Cardiology in the Young最新文献

Children with congenital heart defects (CHD) are often short/lightweight relative to peers. Limited growth, particularly height, may reflect energy deficits impacting physical activity. Latent class analyses of growth from birth and Bruce treadmill exercise data retrospectively identified for height, weight, and body mass index z-scores growth trajectories. Linear regression models examined exercise parameters by growth trajectory, adjusting for age/sex/CHD severity. A total of 213 children with CHD (39% female, 12.1 ± 2.9 years) achieved 85.8 ± 10.1% of the predicted peak heart rate. Peak heart rate among children whose height was consistently below average (class 1) was 15.2 ± 4.9 beats/min lower than children with other height trajectories. These children also attained a lower percentage of predicted peak heart rate. Children whose weight (p = 0.03) or body mass index (p < 0.001) z-score increased throughout childhood had significantly lower exercise duration (mean difference 1-2 min) than children whose growth trajectories were stable or declined. Children with above-average weight or an increasing body mass index also used a higher percentage of their heart rate reserve at each submaximal exercise stage. A very low height z-score trajectory is associated with decreased exercise capacity that may increase the risk for morbidities associated with a sedentary lifestyle. Future studies should examine potential mechanisms for the observed height deficits, such as an inadequate energy supply that could impact physical activity participation, congestive heart failure, cyanosis, pubertal stage, supplemental feeding history, or familial growth patterns. Prospective studies examining growth in relation to objective measures of daily physical activity are required.

Patients with CHD have an increased risk of necrotising enterocolitis, leading to higher mortality compared to infants without necrotising enterocolitis. Current definitions and treatment recommendations are based on premature infants and accepted criteria for diagnosing or treating necrotising enterocolitis in infants with CHD are lacking. We performed a quality improvement project to develop and implement a diagnostic scoring tool and treatment algorithm for necrotising enterocolitis in infants with CHD, aiming to enhance early diagnosis, categorise disease severity, and expedite safe return to enteral feeding.The scoring tool and algorithm were implemented in children with CHD under six months of age with clinical suspicion of necrotising enterocolitis. Outcome measures included days of nil per os (NPO), duration of antibiotic treatment, time on total parenteral nutrition, and time to full enteral feeding after diagnosis. Balancing measures included progression to surgical necrotising enterocolitis, vasoactive-inotropic score, length of stay, and mortality.Twenty-seven patients were included (14 preintervention and 13 postintervention) and 39 episodes of necrotising enterocolitis were analysed (19 preintervention and 20 postintervention). In the postintervention group, patients were NPO for fewer days (2 vs. 7 days, p = 0.004), had a shorter duration of antibiotic treatment (3 vs. 7 days, p = 0.02), received total parenteral nutrition for fewer days (0 vs. 20 days, p = 0.01), and returned to full feeds more quickly (8 versus 18 days, p = 0.013) without increased progression to surgical necrotising enterocolitis (3 versus 0, p = 0.11).Implementation of a novel necrotising enterocolitis scoring tool and treatment algorithm reduced NPO days, antibiotic duration, and time to full feeds without increasing surgical necrotising enterocolitis in infants with CHD.

Introduction: Poor weight gain in infants with single ventricle cardiac physiology between stage 1 and stage 2 palliative surgeries is associated with worse outcomes. The growth of infants with single ventricle physiology, enrolled in home monitoring programmes in the United Kingdom, has not been widely described.

Aim: To explore the growth of infants with single ventricle physiology supported by a home monitoring programme, at a tertiary centre in the South of England.

Methods: A retrospective review of two cohorts, comparing weight gain amongst infants with single ventricle physiology, before and following the implementation of a home monitoring programme. Inclusion was dependent on a diagnosis compatible with single ventricle physiology during the interstage.

Results: Enrolment into a home monitoring programme (cohort 2) was associated with 55% more infants being discharged home during the interstage period (p < 0.05). Interstage mortality did not differ between cohorts. There were no differences in interstage growth velocity between cohorts (cohort 1 23.98 ± 11.7 g/day and cohort 2 23.82 ± 8.3 g/day); however, infants in cohort 2 experienced less growth deceleration early in life, and achieved catch-up growth at 12-23 months. Interstage nasogastric feeding, regardless of the cohort, was associated with worse growth outcomes.

Conclusion: A home monitoring programme for infants with single ventricle physiology provides the opportunity for infants to be safely discharged home to their families and cared for at home during the interstage. Infants in the home monitoring programme experienced better growth, achieving weight restoration at 12-23 months.

Coarctation of the aorta is a rare congenital cardiovascular anomaly. In some patients, one particular vessel in collateral circulation has achieved recognition as the artery of Hamilton and Abbott. This aberrant pathway can create local turbulence, elevating the risk of aneurysm formation. We report the clinical treatment of a 35-year-old patient with coarctation of the aorta combined with an aneurysm originating from the artery of Hamilton and Abbott to highlight the need for attention to the presence of this artery.

