Pediatric Cardiology最新文献

Background: Congenital Heart Disease stands as a prominent cause of infant mortality, with notable disparities in surgical outcomes evident between high-income and low- to middle-income countries.

Objective: This study presents a collaborative partnership between a local governmental entity and an international private organization to establish a high-quality Pediatric Cardiac Surgery Program in a post-conflict limited resource country, Iraq.

Methods: A descriptive retrospective study analyzed pediatric cardiac surgery procedures performed by a visiting pediatric heart surgery team from October 2021 to October 2022, funded by the Ministry of Health (MOH). We used the STS-EACTS complexity scoring model (STAT) to assess mortality risks associated with surgical procedures.

Results: A total of 144 patients underwent 148 procedures. Infants comprised 58.3% of the patients. The most common anomalies included tetralogy of Fallot, ventricular septal defect, and various single ventricle categories, constituting 76% of the patient cohort. The overall surgical mortality rate was 4.1%, with an observed/expected surgical mortality rate of 1.1 (95% CI 0.5, 2.3). There was no significant difference between our observed surgical mortality in Category 2, 3, and 4 and those expected/reported by the STS-EACTS Database (p = 0.07, p = 0.72, and p = 0.12, respectively). The expenses incurred by the MOH for conducting surgeries in Iraq were lower than the alternative of sending patients abroad for the same procedures.

Conclusion: The partnership model between a local public entity committed to infrastructure development and funding and an international private organization delivering clinical and training services can provide the foundation for building sustainable, high-quality in situ programs in upper-middle-income countries.

Traditional cardiovascular risk factors put patients with congenital heart disease (CHD) at increased risk for cardiovascular morbidity and mortality. The aim of this study was to evaluate whether body mass index (BMI) is associated with health-related quality of life (HRQoL) in patients with variants of Tetralogy of Fallot (TOF). Patients and parents of children with variants of TOF-CHD were asked to fill out the PedsQL 4.0 questionnaire and provide weight and length. Patients were categorized into low, normal, and high BMI percentiles. Other demographic data were obtained from the Swedish national registry for congenital heart disease (SWEDCON). Statistical analyses included non-parametric Mann-Whitney U test, Fisher exact, and Chi-square tests. Eighty-five patients were included. Twelve were overweight or obese, 57 had a normal BMI, and 16 were underweight. There was a significant difference in age and gender between the groups. Comparing overweight/obese children to those with normal BMI, physical and social functioning were impaired, while emotional and school function were comparable between the groups. This applied to both child and parental assessment. When comparing underweight to normal weight children, school functioning assessed by the parent was the only domain significantly different from patients with a normal BMI. Children with variants of TOF and overweight/obesity have lower HRQoL, particularly in physical and social functioning, while underweight children may have impaired school functioning. We suggest that preventive measures aimed at maintaining a normal weight should be taken early in life to reduce long-term cardiovascular risk in the CHD population.

Becker muscular dystrophy (BMD) is an X-linked recessive disorder responsible for mild skeletal muscle involvement and variable degree of cardiomyopathy. The characteristics of cardiac phenotype of BMD in childhood remain elusive. Clinical manifestations, genotype, serum biomarkers, and echocardiogram were retrospectively reviewed in BMD patients. Cardiac phenotype was classified into acute progressive (AP), chronic persistent (CP), and latent (L) groups based upon symptoms and echocardiographic findings. Twenty-five BMD patients were studied over 9.5 ± 2.5 years. Sixteen patients presented initially with variable degree of muscle weakness whereas 9 were asymptomatic. Three patients developed medically refractory heart failure by age 18 with progressive dilated cardiomyopathy (DCM) (AP). Six patients developed mild to moderate left ventricular (LV) systolic dysfunction with LV dilatation but remained asymptomatic (CP). Although 16 patients continued to show normal LV function (L), they demonstrated variable degrees of skeletal muscle involvement. The AP groups presented with significantly larger LV size and LV mass index (LVMI) at the initial encounter than groups CP or L, suggesting early myocardial remodeling predicts rapid disease progression. None presented with atrophic myocardial phenotype commonly observed in Duchenne muscular dystrophy (DMD). Wide availability of genetic testing has changed the scope of clinical presentation of BMD. Cardiomyopathy in BMD presents with a diverse clinical spectrum with variable progression of DCM where larger LV dimension and mass at the time of diagnosis may predict the progressiveness of cardiomyopathy.

