South-to-south collaboration to strengthen the health workforce: the case of paediatric cardiac surgery in Rwanda.

Paediatric cardiovascular diseases have been referred to as diseases of injustice as access to care is inequitable globally. For example, Africa only has 78 cardiac centres, with 22 located in Sub-Saharan Africa. Most of these centres rely on visiting surgical teams to provide clinical care. While visiting surgical teams provide essential care, building a sustainable and locally run cardiac workforce in Africa is critical to addressing these inequities in access to care. This paper considers the role of south-to-south partnerships in building sustainable surgical programmes using Rwanda's paediatric cardiac surgery programme as an example.

Cardiac resynchronization therapy for patients with mild to moderately reduced ejection fraction and left bundle branch block.

BACKGROUND: It is unknown whether cardiac resynchronization therapy (CRT) would improve or halt the progression of heart failure (HF) in patients with mild to moderately reduced ejection fraction (HFmmrEF) and left bundle branch block (LBBB). OBJECTIVE: This study aimed to investigate the outcomes of CRT in patients with HFmmrEF and left ventricular conduction delay. METHODS: A prospective, randomized clinical trial sponsored by the National Heart, Lung, and Blood Institute included 76 patients who met the study inclusion criteria (left ventricular ejection fraction [LVEF] of 36%-50% and LBBB). Patients received CRT-pacemaker and were randomized to CRT-OFF (right ventricular pacing 40 beats/min) or CRT-ON (biventricular pacing 60-150 beats/min). At a 6-month follow-up, pacing programming was changed to the opposite settings. New York Heart Association class, N-terminal pro-brain natriuretic peptide levels, and echocardiographic variables were collected at baseline, 6 months, and 12 months. The primary study end point was the left ventricular end-systolic volume (LVESV) change from baseline, and the primary randomized comparison was the comparison of 6-month to 12-month changes between randomized groups. RESULTS: The mean age of the patients was 68.4 ± 9.8 years (male, 71%). Baseline characteristics were similar between the 2 randomized groups (all P > .05). In patients randomized to CRT-OFF first, then CRT-ON, LVESV was reduced from baseline only after CRT-ON (baseline, 116.1 ± 36.5 mL; CRT-ON, 87.6 ± 26.0 mL; P < .0001). The randomized analysis of LVEF showed a significantly better change from 6 to 12 months in the OFF-ON group (P = .003). LVEF was improved by CRT (baseline, 41.3% ±.7%; CRT-ON, 46.0% ± 8.0%; P = .002). In patients randomized to CRT-ON first, then CRT-OFF, LVESV was reduced after both CRT-ON and CRT-OFF (baseline, 109.8 ± 23.5 mL; CRT-ON, 91.7 ± 30.5 mL [P < .0001]; CRT-OFF, 99.3 ± 28.9 mL [P = .012]). However, the LVESV reduction effect became smaller between CRT-ON and CRT-OFF (P = .027). LVEF improved after both CRT-ON and CRT-OFF (baseline, 42.7% ± 4.3%; CRT-ON, 48.5% ± 8.6% [P < .001]; CRT-OFF, 45.9% ± 7.7% [P = .025]). CONCLUSION: CRT for patients with HFmmrEF significantly improves LVEF and ventricular remodeling after 6 months of CRT. The study provides novel evidence that early CRT benefits patients with HFmmrEF with LBBB.

The Impact of Rapid Handpump Repairs on Diarrhea Morbidity in Children: Cross-Sectional Study in Kwale County, Kenya.

