Yves A. D'udekem

Background: Patients with functional single ventricle (SV) are at risk for adverse outcomes after staged palliation from the superior cavopulmonary connection (SCPC) to the Fontan. Current pre-Fontan assessment by cardiac magnetic resonance and cardiac catheterization includes measuring atrioventricular valve regurgitation, aortopulmonary collateral burden, and pressures. Four-dimensional flow can quantify complex flows representing hemodynamic inefficiency. This study determined the clinical significance of kinetic energy (KE) and viscous energy loss in patients before the Fontan procedure using 4-dimensional flow. Results: This was a retrospective analysis of patients before the Fontan procedure who underwent ferumoxytol-enhanced cardiac magnetic resonance and same-day catheterization. Four-dimensional flow data sets were analyzed using ITFlow (CardioFlowDesign) to measure KE/viscous energy loss in the atrium, SV, and SCPC. A composite outcome was defined by rejected Fontan candidacy, prolonged hospitalization, lymphatic dysfunction, or heart failure. The relationship between these outcomes and KE/viscous energy loss was assessed by bivariable and multivariable logistic regression analyses as appropriate. Sixty-five patients (3.9±1.5 years, 0.64±0.1 m2) were included. Fifty (77%) proceeded to Fontan operation with median hospitalization time of 8.5 (interquartile range, 7-12.7) days. Twenty-six (40%) experienced a composite outcome, including 9 with rejected candidacy. Lower SCPC flow was associated with an outcome (P=0.042). Meanwhile, higher SV KE and lower SCPC KE were independently associated with composite outcome (odds ratio, 3.63 [95% CI, 1.32-13.2]; P=0.0263; odds ratio, 0.906 [95% CI, 0.814-0.980]; P=0.0377). Higher SV KE and lower SCPC KE corresponded to significant atrioventricular valve regurgitation, higher aortopulmonary collateral burden, and higher cathetherization pressures. Conclusions: Four-dimensional flow analysis provides insight into SV hemodynamics and is associated with short-term outcomes. Future work will analyze the longitudinal implications for patients undergoing the Fontan procedure.

Objectives: To determine if a priori standardization of outcome hemostatic definitions alone was adequate to enable useful comparison between two cohorts of pediatric extracorporeal membrane oxygenation (ECMO) patients, managed according to local practice and protocol. Design: Comparison of two separate prospective cohort studies performed at different centers with standardized outcome definitions agreed upon a priori. Setting: General and cardiac PICUs at the Royal Children's Hospital (RCH) in Melbourne, Australia, and the Sophia Children's Hospital (SCH) in Rotterdam, The Netherlands. Patients: Children (0-18 yr old) undergoing ECMO. Interventions: None. Measurements and main Results: Although outcome definitions were standardized a priori, the interpretation of surgical interventions varied. The SCH study included 47 ECMO runs (September 2019 to April 2023), and the RCH study included 97 ECMO runs (September 2016 to Jan 2022). Significant differences in patient populations were noted. RCH patients biased toward frequent cardiac ECMO indications, central cannulation, and cardiopulmonary bypass before ECMO. The frequency of outcome ascertainment was not standardized. Conclusions: This international comparison shows that standardizing hemostatic outcome definitions alone is insufficient for sensible comparison. Uniform interpretation of definitions, consistent frequency of outcome ascertainment, and stratification based on patient populations and ECMO practices are required. Our results highlight the granularity of detail needed for cross-center comparison of hemostatic outcomes in pediatric ECMO. Further work is needed as we move toward potential multicenter trials of pediatric ECMO.

Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals are a challenging congenital anomaly to manage surgically with different centers and strategies producing wide-ranging outcomes. Over the past few decades despite diverging treatment pathways, there is an emerging consensus of how these patients "should" be treated. Quite often a combination of rehabilitation strategy and a unifocalization approach has to be tailored to each patient to address the anatomic and physiological variations that characterize this congenital heart defect. Irrespective of the surgical approach, the goal should be to have a complete repair with acceptable right heart pressure ensuring survival and a good quality of life.

