Cardiology in the Young最新文献

Objective(s): To examine feasibility, acceptability, and preliminary effectiveness of a novel group-based telemedicine psychoeducation programme aimed at supporting psychological well-being among adolescents with Fontan-palliated CHD.

Study design: A 5-week telemedicine psychoeducation group-based programme (WE BEAT) was developed for adolescents (N = 20; 13-18 years) with Fontan-palliated CHD aimed at improving resiliency and psychological well-being. Outcome measures included surveys of resilience (Connor-Davidson Resilience Scale), benefit finding (Benefit/Burden Scale for Children), depression, anxiety, peer relationships, and life satisfaction (National Institutes of Health Patient-Reported Outcomes Measurement Information System scales). Within-subject changes in these outcomes were compared pre- to post-intervention using Cohen's d effect size. In addition, acceptability in the form of satisfaction measures and qualitative feedback was assessed.

Results: Among eligible patients reached, 68% expressed interest in study participation. Of those consented, 77% have been scheduled for a group programme to date with 87% programme completion. Twenty adolescents (mean age 16.1 ± SD 1.6 years) participated across five WE BEAT group cohorts (range: 3-6 participants per group). The majority (80%) attended 4-5 sessions in the 5-session programme, and the median programme rating was a 9 out of 10 (10 = most favourable rating). Following WE BEAT participation, resiliency (d = 0.44) and perceptions of purpose in life increased (d = 0.26), while depressive symptoms reduced (d = 0.36). No other changes in assessed outcome measures were noted.

Conclusions: These findings provide preliminary support that a group-based, telemedicine delivered psychoeducation programme to support psychological well-being among adolescents with CHD is feasible, acceptable, and effective. Future directions include examining intervention effects across diverse centres, populations, and implementation methods.

Background: Adult Kawasaki patients may require intervention for occlusive coronary artery disease. Some adverse effects of first-generation drug-eluting stent implantation with sirolimus have been reported in this population.

Methods: A total of nine lesions in eight (seven males, one female) patients who underwent stent implantations in this population between 2000 and 2021 were reviewed.

Results: The age at stent implantation ranged from 31 to 47 years, with a median of 37 years. There were six lesions treated by primary percutaneous transluminal coronary interventions, and three by elective procedures. A coronary aneurysm was found in two lesions, and coronary artery calcification was found in all culprit lesions. The numbers of everolimus-eluting stents, sirolimus-eluting stents and bare metal stents were six, two, and one, respectively. As anti- thrombotic therapy, aspirin, clopidogrel, and prasugrel were given to four, three, and one, respectively. Warfarin was given to five patients. The follow-up ranged from 2 to 12 years, with a median of 4 years. Follow-up angiograms were performed for eight lesions, at 2 to 38 months, with a median of 11 months. The patency of the target vessel was confirmed in all eight vessels. Slight malapposition, and peri-stent contrast staining were found in two lesions each.

Conclusion: Acute coronary syndrome due to coronary artery lesions caused by Kawasaki disease occurred, even in lesions without any apparent coronary artery aneurysms. In our study, we show safe and efficacious placement of second-generation stent without adverse effects during the short-term follow-up, but long-term follow-up is needed to determine the efficacy and complication.

Background: Endomyocardial biopsy remains the gold standard for cardiac cellular rejection surveillance after heart transplantation. We studied a novel non-invasive index of left ventricular relaxation to detect cardiac cellular rejection in paediatric heart transplant patients.

Methods: This is a single-centre retrospective study of paediatric heart transplant patients who underwent endomyocardial biopsy from June 2014 to September 2021. Left ventricular relaxation index was calculated as the sum of diastolic tissue Doppler imaging velocities (E) of the left ventricular lateral, septal, and posterior walls divided by the percentage of the left ventricular posterior wall thinning by M-mode. Statistical analysis included t-tests and Mann-Whitney tests to compare means and medians between treatment and non-treatment groups. We used the cut-off with the maximum Youden index to compare the sensitivity and specificity of left ventricular relaxation index to detect rejection.

Results: The study included 65 patients who underwent 246 cardiac catheterizations and endomyocardial biopsies. Out of 246, 192 procedures were included and 54 were excluded due to recent transplants or lack of echocardiographic data. A total of 114 demonstrated Grade 0R, 68 Grade 1R, 8 Grade 2R, and 2 Grade 3R allograft rejection. The difference in mean left ventricular relaxation index between treatment versus non-treatment groups (2R, 3R vs. 0R, 1R) was not statistically significant (p = 0.917). A left ventricular relaxation index cut-off of 0.73 had the highest Youden index with good sensitivity (100%) and poor specificity (23%) for detecting rejections with grades 2R and 3R.

