Cardiology in the Young最新文献

Objective: Neurodevelopmental delay is common in children who undergo surgery for Congenital Heart Disease (CHD) in infancy. Cardiac surgery associated acute kidney injury (CS-AKI) occurs frequently in the paediatric cardiac Intensive care unit (ICU). Cardiac surgery associated acute kidney injury (CS-AKI) is associated with worse neurodevelopmental scores and delay in cognitive, language, and motor domains in children with CHD. No known data exist regarding the association of CS-AKI and motor and language subscales. In this study, we explored the relationship between CS-AKI and receptive and expressive language, as well as gross and fine motor delay.

Methods: This was a single centre retrospective observational cohort study. Children who underwent surgery for CHD and developed recurrent CS-AKI in the first year of life who had follow-up neurodevelopmental testing using the Bayley Scale of Infant Development Version III were included. Neurodevelopmental delay subscales assessed included: receptive and expressive language, fine and motor skills.

Results: The study cohort included 203 children. Recurrent CS-AKI was significantly associated with lower scores in receptive and expressive language, as well as fine and gross motor on unadjusted analyses. On adjusted analyses, recurrent CS-AKI was significantly associated with severe receptive language delay.

Conclusion: The independent association of recurrent CS-AKI with severe language delay in children who undergo surgery for CHD in infancy is novel. Our findings may contribute to the understanding of language impairment in this population. Further studies are required to better understand this relationship and any potentially modifiable factors.

Background: When young patients with an oval fossa defect present early with symptoms of heart failure and pulmonary hypertension, surgical treatment is recommended in small bodyweight rather than transcatheter closure.

Methods: Outcomes of device closure in consecutive symptomatic children weighing under 10 kg were compared with young children aged below 4 years but weighing above 10 kg. Transthoracic echocardiography under conscious sedation guided the procedure in all without need for balloon sizing, transesophageal echocardiogram, and intubation anaesthesia. Symptoms, anthropometry, shunt ratio, pulmonary pressures, defect and device size, percentage oversizing, device/body weight ratio, complications, and post-procedural growth spurt were compared.

Results: Ninety-six patients weighing under 10 kg were compared with 160 patients weighing above 10 kg. In total, 83.3% of patients in the study group and 25% of controls were severely malnourished. The median indexed defect size was 35.2 mm/sq.m and 27.4 mm/sq.m, and the device was oversized by 8.7% and 14.2% in the study group and controls, respectively. The device/body weight ratio was 1.93 in study group and 1.4 in controls. Procedure was successful in all except one patient weighing under 10 kg who had a device embolisation. Both groups showed significant growth spurts and proportion, with severe malnutrition reduced to 42% and 11% in the two groups.

Conclusions: Device closure was feasible and safe in patients under 10 kg. Transthoracic echocardiographic imaging on conscious sedation provided adequate guidance. Symptoms and growth significantly improved after intervention. Despite a larger defect size, smaller patients had comparable outcomes. In symptomatic children under 10 kg needing early closure, transcatheter intervention should not be deferred.

Introduction: Tetralogy of Fallot patients face an elevated risk of developing chylothorax and pleural effusions post-surgery. This patient group exhibits risk factors known to compromise the lymphatic system, such as elevated central venous pressure, pulmonary flow changes, and hypoxia. This study investigates the morphology and function of the lymphatic system in tetralogy of Fallot patients through lymphatic magnetic resonance imaging and near-infrared fluorescence imaging, respectively.

Methods: Post-repair tetralogy of Fallot patients aged 6-18 years were recruited, along with age and gender-matched controls. Magnetic resonance imaging was used to assess the morphology of the thoracic lymphatic vessels and the thoracic, while near-infrared fluorescence imaging was used to assess lymphatic activity utilising lymph rate, velocity, and pressure.

Results: Nine patients and 10 controls were included. Echocardiography revealed that 2/3 of the patients had moderate-severe pulmonary regurgitation, while none displayed signs of elevated central venous pressure. Magnetic resonance imaging identified three patients with type 3 (out of 4 types) lymphatic abnormalities, while controls had none. The thoracic ducts showed severe (one patient) and moderate (one patient) tortuosity. Mean thoracic duct diameters were 3.3 mm ±1.1 in patients and 3.0 mm ± 0.8 in controls (p-value = 0.53). Near-infrared fluorescence imaging revealed no anomalous patterns.