BACKGROUND: Handpumps are used by millions of people as their main source of water. Although handpumps represent only a basic form of water provision, there have been continuous efforts to improve the performance of these systems as they are likely to remain in use for many years to come. The introduction of a professional maintenance service in southern Kenya has shown an order of magnitude improvement in operational performance over community-based management, with 90% of handpump faults repaired within 3 days of being reported. One driver behind these efforts is the assumption that a more reliable water supply will lead to a reduction in water-related disease. However, it is not clear if operational improvements lead to health gains. Despite limited empirical evidence, some modeling studies suggest that even short periods of drinking contaminated water can lead to disproportionate negative health impacts. OBJECTIVE: The aim of this study was to assess whether the improvements in operational performance from the rapid professional maintenance of rural handpumps lead to improved household health outcomes. METHODS: From a sample of households using handpumps as their primary water source in Kwale County, Kenya, we measured the 2-week prevalence of World Health Organization-defined diarrhea in children, reported by the adult respondent for each household. We compared the rates before and after a period during which the households' handpumps were being professionally maintained. We then conducted a cross-sectional analysis, fitting logistic regression models with reported diarrhea as the dependent variable and speed of repair as the independent exposure of interest, adjusting for household socioeconomic characteristics; dwelling construction; and Water, Sanitation, and Hygiene (WASH)-related factors. We fitted an additional model to examine select interactions between covariates. RESULTS: Reported diarrhea in children was lower in households whose pumps had been repaired within 24 hours (adjusted odds ratio 0.35, 95% CI 0.24-0.51). This effect was robust to the inclusion of multiple categories of covariates. No reduction was seen in households whose pump repairs took more than 24 hours. Analysis of interaction terms showed that certain interventions associated with improved WASH outcomes were only associated with reductions in diarrhea in conjunction with socioeconomic improvements. CONCLUSIONS: Only pump repairs consistently made within 24 hours of failure led to a reduction in diarrhea in the children of families using handpumps. While the efficacy of reduction in diarrhea is substantial, the operational challenges of guaranteeing same-day repairs limits the effectiveness of even best-in-class pump maintenance. Maintenance regimes that cannot bring handpump downtimes close to zero will struggle to generate health benefits. Other factors that reduce diarrhea prevalence have limited effect in isolation, suggesting that WASH interventions will be more effective when undertaken as part of more holistic poverty-reduction efforts.

Nurse Practitioner Delivered Virtual Care in a Pediatric Specialty Clinic.

INTRODUCTION: Virtual care in pediatric specialty practice has become widely used to increase access to care, one in which nurse practitioners (NPs) can play a vital role. We sought to evaluate the outcomes of virtual care provided by pediatric cardiology and electrophysiology (EP) NPs for pediatric and congenital heart disease patients. METHOD: Retrospective review of all virtual care visits performed by pediatric EP NPs between October 2019 and October 2021 at a single tertiary care center. RESULTS: NPs delivered virtual care for 287 pediatric EP evaluations for 276 patients; 132 (45%) independently, with the remaining 155 collaborating with an electrophysiologist as a shared visit. The mean age was 15.2 ± 8.9 years. DISCUSSION: NPs performed a significant subset of these visits independently and were a vital part of all visits in this study. The role of the NP in specialty pediatric virtual care should continue to be supported and advanced.

Impact and Modifiers of Ventricular Pacing in Patients With Single Ventricle Circulation.

BACKGROUND: Palliation of the single ventricle (SV) circulation is associated with a burden of lifelong complications. Previous studies have identified that the need for a permanent ventricular pacing system (PPMv) may be associated with additional adverse long-term outcomes. OBJECTIVES: The goal of this study was to quantify the attributable risk of PPMv in patients with SV, and to identify modifiable risk factors. METHODS: This international study was sponsored by the Pediatric and Congenital Electrophysiology Society. Centers contributed baseline and longitudinal data for functionally SV patients with PPMv. Enrollment was at implantation. Controls were matched 1:1 to PPMv subjects by ventricular morphology and sex, identified within center, and enrolled at matched age. Primary outcome was transplantation or death. RESULTS: In total, 236 PPMv subjects and 213 matched controls were identified (22 centers, 9 countries). Median age at enrollment was 5.3 years (quartiles: 1.5-13.2 years), follow-up 6.9 years (3.4-11.6 years). Median percent ventricular pacing (Vp) was 90.8% (25th-75th percentile: 4.3%-100%) in the PPMv cohort. Across 213 matched pairs, multivariable HR for death/transplant associated with PPMv was 3.8 (95% CI 1.9-7.6; P < 0.001). Within the PPMv population, higher Vp (HR: 1.009 per %; P = 0.009), higher QRS z-score (HR: 1.19; P = 0.009) and nonapical lead position (HR: 2.17; P = 0.042) were all associated with death/transplantation. CONCLUSIONS: PPMv in patients with SV is associated with increased risk of heart transplantation and death, despite controlling for increased associated morbidity of the PPMv cohort. Increased Vp, higher QRS z-score, and nonapical ventricular lead position are all associated with higher risk of adverse outcome and may be modifiable risk factors.

