Tetralogy of Fallot in Low- and Middle-Income Countries.

Low- and middle-incomes countries (LMICs) have limited resources for the diagnosis and treatment of congenital heart diseases such as tetralogy of Fallot. This is in part due to lack of infrastructures, financial means, and expertise. As a result, patients undergo surgery much later than in high-income countries. This delay in treatment results in right ventricular dysfunction, cardiac arrhythmias, and poor psychomotor development-complications that are all related to chronic hypoxia. There are limited data and a few small studies of patients treated for tetralogy of Fallot in LMICs, and, therefore, the aim of this review is to analyse and summarize the surgical outcomes of this LMIC population.

Dans les pays à revenu faible ou intermédiaire (PRFI), les ressources sont limitées pour diagnostiquer et prendre en charge les cardiopathies congénitales comme la tétralogie de Fallot. Cette situation est attribuable en partie au manque d'infrastructures, de moyens financiers et d'expertise. Les patients subissent donc une correction chirurgicale beaucoup plus tard que dans les pays à revenu élevé. Les délais de traitement peuvent entraîner une dysfonction ventriculaire droite, une arythmie cardiaque et des problèmes de développement psychomoteur : des complications toutes liées à l'hypoxie chronique. Il existe des données limitées et quelques études de faible envergure sur des patients traités pour une tétralogie de Fallot dans les PRFI. L'objectif du présent article de synthèse est donc d'analyser et de résumer les issues des interventions chirurgicales dans les PRFI pour cette population.

The Ross-Konno Procedure With or Without Mitral Valve Surgery: A Systematic Review With Individual Data Pooling.

Background: The Ross-Konno procedure is a technically demanding surgical option to treat multilevel left ventricular outflow tract obstruction. Methods: A systematic review with pooled analyses was conducted according to PRISMA criteria on studies published between January 2000 and May 2022 that assessed outcomes following the Ross-Konno intervention in children. Individual patient data were extracted from published Kaplan-Meier curves using digitalization software. Overall survival and freedom from reintervention were assessed by time-to-event approaches. Determinants of one-year survival were investigated by meta-regression analyses. Results: Ten studies with a total population of 274 patients were included. The overall pooled early (≤30 days) survival rate was 86.9% (95% CI [87.6%-78.4%]). Five-year survival rates in patients without and with (N = 50 [18.2%] of 274 total patients) concomitant mitral valve surgery were 82.5% (95% CI [87.6%-77.4%]) versus 56.1% (95% CI [74.1%-38.1%]), hazard ratio 2.67, 95% CI (1.44-4.93), P < .0001. Five- and ten-year freedom from pulmonary autograft reoperation rates were 93.5% and 90.9%, respectively. Five- and ten-year freedom from right ventricular outflow tract reoperation rates were 74.3% and 57.3%, respectively. By meta-regression analysis, resection of endocardial fibroelastosis (N = 32 [11.7%] of 274 total patients) was associated with superior one-year survival (P = .027). Conclusion: The Ross-Konno procedure is associated with substantial early mortality and gradual attrition thereafter. Mortality is higher in patients with concomitant mitral valve surgery. Resection of endocardial fibroelastosis is associated with superior survival. Right ventricular outflow tract reinterventions are common.

Language brain responses and neurodevelopmental outcome in preschoolers with congenital heart disease: A fNIRS study.