Children with congenital heart defects who undergo cardiopulmonary bypass (CPB) surgery are at risk for delayed or impaired neurodevelopmental outcomes. Nitric oxide (NO) added to the CPB oxygenator may reduce systemic inflammation due to CPB and improve recovery from surgery, including improved neurodevelopmental outcomes. To investigate neurodevelopment, health-related quality of life (HRQOL), and factors associated with impaired neurodevelopment at 12 months post surgery in infants who received CPB with NO or standard CPB. This double-masked randomized clinical trial was conducted in 6 centers in Australia, New Zealand, and the Netherlands between July 19, 2017, and April 28, 2021, with a preplanned prospective follow-up 12 months postrandomization completed on August 5, 2022. The cohort included 1364 infants younger than 2 years who underwent open heart surgery with CPB for congenital heart disease. The intervention group received NO 20 ppm into the CPB oxygenator. The control group received standard CPB. The primary outcome was neurodevelopment, defined as the Ages and Stages Questionnaire, Third Edition (ASQ-3) total score. Secondary outcomes were HRQOL and functional status as measured by Pediatric Quality of Life Inventory and modified Pediatric Overall Performance Category scores, respectively. Sensitivity analyses modeled the outcome for patients lost to follow-up. Of 1318 infants alive 12 months after randomization, follow-up was performed in 927, with 462 patients in the NO group and 465 in the standard care group (median [IQR] age at follow-up, 16.6 [13.7-19.8] months; median [IQR] time since randomization, 12.7 [12.1-13.9] months; 516 male [55.7%]). There were no differences between the NO and standard care groups in ASQ-3 total score (mean [SD], 196.6 [75.4] vs 198.7 [73.8], respectively; adjusted mean difference, -2.24; 95% CI, -11.84 to 7.36). There were no differences in secondary outcomes. Prematurity (gestational age <37 weeks), univentricular lesions, congenital syndromes, and longer intensive care unit length of stay were associated with lower ASQ-3 total scores in adjusted multivariable analyses. In this randomized clinical trial of infants with congenital heart disease, NO administered via the CPB oxygenator did not improve neurodevelopmental outcomes or HRQOL 12 months after open heart surgery. Further research should explore homogenous cohorts with higher surgical risk and higher-dose or alternative therapies. ANZCTR Identifier: ACTRN12617000821392.

Objective: To examine global and health-related quality of life (QOL) among parents of individuals with Fontan physiology and determine associations with sociodemographic, parent and child-related health, psychological, and relational factors. Methods: Parents participating in the Australian and New Zealand Fontan Registry (ANZFR) QOL Study (N = 151, Parent Mean age = 47.9 ± 10.2 years, age range: 31.6-79.6 years, 66% women; child Mean age = 16.3 ± 8.8, age range: 6.9-48.7 years, 40% female) completed a series of validated measures. Health-related QOL was assessed using the PedsQL 4.0 Core Generic Scales for adults and global QOL was assessed using a visual analogue scale (0-10). Results: Most parents (81%) reported good global QOL (≥ 6), consistent with broader population trends. Nearly one-third of parents (28%) reported at-risk health-related QOL (based on total PedsQL scores) with physical functioning most affected (44%). Psychological factors, including psychological stress and sense of coherence, emerged as the strongest correlates of global and health-related QOL, explaining an additional 16 to 30% of the variance (using marginal R2). Final models explained 35 and 57% and of the variance in global and health-related QOL, respectively (marginal R2). Relational factors, including perceived social support and family functioning contributed minimally when analyzed alongside psychological variables. Conclusions: While parents of individuals with Fontan physiology report good global QOL, challenges in health-related QOL exist. We identified key psychological, sociodemographic, and health-related factors associated with parental QOL outcomes. These data may aid early identification of physical and psychosocial difficulties and guide targeted health resource allocation for this population.

Tetralogy of Fallot for Life