Pediatric Cardiology最新文献

Cardiac troponin is commonly used to screen for cardiac diagnoses in pediatric patients, as it is only released by myocardial tissue. There is limited data regarding high-sensitivity troponin T in pediatric populations and its clinical interpretation. We sought to determine how high-sensitivity troponin values are associated with myocarditis diagnosis. High sensitivity troponin levels were reviewed for pediatric patients at our center from February 2022 to February 2023. Basic demographic and presenting data (including age, gender, body mass index), and diagnoses (cardiac diagnosis, including myocarditis, vs non-cardiac) were compared for patients with elevated initial troponin levels (≥ 12 ng/L) vs. those with non-elevated values. Of the 308 patients included, 91 (29.5%) had elevated hs-cTnT and 45 (14.6%) had a cardiac diagnosis, of whom 8 (2.5%) were ultimately diagnosed with acute myocarditis. There was no meaningful difference in demographic characteristics between the elevated and non-elevated hs-cTnT groups. For patients with diagnosis of myocarditis (n = 8), median peak levels were 506.5 ng/L (182.0 to 1184.0) versus 6.0 ng/L (< 6.0 to 13.5) for those with all other diagnoses (n = 300) (p < 0.001). A high sensitivity troponin cut-off value of 90 ng/dL was established for diagnosis of myocarditis, providing high sensitivity (100%) and specificity of (95%).

Lack of appropriate durable Ventricular Assist Devices (VADs) that can be managed at home for very young children, and long wait times for transplant have led to search for alternate easily reproducible therapies. In the past decade or more, pulmonary artery banding (PAB), as a new indication of an old technology, has been gradually carried out worldwide as an alternative to mechanical circulatory support in children with end-stage left ventricular dilated cardiomyopathy (DCM) complicated with heart failure(HF), and has achieved encouraging early and mid-term outcomes. Technically, PAB is simple, safe, and effective. This is a promising therapeutic strategy, especially in developing countries where heart transplantation is difficult to implement. As a transition before transplantation, and even a potential treatment, PAB brings more options and hope for children with heart failure who are waiting for transplantation and are refractory to drug therapy. This article reviews the past and current situation, the mechanism, the surgical timing, and application prospect of PAB in the treatment of DCM complicated with heart failure.

This study evaluates a novel surgical technique inspired by the hybrid Norwood procedure, which involves ductal graft placement and bilateral pulmonary artery banding. This technique is designed to avoid the high-risk classical Norwood stage 1 operation and to avoid the need for first stage invasive catheter interventions and interstage catheter interventions when ductal stent placement is not feasible in developing countries with limited resources. Between June 2015 and December 2022, 14 patients with hypoplastic left heart syndrome and variants were treated using this procedure at our center. Polytetrafluoroethylene graft was used for ductal graft procedure and polytetrafluoroethylene bands were used for bilateral pulmonary banding. Atrial septectomy was done selectively. Patients with adequate left heart growth underwent biventricular repair and pulmonary debanding. Patients without left heart growth underwent cavopulmonary anastomosis or necessary intermediate surgical revisions. Seven patients out of 14 reached the second stage. Two received biventricular repair, three underwent bidirectional cavopulmonary anastomosis, and two required repeat palliations other than bidirectional cavopulmonary anastomosis. Modifications of the Norwood procedure is an ongoing process due to the complex nature of hypoplastic left heart syndrome and variants. As experience grows, the mortality associated with ductal graft and bilateral pulmonary banding procedure may decrease, making this technique an alternative for centers where classical Norwood operation or hybrid palliation is not possible, especially in developing countries with limited resources. We might predict promising outcomes, especially for patients with hypoplastic left heart complex where biventricular repair is expected.

