The article "The Influence of Arrhythmia on the Outcomes of Pediatric Patients with Idiopathic Dilated Cardiomyopathy" by Mohammad Dalili et al. provides valuable insights into the impact of arrhythmia on idiopathic dilated cardiomyopathy outcomes. However, it presents with certain limitations. The study does not consider the link between arrhythmia outcomes and key confounding factors, which can potentially impact the outcome at hand. Moreover, the follow-up interval of year potentially limits the understanding of the longer term implications of arrhythmia in pediatric idiopathic dilated cardiomyopathy. Future research should consider the interplay between these confounding factors and arrhythmias associated with dilated cardiomyopathy, and implement longer follow-up intervals.

The Catheterization RISk Score for Pediatrics (CRISP) has been validated and widely adopted as a model to predict adverse outcomes. We sought to determine if the addition of three modifiers (NYHA Class > 2, prior sternotomies > 2, and BMI > 30) to the CRISP score would better predict adverse events (AE) in adults undergoing cardiac catheterization at our institution. All adults (> 18years) who underwent cardiac catheterization at the Children's Hospital Colorado from November 2016 to November 2021 were included. Baseline and modified CRISP scores with adult modifiers and AEs were analyzed. Of a total of 401 cases, there were 27 AEs (6.7%). A higher CRISP score was associated with an increased risk of AEs (p = 0.004). A ROC analysis of the CRISP score gave a AUC of 66% with a cut-off point of 5.5 giving a sensitivity of 74% and specificity of 52%. The modifiers individually did not significantly correlate with AEs. Higher modified CRISP score was similarly associated with an increased risk of AEs (p = 0.01). ROC analysis for the modified CRISP score gave an AUC of 64%. A cut-off point of 6.5 gave the best result with a sensitivity of 59% and specificity of 61%. A modified CRISP score which included adult modifiers similarly predicts AEs in adults with congenital heart disease. These adult modifiers are easy to incorporate into the CRISP score. The CRISP score also predicted AEs in adults with congenital heart disease.

Patients with a Fontan circulation are at risk for chronic kidney disease (CKD), which is defined as persistently reduced glomerular filtration rate (GFR) <60ml/min/1.732 or elevated marker of kidney injury such as urinary albumin-to-creatinine ratio (UACR) >30mg/g. We determined the prevalence of albuminuria in patients with a Fontan circulation. The MEDLINE, EMBASE, Trip, and Cochrane databases were searched for studies reporting the prevalence of albuminuria in Fontan patients. Case reports, reviews, and univentricular patients pre-Fontan completion or post-heart transplantation were excluded. Studies were assessed for potential confounders and measurement, patient selection, intervention, and reporting biases, reported in a table. After systematic review, the pooled prevalence of albuminuria was calculated using the quality effects model for meta-analysis. Secondary outcomes were the clinical determinants of albuminuria. Thirteen studies were included in the systematic review, of which 11 were included in the meta-analysis (6 prospective, sample size per study 25-195 patients, 873 patients in total). The pooled prevalence of albuminuria was 28.4% (95% confidence interval 23.5-33.5%). GFR was mostly preserved in these patients. Albuminuria was associated with elevated systemic venous pressure in 5 studies. Other associations were inconclusive. The main limitations of our study are the predominantly retrospective and cross-sectional nature of the included studies with small sample sizes and heterogeneous study populations. Our findings show albuminuria is more prevalent than reduced GFR in patients with a Fontan circulation, implicating the potential value of UACR in addition to GFR when screening for CKD in these patients.

This article provides a historical review and a current perspective on the procedures used to palliate cyanosis in ductal-dependent infants. Eighty years ago, Helen Taussig, Alfred Blalock, and Vivien Thomas developed the first effective treatment. The Blalock-Taussig-Thomas (BTT) shunt is the historical predecessor of both the contemporary-modified BTT shunt and interventional stenting of the Patent Ductus Arteriosus (PDA). The surgical shunt was firmly established therapy before catheterization was born, and PDA stenting was not possible until the technologies designed to address coronary heart disease were developed. Coronary stents and guidewires are currently used in all cases of PDA stenting. The momentum of long-established therapy and the lack of a purpose-built technology have inhibited clinical adoption of PDA stenting. Nevertheless, available clinical outcomes, though limited, appear to favor PDA stenting, and current first-line therapy may be shifting from the modified BTT shunt to PDA stenting. More definitive data should arise from a randomized controlled trial.

