Pediatric Cardiology最新文献

Frequent ventricular ectopy (VE) ≥ 10% in children with structurally normal hearts and the development of ventricular dysfunction is not well described. We aim to describe frequent VE ≥ 10% in children with structurally normal hearts and characterize the development of dysfunction. Patients with VE burden ≥ 10% on 24-h Holter performed between 2010 and 2019 were included in this retrospective review. Patients with structural heart disease and cardiomyopathy preceding the onset of VE were excluded. Medical records, electrocardiogram, Holter, and transthoracic echocardiogram data were analyzed. Patients were classified based on maximum VE burden on Holter ("frequent" 10-29% versus "very frequent" ≥ 30%), presence of runs of VE, VE morphology on ECG, coupling interval, and development of dysfunction. Two hundred thirty-four patients met inclusion criteria, 187 with frequent VE and 47 with very frequent VE. Seventeen (7%) patients developed ventricular dysfunction, the majority of whom had mild dysfunction. Very frequent VE > 30% and non-sustained ventricular tachycardia (NSVT) on Holter were associated with dysfunction. There was no association between coupling interval or prematurity index and dysfunction. Ventricular dysfunction is uncommon in children with structurally normal hearts and frequent VE burden > 10%, though VE burden > 30% and presence of NSVT were associated with dysfunction. Previously described characteristics to delineate higher risk VE based on coupling interval were not associated with dysfunction.

Severe arrhythmias may occur early after open heart surgery. Because younger patients do not usually show any specific symptoms, presently Holter monitoring is routinely performed for 24 h predischarge at our centre to prevent adverse outcomes. It is unknown whether this test is truly justified in this patient population. Retrospective single-centre analysis of all consecutive patients younger than 19 years old after open heart surgery 2013-2019 who underwent routine Holter monitoring before hospital discharge. Patients with permanent pacemakers and patients who died during this hospital stay were excluded. The cohort was divided into two groups depending on whether severe arrhythmia occurred or not. The study includes 790 Holter recordings from 666 patients with a median age of 0.5 years (IQR 0.23-3.08), performed at a median time of 8 days (IQR 6-15) postoperatively. Postoperative arrhythmia was detected in 554 of 790 24-h Holter recordings (70%); in 47 of 790 (6%), this arrhythmia was classified as severe. The most common severe arrhythmias were premature ventricular contractions (n = 26/47) and long pauses (n = 14/47). A longer aortic cross-clamp time (mean 94.5 (SD ± 53.0) versus 68.1 (SD ± 51.9) min, p = 0.001) was associated with the occurrence of severe postoperative arrhythmia. Severe arrhythmias are rare in predischarge assessments after open heart surgery in children. In current postoperative monitoring at our centre, the diagnostic yield of ECG Holter monitoring for 24 h is too low to justify routine screening in all paediatric patients after open heart surgery.

Background: Matrix metalloproteinase 7 (MMP-7) is a novel biomarker for diagnosis of biliary atresia (BA), the most common cholestatic liver disease in infancy. There is a pressing need to determine the utility of MMP-7 levels in infants with congenital heart disease (CHD) to avoid unnecessary invasive diagnostic procedures in this high-risk population. We investigated the utility of MMP-7 in discriminating BA from non-BA cholestasis in infants with CHD and whether MMP-7 elevation was present in infants requiring treatment for clinically significant PH.

Methods: This is a single-center cross-sectional study including infants < 180 days of age with cholestasis and serum MMP-7 levels collected from 2019 to 2023. Demographic data and descriptive statistics were summarized with medians with interquartile ranges and frequencies with percentages. Median MMP-7 levels were assessed via Wilcoxon rank-sum test.

Results: A total of 149 patients were included. Patients with CHD had significantly elevated MMP-7 levels relative to the non-CHD cohort (50 vs. 34 ng/mL, p = 0.009). Sub-analysis comparing infants with and without PH revealed significantly elevated median MMP-7 levels in those with clinically significant PH (125 vs. 39 ng/mL, p = 0.010). CHD patients with PH had greater median MMP-7 compared to CHD patients without PH (154 vs 43 ng/mL, p = 0.028).

Conclusion: Serum MMP-7 levels in infants with congenital heart disease with cholestasis (CHD-C) were significantly elevated compared to those with cholestasis alone. MMP-7 may help identify non-BA cholestatic infants who have concurrent clinically significant pulmonary hypertension. Larger, prospective studies are needed to validate this finding and establish CHD-specific MMP-7 cut-offs.