Neurodevelopmental disabilities affect up to 50% of survivors of congenital heart disease (CHD). Language difficulties are frequently identified during preschool period and can lead to academic, social, behavioral, and emotional difficulties. Structural brain alterations are associated with poorer neurodevelopmental outcomes in patients with CHD during infancy, childhood, and adolescence. However, evidence is lacking about the functional brain activity in children with CHD and its relationship with neurodevelopment. This study therefore aimed to characterize brain responses during a passive story-listening task in 3-year-old children with CHD, and to investigate the relationship between functional brain patterns of language processing and neurodevelopmental outcomes. To do so, we assessed hemodynamic concentration changes, using functional near-infrared spectroscopy (fNIRS), and neurodevelopmental outcomes, using the Wechsler Preschool and Primary Scale of Intelligence - 4th Edition (WPPSI-IV), in children with CHD (n = 19) and healthy controls (n = 23). Compared to their healthy peers, children with CHD had significantly lower scores on the Verbal comprehension index (VCI), the Vocabulary acquisition index (VAI), the General ability index (GAI), and the Information and the Picture Naming subtests of the WPPSI-IV. During the passive story-listening task, healthy controls showed significant hemodynamic brain responses in the temporal and the temporal posterior regions, with stronger activation in the temporal posterior than in the temporal regions. In contrast, children with CHD showed reduced activation in the temporal posterior regions compared to controls, with no difference of activation between regions. Reduced brain responses in the temporal posterior regions were also correlated with lower neurodevelopmental outcomes in both groups. This is the first study that reveals reduced brain functional responses in preschoolers with CHD during a receptive language task. It also suggests that the temporal posterior activation could be a potential brain marker of cognitive development. These findings provide support for the feasibility of identifying brain correlates of neurodevelopmental vulnerabilities in children with CHD.

Impact of Fontan Fenestration on Adverse Cardiovascular Outcomes: A Multicentre Study.

BACKGROUND: Fenestrating a Fontan baffle has been associated with improved perioperative outcomes in patients with univentricular hearts. However, longer-term potential adverse effects remain debated. We sought to assess the impact of a fenestrated Fontan baffle on adverse cardiovascular events including all-cause mortality, cardiac transplantation, atrial arrhythmias, and thromboemboli. METHODS: A multicentre North American retrospective cohort study was conducted on patients with total cavopulmonary connection Fontan baffle, with and without fenestration. All components of the composite outcome were independently adjudicated. Potential static and time-varying confounders were taken into consideration, along with competing risks. RESULTS: A total of 407 patients were followed for 10.4 (7.1-14.4) years; 70.0% had fenestration of their Fontan baffle. The fenestration spontaneously closed or was deliberately sealed in 79.9% of patients a median of 2.0 years after Fontan completion. In multivariable analysis in which a persistent fenestration was modelled as a time-dependent variable, an open fenestration did not confer a higher risk of the composite outcome (hazard ratio, 1.18; 95% confidence interval, 0.71-1.97; P = 0.521). In secondary analyses, an open fenestration was not significantly associated with components of the primary outcome: that is, mortality or transplantation, atrial arrhythmias, or thromboemboli. However, sensitivity analyses to assess the possible range of error resulting from imprecise dates for spontaneous fenestration closures could not rule out significant associations between an open fenestration and atrial arrhythmias or thromboemboli. CONCLUSIONS: In this multicentre study, no significant association was identified between an open fenestration in the Fontan baffle and major adverse cardiovascular events.

Determining Optimal Mean Arterial Blood Pressure Based on Cerebral Autoregulation in Children after Cardiac Surgery.

To evaluate the feasibility of continuous determination of the optimal mean arterial blood pressure (opt-MAP) according to cerebral autoregulation and to describe the opt-MAP, the autoregulation limits, and the time spent outside these limits in children within 48 h of cardiac surgery. Cerebral autoregulation was assessed using the correlation coefficient (COx) between cerebral oxygenation and MAP in children following cardiac surgery. Plots depicting the COx according to the MAP were used to determine the opt-MAP using weighted multiple time windows. For each patient, we estimated (1) the time spent with MAP outside the autoregulation limits and (2) the burden of deviation, defined as the area between the MAP curve and the autoregulation limits when the MAP was outside these limits. Fifty-one patients with a median age of 7.1 (IQR 0.7-52.0) months old were included. The opt-MAP was calculated for 94% (IQR 90-96) of the monitored time. The opt-MAP was significantly lower in neonates < 1 month old. The patients spent 24% (18-31) of the time outside of the autoregulation limits, with no significant differences between age groups. Continuous determination of the opt-MAP is feasible in children within the first 48 h following cardiac surgery.

