Pediatric Cardiology最新文献

To evaluate the association between initial management strategy of neonatal symptomatic Tetralogy of Fallot (sTOF) and later health-related quality of life (HRQOL) outcomes. We performed a multicenter, cross-sectional evaluation of a previously assembled cohort of infants with sTOF who underwent initial intervention at ≤ 30 days of age, between 2005 and 2017. Eligible patients' parents/guardians completed an age-appropriate Pediatric Quality of Life Inventory, a Pediatric Quality of Life Inventory Cardiac Module Heart Disease Symptoms Scale, and a parental survey. The association between treatment strategy and HRQOL was evaluated, and the entire sTOF cohort was compared to published values for the healthy pediatric population and to children with complex congenital heart disease and other chronic illness. The study cohort included 143 sTOF subjects, of which 59 underwent a primary repair, and 84 had a staged repair approach. There was no association between initial management strategy and lower HRQOL. For the entire cohort, in general, individual domain scores decreased as age sequentially increased. Across domain measurements, mean scores for the sTOF cohort were significantly lower than the healthy pediatric population and comparable to those with other forms of complex CHD and other chronic health conditions. The presence of a genetic syndrome was significantly associated with a poor HRQOL (p = 0.003). Initial treatment strategy for sTOF was not associated with differences in late HRQOL outcomes, though the overall HRQOL in this sTOF cohort was significantly lower than the general population, and comparable to others with chronic illness.

Coronary events are life-threatening long-term complications of the arterial switch operation for complete transposition of the great arteries. The aim of our study was to assess the dimensions of the reimplanted coronary arteries and their relationship with the various geometric characteristics to gain a better understanding of the involved mechanisms. Coronary computed tomography angiography (CCTA) scans of 78 asymptomatic pediatric patients were performed at the age of 10.7 (6.3-17.8) years. The position of the ostia, the branching angles, and the diameters of the coronary arteries were determined in a subgroup of 51 patients presenting the usual preoperative coronary anatomy. Mean Z-score of the left main coronary artery (LMCA), left anterior descending coronary artery (LAD), left circumflex coronary artery (LCX), and right coronary artery (RCA) diameters were 0.7 ± 1.2, - 0.4 ± 1.1, - 0.3 ± 1.1, and - 0.3 ± 1.1, respectively. The Z-score of the ostial LMCA diameter had a positive correlation with the remoteness from the main pulmonary artery (p < 0.001) and the branching angle (p = 0.001). The ostial diameter Z-score of the LMCA had a negative correlation with the interval between the arterial switch operation and the CCTA (p = 0.004). Even though most of the coronary diameters fell within the accepted range, which suggests normal overall development, acute branching angle and more anterior origin were associated with smaller ostial coronary artery diameter Z-scores. To prove the clinical relevance of the smaller ostial diameter of high-risk left coronary arteries and the decrease of ostial coronary artery Z-scores over time needs further follow-up studies.

There is a paucity of literature describing long-term outcomes of patients with coronary artery fistula with most manuscripts focusing on those requiring interventions. We describe single-center outcomes of coronary artery fistulas including those not requiring intervention. We performed a retrospective review of the electronic medical record and identified all patients with a diagnosis of coronary artery fistula over the last 10 years. 158 patients were identified with a coronary artery fistula. The mean age at diagnosis was 5.8 years (SD ± 5.9). There was a male (55%, n = 87) predominance. Concomitant congenital heart lesion was present in 49% (n = 77) and a genetic anomaly was found in 18% (n = 29). No ischemic changes on electrocardiogram or ECG-stress test were observed. The mean follow-up was 5.0 (SD ± 3.8) years. Most patients (94%, n = 149) did not undergo an intervention. Of those 63% (n = 94) had at least one follow-up echocardiogram. There was spontaneous coronary artery fistula closure in 44% (n = 41), 8% (n = 8) decreased in size, and 48% (n = 45) were unchanged. No patient had enlargement of the coronary artery fistula over time. Additionally, tiny and small coronary artery fistulas showed no significant clinical changes in coronary artery dimensions, left ventricle dimensions and function over time. Seven patients required intervention; two patients underwent surgical ligation and five underwent catheter-based intervention. Most patients with coronary artery fistula in our cohort did not require intervention and over half either closed spontaneously or decreased in size with routine follow-up.