Background: Mitral annular disjunction (MAD) has increasingly been recognized as a marker for adverse cardiovascular events in Marfan syndrome (MFS). As recent adult data links MFS with left ventricular (LV) dilation and reduced ejection fraction (LVEF), we hypothesized that MAD may be associated with LV dilation in pediatric MFS patients.

Methods: A retrospective analysis was performed among MFS patients < 19 years old at initial cardiac MRI (CMR). MAD and mitral valve prolapse (MVP) were assessed by CMR or most proximate echo. CMR-derived left ventricular end-diastolic (LVEDV) and end-systolic (LVESV) volumes were measured. Indexed volumes, absolute and indexed z-scores, and LVEF were calculated. The combined volume load from mitral and aortic regurgitation was indexed to LV stroke volume, allowing exclusion of patients with greater than mild volume load or prior MV intervention. MAD association with LV volumes and z-scores was then assessed.

Results: Forty-two patients were analyzed (median age 13.5 years old, IQR [10.9, 15.3]). MAD was present in 28 patients (66.7%), and MVP was present in 13 patients (31.0%). Absolute LVEDV z-score was > 2 in 35.7% of patients, LVESV z-score was > 2 in 42.9%, and LVEF was < 55% in 45.2%. In multivariable analysis including MVP, MAD remained independently associated with elevated absolute LVESV z-score > 2 (RR 3.88, 95% CI 1.02-14.69, p = 0.046).

Conclusion: MAD was associated with CMR-derived volume-load-independent LV dilation among pediatric MFS patients. Prospective studies are needed to further understand this association and its relationship with LV dilation over time.

Hybrid stage 1 palliation (HS1P) is used as an alternative to the surgical Norwood for single ventricle patients and specific other conditions. Necrotizing enterocolitis (NEC) occurs in 5%-18% of infants after surgical Norwood, contributing to significant morbidity. We sought to compare the NEC incidences in HS1P versus Norwood patients. A single-center retrospective cohort study of all infants undergoing HS1P or Norwood over a 12-year period. Demographics, underlying cardiac diagnosis, surgical details, and NEC episodes were queried for each patient. The cumulative incidences of NEC between HS1P and Norwood patients were compared. A total of 305 infants were included; 200 Norwood and 105 HS1P. HS1P infants were more often premature (gestational age < 37 weeks; 23.8% vs 1.5%) and had lower birth weight (mean 2.8 ± 0.66 kg versus 3.3 ± 0.47 kg) than their Norwood counterparts. Most infants who underwent the Norwood procedure had hypoplastic left heart syndrome (79.5%), with variable underlying cardiac diagnosis for those who underwent HS1P. Incidence of NEC was significantly higher in the HS1P group (53.3% versus 37.0%, p = 0.01), with increased risk independent of birth weight or underlying cardiac diagnosis (adjusted odds ratio 1.8, p = 0.03). Infants after HS1P had a median of 2 episodes of NEC (interquartile range [IQR] 1-3) versus 1 episode in the Norwood group (IQR 1-2). Infants after HS1P are at higher risk of NEC in comparison to infants after the Norwood procedure. More studies are needed to help predict which infants after HS1P are at highest risk for NEC.

This study aimed to investigate left-sided atrioventricular valve outcome after the repair of complete atrioventricular septal defect from single-institutional retrospective chart review. From 1998 to 2022, 55 patients with complete atrioventricular septal defect and balanced 2 ventricles underwent biventricular repair. Median age and weight at repair were 5.5 months old [interquartile range, 3.4-9.1] and 4.6 kg [3.9-5.9]. Analyzed possible risk factors were Rastelli classification, low birth weight < 2.5 kg, preterm < 37 weeks, proceeding pulmonary artery banding, Down syndrome, persistent left superior vena cava, tetralogy of Fallot, early surgical era < 2010, preoperative left-sided atrioventricular valve regurgitation > mild, modified single-patch repair, small left mural leaflet, and complete left-sided atrioventricular valve cleft closure. No patients underwent left-sided atrioventricular valve replacement at the timing of repair. No patient required subsequent single ventricular conversion. The median follow-up period in survivors was 11.0 years. The survival rate at 15 years from repair was 96.4%. Freedom from moderate or greater left-sided atrioventricular valve regurgitation rate at 10 years was 58.4%. After elimination of 3 patients undergone complete closure then experienced tone of closed cleft, complete cleft closure was significant inhibitor for moderate or greater left-sided atrioventricular valve regurgitation (p = 0.034, Odds ratio: 0.36, 95% Confidence intervals: 0.14-0.93). Twelve patients underwent 15 reoperations for left-sided atrioventricular valve. All 4 patients who developed left-sided atrioventricular valve stenosis and backward pulmonary hypertension required prosthetic valve replacement. Incomplete cleft closure was the only risk factor for significant postoperative left-sided atrioventricular valve regurgitation. Already small left-sided atrioventricular valve by previous repair developed significant regurgitation, it is no longer repairable therefore required to be replaced.