Dobutamine stress cardiac magnetic resonance (dsCMR) has demonstrated value in identifying patients at risk for adverse cardiovascular events in adults with coronary artery disease, but its prognostic value in younger patients is unknown. This study sought to evaluate the relationship between dsCMR results and the risk of subsequent cardiovascular events in children. Patients age < 23 years who underwent dsCMR at Boston Children's Hospital were eligible for inclusion. Patients were excluded if no follow-up data were available after dsCMR or the dsCMR protocol was not completed. Data regarding the presence of inducible regional wall motion abnormalities and subsequent cardiovascular events during follow-up were analyzed. Cardiovascular events included myocardial infarction, surgical or catheter-based coronary artery intervention, and ischemic symptoms. Among 80 dsCMR studies in 64 patients with median age 5.3 years (range 0.5-22.4 years), 3 patients had a positive dsCMR with inducible regional wall motion abnormalities. Over a median follow-up of 7.4 years (IQR 4.0-11.2), 2 of these patients underwent surgical intervention (coronary artery bypass grafting and unroofing of a myocardial bridge) and the third remained asymptomatic with no interventions. Among 61 patients with a negative dsCMR, none experienced myocardial infarction, death, or aborted sudden death. Four underwent cardiac interventions (cardiac transplantation, coronary artery bypass grafting, and unroofing) due to symptoms or catheterization findings. In pediatric patients undergoing dsCMR, wall motion abnormalities indicating inducible ischemia were uncommon. Those with a negative dsCMR were unlikely to experience an adverse cardiac event or undergo a cardiac intervention on medium-term follow-up.

There is a dearth of systematic data on efficacy and safety of ibutilide in children and adults with CHD and also in pediatric age-group patients. To determine the efficacy and safety of ibutilide in these populations, and to characterize the determinants of its efficacy. Patients with CHD and children < 18 years old with structurally normal hearts were recruited between Jan 1, 2010 and Dec 1, 2020. Logistic regression models were used to determine the effect of covariates on odds of successful cardioversion with ibutilide. 53 patients underwent 105 chemical cardioversions (age: 27.5 ± 10.4 years [75% males], 80% atrial flutter) with ibutilide during the study period. The overall rate of successful cardioversion with ibutilide in our cohort was 70.4% (mean ibutilide dose: 1.5 ± 0.65 mg). Presentation in atrial flutter (P = 0.08) and male gender (0.07) showed a trend towards significance. Adverse effects were reported after 8 ibutilide administrations (7.6%, n = 6/8 [75%] female).

Necrotizing enterocolitis (NEC) is a known cause of morbidity and mortality in infants with congenital heart disease (CHD), but reports about the burden of cardiogenic NEC frequently conflict. To synthesize the extant literature on the incidence, risk factors, and prognosis of NEC in patients with CHD. Medline, Cochrane, and EMBASE were searched from 1946 through 2023 for studies of NEC in infants 0-12 months of age with CHD. Risk of bias was assessed with validated tools for incidence and risk factors. Pooled estimates were meta-analyzed by risk of bias or synthesized without meta-analysis. Eighty-six studies with a total of 67,924 participants were included. The incidence of cardiogenic NEC was 7.1% (95% CI 4.7-10.5%) in term infants and 13.0% (10.2-16.5%) in low birthweight preterm infants. NEC required surgery in 0.8% (0.5-1.1%) of term and 2.7% (2.0-3.7%) of premature infants, respectively. Only gestational age and birth weight were consistently associated with risk of NEC. Restricting pooled estimates to studies of moderate or low risk of bias significantly reduced the number of studies included. Necrotizing enterocolitis is a common cause of morbidity in infants with CHD, but additional research is needed to determine which infants are at highest risk of developing NEC and would benefit most from a change in management. This systematic review and meta-analysis was conducted according to a prespecified protocol registered at the Prospective Register of Systematic Reviews (CRD42021282114).

The Fontan procedure is used to palliate complex forms of congenital heart disease. This results in adverse hepatic sequelae now known as Fontan-associated liver disease (FALD). Conventional laboratory measures of liver disease do not correlate well with FALD severity. Cytokeratin-18 (CK-18) is a measure of cell death and is sensitive in detecting other causes of liver disease. Our aim was to assess the use of a novel measure of liver disease, CK-18, in Fontan patients. This is a single-center, prospective, cross-sectional study of Fontan patients aged 8-21 years old. We performed ultrasound elastography, echocardiography, magnetic resonance imaging, and serum laboratory testing. Novel laboratory test CK-18 levels in Fontan subjects were compared to healthy age-matched controls. Thirteen Fontan patients were evaluated with a median age 15 years (10, 14), 4 Hypoplastic left heart syndrome, 11 were male, and 5 were symptomatic. Fontan patients had normal AST/ALT, but a significantly elevated liver stiffness by elastography (median 13.4 kPa). Hepatic stiffness by elastography was associated with diastolic-indexed (rho = 0.58, p = 0.04) ventricular volumes. Compared to 10 aged-matched controls, CK-18 was higher in the Fontan group-cleaved CK-18 protein (p < 0.01) and full CK-18 protein, (p = 0.02). CK-18 was positively associated with AST and ALT. Elevated CK-18 levels were found in Fontan patients compared to controls suggesting hepatic cell death even in these relatively healthy Fontan patients. CK-18 was elevated prior to changes in traditional testing. CK-18 may be a useful sensitive marker of liver disease in FALD.

