No abstract

Coarctation of the aorta is the more common lesion of the two, with an estimated incidence of four in every 10,000 live births in the United States of America. Interrupted aortic arch is rarer, with an incidence of 19 per one million live births. 1 There is a spectrum of pathology of obstruction of the aortic arch, ranging from coarctation of the aorta with and without hypoplasia of the arch to interrupted aortic arch. Both these lesions are frequently encountered in congenital cardiology practice, and will be discussed in the remainder of this article. Obstruction of the aortic arch in the setting of hypoplastic left heart structures or atresia of the aortic valve is beyond the scope of this review and will not be discussed further.

Children and adolescents with cardiac disease (CCD) have significant morbidity and lower quality of life. However, there are no broadly applicable tools similar to the frailty score as described in the elderly, to define functional phenotype in terms of physical capability and psychosocial wellbeing in CCD. The purpose of this study is to investigate the domains of the frailty in CCD. We prospectively recruited CCD (8-17.5 years old, 70% single ventricle, 27% heart failure, 12% pulmonary hypertension; NYHA classes I, II and III) and age and gender matched healthy controls (total n = 56; CCD n = 34, controls n = 22; age 12.6 ± 2.6 years; 39.3% female). We measured the five domains of frailty: slowness, weakness, exhaustion, body composition and physical activity using developmentally appropriate methods. Age and gender-based population norms were used to obtain Z scores and percentiles for each measurement. Two-tailed t-tests were used to compare the two groups. The CCD group performed significantly worse in all five domains of frailty compared to healthy controls. Slowness: 6-min walk test with Z score −3.

Introduction: The Fontan procedure is the final stage of surgical palliation for the children with functionally single ventricle anatomy. The post-operative medical management of this patient population can be variable and hospital length of stay prolonged. The purpose of this quality improvement project was to determine if the implementation of an evidence-based clinical pathway for post-operative management of the Fontan patient at a large Midwestern academic paediatric medical centre would standardise care and decrease length of stay. Materials and methods: The clinical pathway was developed using key components from three published pathways for the Fontan procedure from other paediatric institutions across the United States. Components of the clinical pathway included (1) supplemental oxygen until pleural drainage tubes are removed, (2) fluid restriction to 80% daily maintenance and a prescribed low-fat diet, (3) aggressive and standardised diuretic therapy while inpatient and (4) central venous access. The pathway was trialed using Plan-Do-Study-Act cycles in 2016, implemented in 2017 and sustained in 2018–2019. A retrospective electronic medical record review was performed to compare key outcomes from pre-pathway (2014–2015, 37 patients) with post-pathway implementation (2017–2018, 30 patients). Results: Adherence to the pathway was nearly 100% with a statistically significant decrease in length of stay from 12 to 9 days (p = 0.007) and no increase in readmissions. Conclusion: Standardising care can improve clinical and financial outcomes for the Fontan patient population without negatively impacting quality of care, thus providing a positive benefit to the healthcare institution, industry and patient.

Background Our Cardiac High Acuity Monitoring Program (CHAMP) uses home video telemetry (HVT) as an adjunct to monitor infants with single ventricle during the interstage period. This study describes the development of an objective early warning score using HVT, for identification of infants with single ventricle at risk for clinical deterioration and unplanned hospital admissions (UHA). Methods and Results Six candidate scoring parameters were selected to develop a pragmatic score for routine evaluation of HVT during the interstage period. We evaluated the individual and combined ability of these parameters to predict UHA. All infants with single ventricle monitored at home by CHAMP between March 2014 and March 2018 were included. Videos obtained within 48 hours before UHA were compared with videos obtained at baseline. We used binary logistic regression models and receiver operating characteristic curves to evaluate the parameters' performance in discriminating the outcome of interest. Thirty‐nine subjects with 64 UHA were included. We compared 64 pre‐admission videos to 64 paired baseline videos. Scoring was feasible for a mean of 91.6% (83.6%–98%) of all observations. Three different HVT score models were proposed, and a final model composed of respiratory rate, respiratory effort, color, and behavior exhibited an excellent discriminatory capability with an area under the receiver operating characteristic curve of 93% (89%–98%). HVT score of 5 was associated with specificity of 93.8% and sensitivity of 88.7% in predicting UHA. Conclusions We developed a feasible and reproducible HVT score that can serve as a tool to predict UHA in infants with single ventricle. Future directions involve prospective, multicenter validation of this tool.

