Cardiology in the Young最新文献

Background: Complexity stratification for CHD is an integral part of clinical research due to its heterogenous clinical presentation and outcomes. To support our ongoing research efforts into CHD requiring disease severity stratifications, a simplified CHD severity classification system was developed and verified, with potential utility for clinical researchers without specialist CHD knowledge or access to clinical/medical records.

Method: A two-tiered analysis approach was undertaken. First-tier analysis included the audit of a comprehensive system based on: i) timing of intervention, ii) cardiac morphology, and iii) cardiovascular physiology using real patient data (n = 30), across 10 common CHD lesions. Second-tier analysis allowed for a simplified version of the classification system using morphology as a stand-alone predictor. Twelve clinicians of varying specialities involved in CHD care ranked 10 common lesions from least to most severe based on typical presentation and clinical course.

Results: First-tier analysis identified that cardiac morphology was the principal driver of complexity. Second-tier analysis largely confirmed the ranking and classification of the lesions into the broad CHD severity groups, although some variation was noted, specifically among non-cardiac specialists. This simplified version of the classicisation system, with morphology as a stand-alone predictor of severity, allowed for effective stratification for the purposes of analysis.

Conclusion: The findings presented here support this comprehensive and simple CHD severity classification system with broad utility in CHD research, particularly among clinicians and researchers with limited knowledge of CHD. The model may be applied to produce locally relevant research tools.

Background: Rastelli surgery is used for the correction of several CHDs. Although late-onset cardiac arrhythmias have emerged as a major complication after corrective surgeries, there is a paucity of data on arrhythmias after Rastelli surgery.

Methods: This retrospective cohort study was conducted on patients who had undergone Rastelli surgery and have been followed at the adult CHD clinic at our hospital.

Results: A total of 55 patients (36.4% female, age 22.2 ± 6.4 years) were followed for a median period of 24.2 (20.6-31.0) years. Tachyarrhythmias occurred in 21 (38.4 %) patients (n = 15 for atrial tachycardia, 5 for ventricular tachycardia, and 1 for both atrial and ventricular tachycardia). Older age at surgery was significantly associated with the risk of tachyarrhythmias (P = 0.022). Bradyarrhythmia occurred in 12 (21.8%) patients and consisted of perioperative AV block (n = 5), late AV block (n = 1), and sinus node dysfunction (n = 6). Nine (16.4%) patients underwent catheter ablation. The mechanisms of atrial arrhythmias include cavotricuspid isthmus-dependent and surgical scar-dependent intra-atrial reentrant tachycardias. Among the three patients who underwent ablation for ventricular tachycardia, all circuits were dependent on the scar at the base of the right ventricle to pulmonary artery conduit. Median survival free from any event (arrhythmia, death, or heart failure) was 31.6 (28.1-35.1) years after Rastelli surgery.

Conclusions: The prevalence of arrhythmias late after Rastelli surgery is substantial and increases in the second decade after surgery. Older age at surgery is associated with a higher prevalence of arrhythmias.

Fetal brain size is decreased in some children with complex CHDs, and the distribution of blood and accompanying oxygen and nutrients is regionally skewed from early fetal life dependent on the CHD. In transposition of the great arteries, deoxygenated blood preferentially runs to the brain, whereas the more oxygenated blood is directed towards the lungs and the abdomen. Knowledge of whether this impacts intrauterine organ development is limited. We investigated lung, liver, and total intracranial volume in fetuses with transposition of the great arteries using MRI.Eight fetuses with dextro-transposition and without concomitant disease or chromosomal abnormalities and 42 fetuses without CHD or other known diseases were scanned once or twice at gestational age 30 through 39 weeks. The MRI scans were conducted on a 1.5T system, using a 2D balanced steady-state free precession sequence. Slices acquired covered the entire fetus, slice thickness was 10 mm, pixel size 1.5 × 1.5 mm, and scan duration was 30 sec.The mean lung z score was significantly larger in fetuses with transposition compared with those without a CHD; mean difference is 1.24, 95% CI:(0.59;1.89), p < 0.001. The lung size, corrected for estimated fetal weight, was larger than in the fetuses without transposition; mean difference is 8.1 cm³/kg, 95% CI:(2.5;13.7 cm³/kg), p = 0.004.In summary, fetuses with dextro-transposition of the great arteries had both absolute and relatively larger lung volumes than those without CHD. No differences were seen in liver and total intracranial volume. Despite the small number of cases, the results are interesting and warrant further investigation.

