Advocacy at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.

Preliminary findings on the use of intravascular ultrasound in the assessment of pediatric pulmonary vein stenosis.

OBJECTIVE: Determine the feasibility of performing intravascular ultrasound (IVUS) in pediatric pulmonary vein stenosis (PVS) and investigate whether IVUS can delineate the mechanism of PVS. BACKGROUND: The use of IVUS in pediatric patients with PVS has not been reported. METHODS: Retrospective, single center, cohort analysis of all patients who underwent IVUS of pulmonary veins from August 2016 to December 2019. RESULTS: IVUS was performed on 81 pulmonary veins in 50 pediatric patients (median age = 1.7 years [0.9, 3.1], median weight = 8.6 kg [7.3, 11.8]). All veins accepted the IVUS catheter (.014 or .018), with adequate visualization in 88% (71/81) of imaged veins, and improvement in visualization in the more recent period (23/24; 96%). Veins were categorized as having presumed intimal thickening (PIT) with luminal narrowing (n = 36), ostial narrowing without PIT (n = 14), distortion/compression (n = 6), normal (n = 2), and stent with (n = 9) or without in-stent stenosis (n = 4). In veins with at least 6 months of follow up, (re)intervention occurred more commonly in veins with PIT (14/19; 74%) versus veins without PIT (3/13; 23%; p = 0.01). There were no IVUS related adverse events. CONCLUSION: IVUS can be used safely in pediatric patients and can reliably demonstrate vein lumen and wall architecture. With further refinement, IVUS has the potential to differentiate intimal neo-proliferation from other mechanisms of obstruction. The exact role of IVUS in the assessment of pediatric PVS is yet to be determined.

Pilot phase experience of the International Quality Improvement Collaborative catheterization registry.

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.

Systemic Sirolimus to Prevent In-Stent Stenosis in Pediatric Pulmonary Vein Stenosis.

Evaluate the efficacy of systemic sirolimus (rapamycin) in preventing in-stent stenosis (ISS) in pediatric intraluminal pulmonary vein stenosis (PVS). Report the adverse events related to sirolimus therapy. There is a high incidence of ISS following stent implantation in PVS. The use of sirolimus in preventing ISS has not been reported. Retrospective review of all patients who received sirolimus (8 week course) for treatment of ISS for PVS between January 2013 and June 2018. Forty stents (37 bare metal, 3 drug-eluting) in 20 patients were treated with sirolimus; 20 at the time of implantation (primary prevention [1P]) and 20 following documented ISS requiring transcatheter reintervention (secondary prevention [2P]). Treated patients were young (median 2 y/o [0.7-5.7]) and most had PVS associated with congenital heart disease (75%, 15/20; 4/15 with TAPVC). In the 1P group, 85% (17/20) of stents were without significant (< 50%) ISS at median of 102 days (range 56-527); the growth rate of ISS in this group was 7.5 ± 7.1%/month. In the 2P group, most stents had a slower growth rate of ISS after sirolimus therapy compared to pre-treatment (median 3.7 [- 0.2 to 13.1] vs. 10.4 [1.3 to 19.5] %/month; p < 0.001). One patient developed pneumonia on drug while concurrently taking another immunosuppressive agent. No other serious adverse events were related to sirolimus therapy. Systemic sirolimus slows the growth rate of ISS following stent implantation in PVS compared to pre-treatment rates and was administered safely in a small number of pediatric patients with complex heart disease.

Experience participating in the American College of Cardiology Quality NetworkTM: paediatric and adult congenital cardiology collaborative quality improvement.

