent an arterial switch operation following which he developed hypotension attributed to left ventricular failure. During cardiopulmonary resuscitation decision was made to place him on cardiac bypass again. Due to limited resources and unavailability of a specialized extracorporeal membrane oxygenator machine, the CPB was modified and converted an ECMO. The neonate was successfully decannulated after 72 hours and discharged home after 3 weeks of the operation without any sequel. ECMO is a viable option in developing countries and may help in improving the outcome especially in neonatal congenital heart disease.

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., (© 2013 Wiley Periodicals, Inc.)

A relatively rare occurrence, the incidence of ventricular septal defect (VSD) complicating penetrating cardiac trauma has been reported at 4.5%. Closing such defects may be challenging especially in an unstable patient where cardiopulmonary bypass may exponentially increase the surgical risk. In such patients, catheter-based device closure is a reliable and effective alternative. We describe case of a 30 year old man who presented with a stab wound to his anterior mediastinum. His injuries involved laceration to right and left ventricles and a VSD. His lacerations were repaired on a beating heart and the VSD was not addressed due to patient hemodynamic instability. The VSD was semi-electively closed using a 24 mm Amplatzer™ device as the patient demonstrated significant left to right shunt. Post-device closure, the patient developed hemolysis attributed to an intra- device residual leak. The hemolysis resolved without any complications by conservative medical management. At latest follow-up the patient is in NYHA functional class I-II., (Copyright © 2013 Wiley Periodicals, Inc.)