Objective: Our objective was to assess the predictive value of physiologic dead space fraction for mortality in patients undergoing the comprehensive stage 2 operation.

Methods: This was a single-centre retrospective observational study conducted at a quaternary free-standing children's hospital specialising in hybrid palliation of single ventricle cardiac disease. 180 patients underwent the comprehensive stage 2 operation. 76 patients (42%) underwent early extubation, 59 (33%) standard extubation, and 45 (25%) delayed extubation. We measured time to extubation, post-operative outcomes, length of stay and utilised Fine gray models, Youden's J statistic, cumulative incidence function, and logistic regression to analyse outcomes.

Results: Delayed extubation group suffered significantly higher rates of mortality (31.1% vs. 6.8%), cardiac arrest (40.0% vs. 10.2%), stroke (37.8% vs. 11.9%), and need for catheter (28.9% vs. 5.1%) and surgical intervention (24.4% vs. 8.5%) (P < 0.001). Physiologic dead space fraction was significantly higher in the delayed extubation group and in non-survivors with a value of 0.3, which was found to be the discriminatory point by Youden's J statistic. For a 0.1 unit increase in physiologic dead space fraction on post-operative day 1, the odds of a patient expiring increase by a factor of 2.26 (95% CI 1.41-3.97, p < 0.001) and by a factor of 3.79 (95% CI 1.65-11.7, p 0.01) on post-operative day 3.

Conclusions: Delayed extubation impacts morbidity and mortality in patients undergoing the comprehensive stage 2 operation. Increased physiologic dead space fraction in the first 60 hours after arrival to the ICU is associated with higher mortality.

Background: Children with left aortic arch and aberrant right subclavian artery may present with either respiratory or swallowing symptoms beyond the classically described solid-food dysphagia. We describe the clinical features and outcomes of children undergoing surgical repair of an aberrant right subclavian artery.

Materials and methods: This was a retrospective review of children undergoing repair of an aberrant right subclavian artery between 2017 and 2022. Primary outcome was symptom improvement. Pre- and post-operative questionnaires were used to assess dysphagia (PEDI-EAT-10) and respiratory symptoms (PEDI-TBM-7). Paired t-test and Fisher's exact test were used to analyse symptom resolution. Secondary outcomes included perioperative outcomes, complications, and length of stay.

Results: Twenty children, median age 2 years (IQR 1-11), were included. All presented with swallowing symptoms, and 14 (70%) also experienced respiratory symptoms. Statistically significant improvements in symptoms were reported for both respiratory and swallowing symptoms. Paired (pre- and post-op) PEDI-EAT-10 and PEDI-TBM-7 scores were obtained for nine patients, resulting in mean (± SD) scores decreasing (improvement in symptoms) from 19.9 (± 9.3) to 2.4 (± 2.5) p = 0.001, and 8.7 (± 4.7) to 2.8 (± 4.0) p = 0.006, respectively. Reoperation was required in one patient due to persistent dysphagia from an oesophageal stricture. Other complications included lymphatic drainage (n = 4) and transient left vocal cord hypomobility (n = 1).

Conclusion: Children with a left aortic arch with aberrant right subclavian artery can present with oesophageal and respiratory symptoms beyond solid food dysphagia. A thorough multidisciplinary evaluation is imperative to identify patients who can benefit from surgical repair, which appears to be safe and effective.

Introduction: Ductal stenting in late presenters with transposition of great arteries with intact ventricular septum retrains the left ventricle before arterial switch operation. However, the experience is limited for its efficacy and safety. This study aims to highlight the efficacy and safety of ductal stenting for retraining the left ventricle.

