Outcomes after pediatric pulmonary valve replacement in patients with tetralogy of Fallot.

BACKGROUND: People with tetralogy of Fallot (TOF) may require a transannular patch during primary repair, which leads to pulmonary valve regurgitation. Pulmonary valve replacement (PVR) is performed to prevent complication of chronic pulmonary regurgitation, but the optimal timing of PVR remains a matter of debate. This study aimed at assessing the association of PVRs performed <18 years of age on the rate of hospitalizations, interventions, and mortality. METHODS: This is a retrospective observational cohort of people with TOF born in Québec between 1982 and 2015, combining clinical and administrative data. Marginal means/rates models and survival curves were used to compare outcomes between patients with pediatric PVR (<18 years) and those without. Outcomes of interest were rates of cardiac hospitalizations, all-cause hospitalizations, cardiac interventions, and mortality. Groups were balanced using models weighed on the inverse probability of receiving pediatric PVR. RESULTS: Of the 316 eligible patients, 58 (18.4%) received a pediatric PVR. Compared to patients not receiving pediatric PVR, they were at increased risk of cardiac hospitalizations, although the rates of cardiac hospitalization were low: 0.50 versus 0.09 hospitalizations per 20 years [Hazard ratio (HR)=4.71 (95%CI 2.22-9.96)]. Patients receiving a pediatric PVR had a comparable risk of all-cause hospitalizations [HR=0.95 (95%CI 0.71-1.26)] and of cardiac interventions [HR=1.13 (95%CI 0.72-1.77)]. CONCLUSIONS: Patients who underwent pediatric PVR had higher rates of cardiac hospitalizations, but similar rates of all-cause hospitalizations, cardiac procedures, and mortality. In this observational cohort, pediatric PVR was not associated with an improved outcome.

The Quebec Congenital Heart Disease Registry: A Model of Prospective Databank to Facilitate Research in Congenital Cardiology.

Background: A national registry of congenital heart disease (CHD) would facilitate project initiation, decrease costs, increase statistical power, and avoid duplication. Establishing such registries poses numerous challenges, but the current Canadian research ecosystem in CHD is well positioned to meet them. We assessed the feasibility of building a province-wide CHD registry by automatically identifying people with CHD and extracting their native cardiac anatomy from multiple clinical data sources, without the need for manual data entry. Methods: We designed a CHD registry of all fetuses and children with at least 1 echocardiographic report confirming CHD since 2000. We interfaced the registry with several clinical and echocardiography data sources from all paediatric cardiology programmes in Québec. Results: We extracted 885,287 echocardiogram reports and 70,121 clinical records. We identified CHD in 43,452 children and 4682 fetuses. There were 1128 (2.3%) cases with files in multiple institutions, and patients with more complex CHD were 3 times more likely to be seen in more than 1 institution. So far, the registry has been used to build and link CHD cohorts for 7 distinct projects. Conclusions: We demonstrated the feasibility of a baseline CHD registry in Québec without the need for manual data entry, in which other CHD research projects could be nested. This could serve as a blueprint to expand the registry and to develop an integrated approach where data gathered in caring for patients with CHD serve as data layers that incrementally contribute to a national cohort, for which data remain easily accessible and usable.

Contexte: Un registre national des cardiopathies congénitales (CC) pourrait faciliter le lancement de projets de recherche, en diminuer les coûts, en améliorer la puissance statistique tout en évitant les redondances. La mise en place de tels registres pose de nombreux défis, mais l'écosystème de recherche canadien dans le domaine de la CC est bien placé pour y répondre. Nous avons évalué la faisabilité de la mise en place d'un registre des CC à l'échelle provinciale par l'identification automatique des personnes atteintes de CC et l'extraction de leur anatomie cardiaque native à partir de plusieurs sources de données cliniques, sans nécessiter de saisie manuelle de données. Méthodologie: Nous avons conçu un registre des CC incluant tous les fœtus et les enfants pour qui au moins un rapport d'évaluation électrocardiographique confirmait la présence d'une CC depuis 2000. Le registre a été mis en relation avec plusieurs sources de données cliniques et échocardiographiques provenant de tous les programmes en cardiologie pédiatrique au Québec. Résultats: Nous avons extrait 885 287 rapports d'échocardiographie et 70 121 dossiers cliniques. La présence d'une CC a été établie chez 43 452 enfants et 4 682 fœtus. Dans 1 128 cas (2,3 %), un dossier existait dans plus d'un établissement. Les patients présentant des CC plus complexes étaient 3 fois plus susceptibles d'être suivis dans plus d'un établissement. Jusqu'à présent, le registre a été utilisé pour établir et mettre en relation des cohortes de patients atteints de CC pour sept projets de recherche distincts. Conclusions: Nous avons démontré la faisabilité de la mise en place d'un registre de référence des CC au Québec sans recours à la saisie manuelle de données, dans lequel peuvent se nicher d'autres projets de recherche sur les CC. Notre démarche pourrait servir de prototype pour une expansion du registre et pour une approche d'intégration des données recueillies dans la prestation de soins aux patients atteints de CC, afin de former des couches de données qui s'ajoutent au fur et à mesure à une cohorte nationale, avec des données faciles à obtenir et à utiliser.