Clinical characteristics and long-term outcomes of catheter ablation in young adults with atrial fibrillation.

PURPOSE: We aim to describe the long-term safety and efficacy of catheter ablation (CA) in young patients (<30 years) with atrial fibrillation (AF). METHODS: This was a retrospective study of patients aged 18-30 who underwent CA for AF, and clinical characteristics and long-term outcomes are reported. Survival analyses were performed between the study group and a propensity-matched older cohort (>30 years, mean age: 58±10 years). RESULTS: From January 2000 to January 2019, a 1st CA (radiofrequency energy n=72, cryoballoon n=10), was performed in 82 patients (mean age 26±4 years, paroxysmal n=61, persistent n=14, longstanding persistent n=7), among 6336 consecutive patients with AF. During a follow-up of 5±5 years, 56% and 30% of the patients with paroxysmal and non-paroxysmal AF were arrhythmia free without antiarrhythmic drug (AAD) therapy after a single CA (P=0.02). After 1.5±0.8 CA procedures, 76% and 75% of the patients with paroxysmal AF and non-paroxysmal AF were arrhythmia free without AADs (P=0.54). Compared to a propensity-matched group of older patients, young patients were as likely to remain in sinus rhythm after CA (P=0.47), however after fewer repeat CAs (1.5±0.8 vs 1.9±0.9, P<0.009). There were no long-term adverse outcomes associated with CA. CONCLUSIONS: CA is a safe and effective treatment of AF in young patients with comparable outcomes to the older patients, however after fewer procedures.

The impact of CIEDs with automatic "wireless" remote monitoring on efficiency.

BACKGROUND: A benefit of automatically transmitting or "wireless" CIEDs (W-CIED) is the prompt detection of device malfunction and arrhythmias. We hypothesized that the use of W-CIEDs would improve the efficiency of remote monitoring by decreasing unnecessary CIED remote transmissions because of the automatic detection of abnormalities. OBJECTIVE: To compare the frequency of patient-initiated transmissions in patients with W-CIEDs versus non-wireless CIEDs (NW-CIED) at a single pediatric and congenital heart center. METHODS: Retrospective cohort study of patients with W-CIEDs followed over a 2-year period compared to a similar cohort of patients with NW-CIED. All CIED remote transmissions during were reviewed for indication and outcome. RESULTS: The W-CIED cohort had 87 patients; mean age 20 ± 13 years; NW-CIED cohort had 220 patients; mean age 22 ± (13) years. The mean number of symptomatic patient-initiated transmissions per patient was 0.93 ± 2.65 in the W-CIED cohort versus 0.39 ± 0.64 in the NW-CIED cohort (p ≤ .001). The mean number of asymptomatic patient-initiated transmission sent per patient in the W-CIED cohort was 1.86 ± 2.59 versus 0.81 ± 1.41 in the NW-CIED cohort (p ≤ .0001). Type of device, age, and presence of congenital heart disease were not significantly associated with the incidence of patient-initiated remote monitoring transmissions. CONCLUSIONS: The frequency of patient-initiated transmission was higher in the W-CIED cohort, contradictory to the study hypothesis. This may reflect a lack of patient understanding of the benefit or functionality of W-CIEDs and may be mitigated by education to both providers and patients.

Acute outcomes of three-dimensional mapping for fluoroscopy reduction in paediatric catheter ablation for supraventricular tachycardia.

