UNASSIGNED: For infants with single ventricle heart disease, the time after stage 2 procedure (S2P) is believed to be a lower risk period compared with the interstage period; however, significant morbidity and mortality still occur.
UNASSIGNED: This study aimed to identify risk factors for mortality or transplantation referral between S2P surgery and the first birthday.
UNASSIGNED: Retrospective cohort analysis of infants in the National Pediatric Cardiology Quality Improvement Collaborative who underwent staged single ventricle palliation from 2016 to 2022 and survived to S2P. Multivariable logistic regression and classification and regression trees were performed to identify risk factors for mortality and transplantation referral after S2P.
UNASSIGNED: Of the 1,455 patients in the cohort who survived to S2P, 5.2% died and 2.3% were referred for transplant. Overall event rates at 30 and 100 days after S2P were 2% and 5%, respectively. Independent risk factors for mortality and transplantation referral included the presence of a known genetic syndrome, shunt type at stage 1 procedure (S1P), tricuspid valve repair at S1P, longer time to extubation and reintubation after S1P, ≥ moderate tricuspid regurgitation prior to S2P, younger age at S2P, and the risk groups identified in the classification and regression tree analysis (extracorporeal membrane oxygenation after S1P and longer S2P cardiopulmonary bypass time without extracorporeal membrane oxygenation).
UNASSIGNED: Mortality and transplantation referral rates after S2P to 1 year of age remain high ∼7%. Many of the identified risk factors after S2P are similar to those established for interstage factors around the S1P, whereas others may be unique to the period after S2P.

Despite improvement in hemodynamics, children with single ventricle heart disease remain on feeding tubes long after stage 2 palliation (S2P). Use of a hunger provocation method in a multidisciplinary team setting has been successful at weaning these children from feeding tubes. The objective of this study is to describe patient characteristics and outcomes in the single ventricle population who underwent a formal tube weaning process using a standardized hunger provocation method. Single ventricle patients after S2P from six centers were included. Patient data collected included baseline demographics, swallow evaluation results, and feeding characteristics such as percent oral intake at the start of tube wean. Tube wean data included tube weaning process and duration, interruptions to the tube wean, adverse events, and weights before, during, and after the tube wean. 94% (60 of 64) of patients achieved oral independence. The median time to tube wean was 12.5 days. 62% of patients had transient weight loss during the tube wean. 61% of the cohort was taking less than 10% goal volumes by mouth with 90% of those patients successfully tube weaned. All patients with history of aspiration were successfully tube weaned. 75% of successfully weaned patients were above baseline weight at 1-month post-tube wean. The most common cause of tube wean interruption was contraction of a viral illness. Use of a standardized tube weaning process via hunger provocation method is both feasible and safe in the single ventricle population, resulting in successful feeding tube removal in a timely manner with minimal adverse effects.

UNASSIGNED: Infants with single ventricle heart disease and severe atrioventricular valve regurgitation have poor outcomes following conventional staged palliation. As such, ventricular assist device (VAD) placement along with hybrid stage 1 palliation has been proposed as a bridge to heart transplant. We present a novel surgical technique for VAD implantation concurrent with hybrid stage 1 that avoids cardiopulmonary bypass.
UNASSIGNED: We performed a retrospective review of our institutional experience with this novel surgical technique.
UNASSIGNED: Three patients (weight, 2.7-3.5 kg; age, 3 to 5 days) underwent hybrid stage 1 with VAD placement, consisting of bilateral 3.5-mm expandable polytetrafluoroethylene (PTFE) pulmonary artery bands, a ductal stent, a 6-mm Berlin Heart outflow cannula onto the main pulmonary trunk with a 10-mm graft, a 6-mm Berlin Heart outflow cannula onto the right atrium, and a 10-mL Berlin Heart pump. In patients with severe aortic arch hypoplasia or coarctation, a 4-mm PTFE graft was sewn from the VAD outflow graft to the innominate artery to protect coronary and cerebral perfusion. Procedures were performed off bypass with minimal blood product use. Patients were extubated on postoperative days 2, 2, and 5. There were no procedural complications. All patients were transferred out of the intensive care unit and demonstrated appropriate weight gain. Anticoagulation strategy was bivalirudin and antiplatelet therapy. The patients underwent transplantation after 149 days, 157 days, and 288 days of support.
UNASSIGNED: Off-pump single ventricle VAD placement is technically feasible and can be done at the time of hybrid stage 1 palliation with minimal operative morbidity as a bridge to transplant.

