Lived experience of fetal alcohol spectrum disorder: A qualitative scoping review.

AIM: To explore the lived experiences of individuals with fetal alcohol spectrum disorder (FASD), their caregivers, and professionals working with individuals with FASD. METHOD: We conducted a scoping review using qualitative methods to explore what it is like to live with FASD from the perspective of those living with FASD, their caregivers, and stakeholders experienced in working with individuals with FASD. We searched electronic databases and grey literature for research published between 2005 and 2022. RESULTS: The 47 studies included in this scoping review show that FASD affects people's lives on a daily basis. Individuals with FASD are aware of their disability and its effects. Caregivers face daily challenges raising children with FASD and are often left unsupported by professionals. Professionals are unprepared and frustrated when supporting someone with FASD. INTERPRETATION: FASD affects individuals, caregivers, and families on a daily basis; and they require improved support and funding.

Between reproductive rights and sex selection in New Zealand's abortion reforms: practitioner dilemma in institutionalising 'choice' and 'agency'.

In 2020, the New Zealand (NZ) Parliament voted to decriminalise abortion. Although NZ's abortion law formally opposes sex selective abortions, there is considerable complexity in the gender politics of 'choice' and 'agency' in multi-ethnic societies, and interpretations of reproductive rights for ethnic minority women and for the girl child, respectively. This paper explores these complexities through the perspectives of reproductive and maternity care practitioners who are situated at the interface of legal systems, health service provision, and delivery of culturally sensitive care. Thirteen practitioners were interviewed as part of this study. The analysis highlights strains in framings of 'reproductive choice' (underpinned by western liberal notions of rights) and 'gender equality' (abortion rights that acknowledge the complexity of cultural son-preference) for ethnic minority women. These tensions are played out in three aspects of the post-reform landscape: (a) everyday practice and accountability; (b) consumerism and choice; (c) custodianship and gender rights. The findings point to the limitations in operationalising choices for ethnic women in health systems wherein trust deficit prevails, and cultural dynamics render complex responses to abortion. They also highlight reconfigurations of client-expert relationships that may have implications for practitioners' abilities to advocate for ethnic women's rights against cultural influences.

Donor considerations in pediatric heart transplantation.

Donors for pediatric heart transplantation are accepted based on variety of donor factors. There is wide variability in practice across centers and lack of evidence to guide standardized approach for some donor characteristics. This article reviews current practice and evidence for donor evaluation in pediatric heart transplantation.

Chronotropic incompetence in paediatric heart transplant recipients with prior congenital heart disease.

BACKGROUND: Cardiopulmonary exercise testing has been used to measure functional capacity in children who have undergone a heart transplant. Cardiopulmonary exercise testing results have not been compared between children transplanted for a primary diagnosis of CHD and those with a primary diagnosis of cardiomyopathy despite differences in outcomes. This study is aimed to compare cardiopulmonary exercise testing performance between these two groups. METHODS: Patients who underwent heart transplant with subsequent cardiopulmonary exercise testing at least 6 months after transplant at our institution were identified. They were then divided into two groups based on primary cardiac diagnosis: CHD or cardiomyopathy. Patient characteristics, echocardiograms, cardiac catheterisations, outcomes, and cardiopulmonary exercise test results were compared between the two groups. RESULTS: From the total of 35 patients, 15 (43%) had CHD and 20 (57%) had cardiomyopathy. Age at transplant, kidney disease, lung disease, previous rejection, coronary vasculopathy, catheterisation, and echocardiographic data were similar between the groups. Mean time from transplant to cardiopulmonary exercise testing, exercise duration, and maximum oxygen consumption were similar in both groups. There was a difference in heart rate response with CHD heart rate response of 63 beats per minute compared to cardiomyopathy group of 78 (p = 0.028). Patients with CHD had more chronotropic incompetence than those with cardiomyopathy (p = 0.036). CONCLUSION: Primary diagnosis of CHD is associated with abnormal heart rate response and more chronotropic incompetence compared to those transplanted for cardiomyopathy.

Monocusp valve placement in children with tetralogy of Fallot undergoing repair with transannular patch: A functioning pulmonary valve does not improve immediate postsurgical outcomes.

INTRODUCTION: In patients with tetralogy of Fallot (TOF), use of transannular patch (TAP) may be required in order to relieve significant right ventricular outflow tract obstruction, subsequently resulting in pulmonary insufficiency (PI). The monocusp valve has been used to temporarily reduce insufficiency in hopes to improve short and midterm outcomes. The purpose of this study was to assess for potential benefits of the monocusp valve in this subset of patients. DESIGN: Between 2005 and 2016, 119 patients with TOF with pulmonary stenosis who underwent repair with TAP were analyzed, 43 (36.1%) had a monocusp valve placed. Immediate outcomes were assessed by postoperative echocardiograms, ICU data including time to extubation, chest tube duration, reintervention, length of stay, and mortality. RESULTS: Median age of repair was similar for monocusp group at 143.5 days and nonmonocusp at 137.0 days (P = .93). Peak preoperative right ventricular outflow tract obstruction was higher in the monocusp group (80 mm Hg vs. 70 mm Hg, P ≤ .01). Patients who had monocusp placed had longer bypass time. There was less PI for monocusp group immediately after repair and at discharge (P ≤ .01). There was no difference in days of intubation, chest tube duration, length of hospitalization, reintervention rates, or mortality. CONCLUSION: Decreasing the degree of PI with a monocusp valve in patients undergoing repair for TOF repair with TAP does not improve clinical outcomes in the immediate postoperative period.