Conclusion: Despite no presence of clinical lymphatic disease, 3/9 of the repaired tetralogy of Fallot patients exhibited lymphatic morphological abnormalities. The significance of these anomalies remains uncertain currently. Further research is needed to determine whether these lymphatic alterations in this patient cohort are a result of congenital malformations, haemodynamic shifts, or prenatal and early-life saturation levels.

Background: Given increased survival for adults with CHD, we aim to determine outcome differences of infective endocarditis compared to patients with structurally normal hearts in the general population.

Methods: We conducted a retrospective cross-sectional study identifying infective endocarditis hospitalisations in patients 18 years and older from the National Inpatient Sample database between 2001 and 2016 using International Classification of Disease diagnosis and procedure codes. Weighting was used to create national annual estimates indexed to the United States population, and multivariable logistic regression analysis determined variable associations. Outcome variables were mortality and surgery. The primary predictor variable was the presence or absence of CHD.

Results: We identified 1,096,858 estimated infective endocarditis hospitalisations, of which 17,729 (1.6%) were adults with CHD. A 125% increase in infective endocarditis hospitalisations occurred for adult CHD patients during the studied time period (p < 0.001). Adults with CHD were significantly less likely to experience mortality (5.4% vs. 9.5%, OR 0.54, CI 0.47-0.63, p < 0.001) and more likely to undergo in-hospital surgery (31.6% vs. 6.7%, OR 6.49, CI 6.03-6.98, p < 0.001) compared to the general population. CHD severity was not associated with increased mortality (p = 0.53). Microbiologic aetiology of infective endocarditis varied between groups (p < 0.001) with Streptococcus identified more commonly in adults with CHD compared to patients with structurally normal hearts (36.2% vs. 14.4%).

Conclusions: Adults with CHD hospitalised for infective endocarditis are less likely to experience mortality and more likely to undergo surgery than the general population.

An anomalous origin of the right coronary artery from the pulmonary artery case report. The diagnosis was made by angiotomography. Reimplantation of the right coronary artery into the ascending aorta and reconstruction of the pulmonary artery were conducted.

Background: Mobile health has been shown to improve quality, access, and efficiency of health care in select populations. We sought to evaluate the benefits of mobile health monitoring using the KidsHeart app in an infant CHD population.

Methods: We reviewed data submitted to KidsHeart from parents of infants discharged following intervention for high-risk CHD lesions including subjects status post stage 1 single ventricle palliation, ductal stent or surgical shunt, pulmonary artery band, or right ventricular outflow tract stent. We report on the benefits of a novel mobile health red flag scoring system, mobile health growth/feed tracking, and longitudinal neurodevelopmental outcomes tracking.

Results: A total of 69 CHD subjects (63% male, 41% non-white, median age 28 days [interquartile range 20, 75 days]) were included with median mobile health follow-up of 137 days (56, 190). During the analytic window, subjects submitted 5700 mobile health red flag notifications including 245 violations (mean [standard deviation] 3 ± 3.96 per participant) with 80% (55/69) of subjects submitting at least one violation. Violations precipitated 116 interventions including hospital admission in 34 (29%) with trans-catheter evaluation in 15 (13%) of those. Growth data (n = 2543 daily weights) were submitted by 63/69 (91%) subjects and precipitated 31 feed changes in 23 participants. Sixty-eight percent of subjects with age >2 months submitted at least one complete neurodevelopment questionnaire.

Conclusion: In our initial experience, mobile health monitoring using the KidsHeart app enhanced interstage monitoring permitting earlier intervention, allowed for remote tracking of growth feeding, and provided a means for tracking longitudinal neurodevelopmental outcomes.

Adolescents with severe cardiogenic shock can present to both paediatric and adult centres. We present six adolescent children who had extracorporeal membrane oxygenation consultation fast-tracked with clinical care input from the adult multidisciplinary team, including interhospital transfers on extracorporeal membrane oxygenation. After recovery on conventional cardiogenic shock care or extracorporeal membrane oxygenation, or bridge to transplant, all had favourable neurologic outcome.