OBJECTIVE: Fetuses with single ventricle physiology (SVP) exhibit reductions in fetal cerebral oxygenation with associated delays in fetal brain growth and neurodevelopmental outcomes. Maternal supplemental oxygen (MSO) has been proposed to improve fetal brain growth but current evidence on dosing, candidacy, and outcomes are limited. In this pilot study, we evaluated the safety and feasibility of continuous low-dose MSO in the setting of SVP.
METHODS: This single-centre, open-label, pilot phase 1 safety and feasibility clinical trial included 25 pregnant individuals with a fetal diagnosis of SVP. Participants self-administered continuous supplemental oxygen using medical-grade oxygen concentrators for up to 24 hours per day from the second half of gestation until delivery. The primary aim was the evaluation of the safety profile and feasibility of MSO. A secondary preliminary analysis was performed to assess the impact of MSO on the fetal circulation by echocardiography and late-gestational cardiovascular magnetic resonance, early outcomes including brain growth and pre-operative brain injury, and 18-month neurodevelopmental outcomes by the Bayley Scales of Infant and Toddler Development 3rd Edition compared to a contemporary fetal SVP cohort that received standard of care (SOC).
RESULTS: Among 25 participants, the average maternal age at conception was 35 years, and fetal SVP diagnoses included 16 right ventricle dominant, 8 left ventricle dominant, and 1 indeterminant ventricular morphology. Participants started the trial at approximately 29.3 gestational weeks and took MSO for a median 16.1 hours per day for 63 days, cumulating a median 1029 hours of oxygen intake from enrollment until delivery. The only treatment-associated adverse events were nasal complications that were typically resolved by attaching a humidifier unit to the oxygen concentrator. No premature closure of the ductus arteriosus or unexpected fetal demise was observed. In the secondary analysis, MSO was not associated with any changes in fetal growth, middle cerebral artery pulsatility index, cerebroplacental ratio, nor head circumference to abdominal circumference ratio Z-scores over gestation compared to SOC. Although MSO was associated with changes in umbilical artery pulsatility index Z-score over gestation compared to SOC (p=0.02), this was likely due to initial baseline differences in placental resistance. At late-gestational cardiovascular magnetic resonance, MSO was not associated with any significant increase in umbilical vein oxygen saturation, fetal oxygen delivery, or fetal cerebral oxygen delivery. Similarly, we observed no differences in newborn outcomes including brain volume and pre-operative brain injury, nor mortality by 18 months of age, nor neurodevelopmental outcomes at 18 months of age.
CONCLUSIONS: This pilot phase 1 clinical trial indicates low-dose maternal supplemental oxygen therapy is safe and well tolerated in pregnancies diagnosed with fetal SVP. However, our protocol was not associated with any significant changes in fetal circulatory physiology or improvements in early neurologic or neurodevelopmental outcomes. This article is protected by copyright. All rights reserved.

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Ensuring the quality of interstage management of infants with single ventricle heart disease (SVHD) residing in rural communities is difficult. Tailored care coordination through parental discharge education, formal and informal care team and family communication, adequate access to healthcare, and informed provider handoffs are crucial to the infant\'s well-being and survival.
To discuss the state of the science related to care coordination factors and infant wellbeing during the interstage period.
An integrative review approach to synthesize findings across studies was used. Through constant comparative analysis, all articles were read and coded, broken down into \"data bits\" or key phrases.
Four major themes were inductively derived: 1) education and confidence-building, 2) communication for building relationships, 3) social work and related mental health support, and 4) availability of resources.
Despite advances in cardiac surgery and related interventions, a clear gap exists regarding care coordination factors and infant well-being, especially in rural communities.