BACKGROUND: Catheter ablation is a safe and effective therapy for the treatment of supraventricular tachycardia in children. Current improvements in technology have allowed progressive reduction in radiation exposure associated with the procedure. To assess the impact of three-dimensional mapping, we compared acute procedural results collected from the Catheter Ablation with Reduction or Elimination of Fluoroscopy registry to published results from the Prospective Assessment after Pediatric Cardiac Ablation study. METHODS: Inclusion and exclusion criteria from the Prospective Assessment after Pediatric Cardiac Ablation study were used as guidelines to select patient data from the Catheter Ablation with Reduction or Elimination of Fluoroscopy registry to compare acute procedural outcomes between cohorts. Outcomes assessed include procedural and fluoroscopy exposure times, success rates of procedure, and complications. RESULTS: In 786 ablation procedures, targeting 498 accessory pathways and 288 atrioventricular nodal reentrant tachycardia substrates, average procedural time (156.5 versus 206.7 minutes, p < 0.01), and fluoroscopy time (1.2 versus 38.3 minutes, p < 0.01) were significantly shorter in the study group. Success rates for the various substrates were similar except for manifest accessory pathways which had a significantly higher success rate in the study group (96.4% versus 93.0%, p < 0.01). Major complication rates were significantly lower in the study group (0.3% versus 1.6%, p < 0.01). CONCLUSIONS: In a large, multicentre study, three-dimensional systems show favourable improvements in clinical outcomes in children undergoing catheter ablation of supraventricular tachycardia compared to the traditional fluoroscopic approach. Further improvements are anticipated as technology advances.

Subcutaneous Implantable Cardioverter-Defibrillators in Pediatrics and Congenital Heart Disease: A Pediatric and Congenital Electrophysiology Society Multicenter Review.

OBJECTIVES: The primary goal of this study was to evaluate the implant experience and midterm results of subcutaneous implantable cardioverter-defibrillators (S-ICDs) in pediatric patients and those with congenital heart disease. BACKGROUND: The S-ICD was developed to avoid the lead-related complications associated with transvenous systems. The absence of intravascular or intracardiac components offers potential advantages to pediatric patients and those with congenital heart disease. METHODS: This international, multicenter, retrospective, standard-of-care study was conducted through the Pediatric & Congenital Electrophysiology Society. Complications at 30 and 360 days, inappropriate shocks, and delivery of appropriate therapy were assessed. RESULTS: The study included 115 patients with a median follow-up of 32 (19 to 52) months. Median age was 16.7 years (14.8 to 19.3 years), 29% were female, and 55% had a primary prevention indication. Underlying disease substrate was cardiomyopathy (40%), structural heart disease (32%), idiopathic ventricular fibrillation (16%), and channelopathy (13%). The complication rate was 7.8% at 30 days and 14.7% at 360 days. Overall, inappropriate shocks occurred in 15.6% of patients, with no single clinical characteristic reaching statistical significance. At implant, 97.9% of patients had successful first shock conversion with 96% requiring ≤65 J. Appropriate therapy was delivered to 11.2% of patients with an annual incidence of 3.9% and an acute first shock conversion success rate of 92.5%. CONCLUSIONS: This study found that in a heterogeneous population of pediatric patients and those with congenital heart disease, the S-ICD had comparable rates of complications, inappropriate shocks, and conversion efficacy compared with previously published studies on transvenous systems in similar populations.

Frequency of CIED remote monitoring: A quality improvement follow-up study.

BACKGROUND: Based on the findings of a prior study of CIED (Cardiac Implantable Electrical Device) remote monitoring (RM) frequency at the same center, the University of Michigan Congenital Heart Center (UMCHC) instituted a quality improvement (QI) change to reduce the frequency of routine CIED RM from every 2 months to every 3 months. The objective of this study is to determine the impact of this QI initiative to reduce workload without compromising patient care. METHODS: This is a single-center, retrospective cohort study of all UMCHC patients with CIEDs followed via Medtronic CareLink CIED remote monitoring system from July 2015 to June 2017, after the QI change in 2014. The primary outcome was success of transition to new monitoring schedule. Secondary outcomes included complications, incidence of actionable events (AES), patient compliance, and change in workload. Outcomes were compared to the prior study. RESULTS: There were 325 patients (mean age was 24  ±  14 years) included, of who 293 (90%) completely transitioned to the new RM schedule. During the study period, 96 transmissions included AES (4% of total), of which 50 (52%) were asymptomatic and discovered on routine monitoring. No patient experienced a complication attributable to decreased RM frequency. The mean number of interrogations decreased by 1.6 per patient over the 2-year period compared to prior study. CONCLUSIONS: This study demonstrated successful implementation of a QI initiative to reduce CIED monitoring frequency at a single center with no patient adverse events. The intervention reduced workload and potentially improved patient compliance with routine RM.