BACKGROUND: The American College of Cardiology Quality Network enables national benchmarking and collaborative quality improvement through vetted metrics. We describe here our initial experience with the Quality Network. METHODS: Quarterly data for metrics pertaining to chest pain, Kawasaki disease, tetralogy of Fallot, elevated body mass index, and others were shared with the collaboratives for benchmarking. National improvement efforts focussed on counselling for elevated body mass index and 22q11.2 testing in tetralogy of Fallot. Improvement strategies included developing multi-disciplinary workgroups, educational materials, and electronic health record advances. RESULTS: Chest pain metric performance was high compared with national means: obtaining family history (90-100% versus 51-77%), electrocardiogram (100% versus 89-99%), and echocardiogram for exertional complaints (95-100% versus 74-96%). Kawasaki metric performance was high, including obtaining coronary measurements (100% versus 85-97%), prescribing aspirin (100% versus 86-99%), follow-up with imaging (100% versus 85-98%), and documenting no activity restriction without coronary aneurysms (83-100% versus 64-93%). Counselling for elevated body mass index was variable (25-75% versus 31-50%) throughout quality improvement efforts. Testing for 22q11.2 deletion in tetralogy of Fallot patients was consistently above the national mean (60-85% versus 54-68%) with improved genetics data capture. CONCLUSION: The Quality Network promotes meaningful benchmarking and collaborative quality improvement. Our high performance for chest pain and Kawasaki metrics is likely related to previous improvement efforts in chest pain management and a dedicated Kawasaki team. Uptake of counselling for elevated body mass index is variable; stronger engagement among numerous providers is needed. Recommendations for 22q11.2 testing in tetralogy of Fallot were widely recognised and implemented.

Adjunct Targeted Biologic Inhibition Agents to Treat Aggressive Multivessel Intraluminal Pediatric Pulmonary Vein Stenosis.

OBJECTIVE: To evaluate the use of imatinib mesylate with or without bevacizumab targeting neoproliferative myofibroblast-like cells with tyrosine kinase receptor expression, as adjuncts to modern interventional therapies for the treatment of multivessel intraluminal pulmonary vein stenosis (PVS). We describe the 48- and 72-week outcomes among patients receiving imatinib mesylate with or without bevacizumab for multivessel intraluminal PVS. STUDY DESIGN: This single-arm, prospective, open-label US Food and Drug Administration approved trial enrolled patients with ≥2 affected pulmonary veins after surgical or catheter-based relief of obstruction between March 2009 and December 2014. Drug therapy was discontinued at 48 weeks, or after 24 weeks of stabilization, whichever occurred later. RESULTS: Among 48 enrolled patients, 5 had isolated PVS, 26 congenital heart disease, 5 lung disease, and 12 both. After the 72-week follow-up, 16 patients had stabilized, 27 had recurred locally without stabilization, and 5 had progressed. Stabilization was associated with the absence of lung disease (P = .03), a higher percentage of eligible drug doses received (P = .03), and was not associated with age, diagnosis, disease laterality, or number of veins involved. Survival to 72 weeks was 77% (37 of 48). Adverse events were common (n = 1489 total), but only 16 were definitely related to drug treatment, none of which were serious. CONCLUSION: Survival to 72 weeks was 77% in a referral population with multivessel intraluminal PVS undergoing multimodal treatment, including antiproliferative tyrosine kinase blockade. Toxicity specific to tyrosine kinase blockade was minimal.

Impact of a Standardized Clinical Assessment and Management Plan (SCAMP®) on growth in infants with CHD.

BACKGROUND: Growth failure is prevalent among infants with CHD. A Standardized Clinical Assessment and Management Plan was introduced at Boston Children's Hospital's cardiac medical ward to identify patients with growth failure, evaluate relevant contributing conditions, and recommend a management plan including collaboration with nutrition physicians. OBJECTIVE: The objective of this study was to determine whether enrolled patients had improved growth compared with historical controls. METHODS: A total of 29 patients were enrolled in the period July, 2013-June, 2014. In all, 42 historical controls who met eligibility criteria for enrolment were selected for comparison from patients admitted to the same ward in the period June, 2010-June, 2011. Patients with CHD aged <1 year , with growth failure defined as weight-for-age z-score <-2, or failure to sustain adequate weight gain were eligible for participation. Primary outcome was change in weight-for-age z-score from enrolment to most recent weight measurement among patients with at least 6 months of follow-up. RESULTS: Control patients were older at baseline admission weight (118 versus 95 days, p=0.33), and had a higher weight-for-age z-score, -2.9 (-3.1, -2.6) versus -3.7 (-4.3, -3.0) (p=0.02), compared with enrolled patients. Enrolled patients had greater gain in weight-for-age z-score, 2.7 (2.0, 3.4) versus 1.8 (1.5, 2.2) (p=0.03), from baseline to most recent follow-up. CONCLUSION: Patients enrolled in a nutrition-focused protocol had greater weight improvement than historical controls. Identification of growth failure and collaboration with a nutrition support team was associated with improved weight gain among CHD patients experiencing growth failure. CHD programmes should consider a structural approach, including nutrition expertise to address growth failure.