Approximately 1% of all live births in the USA are affected by congenital heart disease (CHD), the leading cause of congenital defect-related illness and infant death. Although technological innovations have improved CHD diagnosis in utero, variation among fetal cardiac counseling practices persists. Our study aims to evaluate physician counseling content based on cardiac defect complexity. We conducted an anonymous survey of providers who perform fetal cardiac counseling. Participants stated their counseling practices for 21 CHD lesions, choosing from postnatal surgical intervention, termination of pregnancy, and/or postnatal palliative comfort care. CHD lesions were divided by severity into three groups. Chi-square tests were performed to assess whether counseling content varied based on provider response to the statement "some life is always better than no life at all." There were 138 respondents, with postnatal surgical intervention counseling frequency consistent among all lesions. Inclusion of termination and palliative/comfort care in counseling increased in frequency as disease severity increased, with the former being counseled more frequently. For tetralogy of Fallot (p = 0.02), interrupted aortic arch (p = 0.03), hypoplastic left heart syndrome (p = 0.03), and pulmonary atresia with intact ventricular septum (p = 0.03), those providers who disagreed with the statement that "some life is always better than no life at all" were more likely to counsel termination of pregnancy and/or palliative care compared to their counterparts. Our study found that the interventions and options included in fetal cardiac counseling vary based on complexity. Moreover, physicians' personal beliefs tended to influence counseling practices in some moderate or severe defects.

L-transposition of the great arteries (L-TGA) represents a spectrum of congenital heart defects (CHD) associated with atrioventricular block (AVB). However, the incidence and prognosis of postoperative AVB among patients with variants of L-TGA is uncertain. Assess the incidence and risk factors for postoperative AVB requiring permanent pacemaker (PPM) implantation for pediatric patients with L-TGA undergoing cardiac surgery. Single-center retrospective analysis of patients with L-TGA who underwent cardiac surgery from 2000 to 2022. Patients with postoperative AVB and those requiring PPM implantation were compared to those who did not receive a PPM. 161 cardiac surgeries were performed in 75 patients with L-TGA. Postoperative AVB occurred in 8 cases (5%) with 5 cases (3%) requiring PPM. Univariate analysis identified risk factors for PPM which included ventricular septal defect (VSD) intervention [odds ratio (OR) 58.4, p < 0.01], pre-operative non-single-ventricle physiology (OR 13.3, p = 0.02), and longer pre-operative PR interval Z-score (OR 3.3, p < 0.01). Excluding biventricular conversions, no surgery (n = 116) in patients with univentricular circulation required a PPM in the postoperative period due to AVB. Multivariate analysis did not identify any statistically significant risk factors for AVB requiring PPM. The overall risk of postoperative AVB in L-TGA was 5% despite the anatomic vulnerability of the conduction system. However, L-TGA patients appear less likely to recover AV conduction than all comers to CHD surgery (38% L-TGA vs 60% overall, p < 0.01) and may be considered for earlier PPM placement.

We reviewed the outcomes of truncus arteriosus repair (primary vs. staged repair incorporating bilateral pulmonary artery banding), focusing on survival, reintervention, and functional data. We analyzed 39 patients who underwent a first intervention for truncus arteriosus (staged, n = 19; primary, n = 20) between 1992 and 2022. The median follow-up period was 8.0 (2.2-13.2) years. Survival, freedom from reoperation, and freedom from catheter intervention were estimated using the Kaplan-Meier method. High-risk patients were defined as those with a weight ≤ 2.5 kg, ≥ moderate truncal valve regurgitation, interrupted aortic arch, or preoperative shock. In the staged group, patients with a median weight of 2.6 kg had a median intensive care unit stay of 5 days and no hospital mortality after bilateral pulmonary artery banding. At repair, the staged group had a larger conduit for the right ventricular outflow tract (14 vs. 12 mm; P = .008). Catheter intervention on the branch pulmonary artery was required in 67% of patients in the staged group, but right ventricular end-diastolic pressure at follow-up was comparable between the groups (P = .541). Survival rates were higher among high-risk patients in the staged group (87.5% vs. 21.4% at 15 years; P = .004) but were comparable between groups for standard-risk patients (P = 1.000). Bilateral pulmonary artery banding was a safe, effective procedure. Reintervention for branch pulmonary artery was common but did not affect functional outcomes. Staged repair may play a pivotal role regarding survival in high-risk patients, and risk stratification is vital.