Conclusion: Left ventricular relaxation index, a novel index of left ventricular relaxation, was not a sensitive or specific predictor of cardiac cellular rejection in paediatric heart transplants.

Objectives: To evaluate the motor proficiency, identify risk factors for abnormal motor scores, and examine the relationship between motor proficiency and health-related quality of life in school-aged patients with CHD.

Study design: Patients ≥ 4 years old referred to the cardiac neurodevelopmental program between June 2017 and April 2020 were included. Motor skills were evaluated by therapist-administered Bruininks-Oseretsky Test of Motor Proficiency Second-Edition Short Form and parent-reported Adaptive Behavior Assessment System and Patient-Reported Outcomes Measurement Inventory System Physical Functioning questionnaires. Neuropsychological status and health-related quality of life were assessed using a battery of validated questionnaires. Demographic, clinical, and educational variables were collected from electronic medical records. General linear modelling was used for multivariable analysis.

Results: The median motor proficiency score was the 10^th percentile, and the cohort (n = 272; mean age: 9.1 years) scored well below normative values on all administered neuropsychological questionnaires. In the final multivariable model, worse motor proficiency score was associated with family income, presence of a genetic syndrome, developmental delay recognised in infancy, abnormal neuroimaging, history of heart transplant, and executive dysfunction, and presence of an individualised education plan (p < 0.03 for all predictors). Worse motor proficiency correlated with reduced health-related quality of life. Parent-reported adaptive behaviour (p < 0.001) and physical functioning (p < 0.001) had a strong association with motor proficiency scores.

Conclusion: This study highlights the need for continued motor screening for school-aged patients with CHD. Clinical factors, neuropsychological screening results, and health-related quality of life were associated with worse motor proficiency.

Background: Ebstein's anomaly represents 40% of congenital tricuspid valve abnormalities. Studies about paediatric Ebstein's anomaly patients are limited.

Aim: To evaluate clinical characteristics, treatment (medical/arrhythmia ablation/surgical) results, and outcome of Ebstein's anomaly patients, and to determine factors affecting arrhythmia presence and mortality.

Methods: Clinical data, echocardiography, treatment results, and outcomes of patients followed in our centre between 2000 and 2017 were retrospectively evaluated.

Results: A total of 79 patients (61 children, median diagnosis age: 1.5 years [1 day-24 years]) were included. Eight patients (10.1%) were deceased during the study period. Common associated anomalies were atrial septal defect/patent foramen ovale (56.9%), mitral regurgitation (25.3%), pulmonary stenosis/atresia (17.7%), and ventricular septal defect (16.5%). Genetic diseases/congenital anomalies were present in 5/3.8%. Tricuspid regurgitation was present in 75.9%, and severe in 50%. Arrhythmias were detected in 30.4%, and accessory pathway-mediated re-entrant tachycardia was the most common (67%). Wolff-Parkinson-White syndrome was present in 12.7%. Twenty-one ablation procedures (radiofrequency ablation [85.7%]/cryoablation [14.3%]) were performed in 16 patients (median age: 13.3 years [4.9-17]). Acute success/recurrence rates: 87.5/25%. Surgery was performed in 31.6% (median age: 6.5 years [4 days-29 years]), 7.6% were operated during the first month, and 12.6% during the first year. Second surgery was required in 28%. Perioperative mortality rate was 12%, and median mortality age was 25 days (1 day-17 years). Median follow-up period was 5.3 years (1 day-32 years). Older diagnosis age (p = 0.005) and mild-moderate mitral regurgitation (p = 0.036) were associated with arrhythmias. Younger age at diagnosis (p = 0.012), younger age at first surgery (p = 0.004), surgery before age three years (p = 0.037), and presence of pulmonary atresia (p = 0.000014) were associated with mortality. Gender, diagnosis age, congenital anomalies/genetic disorders, tricuspid regurgitation, arrhythmias, and surgery history did not have an independent effect on survival.

Conclusions: In children and young adults presenting with Ebstein's anomaly, younger age at presentation and at surgery, surgery before age three years, pulmonary atresia were associated with death. Ablation procedures can be successfully performed but recurrence rate is still high.

Background: The Wolff-Parkinson-White pattern is a delta wave frequently detected in school-based cardiovascular screening programs in Japan. Although most children with Wolff-Parkinson-White pattern are asymptomatic, initial symptoms may include syncope or sudden death, necessitating accurate diagnosis and management. Delta waves can also indicate a fasciculoventricular pathway, which poses no risk and does not require management.