Development and psychometric characteristics of the pediatric inpatient experience survey (PIES).

OBJECTIVE: To study the psychometric properties of the Pediatric Inpatient Experience Survey (PIES), a mail and phone survey for parent reporting of family-centered aspects of inpatient care experiences. DESIGN: Two waves of cross-sectional survey data were collected by mail and phone in 2009 to design a measurement instrument with good psychometric characteristics. Additional cross-sectional data from a mail administration in 2011 confirmed the measurement domains. SETTING: Free-standing pediatric hospital in the northeastern USA. PARTICIPANTS: A convenience sample of English-speaking parents of hospitalized children, stratified by patient type (medical versus surgical) and previous stays at this hospital (yes versus no), constituted the instrument design phase. Four hundred and seventy-nine (63%) of those approached agreed to participate and were randomly assigned to mail or phone survey administration. Four hundred and one of these respondents completed the first wave of the survey and 354 respondents completed the second wave. A shortened instrument was mailed to parents randomly selected from patient discharge records. Data from 929 parents (response rate: 36.2%) were used for confirmatory analysis of the created measurement domains. MAIN OUTCOME MEASURES: The main outcome measures of this psychometric validation study were individual item performance, test-retest reliability, internal consistency, and construct validity. RESULTS: The resulting survey includes 61 items with 35 rating items with satisfactory test-retest reliability loading on eight domains. The factor structure was supported by Cronbach's alpha and confirmatory factor analysis. The survey supported construct validity in distinguishing between medical versus surgical and first time versus previous hospital stay groups known to differ with regard to satisfaction. Comparing mail and phone administrations, differences in scores were exacerbated in domain scores and showed the need for mode adjustment. CONCLUSION: PIES shows satisfactory test-retest reliability, internal consistency, and construct validity. A new domain measuring emotional connectedness to staff and the hospital is highly correlated with overall satisfaction.

Evaluation of Primary Pulmonary Vein Stenosis in Children: Comparison of Radionuclide Perfusion Lung Scan and Angiography.

OBJECTIVE: The purpose of this study was to evaluate the utility of radionuclide perfusion lung scanning in the evaluation of primary pulmonary vein stenosis (PVS) in pediatric patients by comparing it with angiography. MATERIALS AND METHODS: We retrospectively identified pediatric patients with primary PVS who underwent both lung scanning and angiography. A cohort of 34 patients was evaluated. The presence of PVS in the right upper, right lower, left upper, and left lower pulmonary veins on angiograms was recorded. Two nuclear medicine physicians evaluated the lung scans for perfusion defects. Agreement between lung scan and angiographic findings was assessed with contingency tables. Sensitivity and specificity of lung scanning for accurate detection of PVS with angiographic findings as the reference standard were assessed by ROC analysis. RESULTS: Angiography depicted PVS in 90 of the total 136 pulmonary veins (66%). Lung scans correctly depicted 65 (72%) of the cases of PVS diagnosed with angiography. The sensitivity and specificity of lung scans were 76.0% and 88.9% for the right upper pulmonary vein, 70.6% and 94.1% for the right lower pulmonary vein, 77.3% and 58.3% for the left upper pulmonary vein, and 65.4% and 87.5% for the left lower pulmonary vein. CONCLUSION: Lung scan findings correlate with angiographic findings in the detection of primary PVS in pediatric patients. Perfusion lung scanning may have a role in angiographically diagnosed PVS by noninvasively showing relative perfusion at the tissue level.

Systemic Embolic Complications of Pulmonary Vein Angioplasty in Children.