Survivors of palliative surgery for single ventricle heart disease (SVHD) are at risk of poor neurodevelopmental outcomes and reduced exercise capacity. In healthy populations, reduced exercise capacity is related to decreased cognition suggesting a possible relationship between exercise capacity and neurodevelopment. Using cardiopulmonary exercise testing (CPET) and neuropsychological testing (NPT) as indicators of exercise capacity and neurodevelopment, respectively, we hypothesized that in SVHD, higher CPET measures are related to better NPT performance. Patients were retrospectively identified. CPET variables included VO2max, anaerobic threshold, peak heart rate, ventilatory efficiency, and respiratory exchange ratio. NPT instruments were divided into domains measuring attention, executive functioning, adaptive functioning, and emotional functioning. Linear regression was used to test for associations between CPET and NPT. 23 subjects with SVHD met inclusion criteria. On both CPET and NPT, the cohort scored worse than healthy, age-matched subjects. Higher VO2max and anaerobic threshold were associated with better parent-rated overall adaptive functioning (p = 0.01 and p = 0.02, respectively). Higher peak heart rate was related to better sustained visual attention (p = 0.01). In SVHD, CPET measures indicating better exercise capacity were positively associated with a subset of scores on NPT. Larger, multisite studies implementing cardiorespiratory fitness intervention and incorporating cognitive outcome measures will be needed to better characterize the relationship between neurodevelopment and functional capacity in this population. Results may assist in providing anticipatory guidance and optimizing post-Fontan developmental trajectories.

OBJECTIVE: Single ventricle heart disease (SVHD) patients show injury in brain sites that regulate autonomic, mood, and cognitive functions. However, the nature (acute or chronic changes) and extent of brain injury in SVHD are unclear. Our aim was to examine regional brain tissue damage in SVHD over controls using DTI-based mean diffusivity (MD), axial diffusivity (AD), radial diffusivity (RD), and fractional anisotropy (FA) procedures.
METHODS: We collected two DTI series (3.0-T MRI), mood and cognitive data, from 27 SVHD and 35 control adolescents. Whole-brain MD, AD, RD, and FA maps were calculated from each series, realigned and averaged, normalized to a common space, smoothed, and compared between groups using ANCOVA (covariates, age and sex; false discovery rate, p < 0.05). Region-of-interest analyses were performed to calculate MD, AD, RD, and FA values for magnitude assessment between groups.
RESULTS: SVHD patients showed impaired mood and cognitive functions over healthy adolescents. Multiple brain sites in SVHD showed increased MD values, including the insula, caudate, cingulate, hypothalamus, thalamus, medial prefrontal and frontal cortices, parahippocampal gyrus, hippocampus, precentral gyrus, amygdala, cerebellum, corpus callosum, basal forebrain, mammillary bodies, internal capsule, midbrain, fornix, and occipital, parietal, and temporal cortices, indicating chronic tissue changes. Similar areas showed either increased AD or RD values, with RD changes more enhanced over AD in SVHD compared to controls. Few brain regions emerged with increased or decreased FA values in SVHD patients over controls.
CONCLUSIONS: SVHD adolescents, more than a decade from their last surgical procedure, show widespread brain abnormalities in autonomic, mood, and cognitive regulatory areas. These findings indicate that brain injury is in a chronic stage in SVHD with predominantly myelin changes that may result from previous hypoxia/ischemia- or developmental-induced processes.

To our knowledge, successful breastfeeding in the population with single ventricle congenital heart disease has not been reported in the literature, particularly during the interstage period.
A retrospective case study including inpatient nutrition and a complete history of daily logs with the home surveillance monitoring program was performed.
Successful full breastfeeding (exceeding prescribed weight growth goals) after Stage I surgery was achieved during the interstage period. The infant was discharged at 3.41 kg, not consistently breastfeeding, and progressed to 7.05 kg at 5 months of age, fully breastfeeding.
Supporting breastfeeding for infants who have undergone repairs for single ventricle anatomy can be challenging but can be accomplished. It requires a concerted team effort, clear communication, and collaboration among caregivers, the mother, and her supporters.