Early cardiac function and death, severe bronchopulmonary dysplasia and pulmonary hypertension in extremely preterm infants.

BACKGROUND: Association between early cardiac function and neonatal outcomes are scarcely reported. The aim of the current study was to describe this association with death, severe bronchopulmonary dysplasia (BPD) and BPD-related pulmonary hypertension (PH). METHODS: Retrospective cohort study of infants <29 weeks born between 2015 and 2019. Infants with clinically acquired echocardiography at ≤21 days after birth were included and data were extracted by an expert masked to outcomes. RESULTS: A total of 176 infants were included. Echocardiogram was performed at a median of 9 days (IQR 5-13.5). Of these, 31 (18%) had death/severe BPD and 59 (33.5%) had death/BPD-related PH. Infants with death/severe BPD were of lower birth weight (745 [227] vs 852 [211] grams, p = 0.01) and more exposed to invasive ventilation, late-onset sepsis, inotropes and/or postnatal steroids. Early echocardiograms demonstrated decreased right ventricular [Tricuspid Annular Plane Systolic Excursion: 5.2 (1.4) vs 6.2 (1.5) cm, p = 0.03] and left ventricular function [Ejection fraction 53 (14) vs 58 (10) %, p = 0.03]. Infants with death/BPD-related PH had an increased Eccentricity index (1.35 [0.20] vs 1.26 [0.19], p = 0.02), and flat/bowing septum (19/54 [35%] vs 20/109 [18%], p = 0.021). CONCLUSIONS: In extremely premature infants, altered ventricular function and increased pulmonary pressure indices within the first 21 days after birth, were associated with the combined outcome of death/severe BPD and death/BPD-related PH. IMPACT: Decreased cardiac function on echocardiography performed during first three weeks of life is associated with severe bronchopulmonary dysplasia in extremely premature infants. In extreme preterm infants, echocardiographic signs of pulmonary hypertension in early life are associated with later BPD-related pulmonary hypertension close to 36 weeks post-menstrual age. Early cardiac markers should be further studied as potential intervention targets in this population. Our study is adding comprehensive analysis of echocardiographic data in infants born below 29 weeks gestational age.

Determinants and Clinical Outcomes of Patients With Tetralogy of Fallot Lost to Cardiology Follow-up.

BACKGROUND: Various rates of loss to follow-up (LTFU) have been reported in patients with congenital heart disease, but return to follow-up is rarely considered in those analyses. Outcomes of LTFU patients are difficult to assess because the patients no longer attend cardiac care. We leveraged data from the TRIVIA cohort, which combines more than 30 years of clinical and administrative data, allowing us to study outcomes even after LTFU. METHODS: This population-based cohort included 904 patients with tetralogy of Fallot (TOF) born from 1982 to 2015 in Québec, Canada. Risk factors for LTFU and outcomes were calculated by Cox models and marginal means/rates models. Outcomes of LTFU patients were compared with propensity score-matched non-LTFU patients. RESULTS: The cumulative risk of experiencing 1 episode of LTFU was 50.3% at 30 years. However, return to follow-up was frequent and the proportion of patients actively followed was 85.9% at 10 years, 76.4% at 20 years, and 70.6% at 30 years. Factors associated with a reduced risk of LTFU were primary repair with conduit (hazard ratio [HR] 0.29, 95% confidence interval [CI] 0.15-0.58) and transannular patch (HR 0.60, 95% CI 0.46-0.79). LTFU patients had lower rates of cardiac hospitalisations (HR 0.49, 95% CI 0.42-0.56) and cardiac interventions (HR 0.32, 95% CI 0.25-0.42), but similar rates of cardiac mortality (HR 0.95, 95% CI 0.24-3.80). CONCLUSIONS: There was a lower proportion of LTFU patients compared with previous studies. Factors associated with lower rates of LTFU were conduits and non-valve-sparing surgery. LTFU patients had lower rates of cardiac procedures and cardiac hospitalisations.

