Patients with hypoplastic left heart syndrome who develop a restrictive atrial septum prenatally have higher rates of postnatal mortality compared to those without atrial restriction. It is not understood whether the gestational age at which restriction becomes apparent echocardiographically impacts clinical outcomes.

A single-centre, retrospective cohort study was performed, including 196 patients with hypoplastic left heart syndrome from 2008 to 2022. A restrictive atrial septum was diagnosed based on fetal echocardiographic findings of an intact atrial septum, antegrade:retrograde pulmonary vein Doppler velocity time integral ratio <3, and/or the presence of a decompressing vein. Patients were grouped as follows: 1. Without Restriction, 2. Early Restriction (<30 weeks’ gestation), and 3. Late Restriction (≥30 weeks’ gestation), based on the gestational age at which diagnostic criteria for restriction were met. Comparative postnatal overall survival and transplant-free survival analyses were performed using the Kaplan–Meier method.

Overall mortality rates were higher in the Early group (62%, n = 13) compared to the Late group (29%, n = 7) and the group without atrial restriction (40%). Similarly, transplant rates were higher in the Early group (23%) compared to the Late group (14%) and those without restriction (14%). Overall survival and transplant-free survival were not statistically different among the groups.

While not statistically significant in this cohort with a small number of patients with atrial restriction, our data suggest that patients with hypoplastic left heart syndrome who meet diagnostic criteria for atrial restriction later in gestation may have outcomes more similar to those without atrial restriction.

Fenestration in the Fontan procedure was introduced to improve early postoperative outcomes by reducing systemic venous pressure and augmenting preload to the systemic ventricle. It is believed to decrease pleural drainage, shorten hospital stay, and reduce complications. However, fenestration may result in systemic desaturation and carries a potential risk of paradoxical thromboembolism. The benefits of routine fenestration remain controversial. This study aimed to evaluate whether adding a fenestration influences early postoperative outcomes.

Between March 2024 and November 2025, 52 patients underwent Fontan completion at our institution. Forty patients met the inclusion criteria and were prospectively divided into two equal groups: non-fenestrated (Group 1, n = 20) and fenestrated (Group 2, n = 20). Twelve patients were excluded due to a primary Fontan procedure, elevated pulmonary vascular resistance, increased transpulmonary gradient, or significant branch pulmonary artery distortion. Demographic and perioperative parameters, including ventilation duration, inotrope duration, chest drain duration and volume, ICU stay, and total hospital stay, were analysed.

Baseline demographic and intraoperative variables were comparable between groups. Duration of mechanical ventilation, ICU stay, total chest drainage, and cardiopulmonary bypass time were similar. Chest drain duration was shorter in the fenestrated group (8.1 ± 4.2 vs. 10.2 ± 5.3 days), but this difference was not statistically significant (p = 0.173). Discharge oxygen saturation was significantly lower in the fenestrated group (88.9 ± 5.6% vs. 93.3 ± 3.5%; p = 0.0072).

In the present cohort, fenestration was not associated with a statistically significant improvement in most early perioperative outcomes. Larger studies are needed to define its role.

As patients progress through single-ventricle palliation, changing haemodynamics and patient conditions can prevent progression to Fontan. We sought to determine the incidence of Fontan completion at our institution and to investigate the reasons for non-Fontan candidacy.

Patients who underwent superior cavopulmonary anastomosis from 2010 to 2020 at a single institution were included. Pre-Fontan testing was reviewed for all patients, and the primary reason for non-candidacy was determined based on a review of the electronic medical records.

Of the 427 patients included, 396 (93%) underwent Fontan or were referred for Fontan at the time of the study. Reasons for non-Fontan candidacy in the remaining 31 patients included cardiac reasons (n = 26), mainly univentricular dysfunction or atrioventricular valve regurgitation, respiratory conditions (n = 3), or miscellaneous reasons (n = 2). The patients who were non-Fontan candidates due to respiratory conditions and miscellaneous reasons are all alive without further palliation at the time of the study. There are 17 patients (17/31; 55%) in the non-candidacy group who had a heart transplant or were listed for transplant at the time of the study. Non-Fontan candidates had a higher incidence of moderate or severe atrioventricular valve regurgitation prior to superior cavopulmonary anastomosis.

Thirty-one patients (31/427; 7%) were not Fontan candidates at our centre. Our results demonstrate that the reasons for non-Fontan candidacy after completion of stage 2 are broadly due to echocardiographic findings and respiratory concerns. Predicting non-Fontan candidacy prior to superior cavopulmonary anastomosis remains difficult.

Neonates may require increased red cell mass to optimise oxygen content after stage 1 palliation; however, data informing transfusion practices are limited. We hypothesise there is a patient-, provider-, and institution-based heterogeneity in red cell transfusion decision-making after stage 1 palliation.

We conducted an online survey of Pediatric Cardiac Intensive Care Society practitioners in 2016. Respondents answered scenario-based questions that defined transfusion indications and identified haematocrit transfusion thresholds. Respondents were divided into restrictive and liberal groups based on a haematocrit score. Fisher’s exact test was used to determine the associations between transfusion likelihood and patient, provider, and institutional characteristics. Bonferroni correction was applied to adjust the p-value to 0.004 for multiple comparisons.

There was a 21% response rate (116 responses). Most were male (58.6%), attending physicians (85.3%) with >5 year of intensive care experience (88.7%) and subspeciality training in critical care medicine (47.4%). The majority of institutions were academic (96.6%), with a separate cardiac ICU (86.2%), and performed >10 stage 1 palliation cases annually (68.1%). After Bonferroni correction, there were no significant patient, respondent, or institutional differences between the restrictive and liberal groups. No respondent or institutional characteristics influenced transfusion decision-making after stage 1 palliation.

Decision-making around red cell transfusion after stage 1 palliation is heterogeneous. We found no clear relationships between patient, respondent, or institutional characteristics and transfusion decision-making among surveyed respondents. Given the lack of existing data informing red cell transfusion after stage 1 palliation, further studies are necessary to inform evidence-based guidelines.