To determine: (i) frequency of probable post-traumatic stress disorder (PTSD) in mothers of infants after complex cardiac surgery (CCS), (ii) predictors of probable PTSD, and (iii) impact on child neurodevelopment. The Impact of Event Scale-Revised (IES-R) was administered to 60 mothers of infants ≥ 6months after CCS at ≤ 6weeks of age. The IES-R measures response to a specific traumatic event: Scores < 24, no concern; 24-32, clinical concern; and ≥ 33, probable diagnosis of PTSD. Post-survey childhood outcomes obtained at 21-months used Bayley Scales of Infant and Toddler Development-Third Edition. Multiple logistic and linear regressions were used to predict high IES-R scores from peri-operative and demographic variables and determine association between maternal IES-R scores and outcomes, reported as Odds Ratio (OR) and Effect Size (ES) with 95% Confidence Intervals (95% CI). IES-R scores ≥ 33 occurred in 14/60 (23%) of mothers. Significant risk factors for IES-R ≥ 33 were days of ventilation after first surgery, OR 1.149 (95% CI 1.037, 1.273), p = 0.008, and birth weight z-scores, OR 0.352 (0.140, 0.881), p = 0.026. Bayley cognitive, language, and motor scores were significantly lower for children whose mothers had IES-R ≥ 33. The IES-R was independently associated with cognitive, ES -0.23 (95%CI -00.39, -00.08), p = 0.036 and language, ES -00.17 (95%CI -00.33, -00.06), p = 0.043 scores. Probable PTSD occurred in 23% of mothers ≥ 6month after discharge of their infant following CCS. Toddlers of mothers with probable PTSD had lower cognitive and language scores suggesting a relation between PTSD and development requiring further study.

The present study is the first meta-analysis comparing long-term outcomes in patients undergoing correction for anomalous left coronary artery (ALCAPA) regarding concomitant mitral valve surgery. A systematic literature review was conducted to identify all relevant studies with comparative data on mitral valve surgery performed during surgery for ALCAPA correction. Predefined primary end points included mortality and mitral valve (re)operation. Echocardiographic outcomes were assessed when available (including severity of mitral regurgitation). Fifty-three relevant retrospective studies with comparative data for ALCAPA patients with concomitant mitral valve surgery were identified from the existing literature. The study population included 3,851 patients, of whom 55% were girls. The mean age at surgery was 6months [0-99months]. The mean follow-up after surgery was 22.7years [6.3-37.2]. Among patients with available echocardiographic data at the time of ALCAPA correction, 283 had no mitral regurgitation (13%), 642 had mild mitral regurgitation (30%), 756 had moderate mitral regurgitation (36%), and 437 had severe mitral regurgitation (21%). A subgroup of patients who underwent mitral valve surgery (yMVS; n = 753; 19.5% of the total population) was identified. A total of 228 patients died during follow-up (5.9%), with no differences among patients who underwent vs those who did not undergo mitral valve concomitant surgery at baseline (p = ns). Among the 1,536 patients with available follow-up echocardiographic data, 519 had no mitral regurgitation (34%), 663 had mild mitral regurgitation (43%), 268 had moderate mitral regurgitation (17%), and 86 had severe mitral regurgitation (6%). Overall, during follow-up, 91 patients underwent mitral valve surgery (first MVS for 20 pts and reintervention for 71 pts, representing a 10% reintervention rate). In regression analysis, we found no association between the mean age at first surgery and the need for reoperation (p = 0.458). Mitral valve surgery is performed in about 20% of patients undergoing ALCAPA correction. Indications for MVS vary among centers, although the severity of MV regurgitation was the most common criterion (more than 50% of the yMVS group). Of yMVS patients, approximately 10% underwent a second MVS during follow-up, regardless of age at first surgery. Overall, long-term mortality in these patients is over 5%, with no evident impact related to concomitant MV surgery.