Pulmonary vein stenosis (PVS) carries significant morbidity and mortality for affected children, and its management often requires multiple angioplasty procedures. PVS angioplasty can be complicated by systemic embolic events such as stroke, but incidence and risk factors are poorly understood. We reviewed pediatric catheterizations involving PVS angioplasty and/or stent placement performed at Boston Children's Hospital between July 2005 and February 2014. A total of 406 cases were performed in 144 distinct patients. Patients underwent a median of two catheterizations, at median age 1 year and weight 6.9 kg. Eleven (2.7 %) catheterizations were complicated by clinically apparent systemic embolic events, comprising 10 strokes (one with associated hepatic embolism) and 1 renal infarct. Prevalence of clinically evident stroke among this cohort was 7.6 %. Using a prior (uncomplicated) catheterization to allow each patient to serve as their own control, we sought to identify potentially modifiable risk factors for systemic embolic events. Although this analysis was limited by low power, complicated and uncomplicated angioplasties did not appear to differ in case time, contrast dose, anticoagulation management, use of cutting balloons, number of catheter exchanges, or size of long sheath used. Significant non-embolic adverse events were common, occurring in 25 % of catheterizations. Systemic embolism appears to complicate PVS angioplasty at a rate much higher than that described for other congenital catheterizations. This risk may be inherent to the procedure rather than related to any modifiable or operator-dependent factors.

Impact of prenatal diagnosis in survivors of initial palliation of single ventricle heart disease: analysis of the National Pediatric Cardiology Quality Improvement Collaborative database.

Among infants with single ventricle congenital heart disease (SVD) requiring Stage I palliation (S1P), the impact of prenatal diagnosis (PD) on outcomes has been variably characterized. We investigated the impact of PD in a large multi-center cohort of survivors of S1P in the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) registry. Retrospective analysis of demographic and outcomes data among infants enrolled in the NPCQIC database; eligibility includes SVD requiring S1P and survival to discharge. From 43 contributing surgical centers, 591 infants had data available through time of BDG (519) or interstage death (55). Median gestational age was 39 weeks (31-46), and 66% had variants of hypoplastic left heart syndrome. PD was made in 445 (75%), with significant variation by center (p = 0.004). While infants with PD had slightly lower gestational age at birth (p < 0.001), there were no differences in birth weight, the presence of major syndromes or other organ system anomalies. Those without PD were more likely to have atrioventricular valve regurgitation (p = .002), ventricular dysfunction (p = 0.06), and pre-operative risk factors including acidosis (p < 0.001), renal insufficiency (p = 0.007), and shock (p = 0.05). Post-operative ventilation was shorter in the PD group (9 vs. 12 d, p = 0.002). Other early post-operative outcomes, interstage course, and outcomes at BDG were similar between groups. In a large cohort of infants with SVD surviving to hospital discharge after S1P, PD showed significant inter-site variation and was associated with improved pre-operative status and shorter duration of mechanical ventilation. The significance of such associations merits further study.

Predictors of prolonged length of intensive care unit stay after stage I palliation: a report from the National Pediatric Cardiology Quality Improvement Collaborative.

The objective of this study is to identify predictors of prolonged intensive care unit (ICU) length of stay (LOS) for single ventricle patients following Stage I palliation. We hypothesize that peri-operative factors contribute to prolonged ICU stay among children with hypoplastic left heart syndrome (HLHS) and its variants. In 2008, as a part of the Joint Council on Congenital Heart Disease initiative, the National Pediatric Cardiology-Quality Improvement Collaborative established a data registry for patients with HLHS and its variants undergoing staged palliation. Between July 2008 and August 2011, 33 sites across the United States submitted discharge data essential to this analysis. Data describing the patients, their procedures, and their hospital experience were entered. LOS estimates were generated. Prolonged LOS in the ICU was defined as stay greater than or equal to 26 days (i.e., 75th percentile). Statistical analyses were carried out to identify pre-operative, operative, and post-operative predictors of prolonged LOS in the ICU. The number of patients with complete discharge data was 303, and these subjects were included in the analysis. Univariate and multivariate analyses were performed. Multivariate analysis revealed that lower number of enrolled participants (e.g., 1-10) per site, the presence of pre-operative acidosis, increased circulatory arrest time, the occurrence of a central line infection, and the development of respiratory insufficiency requiring re-intubation were associated with prolonged LOS in the ICU. Prolonged LOS in the ICU following Stage I palliation in patients with HLHS and HLHS variant anatomy is associated with site enrollment, circulatory arrest time, pre-operative acidosis, and some post-operative complications, including central line infection and re-intubation. Further study of these associations may reveal strategies for reducing LOS in the ICU following the Norwood and Norwood-variant surgeries.