Association of Gestational Age at Birth With Left Cardiac Dimensions at Near-Term Corrected Age Among Extremely Preterm Infants.

BACKGROUND: Remodeling and altered ventricular geometry have been described in adults born preterm. Although they seem to have an adverse cardiac phenotype, the impact of various degrees of prematurity on cardiac development has been scarcely reported. In this study, we evaluated the impact of gestational age (GA) at birth on cardiac dimensions and function at near-term age among extremely preterm infants. METHODS: This is a retrospective single-center cohort study of infants born at <29 weeks of GA between 2015 and 2019. Infants with available clinically acquired echocardiography between 34 and 43 weeks were included. Two groups were investigated: those born <26 weeks and those born ≥26 weeks. All measurements were done by an expert masked to clinical data using the raw images. The primary outcome was measurements of cardiac dimensions and function based on GA group. Secondary outcomes were the association between cardiac dimensions and postnatal steroid exposure and with increments of GA at birth. RESULTS: A total of 205 infants were included (<26 weeks, n = 102; ≥26 weeks, n = 103). At time of echocardiography, weight (2.4 ± 0.5 vs 2.5 ± 0.5 kg, P = .86) and age (37.2 ± 1.6 vs 37.1 ± 1.9 weeks, P = .74) were similar between groups. There was no difference in metrics of right-sided dimensions and function. However, left-sided dimensions were decreased in infants born <26 weeks, including systolic left ventricle (LV) diameter (1.06 ± 0.20 cm vs 1.12 ± 0.18 cm, P = .02), diastolic LV length (2.85 ± 0.37 vs 3.02 ± 0.57 cm, P = .02), and estimated LV end-diastolic volume (5.36 ± 1.69 vs 6.01 ± 1.79 mL, P = .02). CONCLUSIONS: In our cohort of very immature infants, birth at the extreme of prematurity was associated with smaller left cardiac dimensions around 36 weeks of corrected age. Future longitudinal prospective studies should evaluate further the impact of prematurity on LV development and performance and their long-term clinical impact.

Newborns with a Congenital Heart Defect and Diastolic Steal Have an Altered Cerebral Arterial Doppler Profile.

OBJECTIVES: To compare trends in the anterior cerebral artery (ACA) Doppler markers of vascular flow for neonates with a congenital heart defect (CHD) with and without diastolic systemic steal during the first 7 days of life. METHODS: Prospective study recruiting newborns (≥35 weeks of gestation) with a CHD. Doppler ultrasound and echocardiography were performed daily from day 1 to 7. The cohort was divided into the presence/absence of holo-diastolic retrograde flow in the postductal aorta ("retrograde") on the last-available echocardiogram. Data extractors were masked to retrograde status. Mixed effect models (random slope/intercept) were constructed using RStudio. RESULTS: We enrolled 38 neonates with CHD. Retrograde aortic flow was present on the last echocardiogram in 23 (61%). Peak systolic velocity and mean velocity increased significantly over time, independent of retrograde status. However, having a "retrograde" flow status conferred a significant decrease over time of their ACA-end-diastolic velocity (ß = -5.75 cm/s, 95% CI -8.38 to -3.12, P < .001, when compared with the nonretrograde group), and a significant increase in the ACA resistive (ß = 0.16, 95% CI 0.10-0.22, P < .001) and pulsatility (ß = 0.49, 95% CI 0.28-0.69, P < .001) indexes. No subject presented retrograde diastolic flow in the ACA. CONCLUSIONS: In neonates with CHD in the first week of life, infants with echocardiographic signs of systemic diastolic steal within the pulmonary circulation have Doppler signs of cerebrovascular steal in the ACA.

The biventricular contribution to chronic pulmonary hypertension of the extremely premature infant.

OBJECTIVE: Evaluate factors associated with significant pulmonary hypertension [PH] (≥2/3 systemic) and its impact on ventricular function at 36 weeks postmenstrual age (PMA). STUDY DESIGN: Retrospective cohort of infants born at <29 weeks who survived to their echocardiography screening for PH at 36 weeks PMA. Masked experts extracted conventional and speckle-tracking echocardiography [STE] data. RESULTS: Of 387 infants, 222 were included and 24 (11%) categorized as significant PH. Significant PH was associated with a decrease in tricuspid annular plane systolic excursion (0.79 vs 0.87 cm, p = 0.03), right peak longitudinal strain [pLS] by STE (-19.6 vs -23.1%, p = 0.003) and left pLS (-25.0 vs -22.7%, p = 0.02). The association between biventricular altered function by STE and significant PH persisted after adjustment for potential confounders - LV-pLS (p = 0.007) and RV-pLS (p = 0.01). CONCLUSION: Our findings are suggestive that premature newborns with significant PH at 36 weeks PMA have a biventricular cardiac involvement to their pathophysiology.

Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot.

Importance: The choice of the right surgical technique for correction of tetralogy of Fallot (TOF) is contentious for patients with a moderate to severe right outflow tract obstruction. The use of a transannular patch (TAP) exposes patients to chronic pulmonary regurgitation, while valve-sparing (VS) procedures may incompletely relieve pulmonary obstruction. Objective: To compare 30-year outcomes of TOF repair after a VS procedure vs TAP. Design, Setting, and Participants: This retrospective population-based cohort study was conducted among all patients with TOF born in the province of Quebec, Canada, from 1980 to 2015 who underwent complete surgical repair. Patients who received a TAP or VS procedure were matched using a propensity score based on preoperative factors in a 1:1 ratio. Data were analyzed from March 2020 through April 2021. Exposures: The study groups were individuals who received TAP and those who received VS. The VS group was further stratified by the presence of residual pulmonary stenosis. Main Outcomes and Measures: The primary outcome was all-cause mortality, with 30-year survival evaluated using Cox proportional-hazards models. Secondary outcomes included the cumulative mean number of cardiovascular interventions, pulmonary valve replacements (PVRs), and cardiovascular hospitalizations were evaluated using marginal means/rates regressions. Results: Among 683 patients with TOF (401 patients who underwent TAP [58.7%] and 282 patients who underwent a VS procedure [41.3%]), adequate propensity score matching was achieved for 528 patients (264 patients who underwent a VS procedure and 264 patients who underwent TAP). Among this study cohort, 307 individuals (58.1%) were men. The median (interquartile range [IQR]) follow-up was 16.0 (8.1-25.4) years, for a total of 8881 patient-years, including 63 individuals (11.9%) followed up for more than 30 years. Individuals who received a VS procedure had an increased 30-year survival of 99.1% compared with 90.4% for individuals who received TAP (hazard ratio [HR], 0.09 [95% CI, 0.02-0.41]; P = .002). Patients who underwent TAP had an increased 30-year cumulative mean number of cardiovascular interventions compared with patients who underwent a VS procedure without residual pulmonary stenosis (2.0 interventions [95% CI, 1.5-2.7 interventions] vs 0.7 interventions [95% CI, 0.5-1.1 interventions]; mean ratio [MR], 0.36 [95% CI, 0.25-0.50]; P < .001) and patients who underwent a VS procedure with at least moderate residual stenosis (1.3 interventions [95% CI, 0.9-1.9 interventions]; MR, 0.65 [0.45-0.93]; P = .02). Results were similar for PVR, with a 30-year cumulative mean 0.3 PVRs [95% CI, 0.1-0.7 PVRs] for patients who underwent a VS procedure without residual pulmonary stenosis (MR, 0.22 [95% CI, 0.12-0.43]; P < .001) and 0.6 PVRs (95% CI, 0.2-1.5 PVRs) for patients with at least moderate residual stenosis (MR, 0.44 [95% CI, 0.21-0.93]; P = .03), compared with 1.4 PVRs (95% CI, 0.8-2.5 PVRs) for the TAP group. No statistically significant difference was found for cardiovascular hospitalizations. Conclusions and Relevance: This study found that patients who underwent a VS procedure had increased 30-year survival, fewer cardiovascular reinterventions, and fewer PVRs compared with individuals who underwent TAP, even in the presence of significant residual pulmonary stenosis. These findings suggest that it is beneficial to perform a VS procedure when possible, even in the presence of moderate residual stenosis, compared with the insertion of a TAP.

Neurocognitive disorders amongst patients with congenital heart disease undergoing procedures in childhood.