A Parent-child yoga intervention for reducing attention deficits in children with congenital heart disease: the Yoga for Little Hearts Feasibility Study Protocol.

INTRODUCTION: Preschoolers and school-aged children with congenital heart disease (CHD) are at higher risk of attention deficit hyperactivity disorder (ADHD) compared with the general population. To this day, no randomised controlled trial (RCT) aiming to improve attention has been conducted in young children with CHD. There is emerging evidence indicating that parent-child yoga interventions improve attention and reduce ADHD symptoms in both typically developing and clinical populations. METHODS AND ANALYSIS: This is a single-blind, two-centre, two-arm trial during which 24 children with CHD and their parents will be randomly assigned to (1) a parent-child yoga intervention in addition to standard clinical care or (2) standard clinical care alone. All participants will undergo standardised assessments: (1) at baseline, (2) immediately post-treatment and (3) 6 months post-treatment. Descriptive statistics will be used to estimate the feasibility and neurodevelopmental outcomes. This feasibility study will evaluate: (1) recruitment capacity; (2) retention, drop-out and withdrawal rates during the yoga programme and at the 6-month follow-up; (3) adherence to the intervention; (4) acceptability of the randomisation process by families; (5) heterogeneity in the delivery of the intervention between instructors and use of home-based exercises between participants; (6) proportion of missing data in the neurodevelopmental assessments and (7) SD of primary outcomes of the full RCT in order to determine the future appropriate sample size. ETHICS AND DISSEMINATION: Ethical approval has been obtained by the Research Ethics Board of the Sainte-Justine University Hospital. The findings will be disseminated in peer-reviewed journals and conferences and presented to the Canadian paediatric grand round meetings. TRIAL REGISTRATION NUMBER: NCT05997680.

Effect of red blood cell storage time in pediatric cardiac surgery patients: A subgroup analysis of a randomized controlled trial.

Objective: This study aimed to determine whether or not transfusion of fresh red blood cells (RBCs) reduced the incidence of new or progressive multiple organ dysfunction syndrome compared with standard-issue RBCs in pediatric patients undergoing cardiac surgery. Methods: Preplanned secondary analysis of the Age of Blood in Children in Pediatric Intensive Care Unit study, an international randomized controlled trial. This study included children enrolled in the Age of Blood in Children in Pediatric Intensive Care Unit trial and admitted to a pediatric intensive care unit after cardiac surgery with cardiopulmonary bypass. Patients were randomized to receive either fresh (stored ≤7 days) or standard-issue RBCs. The primary outcome measure was new or progressive multiple organ dysfunction syndrome, measured up to 28 days postrandomization or at pediatric intensive care unit discharge, or death. Results: One hundred seventy-eight patients (median age, 0.6 years; interquartile range, 0.3-2.6 years) were included with 89 patients randomized to the fresh RBCs group (median length of storage, 5 days; interquartile range, 4-6 days) and 89 to the standard-issue RBCs group (median length of storage, 18 days; interquartile range, 13-22 days). There were no statistically significant differences in new or progressive multiple organ dysfunction syndrome between fresh (43 out of 89 [48.3%]) and standard-issue RBCs groups (38 out of 88 [43.2%]), with a relative risk of 1.12 (95% CI, 0.81 to 1.54; P = .49) and an unadjusted absolute risk difference of 5.1% (95% CI, -9.5% to 19.8%; P = .49). Conclusions: In neonates and children undergoing cardiac surgery with cardiopulmonary bypass, the use of fresh RBCs did not reduce the incidence of new or progressive multiple organ dysfunction syndrome compared with the standard-issue RBCs. A larger trial is needed to confirm these results.

Relationship between 4-month functional brain network topology and 24-month neurodevelopmental outcome in children with congenital heart disease.