Fluid overload is associated with increased morbidity and mortality after pediatric cardiac surgery. Management of fluid overload can be difficult and conventional tools may increase the risk of acute kidney injury. This study aimed to study the effects of aminophylline on fluid balance, urine output, blood urea nitrogen, and serum creatinine. Pediatric cardiac surgical patients who received aminophylline between September 2022 and December 2023 were identified. Data for various clinical parameters before and after an aminophylline dose were collected. Paired univariable analyses and a random forest classifier were conducted to help characterize the effects of aminophylline. A total of 169 aminophylline administrations in 72 unique patients were included in the final analyses. Fluid balance decreased by 115% in the 24 h after aminophylline administration compared to the 24 h preceding. Urine output peaked at 2 h after administration and increased 100% from baseline. Heart rate increased by 5% after administration and peaked between 2 and 4 h after. In pediatric patients after cardiac surgery, a 5 mg/kg dose of aminophylline is safe and is associated with a reduction in fluid balance and increase in urine output without significantly changing blood urea nitrogen or serum creatinine levels.

Hypothesis: The aim of the study was to evaluate the correlation between NTproBNP and left ventricular mass (LVM) in children affected by hypertrophic cardiomyopathy (HCM), both asymptomatic, symptomatic, and with implantable cardioverter-defibrillator (ICD).

Methods: A total of 225 pediatric patients were evaluated and 45 were enrolled in the study because met inclusion criteria (age < 18 years; diagnosis of hypertrophic cardiomyopathy; NTproBNP > 600 pg/ml).

Results: The analysis showed a significant correlation between NTproBNP level and LVM (p = 0.024). ANOVA test showed that high level of NTproBNP and LVM were significantly correlated to ICD implantation (p = 0.001; p = 0.003). Multivariate analysis showed NTproBNP as an independent risk factor for ICD implantation (p = 0.035).

Conclusion: Our study showed that NTproBNP levels directly correlated to LVM and disease progression and it was found to be predictive, together with LVM, of ICD implantation.

To describe critically ill neonates with premature closure of the ductus arteriosus (DA) and assess the frequency as well as predictive factors for extracorporeal membrane oxygenation (ECMO) support in the latter. This was a monocentric retrospective observational study conducted in the NICU of a French academic medical center between 01/01/2013 and 01/01/2023. All neonates diagnosed with premature closure of the DA were included. Exclusion criteria were associated congenital heart disease amenable to urgent surgery or contraindication for ECMO. Eleven neonates with complete premature closure of the DA were included. Births were full-term with a weight of 3.60 kg [3.16-3.89]. Only one case (9%) was diagnosed antenatally. Premature closure of the DA was idiopathic in seven neonates (64%) and associated to maternal exposure to non-steroidal anti-inflammatory drugs (NSAIDs) in three (27%). All newborns had pulmonary hypertension (PH) and right ventricular hypertrophy. Three neonates (27%) were supported on veno-arterial ECMO and appeared to have more pathological adaptation to extra-uterine life, greater need for vasopressor and/or inotropic support, and higher frequency of bi-ventricular failure (100%) or morphological anomaly of the tricuspid valve (67%). No patient died but more than half had non-cardiological sequelae. Veno-arterial ECMO support is not uncommon in critically ill neonates with premature DA closure and appeared to be associated with more pathological adaptation to extra-uterine life, greater need for vasoactive-inotropic support and higher frequency of bi-ventricular failure or morphological anomaly of the tricuspid valve. These parameters can help clinicians to identify neonates likely to require such an assistance.