The diagnosis of coarctation of the aorta (CoA) prior to birth can be challenging due to the physiologic changes during postnatal transition. Prenatal risk stratification can standardize postnatal management and improve outcome. CT Children's Fetal Cardiology created the Antenatal Risk of CoA in Hartford (ARCH) clinical pathway defining four distinct postnatal order sets based on degree of suspicion for ductal dependency on fetal evaluation: low, low-moderate, moderate-high, and high risk. This study aims to evaluate safety and efficacy of the ARCH pathway in neonates with suspected CoA. This study was a single-center, retrospective chart review evaluating maternal-infant dyads with findings concerning for CoA between July 2004 and July 2021, before and after ARCH pathway implementation. Neonates were evaluated for the presence or absence of critical CoA and postnatal clinical data were collected. Statistical analysis was performed using chi square and Fisher's exact test. There were 108 maternal-infant dyads studied, comprising 53 non-pathway patients and 55 ARCH pathway participants. Thirty-three neonates had critical CoA, comprising 23 non-pathway and 10 ARCH pathway subjects. Patients categorized in the high-risk group were highly associated with critical CoA (P = 0.003). Non-pathway neonates with CoA demonstrated higher likelihood of hospital transfer compared to ARCH pathway neonates (56.5% vs. 10.0%, P = 0.021). NICU admission, prostaglandin administration, and intubation were not significantly different between before and after ARCH implementation (P < 0.05). More echocardiograms were performed in ARCH pathway neonates without CoA than their non-pathway counterparts (1.586 vs. 2.133, P = 0.049). The ARCH pathway is a safe, reliable prenatal risk stratification system to help guide management of patients with critical CoA. These results identify effective targets of modification to the pathway to reduce resource utilization without compromising safety.

The incidence of myocarditis and pericarditis has been documented in adolescents after COVID-19 vaccinations. This study aims to assess the risk of myopericarditis in adolescents following COVID-19 vaccination, using a meta-analysis of the published cases. We performed a comprehensive literature search of the following databases on July 5, 2023: MEDLINE, EMBASE, PubMed, and the Cochrane Library. We performed a meta-analysis using a random-effects model to estimate the incidence of myopericarditis per million of administered COVID-19 vaccine doses or COVID-19 infections. A total of 33 studies were included in the meta-analysis. The incidence of myopericarditis per million COVID-19 infections (1583.9 cases, 95% CI 751.8-2713.8) was approximately 42 times higher than that for COVID-19 vaccine administrations (37.6 cases, 95% CI 24.2-53.8). The risk of myopericarditis after COVID-19 vaccination was particularly high among the 16-19 age group (39.5 cases per million, 95% CI 25.8-56.0), males (43.1 cases per million, 95% CI 21.6-71.9), and those who received the second dose (47.7 cases per million, 95% CI 22.2-82.2). There were no significant differences in the incidence of myopericarditis per million COVID-19 vaccine administrations between Europe, the Western Pacific, and the Americas (p = 0.51). Adolescents faced a potential risk of myopericarditis after COVID-19 vaccination, but this risk is less harmful than that of myopericarditis following COVID-19 infection.

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 6 months [0-99 months]. The mean follow-up after surgery was 22.7 years [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.

This retrospective study assessed the efficacy of initial treatment with intravenous immunoglobulin (IVIG), aspirin, and corticosteroids in Kawasaki disease (KD) patients at risk for IVIG resistance. Data from pediatric patients with KD between January 2010 and December 2019 were divided into the IVIG-sensitive and IVIG-resistant groups based on treatment response. Risk factors for IVIG resistance were identified through univariate analysis. Outcomes included comparison of fever resolution time, hospital stay, and coronary artery lesion (CALs) incidence at multiple intervals post-treatment. An observational group (January 2020 to December 2022) was given additional methylprednisolone if they exhibited three or more IVIG resistance risk factors. The IVIG-resistant group had a higher proportion of males and significantly elevated inflammatory markers (CRP, ESR, PCT) and TBIL, NT-proBNP compared to the IVIG-sensitive group (all p < 0.05), while showing lower levels of albumin, ARR (AST/ALT ratio), and serum sodium (all p < 0.05). Both the IVIG-sensitive and observational groups had significantly shorter fever resolution times and hospital stays than the IVIG-resistant group (all p < 0.05). CALs incidence was consistently higher in the IVIG-resistant group across all follow-up periods (all p < 0.05), with significant differences observed between the observational group and IVIG-resistant group at 1-month, 3-month, and 6-month post-treatment (all p < 0.05). Notably, prior to treatment, the observational group had a higher CALs incidence compared to the IVIG-sensitive group (all p < 0.05). In children with KD who exhibit ≥ 3 risk factors for IVIG non-response, initial treatment with IVIG, aspirin, and glucocorticoids can effectively reduce fever duration, hospitalization duration, and incidence of CALs and is considered safe.

