Parents of children with CHD face several barriers when trying to access the services needed to support their child’s development. In fact, current developmental follow-up practices may not identify developmental challenges in a timely manner and important opportunities for interventions may be lost. This study aimed to explore the perspectives of parents of children and adolescents with CHD with respect to developmental follow-up in Canada.

Interpretive description was used as a methodological approach for this qualitative study. Parents of children aged 5–15 years with complex CHD were eligible. Semi-structured interviews that aimed to explore their perspectives regarding their child’s developmental follow-up were conducted.

Fifteen parents of children with CHD were recruited for this study. They expressed that the lack of systematic and responsive developmental follow-up services and limited access to resources to support their child’s development placed an undue burden on their families, and as a result, they needed to assume new roles as case managers or advocates to address these limitations. This additional burden resulted in a high level of parental stress, which, in turn, affected the parent–child relationship and siblings.

The limitations of the current Canadian developmental follow-up practices put undue pressure on the parents of children with complex CHD. The parents stressed the importance of implementing a universal and systematic approach to developmental follow-up to allow for the timely identification of challenges, enabling the initiation of interventions and supports and promoting more positive parent–child relationships.

Diversification of the medical and cardiothoracic surgical workforce represents an ongoing need. A congenital cardiac surgery shadowing programme for undergraduate students was implemented at the University of Florida Congenital Heart Center.

Students shadowing in the Congenital Heart Center from 17 December 2020 through 20 July 2021 were sent a survey through Qualtrics to evaluate the impact of their shadowing experience. The main objectives of the survey were to determine the personal relationship(s) of the students to physicians prior to shadowing, how the presence or absence of physicians in the family of a given student related to the exposure of the student to a medical setting prior to shadowing, and the interest of the students in medicine and cardiothoracic surgery prior to and after the shadowing experience. Survey responses included “Yes/No” questions, scaled responses using a Likert scale, selection lists, and free text responses. When applicable, t-tests were utilised to assess differences between student groups.

Of the 37 students who shadowed during the study period, 26 (70%) responded. Most students were female (58%, n = 15), and the mean age was 20.9 ± 2.4 years. Students spent a mean duration of 95 ± 138 hours shadowing providers as part of the shadowing programme. Likert scale ratings of interest in the professions of medicine, surgery, and cardiothoracic surgery all increased after the shadowing experience (p < 0.01). Students with a family member in medicine had more clinical exposure prior to the shadowing programme (p < 0.01).

A surgical shadowing programme at a Congenital Heart Center may have an important formative impact on the views of undergraduate students regarding potential careers in surgery and medicine. Additionally, students without family members in medicine tend to have less prior exposure to medicine and could likely benefit more from this type of shadowing programme.

Adults with CHD are a growing patient group and childbirth is a relatively new phenomenon. EQ-5D is commonly used to measure health-related quality of life. We sought to investigate EQ-5D status before, during, and after pregnancy in women with CHD.

We identified 128 pregnancies in 86 CHD women giving birth in Skåne County during 2009–2021. Repeated measures ANOVA was performed to test for differences between the five EQ-5D domains, EQ-VAS, and EQ-index over time points before, the second trimester, the third trimester, and after pregnancy.

Mean age at estimated childbirth was 30.3 (± 4.7) years; 56.25% of births were vaginal deliveries and 43.75% were Caesarean sections. The cohort consisted of patients with double outlet right ventricle (4.7%), transposition (Mustard/Senning 2.3%, arterial switch 4.7%), aortic anomalies (19.5%), Fallot’s anomaly (16.4%), single ventricle (3.9%), shunt lesions (11.7%), cardiomyopathies (4.7%), coronary anomalies (1.6%), arrythmias (0.8%), and valve lesions: aortic (19.5%), mitral (5.5%), and pulmonary (4.7%). The women reported significantly worse mobility (p = 0.007) and higher pain/discomfort (p = 0.049) at trimester 3 compared to before pregnancy. The women had lower EQ-5D index during trimester 3 compared to after pregnancy (p = 0.004). We saw worse mobility during Tri 2 comparing multiparity with primiparity (p = 0.046). Looking at delivery mode, we noted significantly higher anxiety/depression before pregnancy (p =0.023) in women that had a Caesarean section.

In this study, women with CHD reported worse mobility and a higher pain level during Tri 3, although the overall health-related quality of life is acceptably high.

To evaluate early- and long-term outcomes of the surgical treatment for coarctation of the aorta based on a new classification system.

A retrospective clinical review of 111 patients with coarctation of the aorta who underwent surgery (March 2011 to August 2020) was performed. We categorised coarctation of the aorta into type I, with all three head vessels tightly packed; type II, with the left subclavian artery separated from the two other head vessels; and type III, with all three head vessels separated from one another. Each type included subtype a, with a short isthmic portion, and subtype b, with a long isthmic portion.

The median patient age and weight at operation were 8 (range, 1–1490) days and 3.2 (range, 1.9–18.5) kg, respectively. Extended end-to-end anastomosis was performed via sternotomy in 54, via thoracotomy in 12, end-to-side anastomosis in 31, autologous main pulmonary artery patch augmentation in 12, and modified end-to-end anastomosis combined with subclavian artery flap aortoplasty in two patients. There was one (0.9%) case of early mortality and 12 (10.8%) cases of post-operative complications. Two (1.8%) late deaths occurred during follow-up. Five (4.5%) patients underwent balloon dilatation and three (2.7%) underwent reoperation for restenosis of coarctation of the aorta. All patients with type Ia (21 patients, 18.9%) underwent extended end-to-end anastomosis via sternotomy or thoracotomy.