Preoperative Malnutrition Increases Risk of In-Hospital Mortality, Major Infection, and Longer Intensive Care Unit Stay After Ventricular Septal Defect Closure.

BACKGROUND: High energy requirements and poor feeding can lead to growth failure in patients with ventricular septal defect (VSD), but effects of preoperative malnutrition on surgical outcomes are poorly understood, especially in low-resource settings. METHODS AND RESULTS: We analyzed a cohort of children <5 years of age undergoing VSD closure at 60 global centers participating in the International Quality Improvement Collaborative for Congenital Heart Disease, 2015 to 2020. We calculated adjusted odds ratios (ORs) for in-hospital death and major infection and adjusted coefficients for duration of intensive care unit stay for 4 measures of malnutrition: severe wasting (weight-for-height Z score, <-3), moderate wasting (-3

Social Determinants of Health Including Child Opportunity Index Leading to Gaps in Care for Patients With Significant Congenital Heart Disease.

BACKGROUND: Gaps in care (GIC) are common for patients with congenital heart disease (CHD) and can lead to worsening clinical status, unplanned hospitalization, and mortality. Understanding of how social determinants of health (SDOH) contribute to GIC in CHD is incomplete. We hypothesize that SDOH, including Child Opportunity Index (COI), are associated with GIC in patients with significant CHD. METHODS AND RESULTS: A total of 8554 patients followed at a regional specialty pediatric hospital with moderate to severe CHD seen in cardiology clinic between January 2013 and December 2015 were retrospectively reviewed. SDOH factors including race, ethnicity, language, and COI calculated based on home address and zip code were analyzed. GIC of >3.25 years were identified in 32% (2709) of patients. GIC were associated with ages 14 to 29 years (P<0.001), Black race or Hispanic ethnicity (P<0.001), living ≥150 miles from the hospital (P=0.017), public health insurance (P<0.001), a maternal education level of high school or less (P<0.001), and a low COI (P<0.001). Multivariable analysis showed that GIC were associated with age ≥14 years, Black race or Hispanic ethnicity, documenting <3 caregivers as contacts, mother's education level being high school or less, a very low/low COI, and insurance status (C statistic 0.66). CONCLUSIONS: One-third of patients followed in a regional referral center with significant CHD experienced a substantial GIC (>3.25 years). Several SDOH, including a low COI, were associated with GIC. Hospitals should adopt formal GIC improvement programs focusing on SDOH to improve continuity of care and ultimately overall outcomes for patients with CHD.

Acute outcome of stent therapy for coarctation of the aorta: results of the coarctation of the aorta stent trial.

OBJECTIVES: The coarctation of the aorta stent trial (COAST) is a pivotal trial of the NuMED Cheatham Platinum Stent(®) for treatment of coarctation of the aorta (CoA). BACKGROUND: CoA is a congenital obstruction to flow through the aorta. Stents have been used since the 1990s to treat CoA; none have been approved by the Food and Drug Administration for this indication. METHODS: In a prospective, multicenter study 105 patients received stents for treatment of CoA. Data was collected in the catheterization laboratory, on discharge and at 1-month postprocedure. RESULTS: Of 105 patients, 69% were male; 57% had native CoA. Noninvasive, baseline systolic blood pressure (SBP) showed upper extremity (UE) SBP to be 140 ± 16 mm Hg and UE to lower extremity (LE) SBP difference 29 ± 17 mm Hg. At catheterization, baseline ratio of minimum CoA diameter to diameter of descending aorta (CoA:DAo) was 0.46 ± 0.16. Implants (104/105) were successful with one stent migration. There were no deaths or serious complications. Paradoxical hypertension occurred in 6%. Four percent of patients experienced somewhat serious adverse events related to the procedure. All patients achieved relief of ascending aorta to Dao pressure gradient: mean = 2.0 ± 4.0 mm Hg (P < 0.001). The CoA:DAo increased to 0.84 ± 0.18. At 1-month, UE SBP was 120 ± 12 mm Hg, UE to LE SBP difference = -1 ± 12 mm Hg. Ninety-nine percent of patients had UE to LE SBP difference <20 mm Hg. CONCLUSIONS: Stenting of CoA, using the NuMED Cheatham Platinum Stent, is acutely safe and effective for treatment of CoA.