Survivors of complex forms of congenital heart disease (CHD)∗ are at high risk of neurodevelopmental disabilities. Neuroimaging studies have pointed to brain anomalies and immature networks in infants with CHD, yet less is known about their functional network topology and associations with neurodevelopment. To characterize the functional network topology in 4-month-old infants with repaired CHD, we compared graph theory metrics measured using resting-state functional near-infrared spectroscopy (rs-fNIRS) between infants with CHD (n = 22) and healthy controls (n = 30). We also investigated the moderating effect of graph theory metrics on the relationship between group (CHD vs. Controls) and developmental outcomes at 24 months. At 4 months, both groups presented similar functional brain network topology. At 24 months, children with CHD had lower scores on the language scale and the expressive communication subscale of the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III), as well as lower scores on the Grammatical Form scale of the MacArthur-Bates Communicative Development Inventory (MBCDI). The relationship between group and expressive language was moderated by the normalized characteristic path length (λ) and the degree (k). Although infants with CHD have functional brain topology similar to that of healthy controls, our findings suggest that they do not benefit from an optimal functional brain organization in comparison with healthy infants.

Successful Pediatric Heart Transplantation from a Deceased Donor with SARS-Cov-2 Infection: A Case Report.

We report the case of a 3-year-old who was highly sensitized and received cardiac transplantation from a donor in the acute phase of SARS-CoV-2 infection. Despite maximal immunosuppression owing to a positive cross-match and desensitization protocol, the patient's course was favorable.

Hemodynamic assessment in children after cardiac surgery: A pilot study on the value of infrared thermography.

Introduction: Low cardiac output syndrome in the postoperative period after cardiac surgery leads to an increase in tissue oxygen extraction, assessed by the oxygen extraction ratio. Measurement of the oxygen extraction ratio requires blood gases to be taken. However, the temperature of the skin and various parts of the body is a direct result of blood flow distribution and can be monitored using infrared thermography. Thus, we conducted a prospective clinical study to evaluate the correlation between the thermal gradient obtained by infrared thermography and the oxygen extraction ratio in children at risk for low cardiac output after cardiac surgery. Methods: Children aged 0 to 18 years, having undergone cardiac surgery with cardio-pulmonary bypass in a pediatric intensive care unit were included in the study. One to 4 thermal photos were taken per patient using the FLIR One Pro thermal imaging camera. The thermal gradient between the central temperature of the inner canthus of the eye and the peripheral temperature was compared to the concomitant oxygen extraction ratio calculated from blood gases. Results: 41 patients were included with a median age of 6 months (IQR 3-48) with median Risk Adjustment for Congenital Heart Surgery-1 score was 2 (IQR 2-3). Eighty nine thermal photos were analyzed. The median thermal gradient was 2.5 °C (IQR 1,01-4.04). The median oxygen extraction ratio was 35% (IQR 26-42). Nine patients had an oxygen extraction ratio ≥ 50%. A significant but weak correlation was found between the thermal gradient and the oxygen extraction ratio (Spearman's test p = 0.25, p = 0.016). Thermal gradient was not correlated with any other clinical or biologic markers of low cardiac output. Only young age was an independent factor associated with an increase in the thermal gradient. Conclusion: In this pilot study, which included mainly children without severe cardiac output decrease, a significant but weak correlation between thermal gradient by infrared thermography and oxygen extraction ratio after pediatric cardiac surgery was observed. Infrared thermography is a promising non-invasive technology that could be included in multimodal monitoring of postoperative cardiac surgery patients. However, a clinical trial including more severe children is needed.

Generation of functional human T cell development in NOD/SCID/IL2rγnull humanized mice without using fetal tissue: Application as a model of HIV infection and persistence.

