Objective:Hypoplastic left heart syndrome is a single ventricle defect. While staged surgical palliative treatments have revolutionised care, patients with hypoplastic left heart syndrome continue to have significant morbidity and mortality. In 2017, the National Pediatric Cardiology Quality Improvement Collaborative recommended all single ventricle patients to receive a prenatal palliative care consult. This study aimed to elucidate provider perspectives on the implementation of prenatal palliative care consults for families expecting a child with hypoplastic left heart syndrome.Methods:An online survey was administered to obstetric and paediatric providers of relevant disciplines to assess their experience with palliative care involvement in hypoplastic left heart syndrome cases.Results:Nearly, all physicians (97%) and most registered nurses (79%) agreed that the initial palliative care consult for patients with hypoplastic left heart syndrome should occur during the prenatal period. Respondents also indicated that prenatal palliative care consults should also be offered in a variety of other CHD conditions. Participants believed positive aspects of this new referral protocol included an expanded support network for families, decreased family stress during the postnatal period, increased patient education about what to expect during the postnatal period, and continuity of care.Conclusion:Multidisciplinary healthcare professionals believe that prenatal palliative care consults provide a variety of benefits for patients and families with hypoplastic left heart syndrome. Additional, multi-centre research is necessary to evaluate whether prenatal palliative care consults should become standard of care for families expecting a child with a single ventricle defect.

Late diagnosis of critical congenital heart disease (CCHD) is associated with higher levels of morbidity and mortality in neonates. Nearly all states have passed laws mandating universal pulse oximetry screening (POxS) of newborns to improve early detection rates of CCHD. We performed a retrospective chart review of all transthoracic echocardiograms (TTEs) interpreted by our institution on patients between 0 and 30 days of life in the years 2010 (prior to POxS) and 2015 (after POxS). Between 2010 and 2015, the number of neonatal TTEs interpreted by our institution decreased by 18.2%. In 2015, there were 46 neonates diagnosed with CCHD with a 78% prenatal detection rate. There was only one case of a true-positive POxS. Our study demonstrated that the initiation of POxS coincided with a significant decrease in neonatal TTEs, suggesting universal POxS may impart reassurance to primary providers leading to a decrease in TTE utilization.

Introduction: Hypoplastic left heart syndrome (HLHS) carries significant morbidity and mortality. Transthoracic echocardiogram (echo) assessment of right ventricular (RV) function and tricuspid regurgitation (TR) correlate with HLHS outcomes. There are limited reports on RV function and TR in fetal HLHS and postnatal outcomes. We aim to correlate RV function and TR on fetal-echo (F-echo) with transplant-free survival (survival), hospital length of stay after initial surgery (LOS), and timing of Glenn (BDG) in HLHS patients. Methods: Imaging and clinical data of fetuses diagnosed with HLHS between February 2014 and September 2020 at our institution were retrospectively reviewed. Measurements of RV myocardial performance index (MPI) and fractional area change (FAC) were made on initial F-echo. The RV shortening fraction (SF), presence/degree of TR, and RV size by diastolic short-axis dimension were recorded. Postnatal outcomes included survival, LOS and age at BDG. Early BDG was a repair at <4 months. Mann-Whitney U test correlated F-echo measures and postnatal outcomes. Results: F-echo was available in 69 subjects; 22 excluded for termination/fetal demise, 9 for genetic syndromes, resulting in 38 subjects. Mean age at initial F-echo was 31.4±0.9wks, 11 referred >30 weeks from regional cardiologists (adjusted mean age 23.1±0.4wks). Subject age at time of review ranged from 7 months to 6.5 years. Eight subjects died (median age 2 months) and 3 required transplant (median age 8 months). F-echo RV MPI was significantly higher (0.372±0.09;p=0.005) and RV FAC was significantly lower (43%±6.4%;p=0.016) in HLHS non-survivors versus survivors. Fetal RV size and presence of TR were not significantly different. Subjects with initial LOS >30 days were more likely to have fetal TR (p<0.001) and RV dilation (p=0.019), but no significant RV MPI and FAC difference. No fetal measures correlated with need for an early BDG. Conclusion: F-echo evaluation of RV MPI and FAC in HLHS correlates with transplant-free survival. Presence of fetal TR and RV dilation correlate with a longer LOS. F-echo measures of RV function and TR function may inform prenatal counseling and postnatal outcomes for fetal HLHS.