Cardiac-Urogenital Syndrome (CUGS) is a recently identified genetic disease characterized by urogenital, diaphragmatic, ophthalmic, and cardiac abnormalities caused by heterozygous pathogenic variants in the Myelin Regulatory Factor (MYRF) gene. The complete spectrum of disease characteristics and prevalence is not yet defined. This report documents the first known cases of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in MYRF-associated Cardiac-Urogenital Syndrome (MYRF-CUGS).

Studies have suggested that pediatric patients with heart transplants (HT) due to congenital heart disease (CHD) perform differently on cardiopulmonary exercise testing compared to pediatric patients with HT due to cardiomyopathy (CM). However, it is not known if this relationship changes over time. The aim of this study was to examine the differences in cardiopulmonary exercise test (CPET) parameters over time between patients with HT due to CHD versus CM. A large single-institution CPET database was used for this study. We conducted a retrospective cohort study of 250 total CPETs from 93 unique patients, examining how patients with HT due to CHD (109 CPETs, 40 unique patients) differed in CPET performance from patients with HT due to CM (141 CPETs, 53 unique patients) from < 2 years post-HT, 2 to < 6 years post-HT, and ≥ 6 years post-HT. There were no differences between patients with HT due to CHD compared to CM in CPETs performed < 2 years post-HT. In CPETs performed 2 to < 6 years post-HT, the CM group had higher maximal HR and percentage of age-predicted maximal heart rate (APMHR) achieved. At ≥ 6 years post-HT, the CM group continued to have higher maximal HR and percentage of APMHR achieved, but also improved HR recovery at one minute. Initial indication for transplant may affect performance on CPETs post-transplant. Patients with HT due to CM have improved chronotropic measures compared to patients with HT due to CHD and these differences are more pronounced with increased time post-HT.

Studies have shown that respiratory muscle training enhances functional capacity and pulmonary function in Fontan patients. However, diaphragm muscle characteristics in Fontan children have not been fully elucidated. The aim of this study was to compare respiratory function, maximal and submaximal functional capacities, and quality of life, as well as to assess diaphragm mobility and thickness, between Fontan patients aged 8 to 12 years and healthy individuals. This cross-sectional study included 45 children-27 Fontan patients, Fontan Group (FG) and 18 healthy control Group (CG) subjects. Different examinations were performed: spirometry was used to analyze pulmonary volume and capacity; manovacuometry was used to determine respiratory muscle strength; chest ultrasound was used to determine diaphragm muscle characteristics; cardiopulmonary exercise and the six-minute walk test (6MWT) were used to determine functional capacity; the AUQEI questionnaire was used to determine quality of life. Descriptive analysis and intergroup comparisons were performed for all the data. Compared with the CG, the FG exhibited impaired pulmonary function and reduced functional capacity. Significant differences in median values were noted for forced expiratory volume in one second (FEV₁): 2.39 L/min, p = 0.002; forced vital capacity (FVC): 1.73 vs. 3.06 L/min, p = 0.002; maximal inspiratory pressure: - 73 vs. - 117 cmH₂O, p = 0.007; absolute peak VO₂: 1.09 vs. 1.51 L/min, p < 0.001; relative peak VO₂: 31.9 vs. 42.5 mL/kg/min, p = 0.003; and 6MWT distance: 420 vs. 586 m, p < 0.001. Diaphragmatic thickness, mobility and quality of life were similar between the groups. Despite the reduced functional capacity, impaired pulmonary volume and capacity, and respiratory muscle weakness of the FG compared to those of the CG, the diaphragm characteristics and quality of life were similar between the groups.