BACKGROUND: Amongst children with congenital heart disease (CHD), earlier age of repair improves cardiovascular outcomes. The effects of early intervention on neurodevelopment remains unclear. We assessed the association between early life repair, cardiopulmonary bypass (CPB) and the incidence of neurocognitive disorders (NCD) amongst CHD patients. METHODS: We created two retrospective cohorts from the Quebec CHD Database; with data from 1988 to 2010. Complexity of reparative procedures for CHD lesions were used as the proxy of CPB exposure with more complex procedure means longer exposure to CPB. Study Population 1 included pediatric patients with a single reparative procedure and compared patients with complex (long CBP) versus isolated shunt (short CBP) lesions. To assess the effects of CPB alone in Study Population 2 we compared patients with isolated atrial septal defects (ASD) who had surgical (short CBP) versus percutaneous (no CBP) repairs. The primary endpoint for both cohorts was development of an NCD. RESULTS: In Study population 1, 1174 patients underwent complex surgical repair and 1620 had a shunt closure. The incidence of NCDs was 2.45/100 person-years in the complex surgery group, and 2.08/100 person-years in the shunt closure group. The following were associated with increased risk of developing a NCD: surgical complexity (Hazard Ratio [HR] = 1.20, 95% Confidence Interval [CI]: 1.01-1.42), younger age at intervention (AAI) (HR = 1.20, 95% CI: 1.16-1.25), male sex (HR = 1.91, 95% CI: 1.61-2.27), and later calendar year at intervention (HR = 1.06, 95% CI: 1.04-1.07). Study population 2 had 527 isolated ASD patients; 202 underwent surgical repair and 325 had percutaneous closure. The incidence of NCDs was not statistically different between groups. Male sex (HR = 1.77, 95% CI: 1.08-2.89) and younger AAI (HR = 1.15, 95% CI: 1.06-1.25) were associated with increased NCD risk. CONCLUSION: Increased surgical complexity, male sex and younger AAI were associated with increased risk of NCDs in pediatric CHD patients. Surveillance protocols should be considered to identify NCDs in CHD patients after cardiac intervention.

The 30-Year Outcomes of Tetralogy of Fallot According to Native Anatomy and Genetic Conditions.

BACKGROUND: The reported survival of tetralogy of Fallot (TOF) is > 97%. Patients with pulmonary atresia and/or genetic conditions have worse outcomes, but long-term estimates of survival and morbidity for these TOF subgroups are scarce. The objective of this study was to describe the 30-year outcomes of TOF according to native anatomy and the coexistence of genetic conditions. METHODS: The TRIVIA (Tetralogy of Fallot Research for Improvement of Valve Replacement Intervention: A Bridge Across the Knowledge Gap) study is a retrospective population-based cohort including all TOF subjects born from 1980 to 2015 in Québec. We evaluated all-cause mortality by means of Cox proportional hazards regression, and cumulative mean number of cardiovascular interventions and unplanned hospitalisations with the use of marginal means/rates models. We computed 30-year estimates of outcomes according to TOF types, ie, classic TOF (cTOF) and TOF with pulmonary atresia (TOF-PA), and the presence of genetic conditions. RESULTS: We included 960 subjects. The median follow-up was 17 years (interquartile range, 8-27). Nonsyndromic cTOF subjects had a 30-year survival of 95% and had undergone a mean of 2.8 interventions and 0.5 hospitalisations per subject. In comparison, TOF-PA subjects had a lower 30-year survival of 78% and underwent a mean of 8.1 interventions, with 4 times as many hospitalisations. The presence of a genetic condition was associated with lower survival (< 85% for cTOF and < 60% for TOF-PA) but similar numbers of interventions and hospitalisations. CONCLUSIONS: The anatomic types and the presence of genetic conditions strongly influence the long-term outcomes of TOF. We provided robust 30-year estimates for key markers of prognosis that may be used to improve risk stratification and provide more informed counselling to families.

The TRIVIA Cohort for Surgical Management of Tetralogy of Fallot: Merging Population and Clinical Data for Real-World Scientific Evidence.

BACKGROUND: Guidelines for surgical management of tetralogy of Fallot (TOF) are often based on low-quality evidence due to the many challenges of congenital heart disease: heterogeneous cardiac anatomy, consequences from surgical interventions arising years later, and scarcity of hard outcomes. The overarching goal of the Tetralogy of Fallot Research for Improvement of Valve replacement Intervention: A Bridge Across the Knowledge Gap (TRIVIA) study is to evaluate the long-term impact of the surgical management strategies in TOF. The specific objectives are: (1) to describe the long-term outcomes of TOF according to the native anatomy and the presence of genetic conditions, (2) to evaluate the long-term outcomes of surgical repair according to associated residual lesions, and (3) to evaluate the impact of paediatric pulmonary valve replacements on the long-term outcomes. METHODS: The TRIVIA study is a population-based cohort including all subjects with TOF in the province of Québec between 1980 and 2017. It links patient-level granular clinical data with long-term administrative health care data. We will evaluate mortality, cardiovascular interventions, and hospitalizations for adverse cardiovascular events using survival Cox models and marginal mean/rates models for recurrent events, respectively. Multivariate multilevel models will correct for potential confounders, and risk score matching will mitigate the potential of confounding by indication. RESULTS: The current TRIVIA cohort includes 1001 eligible subjects with TOF with complete lifelong follow-up for > 98%. The median follow-up is 17.1 years, totalling > 17,000 patient-years. CONCLUSIONS: Universal health insurance data combined with granular clinical data enable the development of population-based cohorts, to which contemporary statistical methods are applied to address important research questions in congenital heart disease research.