Humanization of mice with functional T cells currently relies on co-implantation of hematopoietic stem cells from fetal liver and autologous fetal thymic tissue (so-called BLT mouse model). Here, we show that NOD/SCID/IL2rγnull mice humanized with cord blood- derived CD34+ cells and implanted with allogeneic pediatric thymic tissues excised during cardiac surgeries (CCST) represent an alternative to BLT mice. CCST mice displayed a strong immune reconstitution, with functional T cells originating from CD34+ progenitor cells. They were equally susceptible to mucosal or intraperitoneal HIV infection and had significantly higher HIV-specific T cell responses. Antiretroviral therapy (ART) robustly suppressed viremia and reduced the frequencies of cells carrying integrated HIV DNA. As in BLT mice, we observed a complete viral rebound following ART interruption, suggesting the presence of HIV reservoirs. In conclusion, CCST mice represent a practical alternative to BLT mice, broadening the use of humanized mice for research.

Functional brain connectivity after corrective cardiac surgery for critical congenital heart disease: a preliminary near-infrared spectroscopy (NIRS) report.

Patients with congenital heart disease (CHD) requiring cardiac surgery in infancy are at high risk for neurodevelopmental impairments. Neonatal imaging studies have reported disruptions of brain functional organization before surgery. Yet, the extent to which functional network alterations are present after cardiac repair remains unexplored. This preliminary study aimed at investigating cortical functional connectivity in 4-month-old infants with repaired CHD, using resting-state functional near-infrared spectroscopy (fNIRS). After fNIRS signal frequency decomposition, we compared values of magnitude-squared coherence as a measure of connectivity strength, between 21 infants with corrected CHD and 31 healthy controls. We identified a subset of connections with differences between groups at an uncorrected statistical level of p < .05 while controlling for sex and maternal socioeconomic status, with most of these connections showing reduced connectivity in infants with CHD. Although none of these differences reach statistical significance after FDR correction, likely due to the small sample size, moderate to large effect sizes were found for group-differences. If replicated, these results would therefore suggest preliminary evidence that alterations of brain functional connectivity are present in the months after cardiac surgery. Additional studies involving larger sample size are needed to replicate our data, and comparisons between pre- and postoperative findings would allow to further delineate alterations of functional brain connectivity in this population.

Social cognition and competence in preschoolers with congenital heart disease.

OBJECTIVE: Children born with congenital heart disease (CHD) are at an increased risk for various neurodevelopmental impairments. However, little is known regarding social outcomes associated with CHD, particularly during early childhood. The present study aimed to characterize the sociocognitive profile and to assess the contribution of language, executive functions (EF), and social cognition to social competence (SC) in preschoolers with CHD. METHOD: Five-year-old children with CHD (n = 55) completed a standardized neuropsychological assessment. Performance on sociocognitive skills was compared to test norms using one-sample t tests. Hierarchical regression was conducted to examine the associations between language skills, affect recognition (AR), theory of mind (ToM), EF (performance-based and parent-rated), and social competence. RESULTS: Children with CHD performed significantly worse than norms in language and ToM, whereas EF and social competence appeared generally preserved in our sample. In hierarchical regression analysis, cognitive functions (language score, AR, ToM, EF performance) accounted for a significant 24.3% of the variance. Parent-rated EF added another 24.8% to the total explained variance. CONCLUSIONS: These findings provide new evidence for understanding social cognition and competence among preschoolers with CHD, showing vulnerability in social cognition and language skills but not in social competence more generally. The model suggests a combined contribution of social cognition, language, and EF on social outcomes. Remedial programs addressing these intervention targets could be useful in promoting social development in this vulnerable population. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Canadian Cardiovascular Society 2022 Guidelines for Cardiovascular Interventions in Adults With Congenital Heart Disease.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

The Fontan Circulation: From Ideal to Failing Hemodynamics and Drug Therapies for Optimization.