According to the early and late outcomes observed in this study, surgical treatment of coarctation of the aorta using the new classification system could be safe and low risk.

Coronary artery dilation associated with bicuspid/unicuspid aortic valves is described in adults with limited data in children. We aimed to describe the clinical course of children with bicuspid/unicuspid aortic valves and coronary dilation including coronary Z-score changes over time, association of coronary changes with aortic valve anatomy/function, and complications.

Institutional databases were searched for children ≤18 years with both bicuspid/unicuspid aortic valves and coronary dilation (1/2006-6/2021). Kawasaki disease and isolated supra-/subvalvar aortic stenosis were excluded. Statistics were descriptive with associations measured by Fisher’s exact test and overlapping 83.7% confidence intervals.

Of 17 children, bicuspid/unicuspid aortic valve was diagnosed at birth in 14 (82%). Median age at coronary dilation diagnosis was 6.4 years (range: 0-17.0). Aortic stenosis was present in 14 (82%) [2 (14%) moderate, 8 (57%) severe]; 10 (59%) had aortic regurgitation; 8 (47%) had aortic dilation. The right coronary was dilated in 15 (88%), left main in 6 (35%), and left anterior descending in 1 (6%) with no relationship between leaflet fusion pattern or severity of aortic regurgitation/stenosis on coronary Z-score. Follow-up evaluations were available for 11 (mean 9.3 years, range 1.1–14.8) with coronary Z-scores increasing in 9/11 (82%). Aspirin was used in 10 (59%). There were no deaths or coronary artery thrombosis.

In children with bicuspid/unicuspid aortic valves and coronary dilation, the right coronary artery was most frequently involved. Coronary dilation was observed in early childhood and frequently progressed. Antiplatelet medication use was inconsistent, but no child died nor developed thrombosis.

Infants with truncus arteriosus typically undergo repair by repurposing the truncal valve as the neo-aortic valve and using a valved conduit homograft for the neo-pulmonary valve. In cases where the native truncal valve is too insufficient for repair, it is replaced, but this is a rare occurrence with a paucity of data, especially in the infant population. Here, we conduct a meta-analysis to better understand the outcomes of infant truncal valve replacement during the primary repair of truncus arteriosus.

We systematically reviewed PubMed, Scopus, and CINAHL for all studies reporting infant (<12 months) truncus arteriosus outcomes between 1974 and 2021. Exclusion criteria were studies which did not report truncal valve replacement outcomes separately. Data extracted included valve replacement type, mortality, and reintervention. Our primary outcome was early mortality, and our secondary outcomes were late mortality and reintervention rates.

Sixteen studies with 41 infants who underwent truncal valve replacement were included. The truncal valve replacement types were homografts (68.8%), mechanical valves (28.1%), and bioprosthetic valves (3.1%). Overall early mortality was 49.4% (95% CI: 28.4–70.5). The pooled late mortality rate was 15.3%/year (95% CI: 5.8–40.7). The overall rate of truncal valve reintervention was 21.7%/year (95% CI: 8.4–55.7).

Infant truncal valve replacement has poor early and late mortality as well as high rates of reintervention. Truncal valve replacement therefore remains an unsolved problem in congenital cardiac surgery. Innovations in congenital cardiac surgery, such as partial heart transplantation, are required to address this.

Paediatric heart failure is a common clinical syndrome that may be experienced by children with congenital heart disease (CHD) and/or cardiomyopathy. It is characterised by clinical signs/symptoms which reflect the underlying pathophysiology based on one of three main clinical states: Pulmonary over-circulation, pressure overload, and ventricular failure. Current diagnosis relies on clinical assessment and echocardiogram imaging as cardiac biomarkers has been predominantly scientific to date. This review provides a comprehensive overview of paediatric heart failure pathophysiology and considers the available evidence for cardiac biomarkers in this setting.

A literature review was completed using MEDLINE ALL, EMBASE, and PubMed on 10th November, 2022. Search terms included biomarkers, heart failure, heart defects, congenital heart disease, fontan circulation, single ventricle circulation, cardiomyopathy, and child. This allowed the identification of individual cardiac biomarkers which are the focus of this review. These included NT-proBNP, MR-proANP, MR-proADM, troponin, sST2, galectin 3, and growth differentiation factor-15.

Paediatric studies have established reference ranges for NT-proBNP and troponin for children with structurally normal hearts. Of all the biomarkers reviewed, NT-proBNP appears to correlate most closely with symptoms of heart failure and ventricular dysfunction on echocardiogram. However, there remains limited longitudinal data for NT-proBNP, and no validated reference ranges for patients with CHD and/or cardiomyopathy. None of the other biomarkers reviewed were consistently superior to NT-proBNP.

Further large paediatric studies of patients with heart failure are needed to validate NT-proBNP in CHD and to evaluate the role of novel biomarkers in specific types of CHD, e.g. single ventricle physiology.