CONTEXTE: Les lignes directrices pour la prise en charge chirurgicale de la tétralogie de Fallot (TdF) sont souvent basées sur des données de faible qualité en raison des nombreux défis posés par les maladies cardiaques congénitales : anatomie cardiaque hétérogène, répercussions des interventions chirurgicales survenant des années plus tard et rareté de conclusions solides. L'objectif principal de l'étude TRIVIA ( T etralogy of Fallot R esearch for I mprovement of V alve replacement I ntervention: A Bridge Across the Knowledge Gap) est d'évaluer l'impact à long terme des stratégies de prise en charge chirurgicale dans les TdF. Les objectifs spécifiques sont les suivants : (1) décrire les issues à long terme de la TdF en fonction de l'anatomie native et de la présence de conditions génétiques, (2) évaluer les conséquences à long terme de la chirurgie réparatrice en fonction des lésions résiduelles associées, et (3) évaluer l'impact des remplacements valvulaires pulmonaires pédiatriques sur le pronostic à long terme. MÉTHODES: L'étude TRIVIA est une étude de cohorte basée sur une population comprenant tous les sujets atteints de TdF dans la province du Québec entre 1980 et 2017. Elle fait le lien entre les données cliniques détaillées des patients et les données administratives des soins de santé à long terme. Nous évaluerons la mortalité, les interventions cardiovasculaires et les hospitalisations pour troubles cardiovasculaires en utilisant respectivement des analyses de survie par les modèles de Cox et des modèles de moyennes/taux marginaux pour les événements récurrents. Des modèles multivariés à plusieurs niveaux permettront de corriger les facteurs de confusion potentiels, et l'appariement par des scores de risque atténuera le potentiel de confusion par indication. RÉSULTATS: La cohorte de l'étude TRIVIA comprend actuellement 1001 sujets admissibles atteints de TdF avec un suivi complet tout au long de la vie pour > 98 %. Le suivi médian est de 17,1 ans, pour un total de > 17 000 patients-années. CONCLUSIONS: Les données de l'assurance maladie universelle combinées à des données cliniques détaillées permettent la mise en place de cohortes populationnelles, auxquelles les méthodes statistiques contemporaines pourront être appliquées afin de répondre aux questions importantes pour la recherche portant sur les maladies cardiaques congénitales.

Follow-up after neonatal heart disease repair: watch out for chronic kidney disease and hypertension!

BACKGROUND: With advances in care, neonates undergoing cardiac repairs are surviving more frequently. Our objectives were to 1) estimate the prevalence of chronic kidney disease (CKD) and hypertension 6 years after neonatal congenital heart surgery and 2) determine if cardiac surgery-associated acute kidney injury (CS-AKI) is associated with these outcomes. METHODS: Two-center prospective, longitudinal single-visit cohort study including children with congenital heart disease surgery as neonates between January 2005 and December 2012. CKD (estimated glomerular filtration rate < 90 mL/min/1.73m2 or albumin/creatinine ≥3 mg/mmol) and hypertension (systolic or diastolic blood pressure ≥ 95th percentile for age, sex, and height) prevalence 6 years after surgery was estimated. The association of CS-AKI (Kidney Disease: Improving Global Outcomes definition) with CKD and hypertension was determined using multiple regression. RESULTS: Fifty-eight children with median follow-up of 6 years were evaluated. CS-AKI occurred in 58%. CKD and hypertension prevalence were 17% and 30%, respectively; an additional 15% were classified as having elevated blood pressure. CS-AKI was not associated with CKD or hypertension. Classification as cyanotic postoperatively was the only independent predictor of CKD. Postoperative days in hospital predicted hypertension at follow-up. CONCLUSIONS: The prevalence of CKD and hypertension is high in children having neonatal congenital heart surgery. This is important; early identification of CKD and hypertension can improve outcomes. These children should be systematically followed for the evolution of these negative outcomes. CS-AKI defined by current standards may not be a useful clinical tool to decide who needs follow-up and who does not.