Proximal pulmonary vein stenosis detection in pediatric patients: value of multiplanar and 3-D VR imaging evaluation.

BACKGROUND: One of the important benefits of using multidetector computed tomography (MDCT) is its capability to generate high-quality two-dimensional (2-D) multiplanar (MPR) and three-dimensional (3-D) images from volumetric and isotropic axial CT data. However, to the best of our knowledge, no results have been published on the potential diagnostic role of multiplanar and 3-D volume-rendered (VR) images in detecting pulmonary vein stenosis, a condition in which MDCT has recently assumed a role as the initial noninvasive imaging modality of choice. OBJECTIVE: The purpose of this study was to compare diagnostic accuracy and interpretation time of axial, multiplanar and 3-D VR images for detection of proximal pulmonary vein stenosis in children, and to assess the potential added diagnostic value of multiplanar and 3-D VR images. MATERIALS AND METHODS: We used our hospital information system to identify all consecutive children (< 18 years of age) with proximal pulmonary vein stenosis who had both a thoracic MDCT angiography study and a catheter-based conventional angiography within 2 months from June 2005 to February 2012. Two experienced pediatric radiologists independently reviewed each MDCT study for the presence of proximal pulmonary vein stenosis defined as ≥ 50% of luminal narrowing on axial, multiplanar and 3-D VR images. Final diagnosis was confirmed by angiographic findings. Diagnostic accuracy was compared using the z-test. Confidence level of diagnosis (scale 1-5, 5 = highest), perceived added diagnostic value (scale 1-5, 5 = highest), and interpretation time of multiplanar or 3-D VR images were compared using paired t-tests. Interobserver agreement was measured using the chance-corrected kappa coefficient. RESULTS: The final study population consisted of 28 children (15 boys and 13 girls; mean age: 5.2 months). Diagnostic accuracy based on 116 individual pulmonary veins for detection of proximal pulmonary vein stenosis was 72.4% (84 of 116) for axial MDCT images, 77.5% (90 of 116 cases) for multiplanar MDCT images, and 93% (108 of 116 cases) for 3-D VR images with significantly higher accuracy with 3-D VR compared to axial (z = 4.17, P < 0.001) and multiplanar (z = 3.34, P < 0.001) images. Confidence levels for detection of proximal pulmonary vein stenosis were significantly higher with 3-D VR images (mean level: 4.6) compared to axial MDCT images (mean level: 1.7) and multiplanar MDCT images (mean level: 2.0) (paired t-tests, P < 0.001). Thus, 3-D VR images (mean added diagnostic value: 4.7) were found to provide added diagnostic value for detecting proximal pulmonary vein stenosis (paired t-test, P < 0.001); however, multiplanar MDCT images did not provide added value (paired t-test, P = 0.89). Interpretation time was significantly longer and interobserver agreement was higher when using 3-D VR images than using axial MDCT images or MPR MDCT images for diagnosing proximal pulmonary vein stenosis (paired t-tests, P < 0.001). CONCLUSIONS: Use of 3-D VR images in the diagnosis of proximal pulmonary vein stenosis in children significantly increases accuracy, confidence level, added diagnostic value and interobserver agreement. Thus, the routine use of this technique should be encouraged despite its increased interpretation time.

Can the Public-Private Business Model Provide a Sustainable Quality Pediatric Cardiac Surgery Program in Low- and Middle-Income Countries?