Background: An aberrant right subclavian artery represents the most common aortic arch vascular anomaly. Conventional wisdom states that these anomalies do not result in dysphagia, but rather serve as "red herrings". Clearly, in the vast majority of cases, this holds true. Nonetheless, one should never say never.

Methods: Herein, we present a cohort of four children with debilitating dysphagia resulting from an aberrant right subclavian artery. Subclavian reimplantation via a right posterolateral thoracotomy was performed successfully in all cases.

Results: Dysphagia resolved postoperatively, and all patients were able to advance to a normal diet. They were able to gain appropriate weight postoperatively and continue to do well at most recent clinical follow-up.

Conclusions: This case series suggests that aberrant right subclavian artery anatomy should be considered a potential aetiology of dysphagia, albeit rarely. Surgical intervention for select patients can provide dramatic resolution of symptoms.

Introduction: Bicuspid aortic valve is the most common CHD and commonly associated with activity restrictions that may lead to a sedentary lifestyle known to increase obesity risk. It is unknown whether obesity is associated with changes in aortic dimensions or aortic valve function in young people with bicuspid aortic valve. This study investigates whether overweight and obese children with bicuspid aortic valve have worse aortic valve function or increased aortic dimensions compared to healthy weight children with bicuspid aortic valve.

Methods: This was a single centre retrospective cohort study comprised of patients 5 to 25 years old with a diagnosis of bicuspid aortic valve between 1 January, 2019 and 31 December, 2020. Patients were classified as healthy weight or overweight/obese. Values for aortic dimensions as well as peak and mean aortic valve gradients were obtained from echocardiogram reports.

Results: About 251 patients were analysed. Demographics were similar between groups. When indexed to height, the aortic valve annulus (1.28 ± 0.14 vs. 1.34 ± 0.15, p = 0.001) and sinotubular junctions (1.44 ± 0.21 vs. 1.49 ± 0.24, p = 0.038) were larger in the overweight/obese group, with no differences in aortic root or ascending aorta sizes. The obese/overweight group had a higher peak aortic valve gradient (23.03 ± 1.64 mmHg vs. 16.17 ± 1.55 mmHg, p = 0.003) compared to the healthy weight group.

Conclusion: Healthy weight patients did not have larger aortic dimensions compared to the overweight/obese patients. There was evidence of worsening aortic valve stenosis in overweight/obese patients compared to those at a healthy weight.

Background: Myocardial infarction is rare in children but frequently occurs unexpectedly with atypical presentation. It can cause a progressive lethal course unless prompt treatment is initiated.

Methods: Paediatric cases of myocardial infarction diagnosed by the presence of ischaemic myocardial insults and symptomatic ventricular dysfunction were reviewed retrospectively.

Results: Eighteen patients (5 days to 14 years of age; median 3 months) with myocardial infarction were studied. The aetiology was variable, including congenital coronary anomalies: anomalous left coronary artery from pulmonary artery (five patients), pulmonary atresia with intact ventricular septum with right ventricle-dependent coronary circulation (four), anomalous aortic origin of left coronary artery (three), and Williams syndrome with supravalvar aortic stenosis (one). Two acquired coronary anomalies occurred in one patient with undiagnosed Kawasaki disease with complete thrombotic obstruction of the left coronary artery and another with post-transplant cardiac allograft microangiopathy. Three patients developed thromboembolic coronary artery incidents in normal coronary anatomy. Fourteen patients were less than 1 year of age (78%). Electrocardiographic abnormalities were noted in all patients. Four patients required extracorporeal membrane oxygenation support for severe ventricular dysfunction, none of whom survived. Five patients underwent heart transplant. Five patients died during the same hospitalisation, and one patient died after discharge (overall mortality 33%). Transplant-free survival was 39%.

Conclusion: Most myocardial infarction occurred in infants who presented with abrupt onset of non-specific clinical manifestations with progressively deteriorating haemodynamic status resulting in poor transplant-free survival rate. Early diagnosis and treatment are essential to prevent catastrophic outcomes.