Patent ductal arteriosus stenting is an alternative procedure in patients with pulmonary-ductal-dependent circulation. Stent embolization is one of the major acute complications of ductal stenting procedure. We describe the case of a stent embolization into the abdominal aorta during the deployment of ductal stent in a premature low-weight infant (1.850 kg), affected by critical pulmonary stenosis and duct-dependent pulmonary circulation. The stent was successfully retrieved through a 4F Flexor catheter without vascular complication by using a new approach.

Pulmonary valve replacement in small children is rarely needed; when performed, it is usually done surgically. Herein, we describe a hybrid technique for pulmonary valve replacement through a small subxiphoid incision in a Toddler without utilising the cardiopulmonary bypass machine. The procedural technique is thoroughly explained. Collaboration between paediatric cardiology and cardiovascular surgery teams achieved optimal outcome with minimised invasiveness.

We report an uncommon case of tetralogy of Fallot with absent pulmonary valve and extreme right pulmonary artery dilatation in patient with 22q11.2 deletion syndrome.

Introduction: Interprofessional teams in the pediatric cardiac ICU consolidate their management plans in pre-family meeting huddles, a process that affects the course of family meetings but often lacks optimal communication and teamwork.

Methods: Cardiac ICU clinicians participated in an interprofessional intervention to improve how they prepared for and conducted family meetings. We conducted a pretest-posttest study with clinicians participating in huddles before family meetings. We assessed feasibility of clinician enrollment, assessed clinician perception of acceptability of the intervention via questionnaire and semi-structured interviews, and impact on team performance using a validated tool. Wilcoxon rank sum test assessed intervention impact on team performance at meeting level comparing pre- and post-intervention data.

Results: Totally, 24 clinicians enrolled in the intervention (92% retention) with 100% completion of training. All participants recommend cardiac ICU Teams and Loved ones Communicating to others and 96% believe it improved their participation in family meetings. We exceeded an acceptable level of protocol fidelity (>75%). Team performance was significantly (p < 0.001) higher in post-intervention huddles (n = 30) than in pre-intervention (n = 28) in all domains. Median comparisons: Team structure [2 vs. 5], Leadership [3 vs. 5], Situation Monitoring [3 vs. 5], Mutual Support [ 3 vs. 5], and Communication [3 vs. 5].

Conclusion: Implementing an interprofessional team intervention to improve team performance in pre-family meeting huddles is feasible, acceptable, and improves team function. Future research should further assess impact on clinicians, patients, and families.

Background: Cardiac surgery-associated acute kidney injury (CS-AKI) and fluid overload (FO) are common among neonates who undergo cardiopulmonary bypass, and increase mortality risk. Current diagnostic criteria may delay diagnosis. Thus, there is a need to identify urine biomarkers that permit earlier and more accurate diagnosis.

Methods: This single-centre ancillary prospective cohort study describes age- and disease-specific ranges of 14 urine biomarkers at perioperative time points and explores associations with CS-AKI and FO. Neonates (≤28 days) undergoing cardiac surgery were included. Preterm neonates or those who had pre-operative acute kidney injury were excluded. Urine biomarkers were measured pre-operatively, at 0 to < 8 hours after surgery, and at 8 to 24 hours after surgery. Exploratory outcomes included CS-AKI, defined by the modified Kidney Disease Improving Global Outcomes criteria, and>10% FO, both measured at 48 hours after surgery.

Results: Overall, α-glutathione S-transferase, β-2 microglobulin, albumin, cystatin C, neutrophil gelatinase-associated lipocalin, osteopontin, uromodulin, clusterin, and vascular endothelial growth factor concentrations peaked in the early post-operative period; over the sampling period, kidney injury molecule-1 increased and trefoil factor-3 decreased. In the early post-operative period, β-2 microglobulin and α-glutathione S-transferase were higher in neonates who developed CS-AKI; and clusterin, cystatin C, neutrophil gelatinase-associated lipocalin, osteopontin, and α-glutathione S-transferase were higher in neonates who developed FO.

Conclusion: In a small, single-centre cohort, age- and disease-specific urine biomarker concentrations are described. These data identify typical trends and will inform future studies.

Dilated cardiomyopathy (DCM) is a leading cause of heart failure and the most common indication for a heart transplant. Guidelines are regularly based on studies of adults and applied to the young. Children and adolescents diagnosed with DCM face different lifestyle challenges from individuals diagnosed in adulthood that include medical trauma and are influenced by maturity levels and confidence with advocacy to adults.Using a UK patient-scientist's perspective, we reviewed the age-specific challenges faced by the young with DCM, evaluated current guidelines and evidence, and identified areas requiring further recommendations and research. We highlight the importance of (i) the transition clinic from paediatric to adult services, (ii) repeated signposting to mental health services, (iii) standardised guidance on physical activity, (iv) caution surrounding alcohol and smoking, (v) the dangers of illegal drugs, and (vi) reproductive options and health.Further research is needed to address the many uncertainties in these areas with respect to young age, particularly for physical activity, and such guidance would be welcomed by the young with DCM who must come to terms with being different and more limited amongst healthy peers.