Research establishing factors associated with duration of mechanical ventilation after Tetralogy of Fallot repair, is mainly based on population presenting at early infancy. There are fewer reports regarding repair after infancy, during childhood and preadolescence. To compare two groups of late TOF repair based on post-operative invasive mechanical ventilation duration and explore associations with pre-operative clinical markers of severity of right ventricular outflow tract obstruction. A single-center retrospective cohort study based on medical charts review of patients older than 12 months who underwent primary complete TOF repair between 2017 and 2023. Patients younger than 12 months or those who had distinct anatomical variants were excluded. Pre-operative characteristics reflecting the severity of right ventricular outflow tract obstruction were collected, including baseline oxygen saturation, the occurrence of hypercyanotic spells, maximum right ventricular outflow tract pressure gradient. Perioperative variables included repair type, cardiopulmonary bypass and aortic cross-clamp duration, and rates of residual lesions. 280 patients from 27 countries were screened; 181 remained eligible and were significantly older than previously reported. 129 (71%) were extubated early post-operatively, while 52 (29%) had a longer ventilation course. In a multivariate analysis, only baseline oxygen saturation and repair type maintained a significant association with length of mechanical ventilation. In distinctly older patients undergoing TOF repair, those with lower base line saturation undergoing a non-valve preserving repair are more likely to have a longer course of mechanical ventilation. Other clinical markers reflecting the severity of right ventricular outflow tract obstruction were not independently associated with mechanical ventilation duration.

Lead strangulation is a dangerous complication of epicardial pacemaker insertion. This complication has been increasingly highlighted lately. Our institution has recently identified four cases over the past five years. This study's aim was to 1) identify risk factors for strangulation and 2) prospectively screen existing epicardial pacemaker patients for unrecognized strangulation or features that would prompt closer review. Patients known to the pacemaker clinic with epicardial pacemakers inserted from 2005 to 2023 were included. Electronic health records were used to locate all subjects and gather data. Risk factors were identified using Firth's penalized method of logistic regression. Forty-five patients were included, of which four (8.8%) had evidence of strangulation. Posterior-anterior (PA) chest radiographs all demonstrated characteristic looping patterns of the pacing leads, with confirmation on CT angiography. All affected patients underwent revision surgery. Implantation at a weight of less than 6.5 kg was associated with a significantly increased incidence of strangulation (OR 25, P 0.044). Other factors including lead length, presence of structural cardiac disease, and dual-chamber insertion were not statistically significant. No patients who were prospectively screened were found to have strangulation. Children undergoing insertion of a pacemaker early in infancy are at particularly high risk of strangulation and should be closely monitored following surgery. Regular chest radiography (every three years) to screen for this complication is advised. Larger multi-center studies to pool data for this relatively rare complication may help identify other risk factors for strangulation.

The study sought to assess the clinical utility of complete blood count-derived composite scores, suggesting their potential as markers of inflammation and disease severity in Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) with Kawasaki-like features. This retrospective study analyzed data from 71 KD and 73 MIS-C patients and 70 healthy controls. The KD group showed a higher rate of coronary involvement (26.7% vs. 10.9%), while the MIS-C group had a higher intensive care unit (ICU) admission rate (34.2% vs. 2.8%). Platelet counts, lymphocyte counts, mean platelet volume (MPV), MPV/Lymphocyte (MPVLR), and MPV/Platelet (MPVPR) ratios demonstrated the highest specificities in distinguishing MIS-C than KD (84.5%, 83.1%, 91.1%, 88.7%, and 88.7%, respectively). Monocyte counts, MPV, and MPVPR demonstrated the highest specificities to predictive ICU admission in the MIS-C group (83.3%, 89.6%, and 89.6%, respectively). Lymphocyte counts, platelet/lymphocyte ratio (PLR), neutrophil/lymphocyte ratio (NLR), MPVLR, and Systemic Immune-Inflammation Index (SII) parameters were found to have high negative predictive values for predicting KD patients without coronary artery lesions (CALs) (85.7%, 86.1%, 87.1%, 87.1%, and 85.7%, respectively)., Systemic Inflammation Response Index (SIRI), MPVPR, and CRP were independently predictive of ICU admission in the MIS-C group, and lymphocyte count and IVIG resistance were also identified as significant predictors of CALs in the KD group. NLR, MPVLR, MPVPR, and NPR indices effectively differentiate MIS-C from KD and predict ICU admission in MIS-C. NLR, PLR, MPVLR, and SII are valuable in excluding CALs in KD with high negative predictive values. In addition, SIRI and MPVLR were independent predictors of ICU admission in MIS-C, and lymphocyte count was identified as an independent predictor of CALs in KD.