Paucity of renal follow-up by school age after neonatal cardiac surgery.

INTRODUCTION: There are little data about renal follow-up of neonates after cardiovascular surgery and no guidelines for long-term renal follow-up. Our objectives were to assess renal function follow-up practice after neonatal cardiac surgery, evaluate factors that predict follow-up serum creatinine measurements including acute kidney injury following surgery, and evaluate the estimated glomerular filtration rate during follow-up using routinely collected laboratory values. METHODS: Two-centre retrospective cohort study of children 5-7 years of age with a history of neonatal cardiac surgery. Univariable and multivariable analyses were performed to determine factors associated with post-discharge creatinine measurements. Glomerular filtration rate was estimated for each creatinine using a height-independent equation. RESULTS: Seventeen of 55 children (30%) did not have any creatinine measured following discharge after surgery until the end of study follow-up, which occurred at a median time of 6 years after discharge. Of the 38 children who had the kidney function checked, 15 (40%) had all of their creatinine drawn only in the context of a hospitalisation or emergency department visit. Acute kidney injury following surgery did not predict the presence of follow-up creatinine measurements. CONCLUSIONS: A large proportion of neonates undergoing congenital heart repair did not have a follow-up creatinine measured in the first years following surgery. In those that did have a creatinine measured, there did not appear to be any identified pattern of follow-up. A follow-up system for children who are discharged from cardiac surgery is needed to identify children with or at risk of chronic kidney disease.

Kidney and blood pressure abnormalities 6 years after acute kidney injury in critically ill children: a prospective cohort study.

BACKGROUND: Acute kidney injury (AKI) in pediatric intensive care unit (PICU) children may be associated with long-term chronic kidney disease or hypertension. OBJECTIVES: To estimate (1) prevalence of kidney abnormalities (low estimated glomerular filtration rate (eGFR) or albuminuria) and blood pressure (BP) consistent with pre-hypertension or hypertension, 6 years after PICU admission; (2) if AKI is associated with these outcomes. METHODS: Longitudinal study of children admitted to two Canadian PICUs (January 2005-December 2011). Exposures (retrospective): AKI or stage 2/3 AKI (KDIGO creatinine-based definition) during PICU. Primary outcome (single visit 6 years after admission): presence of (a) low eGFR (<90 ml/min/1.73 m2) or albuminuria (albumin to creatinine ratio >30 mg/g) (termed "CKD signs") or (b) BP consistent with ≥pre-hypertension (≥90th percentile) or hypertension (≥95th percentile). RESULTS: Of 277 children, 25% had AKI. AKI and stage 2/3 AKI were associated with 2.2- and 6.6-fold higher adjusted odds, respectively, for the 6-year outcomes. Applying new hypertension guidelines attenuated associations; stage 2/3 AKI was associated with 4.5-fold higher adjusted odds for 6-year CKD signs or ≥elevated BP. CONCLUSIONS: Kidney and BP abnormalities are common 6 years after PICU admission and associated with AKI. Other risk factors must be elucidated to develop follow-up recommendations and reduce cardiovascular risk.

Pathophysiology, screening and diagnosis of pulmonary hypertension in infants with bronchopulmonary dysplasia - A review of the literature.

Bronchopulmonary dysplasia (BPD) is a common complication of extreme prematurity, which has increased over the last 20 years. BPD is associated with increased morbidities and mortality. It has been increasingly recognized that BPD affects overall lung development including the pulmonary vasculature. More recent studies have demonstrated an increased awareness of pulmonary arterial hypertension (PH) in BPD patients and recent international guidelines have advocated for better screening. This review will describe the current understanding of the pathophysiology of PH in infants with BPD, the in-depth assessment of the available literature linking PH and BPD, and propose an approach of screening and diagnosis of PH in infants with BPD.

Extent, risk factors, and outcome of fluid overload after pediatric heart surgery*.