Fontan palliation results in a hemodynamically complex circulation with multisystem consequences, which in the long term adversely affect many body processes. Systemic venous hypertension, nonpulsatile low-shear pulmonary blood flow, and low cardiac output are the 3 main characteristics of a Fontan circulation, leading to unavoidable slowly progressive failure. An appreciation of how the hemodynamics of a Fontan circulation change with time and relate to the various modes of Fontan circulatory failure is important. Accurate hemodynamic assessment aid this understanding and may permit early identification of potentially treatable drivers of decline. While no evidence-based or guideline-directed pharmacologic management strategy has been established in Fontan patients, understanding the hemodynamics of Fontan circulation failure will assist in the rational selection of potentially helpful drug therapies for individual patients. In this review, we present hemodynamic concepts of the optimal Fontan physiology and Fontan circulatory failure, review practical aspects of invasive hemodynamic assessment, and discuss the role of drug therapies in increasing systemic venous blood flow return and decreasing ventricular filling pressures in Fontan circulation. Often complementary to catheter-based or surgical interventions, pharmacologic management aims at preserving patency of the circuit, adequate systolic and diastolic ventricular function, atrioventricular valve function, an unobstructed ventricular outflow tract, and pulmonary vascular integrity in order to maintain an acceptable cardiac output.

Autograft remodeling after the Ross procedure by cardiovascular magnetic resonance imaging: Aortic stenosis versus insufficiency.

BACKGROUND: Studies suggest that patients undergoing the Ross procedure for aortic insufficiency are at greater risk of autograft dilatation than those with aortic stenosis. By using a tailored Ross technique to mitigate autograft dilatation in patients with aortic insufficiency, we aimed to compare the biomechanical and morphologic remodeling of the autograft at 1 year between patients with aortic insufficiency and patients with aortic stenosis. METHODS: A total of 210 patients underwent a Ross procedure (2011-2016). Of those, 86 patients (mean age 43 ± 13 years; 32% were female) completed preoperative and postoperative cardiovascular magnetic resonance imaging. A total of 71 studies were suitable for analysis: 41 patients with aortic stenosis and 30 patients with aortic insufficiency. Nine healthy adults were used as controls. Autograft root dimensions, individual sinus volumes, and distensibility were measured using cardiovascular magnetic resonance. RESULTS: At 1 year, there was no difference in autograft root dimensions between patients with aortic stenosis (mean annulus 25.1 ± 3.1 mm and sinus diameters 35 ± 4.1 mm) and patients with aortic insufficiency (26.6 ± 3 mm and 37.1 ± 3.5 mm; P = .12 and .06, respectively). Relative sinus of Valsalva volumes were symmetrical in the aortic stenosis (right 34.8% ± 4%, left 33.7% ± 3.5%, noncoronary 31.4% ± 3.2%) and aortic insufficiency groups (34.8% ± 3.9%, 33.8% ± 2.8%, 31.3% ± 3.7%, P = .85, .92, and .82), and similar to those of healthy adults. Aortic root distensibility was reduced in both groups compared with healthy adults (P = .003), but was similar between aortic stenosis (3.12 ± 1.58 × 10-3 mm Hg-1) and aortic insufficiency (3.04 ± 1.15 × 10-3 mm Hg-1; P = .9). CONCLUSIONS: Using a tailored technique, there were no differences in the morphologic or biomechanical remodeling of the autograft root 1 year after the Ross procedure between patients with aortic stenosis and patients with aortic insufficiency. However, autograft roots are stiffer than native aortic roots.

Utility of the Ages and Stages Questionnaires 3rd Edition for Developmental Screening in Children with Surgically Repaired Congenital Heart Disease.

Aim: This study sought to evaluate the accuracy of the Ages and Stages Questionnaires 3rd Edition (ASQ-3) in identifying developmental delay (DD) in children with congenital heart disease (CHD) born at term who underwent surgical repair.Methods: Participants had to complete ASQ-3 and Bayley Scales of Infant and Toddler Development 3rd Edition (BSID-III) at 12 and 24 months. A child was considered at risk of DD for a ASQ-3 domain when he scored below the cutoff (≤-1SD or ≤-2SD). A child had a DD in a BSID-III domain when the score was ≤-1SD. The validity for each ASQ-3 domain and for overall ASQ-3 was measured.Results: At 12 months (n = 64), overall ASQ-3 (≤-2SD) sensitivity was 88%, specificity 74%. At 24 months (n = 82), overall ASQ-3 (≤-2SD) sensitivity was 74%, specificity 88%.Conclusion: The results support the utility of the ASQ-3 for screening the overall risk of DD in children with CHD.