What have we learned in the last 20 years? A comparison of a modern era pediatric and congenital catheter ablation registry to previous pediatric ablation registries.

BACKGROUND: Since the onset of pediatric catheter ablation, the pediatric electrophysiology community has reported outcomes via various registries (PAPCA [Prospective Assessment After Pediatric Cardiac Ablation], PCAR [Pediatric Catheter Ablation Registry]). Most recently, a modern era pediatric and congenital ablation registry (MAP-IT [Multicenter Pediatric and Congenital EP Quality Initiative]) was developed for eventual incorporation into the National Cardiovascular Data Registry (NCDR) IMPACT (Improving Pediatric and Adult Congenital Treatment) registry. OBJECTIVE: The purpose of this study was to describe initial findings from the MAP-IT pilot registry and to compare these findings to earlier registries. METHODS: Before entering the NCDR IMPACT registry, MAP-IT was active at 12 centers (11 in the United States) between October 2014 and April 2016. All electrophysiological studies for patients younger than 21 years and for patients of all ages with structural congenital heart disease were included. We compared the acute success, fluoroscopy and procedural times, and frequency of complications between MAP-IT and the earlier registries. RESULTS: Acute success rates have improved from the initial PCAR registry for both accessory and slow pathway substrates. Both fluoroscopy and procedural times have significantly decreased across the time periods (fluoroscopy time 47.6 ± 40 minutes to 7.0 ± 9.2 minutes; P <.001; procedural time 257 ± 157 minutes to 166 ± 84 minutes; P <.001). CONCLUSION: Acute success rates and fluoroscopy and procedural times in pediatric ablation all have improved over the last 25 years.

Life-Threatening Event Risk in Children With Wolff-Parkinson-White Syndrome: A Multicenter International Study.

OBJECTIVES: This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by comparing those who had experienced a life-threatening event (LTE) with a control population. BACKGROUND: Children with WPW syndrome are at risk of sudden death. METHODS: This retrospective multicenter pediatric study identified 912 subjects ≤21 years of age with WPW syndrome, using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or atrial fibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of ≤250 ms or with hemodynamic compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects. RESULTS: Case subjects (n = 96) were older and less likely than subjects (n = 816) to have symptoms or documented tachycardia. Mean age at LTE was 14.1 ± 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP, SPERRI, or SPPCL ≤250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had ≥2 EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and 15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia. CONCLUSIONS: Young patients may experience LTE from WPW syndrome without prior symptoms or markers of high-risk on EPS.

CHCi - A Dynamic Data Platform for Clinical Data Capture and Use.

All academic medical centers have a strong desire to maximize the value of their clinical data for secondary use purposes such as quality improvement (QI) and research. However, this need has not been adequately fulfilled due in part to the fact that the data capture functions in current electronic health record systems predominantly focus on clinical documentation and billing, lacking the flexibility to allow the collection of additional data elements critical to QI or research. To address this gap, we designed and developed a dynamic data platform to support clinicians' varied needs for recording additional data about their patients outside of direct patient care (e.g. classifying patient conditions based on the inclusion criteria of a research-oriented patient registry). In this paper, we describe the design considerations of this platform such as data models, query functions, coding and controlled vocabulary, user interface design, access control, and data interoperability. In developing the platform, we partnered with the frontline clinicians in an academic congenital heart canter, and adopted the agile software development approach with numerous rounds of evaluation and iterative refinement. Since 2013, this platform has been successfully used to meet the dynamic QI and research data needs of clinicians in the congenital heart center. Future work includes improving the efficiency and effectiveness of the platform and incorporating cutting-edge data interoperability standards.