Methods: We reviewed the medical records of patients referred to our hospital between April 2008 and March 2022 to evaluate the electrocardiographic signs of the Wolff-Parkinson-White pattern. The existence of Wolff-Parkinson-White syndrome and fasciculoventricular pathway were determined based on atrioventricular block and QRS waveform changes after adenosine administration during sinus rhythm.

Results: The study cohort included 127 children (65 males; median age: 12.8 years, resting heart rate: 75 beats/min, PR interval: 109 ms, and QRS duration: 101 ms). The adenosine administration test revealed a fasciculoventricular pathway, Wolff-Parkinson-White syndrome, and indeterminate findings in 64, 54, and 9 children, respectively. More than 60% of children with a QRS duration ≤ 120 ms had a fasciculoventricular pathway. Age ≤ 12 years, QRS duration >120 ms, and type A category (children with R/S ratios >1 in lead V1) were identified as independent risk factors for Wolff-Parkinson-White syndrome. No adverse events were observed in any child.

Conclusions: The adenosine administration test is safe and feasible for differentiating Wolff-Parkinson-White syndrome from fasciculoventricular pathways and can reduce the unnecessary management of children with fasciculoventricular pathways.

We report a case of right ventricle to pulmonary artery conduit angioplasty in which a valvuloplasty balloon ruptured circumferentially and was retained within the conduit. A high-pressure balloon was used to relieve the obstruction and free the ruptured balloon. The procedure was further complicated when the distal part of the balloon broke away from the proximal part during an attempt to retrieve it back into the femoral sheath. This report highlights the inappropriate use of a soft balloon in a calcified xenograft conduit, which led to a series of complications, and discusses potential strategies for managing these complications.

CHDs affecting the right ventricular outflow tract often require repeated surgical or transcatheter interventions due to pulmonary insufficiency or stenosis. This study presents percutaneous implantation of large self-expanding valves in patients with complex right ventricular outflow tract anatomy after prior pulmonary branch stenting.

Purpose: Northwest axis on an electrocardiogram is conventionally thought to be associated with CHD, but there is a paucity of data regarding the types and incidence of CHD associated with this finding. The purpose of this study was to report the types and incidence of CHD found at our institution to determine the efficacy of electrocardiograms as a screening test in infants.

Methods: Retrospective, single-centre study of all infants ≤ 60 days of age who underwent a first-time electrocardiogram from 2015 to 2021. Our inclusion criteria included those found to have a northwest axis on electrocardiogram and who also had an echocardiogram performed. Data were described using standard summary statistics.

Results: Two hundred sixty-eight infants met inclusion criteria (median age 4 days, interquartile range 2-24). The most common reason for an electrocardiogram to be obtained was the presence of a heart murmur (n = 102, 38%). One hundred thirty-one infants (49%) were found to have significant CHD. Cardiac defects included ventricular septal defect (n = 36, 28%), severe pulmonary valve stenosis (n = 17, 13%), atrioventricular canal defect (n = 16, 12%), and single ventricle disease (n = 16, 12%).

Conclusion: In this study, nearly half (49%) of these infants with northwest axis on electrocardiogram were found to have significant CHD. Electrocardiograms remain an important screening tool for infants with findings associated with potential cardiac disease and echocardiogram is warranted for infants found to have northwest axis on a first-time electrocardiogram.

Background: New drugs to target different pathways in pulmonary hypertension has resulted in increased combination therapy, but details of this use in infants are not well described. In this large multicenter database study, we describe the pharmacoepidemiology of combination pulmonary vasodilator therapy in critically ill infants.

Methods: We identified inborn infants discharged home from a Pediatrix neonatal ICU from 1997 to 2020 exposed to inhaled nitric oxide, sildenafil, epoprostenol, or bosentan for greater than two consecutive days. We compared clinical variables and drug utilisation between infants receiving simultaneous combination and monotherapy. We reported each combination's frequency, timing, and duration and graphically represented drug use over time.

Results: Of the 7681 infants that met inclusion criteria, 664 (9%) received combination therapy. These infants had a lower median gestational age and birth weight, were more likely to have cardiac and pulmonary anomalies, receive cardiorespiratory support, and had higher in-hospital mortality than those receiving monotherapy. Inhaled nitric oxide and sildenafil were most frequently used, and utilisation of combination and monotherapy for all drugs increased over time. Inhaled nitric oxide and epoprostenol were used in infants with a higher gestational age, earlier postnatal age, and shorter duration than sildenafil and bosentan. Dual therapy with inhaled nitric oxide and sildenafil was the most common combination therapy.

Conclusion: Our study revealed an increased use of combination pulmonary vasodilator therapy, favouring inhaled nitric oxide and sildenafil, yet with considerable practice variation. Further research is needed to determine the optimal combination, sequence, dosing, and disease-specific indications for combination therapy.