Objectives: Women carrying a fetus diagnosed with congenital heart disease often experience significant distress because of their medical diagnosis. Given the well-documented impact associated with elevated prenatal stress and critical importance of developing targeted interventions, this study aims to examine stressors, coping and resilience resources, and mental health treatment preferences in pregnant women receiving a congenital heart disease diagnosis to inform the development of a psychological intervention to reduce maternal distress prenatally. Methods: Three groups of participants were included consisting of two pregnant women carrying a fetus with congenital heart disease, five women of children (4−16 months) with congenital heart disease, and five paediatric cardiology medical providers. Responses were gathered via semi-structured interviews and analysed using qualitative thematic analysis. Results: Information regarding four broad areas were analysed of emotional distress during pregnancy; experience of initial diagnosis; coping and resilience; and perspectives on a mental health intervention in pregnancy. Anxiety regarding baby’s future, guilt following diagnosis, and various coping strategies emerged as primary themes among the participant sample. Medical staff corroborated mothers’ heightened anxiety and viewed a psychotherapeutic intervention during the prenatal period as essential and complimentary to standard of care. Conclusion: We identified salient themes and preferred components for a future psychological intervention delivered prenatally. Practice Implications: Patients’ and providers’ perspectives regarding the nature of maternal distress, resilience and treatment preferences can inform the development of interventions to support the emotional well-being of pregnant women carrying a fetus with congenital heart disease to optimise care and potentially improve outcomes for fetal brain development.

Background The accuracy of echocardiographic measures of right ventricular (RV) diastolic function has been sparsely studied. Our objective was to evaluate the correlation between echocardiographic and reference standard measures of RV diastolic function derived from micromanometer pressure analysis before and after preload alteration in children. Methods Echocardiograms and micromanometer pressure analyses were prospectively performed before and after fluid bolus in children undergoing right heart catheterization. The isovolumic relaxation time constant (tau) and end-diastolic pressure (EDP) were measured. Conventional and speckle-tracking echocardiographic (STE) parameters of RV systolic and diastolic function were assessed. Normal saline bolus was given to increase RV EDP by 20%. Results 28 studies were performed in 22 patients with congenital heart disease or post heart transplantation. Mean age was 8.7 ± 6.1 yrs. RV longitudinal early diastolic strain rate (EDSR) correlated with tau before (r = 0.57, p = 0.001) and after fluid bolus (r = 0.48, p = 0.008). No conventional echocardiographic measures correlated with tau both before and after fluid bolus. Multiple regression analysis revealed RV EDSR and LV circumferential EDSR as independent predictors of RV tau. There were no independent predictors of EDP. Conclusion RV EDSR appears to correlate with the reference-standard measure of early active ventricular relaxation in children at baseline and after changes in preload. Conventional echocardiographic measures of diastolic function were not predictive of diastolic function after preload alteration. Future studies should assess the prognostic significance of STE measures of diastolic function in this population.

Introduction: Children with a single ventricle post-Fontan palliation are at increased risk of poor outcomes with peak oxygen consumption acting as a surrogate outcome marker. The purpose of this study is to evaluate the relationship between peak oxygen consumption and echocardiographic measures of ventricular function and deformation, including ventricular global longitudinal strain and dyssynchrony, in children and adolescents following Fontan palliation. Methods: Patients (age 8–21 years) with single ventricle post-Fontan palliation were prospectively recruited and participated in an echocardiogram, including views optimised for two-dimensional speckle tracking, and a cardiopulmonary exercise test on a cycle ergometer to maximal volitional fatigue. Results: Thirty-eight patients (mean age 13.7 ± 2.3 years) post-Fontan palliation had either a single left ventricular (n = 20), single right ventricular (n = 14), or biventricular (n = 4) morphology. Peak oxygen consumption (24.9 ± 5.6 ml/kg/minute) was correlated with global longitudinal strain (r = −0.435, p = 0.007), a strain discoordination time to peak index (r = −0.48, p = 0.003), and the presence of an electro-mechanical dyssynchrony strain pattern (p = 0.008). On multivariate regression modelling, these three variables were associated with peak oxygen consumption independently of age and sex. The single right ventricular group had evidence of possible diastolic dysfunction by E/e’ compared to the single left ventricular and biventricular groups (p = 0.001). Conclusions: Strain analysis measures are correlated with peak oxygen consumption in this cohort of children, adolescents, and young adults following Fontan palliation, suggesting that ventricular mechanics may influence the efficiency of the Fontan circulation.