Over 90% of the world's children with congenital heart disease do not have access to cardiac care. Although many models provide pediatric cardiac surgery in low- and middle-income countries, sustainability poses a barrier. We explore one model providing care for the underserved in Chennai, India, that came into existence through trial and error over 30 years across three phases. Phase 1 was a Tamilnadu state government-sponsored program that soon became unsustainable with unmet demands. Phase 2 utilized a grassroots foundation of a public-private partnership (PPP) with few donors and a hospital with suboptimal infrastructure. Phase 3 is the ongoing fine-tuning of the PPP model, with upgraded infrastructure and a well-trained team. Through indigenization, an average cardiac surgery costs Rupees (Rs.) 1,80,000 ($2400). The government funds Rs. 60,000 to 80,000 ($800-$1066.67), and the rest is funded through the fund pool. The goal is to perform 100 free surgeries annually by maintaining a fund pool of Rs. 50 lakhs ($66,666.67), which supplements government funds. This ensures equitable distribution of funds with no compromise on resources (disposables, single-use cannulas, etc). Our model ensures the dignity of the patient, fair compensation for workers, and is practical, affordable, and easily adaptable. Thus far, this model provided free cardiac surgery for 357 children from Risk Adjusted Congenital Heart Surgery Score of 1 to 4, with an overall mortality of 2.73%. The prerequisites for this model are having a "spark plug," a dedicated surgical team, a partnership with state-of-the-art infrastructure, and a steady flow of funds.

Changing epidemiology of congenital heart disease: effect on outcomes and quality of care in adults.

The epidemiology of congenital heart disease (CHD) has changed in the past 50 years as a result of an increase in the prevalence and survival rate of CHD. In particular, mortality in patients with CHD has changed dramatically since the latter half of the twentieth century as a result of more timely diagnosis and the development of interventions for CHD that have prolonged life. As patients with CHD age, the disease burden shifts away from the heart and towards acquired cardiovascular and systemic complications. The societal costs of CHD are high, not just in terms of health-care utilization but also with regards to quality of life. Lifespan disease trajectories for populations with a high disease burden that is measured over prolonged time periods are becoming increasingly important to define long-term outcomes that can be improved. Quality improvement initiatives, including advanced physician training for adult CHD in the past 10 years, have begun to improve disease outcomes. As we seek to transform lifespan into healthspan, research efforts need to incorporate big data to allow high-value, patient-centred and artificial intelligence-enabled delivery of care. Such efforts will facilitate improved access to health care in remote areas and inform the horizontal integration of services needed to manage CHD for the prolonged duration of survival among adult patients.

Exploring procedure duration and risk for serious adverse events during congenital cardiac catheterization.

Objectives: While procedure length is considered an important metric for cardiothoracic surgical procedures, the relationship between procedure length and adverse events (AEs) in congenital cardiac catheterizations has little published data available. Furthermore, most existing congenital cardiac catheterization risk prediction models are built on logistic regression models. This study aimed to characterize the relationship between case length and AE occurrence in congenital cardiac catheterization while adjusting for known risk factors and to investigate the potential role of non-linear analysis in risk modeling. Design: Age, case type, and procedure duration were evaluated for relationships with the primary outcome using logistic regression. Non-linearity of the associations with continuous risk factors was assessed using restricted cubic spline transformations. Setting and participants: All diagnostic and interventional congenital cardiac catheterization cases performed at Boston Children's Hospital between January 1, 2014 and October 31, 2019 were analyzed. Main outcome measure: The primary outcome was defined as the occurrence of any clinically significant (level 3/4/5) AE. Results: A total of 7011 catheterization cases met inclusion criteria, with interventional procedures accounting for 68% of cases. Median case duration was 97 min. A multivariable model including age, procedure type, and case duration showed a significant relationship between case duration and AE occurrence (OR 1.07 per 10 min increase, 95% CI 1.06 to 1.09, p<0.001). Conclusions: This study demonstrated the importance of procedure duration as a potential frontier for procedure risk management. Better understanding of the role of procedure duration in cardiac catheterizations may provide opportunities for quality improvement in patient safety and resource planning.