Methods: Eight children with transposition of great arteries-intact ventricular septum and regressed left ventricle underwent ductal stenting. Serial echocardiographic measurements of left ventricle shape, mass, volume, free wall thickness, and function were done, and arterial switch operation was performed once the left ventricle was adequately prepared. Post-operative outcome in terms of duration of mechanical ventilation, ICU stay, and improvement in left ventricle function were monitored.

Results: The procedure was successful in all patients. Babies were divided into two groups on basis of age at ductal stenting (group 1 age less than 90 days and group 2 age more than 90 days) and were evaluated for the degree of left ventricle retraining as evidenced by echocardiographic parameters and post-operative variables. The left ventricle posterior wall thickness and mass index after ductal stenting increased significantly in both the groups. Postoperatively, one baby of group two expired after seven days due to severe left ventricle dysfunction. Rest babies had an uneventful post-operative ICU stay with no statistical difference in the duration of invasive mechanical ventilation or ICU stay. On six-month follow-up, all surviving babies were doing well with normal left ventricle function.

Conclusion: Ductal stenting is a good alternative measure as compared to surgical procedures for left ventricle retraining in transposition of great arteries with regressed left ventricle.

Introduction: Thromboembolism is a complication in paediatric patients with CHD requiring cardiac surgery. Previous research has focused on post-operative thromboembolism. This study aimed to describe thromboembolism frequency before or after cardiac surgery in children with CHD.

Methods: We performed a single-centre retrospective study from October 2020 to June 2023 (inclusive). Patients were eligible for inclusion if they were <21 years of age and underwent cardiac surgery. Outcomes of interest included the occurrence and characteristics of thromboembolism in the 12 months before and after surgery, antithrombotic therapies, recurrent thromboembolism, and clinically significant bleeding.

Results: Among 260 patients included, 35 (13.5%) developed an index thromboembolism. Twelve (34.3%) patients had an index thromboembolism <12 months before surgery and 23 (65.7%) had an index thromboembolism <12 months after surgery, including 8 (22.9%) patients who had thromboembolism during both exposure periods. The median interquartile range (IQR) time of thromboembolism relative to cardiac surgery was -26 (-4 to -140) days and 15 (4 to 41) days, respectively. Seven (20%) patients had arterial, 18 (51.4%) venous, and 3 (8.6%) had both arterial and venous thromboembolism. Median (IQR) antithrombotic therapy duration was 49 (24-84) days. Nine (25.7%) patients developed recurrent thromboembolism and five (14.3%) patients experienced clinically significant bleeding.

Conclusions: The risk of thromboembolism and recurrence is high both before and after cardiac surgery among paediatric patients with CHD. Prospective multi-centre studies should seek to identify risk factors for preoperative and postoperative thromboembolism to inform the design of future risk-stratified thromboembolism prevention trials in children with CHD.

Background: Outcomes for children with heart disease improved over the past decades. Quality improvement (QI) research in paediatric cardiac critical care is a key driver of improvement. The availability and variability of QI research across the field is unknown. This project represents a step in understanding the role. The Pediatric Cardiac Intensive Care Society (PCICS) can serve to support institutions' needs, drive collaborations, and utilise available infrastructure at member institutions for improvement work.

Methods: The PCICS Quality Improvement and Safety Committee developed a survey to assess the state of QI research. The survey was disseminated over several months and available via QR code at the World Congress of Pediatric Cardiology and Cardiac Surgery in 2023.

Results: Fifty-eight respondents completed the survey representing at least 38 unique institutions. Most respondents participated in QI research (52/58, 90%). Most QI projects were single centre (41% of respondents), and of those, the majority were from a minority of institutions (13 institutions [34% of total institutions]). QI support is available at slightly more than half of units, and 55% (32/58) have access to a QI specialist. QI support and rate of publications is significantly lower for small/medium units as compared to large units. Respondents suggested most interest from PCICS in networking with other members with similar project ideas (50/58, 86%).

Conclusion: PCICS member institutions are committed to QI research, with limitations in support, local specialists, and networking. Increasing connectivity and accessibility to QI resources may reduce burden to individual members and institutions to achieve QI research.

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.

We present a neonatal case of interventricular septal aneurysm associated with right coronary artery fistula. This report is the first to document such a neonatal case, highlighting the importance of early diagnosis and surgical intervention.