Quantification of right ventricular (RV) size and function after tetralogy of Fallot repair is critical for determining timing of reintervention and outcomes. Tetralogy of Fallot patients with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCAs) are a unique group in which the RV is subjected to various loading conditions, allowing for direct comparison. Retrospective evaluation of RV echocardiographic indices in repaired pediatric TOF/PA/MAPCAs patients (2/2002 - 4/2018). Patients were categorized as having conduit stenosis (peak gradient ≥ 2.5m/s) and/or distal pulmonary artery (PA) stenoses (requiring catheter or surgical intervention and > 1/2 systemic RV pressures). A comparison group (N = 9) included distinct age-matched repaired patients without residual lesions. Indexed right heart dimensions were larger in patients with distal PA stenoses (N = 49) compared to conduit stenosis (N = 26), including RV end-diastolic and end-systolic dimensions (p < 0.01), despite earlier time to reintervention (p < 0.0001). RV fractional area change (FAC) was diminished in patients with distal PA stenoses compared to patients without residual lesions (p = 0.006). Patients with conduit stenosis demonstrated a marked decrement in tricuspid annular plane systolic excursion (TAPSE) z-scores. Repaired TOF/PA/MAPCAs patients with residual distal PA stenoses have larger right heart dimensions compared to patients with conduit stenosis, and decreased RV FAC compared to patients without residual lesions at 1-2years after repair, suggesting early surveillance and immediate intervention on stenotic PA segments appears warranted to decrease the risk of further deterioration of RV systolic function.

During surgery for oval fossa defects, inadvertent suturing of pericardial patches to a prominent Eustachian valve diverts the inferior vena caval blood to the left atrium causing iatrogenic cyanosis. A similar complication may arise after surgery for superior sinus venosus defects. Surgical patch in a wrong plane may direct the superior vena cava towards the left atrium. Early post-operative diagnosis leads to immediate correction. Diagnosis delayed beyond many years lead to chronic hypoxemia and secondary polycythemia. This report details the clinical presentation and imaging of this iatrogenic complication in three patients and explains a transcatheter corrective intervention.

Fetoscopic endoluminal tracheal occlusion (FETO) induces lung growth and may improve survival in congenital diaphragmatic hernia (CDH) but the effect on post-natal right (RV) and left (LV) ventricular size and cardiac function is unknown. Quantitative measures of heart size and function including tricuspid annular plane systolic excursion Z-score (TAPSEZ), RV fractional area change (RVFAC), RV global longitudinal and free wall strain (RVGLS, RVFWS), RV/LV ratio, LV eccentricity index (LVEI), and LV M-mode diastolic and systolic Z-scores (LVIDDZ, LVIDSZ) were compared between FETO and control patients on first post-natal echocardiogram, prior to and post CDH repair, and on last available echocardiogram using non-parametric Wilcoxon rank-sum test in a single-center, retrospective cohort study. Linear regression models evaluated change over time, adjusting for clustering and interaction of echocardiogram parameters with time. Thirty-two patients (10 FETO, 22 control) met inclusion criteria. At first echocardiogram, FETO patients demonstrated lower RV/LV ratio and LVEI (p = 0.01 for both) indicating less RV dilation and less ventricular septal displacement, respectively. LV hypoplasia was less severe in FETO patients (p = 0.01 for both LVIDDZ and LVIDSZ) initially. After repair, FETO patients demonstrated better RV systolic function compared to control patients by FAC (p < 0.01), RVGLS (p = 0.02), and RVFWS (p = 0.05). Over time, FETO patients demonstrated greater improvements in RV/LV ratio and LVEI but smaller increases in LV dimensions compared to control patients. Improvements in RV function were similar between the groups. FETO patients demonstrate differences in cardiac size and function compared to control patients.