OBJECTIVES: Fluid overload is associated with poor PICU outcomes in different populations. Little is known about fluid overload in children undergoing cardiac surgery. We described fluid overload after cardiac surgery, identified risk factors of worse fluid overload and also determined if fluid overload predicts longer length of PICU stay, prolonged mechanical ventilation (length of ventilation) and worse lung function as estimated by the oxygenation index. DESIGN: Retrospective cohort study. SETTING: Montreal Children's Hospital PICU, Montreal, Canada. PATIENTS: Patients 18 years or younger undergoing cardiac surgery (2005-2007). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Cumulative fluid overload % was calculated as [(total fluid in - out in L)/admission weight (kg) × 100] and expressed as PICU peak cumulative fluid overload % throughout admission and PICU day 2 cumulative fluid overload %. Primary outcomes were length of stay and length of ventilation. The secondary outcome was oxygenation index. Fluid overload risk factors were evaluated using stepwise linear regression. Fluid overload-outcome relations were evaluated using stepwise Cox regression (length of stay, length of ventilation) and generalized estimating equations (daily PICU cumulative fluid overload % and oxygenation index repeated measures). There were 193 eligible surgeries. Peak cumulative fluid overload % was 7.4% ± 11.2%. Fluid overload peaked on PICU day 2. Lack of past cardiac surgery (p = 0.04), cyanotic heart disease (p = 0.03), and early postoperative fluids (p = 0.0001) was independently associated with higher day 2 fluid overload %. Day 2 fluid overload % predicted longer length of stay (adjusted hazard ratio, 0.95; 95% CI, 0.92-0.99; p = 0.009) and length of ventilation (adjusted hazard ratio, 0.97; 95% CI, 0.94-0.99; p = 0.03). In patients without cyanotic heart disease, worse daily fluid overload % predicted worse daily oxygenation index. CONCLUSION: Fluid overload occurs early after cardiac surgery and is associated with prolonged PICU length of stay and ventilation. Future fluid overload avoidance trials may confirm or refute a true fluid overload-outcome causative association.

Percutaneous angioplasty used to manage native and recurrent coarctation of the aorta in infants younger than 1 year: immediate and midterm results.

Balloon angioplasty (BAP) used to manage native coarctation of the aorta (CoAo) in infants remains controversial. This study aimed to compare short- and midterm results of BAP between native CoAo (NaCo) and postsurgical recoarctations (ReCo) in infants younger than 1 year. This retrospective study compared the clinical, echocardiographic, hemodynamic, and angiographic data for infants who underwent BAP between July 2003 and September 2012. The 12 NaCo and 13 ReCo patients in this study underwent BAP at 4.61 ± 3.69 and 4.88 ± 3.07 months (p = 0.84) and weighed 5.49 ± 2.57 and 6.10 ± 2.11 kg (p = 0.52), respectively. Their respective heights were 60.58 ± 10.58 and 61.15 ± 6.74 cm (p = 0.87). All the ReCo patients had their initial surgery before the age of 3 months. The minimal CoAo diameter was 2.81 ± 0.96 mm in the NaCo group and 2.86 ± 1.0 mm in the ReCo group (p = 0.90). The relative gradient reduction was 62.79 ± 32.43 % in the NaCo group and 73.37 ± 20.78 % in the ReCo group (p = 0.33). The in situ complication rate (pseudoaneurysm) was 8.33 % in the NaCo group and 7.69 % in the ReCo group (p = 0.74). During the early follow-up evaluation, five NaCo patients (41.66 %) presented with recoarctation requiring BAP reintervention within 1.75 ± 1.41 months (all had their initial BAP before 3 months of age) compared with 1 ReCo patient (7.69 %) (p = 0.165). The mean follow-up period was 3.09 ± 2.69 years for the NaCo patients and 3.6 ± 3.18 years for the ReCo patients (p = 0.69), during which the blood pressure gradient was 12.33 ± 9.67 for the NaCo patients and 7.80 ± 8.78 for the ReCo patients (p = 0.17), with corresponding Doppler peak instantaneous gradients of 21.29 ± 11.19 and 16.20 ± 10.23 (p = 0.24). The resultant diameter ratio between the minimal isthmus diameter and the aortic diameter at the diaphragmatic level was 0.81 ± 0.15 in the NaCo group and 0.85 ± 0.12 in the ReCo group (p = 0.53). The immediate and midterm results of BAP for the NaCo and ReCo infants were comparable. Accordingly, BAP seems to be an acceptable alternative to surgery for infants older than 3 months.

Presentation, diagnosis, and medical management of heart failure in children: Canadian Cardiovascular Society guidelines.

Pediatric heart failure (HF) is an important cause of morbidity and mortality in childhood. This article presents guidelines for the recognition, diagnosis, and early medical management of HF in infancy, childhood, and adolescence. The guidelines are intended to assist practitioners in office-based or emergency room practice, who encounter children with undiagnosed heart disease and symptoms of possible HF, rather than those who have already received surgical palliation. The guidelines have been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and are accompanied by practical Recommendations for their application in the clinical setting, supplemented by online material. This work does not include Recommendations for advanced management involving ventricular assist devices, or other device therapies.