Contraceptive use among adolescents and young adults with congenital heart disease (CHD) is notably lower, influenced by multiple critical factors. This critique highlights three key determinants that warrant further investigation in future research. First, studies indicate that hormonal contraceptives, particularly estrogen-containing methods, may elevate the risk of thromboembolic complications, arrhythmias, and heart failure in CHD patients, especially those with Fontan circulation or transposition of the great arteries. Second, menstrual irregularities are prevalent among CHD patients, with a significant proportion facing contraindications to estrogen-based contraceptives, potentially discouraging their use. Lastly, inadequate disease-specific contraceptive counseling contributes to knowledge gaps, limiting informed decision-making regarding reproductive health. Addressing these determinants is essential to optimize contraceptive use and improve reproductive health outcomes in CHD patients.

Patent ductus arteriosus (PDA) stent placement is commonly performed in children with ductal-dependent pulmonary circulation. We report an extremely rare complication during the stenting of a tortuous PDA in a newborn with Tetralogy of Fallot and pulmonary atresia, along with its management. Immediately after stent placement, tissue protrusion inside the stent lumen was observed, causing a reduction in lumen size. This complication was successfully managed by placing a shorter stent inside the original one at the site of the tissue protrusion.

Our aim is to determine the rate of unexpected malrotation identified on routine preoperative upper gastrointestinal (UGI) contrast study in infants with congenital heart disease (CHD) prior to gastrostomy tube (GT) placement and quantify any associated delay in care. We performed a retrospective review of infants with CHD who underwent GT placement following initial cardiac surgery at a single center between 2016 and 2021. Patients were identified in the electronic medical record. Demographic information, indications for GT placement, and clinical course were collected. Variables were compared using Mann-Whitney test, with significance set at p < 0.05. One hundred and thirty-one infants with CHD underwent GT placement after cardiac surgery; 124 (94.7%) underwent preoperative UGI of which 119 (95.2%) were normal. Five studies were read as "could not rule out malrotation" and one infant had malrotation on UGI. Median time from UGI to surgery was 3 days. Median days from consult to GT placement was 3 days among those who underwent UGI and 2 days in those who did not (p = 0.34). Among infants with CHD, the rate of unknown malrotation is low. UGI contrast study prior to GT placement may be associated with surgical delay, unnecessary radiation exposure, and low value healthcare. If a child is tolerating gastric feeds prior to GT placement, routine UGI is unnecessary.

In the pediatric cardiac intensive care unit (PCICU), predicting the complications and long-term impact of extracorporeal membrane oxygenation (ECMO) and cardiopulmonary resuscitation (CPR) is influenced by the providers' perceptions. Little is understood about such perceptions as they relate to provider role and experience. A multi-disciplinary group in the PCICU of a single center was surveyed regarding two patient scenarios: (1) ECMO after cardiac surgery, and (2) cardiac arrest with need for CPR. Respondents indicated their risk tolerance for potential complications of these interventions, and the impact on patient health-related quality of life (HRQoL). Responses were compared based on providers' role and experience. 101 providers were surveyed and were willing to tolerate a 50% risk of death with ECMO or continued CPR but had less tolerance for the risk of other long-term morbidities. For most potential complications, nurses had the lowest risk tolerance and attending physicians the highest. Provider experience had no impact on risk tolerance. All providers underestimated HRQoL scores compared to previously published scores of patients who survived the surveyed scenarios. This survey is one of the first to explore providers' perceptions of ECMO and CPR. PCICU providers are more willing to accept the risk of death than other morbid complications, and poorly predict HRQoL outcomes. Team members have varied risk tolerance for complications and predict their impact differently. Understanding the variability of and influences on perceptions of patient outcomes could help improve communication with patients and families, team dynamics, and decision-making in the PCICU.