The role of parenting stress in anxiety and sleep outcomes in toddlers with congenital heart disease.

Objectives: This retrospective cohort study investigates how parenting stress, measured at 4 months of age by use of a classic three-dimensional parent-reported scale (Parenting Stress Index, 4th Ed. or PSI-4), can predict anxiety symptoms and quality of sleep at 24 months in toddlers with congenital heart disease (CHD). Study Design: Sixty-six toddlers with CHD followed at our cardiac neurodevelopmental follow-up clinic were included in this study. As part of their systematic developmental assessment program, parents completed questionnaires on their stress level (PSI-4) when their child was 4 months old, and on their child's anxiety symptoms and quality of sleep at 24 months. Eight multiple linear regression models were built on the two measures collected at 24 months using the PSI-4 scores collected at 4 months. For each measure, four models were built from the PSI-4 total score and its three subscales (Parental Distress, Parent-Child Dysfunctional Interaction, Difficult Child), controlling for sex and socioeconomic status. Results: The PSI-4 Difficult Child subscale, which focuses on parenting anxiety related to the child's behavioral problems and poor psychosocial adjustment, accounted for 17% of the child's anxiety symptoms at 24 months. The two other PSI-4 subscales (Parental Distress and Parent-Child Dysfunctional Interaction) and the PSI-4 total score did not contribute significantly to the models. None of the four regression models on perceived quality of sleep were significant. It is important to note that 33% of parents responded defensively to the PSI-4. Conclusions: Parenting stress related to the child's behavioral problems and poor psychosocial adjustment, measured when the child is 4 months old, is associated with the child's ulterior anxiety symptoms. As very few standardized tools are available to assess the behavioral and psychoaffective development of infants, this study highlights the importance of early psychosocial screening in parents of infants with CHD. The high rate of significant Defensive Responding Indices reminds us to not take parent reports at face value, as their actual stress levels might be higher.

Ross operation after failure of aortic valve repair.

BACKGROUND: Repair failure remains one of the most important complications of aortic valve reconstruction. Young patients might benefit from a Ross procedure in such a scenario, provided it can be performed safely and with adequate durability. The aim of this study was to assess the safety and clinical outcomes of a Ross operation following a failed repair. METHODS: Between 1996 and 2019, 80 patients (male, 76%; mean age, 31±13 years) underwent a Ross procedure after a median of 6.6 (1.7-15.9) years following an initial aortic valve repair. The previous valve repair was performed for unicuspid (53%), bicuspid (39%), tricuspid (7%), and quadricuspid morphology (1%). Median follow-up after the Ross operation was 2.8 (0.964-13.25) years, mean 5±5 years (92% complete). RESULTS: Median cardiopulmonary bypass and cross-clamp times were 144 [106-154] minutes and 98 [79-113] minutes, respectively. Thirty-two patients (40%) required a concomitant procedure, most commonly, an ascending aortic replacement (n=23). There were no peri-operative deaths, myocardial infarctions, or neurological complications. There was one late death from a non-cardiac cause. At 10 years, overall survival was 99%±1%, similar to that of an age- and gender-matched population. Nine patients required re-intervention after their Ross procedure (five on the autograft and four on the pulmonary conduit). The autograft re-interventions were valve-sparing procedures in all patients. The cumulative incidence of re-intervention on the autograft at 8 years was 5.1%±3.1%. CONCLUSIONS: The stepwise strategy of an initial valve repair followed by Ross operation represents a safe and valid option for failed aortic valve repair. It is associated with low peri-operative morbidity. Mid-term survival is excellent, similar to that of a matched general population. The probability of re-intervention after the Ross procedure appears similar to that of a primary Ross operation, deeming it a warranted consideration in cases of failed aortic valve repair.