Abstract Scimitar syndrome in conjunction with congenital coronary artery anomalies is a rare combination. We describe two siblings with both Scimitar syndrome and an anomalous left circumflex artery from the pulmonary artery, who were both found to have a genetic mutation in the myelin regulatory factor gene. This represents the first familial association between this gene and this CHD combination.
Abstract Background: Parents play a key role in adolescent licensure in the United States, particularly for those with children diagnosed with CHD. Many seek strategies to facilitate discussions about driving safety, yet limited research on parental perceptions restricts resources available for these conversations. Purpose: This study examined parental perspectives on barriers and facilitators to safe, independent driving among adolescents with CHD and impacts of CHD on family driving experiences. Methods: Semi-structured interviews were conducted with 29 parents of adolescents with CHD (93% mothers), and key themes were identified using conventional content analysis. Results: Two themes emerged: (1) parents see driving as a pivotal step towards adolescent independence, leading to both anticipation and apprehension; (2) driving introduces challenges specific to adolescence that may not be evident earlier. Ninety-seven percent of parents reported their adolescent had no current or expected driving restrictions, consistent with reports from parents of adolescents without CHD. Conclusions: Parents perceived minimal CHD impact on driving, but concerns such as fatigue, cognitive effects, and attention deficit hyperactivity disorder were revealed often intensifying during adolescence and unrecognised. Provider-led discussions addressing driving readiness, CHD, and other health issues can support families during transition from adolescence to adulthood.
Abstract A common arterial trunk with an interrupted aortic arch is a rare and complex CHD. Although single-stage neonatal repair can be performed in term infants, prematurity, low birthweight, and complex/multiple defects are major risk factors for in-hospital mortality. We report the first patient-specific, three-dimensional-modelling-guided, off-pump transaortic ductal stenting combined with bilateral pulmonary artery banding, performed as a bridge to successful complete repair in a very-low-birthweight, extremely premature infant with this anomaly.
Abstract Although well-described, there are few published images of spasm of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect. We offer here angiographic evidence of unprecipitated spasm affecting multiple vessels simultaneously.
