Pediatric Cardiology最新文献

To study the association between technique for definitive closure of PDA and peri-procedural opioid utilization in preterm neonates. A retrospective cohort study comprising all premature neonates and infants with a PDA who underwent surgical ligation (SL) between February 2020 and December 2022 or transcatheter PDA closure (TCPC) between August 2020 and February 2023. Intra-procedural and post-procedural opioid use was systematically quantified and compared between the two groups before and after 1:1 propensity score matching. We hypothesized that preterm neonates and infants undergoing TCPC will have an overall reduction in peri-procedural opioid exposure compared to those undergoing SL. The study included 52 preterm infants in the SL group and 74 in the TCPC group. After propensity score matching, infants undergoing TCPC received significantly lower intra-procedural (4.33 vs 5.99 µg/kg, P = 0.037) and post-procedural (0.09 vs 0.32 mg/kg, P < 0.001) opioids. A significantly lower proportion of infants undergoing TCPC were exposed to post-procedural opioids (60.8% vs 92.3%, P = < 0.001). In a multivariable logistic regression model, surgical ligation was associated with sevenfold higher odds of post-procedural opioid exposure compared to TCPC (OR 7.2, 95% CI of 2.1-24.2, P = 0.001). Transcatheter PDA device closure is associated with significantly lower neonatal opioid exposure.

Twin Reversed Arterial Perfusion (TRAP) sequence is a rare complication in monochorionic pregnancies, often leading to fetal heart failure (FHF) in the pump twin. We present a 17-year-old primigravida with a monochorionic monoamniotic twin pregnancy presented with an acardiac twin, single umbilical artery (SUA), and multicystic placenta appearance. Throughout the pregnancy, the pump twin exhibited normal growth without signs of FHF. The infant was delivered at 34 weeks via cesarean section, healthy and without cardiac anomalies. The nonexistence of an impacted intrauterine fetal development and FHF of the pump twin in our case may be explained by the presence of one umbilical artery, resulting in less blood flow to the parasitic twin, which can be accepted as a protective factor for the pump twin circulation. Our hypothesis-generating finding that SUA may play a protective role in the development of FHF in pump-twin is promising; however, it remains speculative, and further research is needed to confirm the protective role of SUA for pump-twin outcomes in TRAP pregnancies.

The number of adults living with congenital heart disease continues to rise, emphasizing the need to understand the challenges they encounter to enhance disease management. This study aimed to evaluate adults with CHD using the International Classification of Functioning (ICF) framework and explore factors associated with community integration. Holistic assessments were conducted encompassing all dimensions of the ICF framework, including Body Structures (echocardiography and electrocardiography findings), Body Functions (6-Minute Walk Test, Muscle Strength, Hand Grip Strength, and Fatigue Severity Scale), Activity-Participation (International Physical Activity Questionnaire, Multidimensional Quality of Life Scale, and Community Integration Questionnaire), and Environmental-Personal factors (Depression, Anxiety, and Stress Scale- 21, and Physical Activity Barriers Scale). Pearson correlation analysis was conducted to examine the relationships between community integration and other parameters, with variables showing significant correlations included in the multiple linear regression analysis. The mean age of 42 CHD participants was 24.61 ± 7.27 years. The Community Integration Questionnaire correlated with the Physical Activity Barriers Scale (r = 0.310, p = 0.046), Multidimensional Quality of Life Scale (r = 0.441, p = 0.003), and 6-min walk test (r = 0.364, p = 0.021). "Access to health personnel," a sub-dimension of the Multidimensional Quality of Life Scale, and 6-min walk test distance predicted the Community Integration Questionnaire score, explaining 30.7% of its variance (r = 0.554; r² = 0.307; F = 8.197; p = 0.001). The findings suggest that community integration in individuals with CHD is linked to body function, activity-participation, and environmental-personal factors. This study highlights the importance of evaluating patient-reported outcome measures in the domain of community integration, especially considering the shifting demographics of congenital heart disease.

KONAR-MF™ device is a novel, cone-shaped, medium profile, self-expanding, double-disk, nitinol occlusive device designed for the ventricular septal defect (VSD). Due to its unique design and easy deployment from either side and multiple occlusion layers, it has also been used to occlude defects other than VSD. A retrospective review was done from 2019 to 2024 from three institutions. All patients where the KONAR-MF™ device was used other than for VSD closure were included in this study. Standard post-procedure follow-up was at 1, 6, and 12 months for all patients. 79 off-label implantations of the KONAR-MF™ device were done for conditions that included 59 shunt lesions (patent ductus arteriosus -34, coronary arteriovenous fistula -12, pulmonary arteriovenous fistula -2, systemic arteriovenous fistula -3, aortopulmonary window -3, aortopulmonary collateral -5, Fontan fenestration closures -7, Fontan antegrade pulmonary valve closures -2, Abernethy malformations -3, decompressing vein -2, and paravalvular leak -2, others 4. The median fluoroscopy time was 10 min (IQR 6-18). The median duration of hospital stay was 2 days (IQR 1-4 days). There were no significant complications. Complete occlusion at the end of the procedure was documented in 72 (91.13%) patients. At a median follow-up of 18 months (IQR 12-28 months) in all except one patient who had mild residual flow with no device-/(procedure) related complications. The unique structure and compact profile of KONAR-MF™ enable a wide range of uses in catheter-based management of various CHDs with the potential to simplify the inventory of the catheterization laboratory.

Pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD) is a severely progressive condition with complex pathogenesis. The aim of this study was to evaluate the contribution of genetic variants to the development of PAH in patients with APAH-CHD. Fifteen children and twenty-seven adults diagnosed with APAH-CHD were enrolled. Targeted next-generation sequencing was performed on PAH-associated genes (ABCC8, ACVRL1, AQP1, ATP13A3, BMPR2, CAV1, GDF2, GGCX, EIF2AK4, ENG, KCNK3, KDR, KLK1, SMAD1, SMAD4, SMAD9, SOX17, TBX4, TET2). A total of 21 distinct variants across 11 different genes were detected in 17 of the 42 patients. (ABCC8 = 2, ACVRL1 = 1, ATP13A3 = 2, BMPR2 = 4, GGCX = 1, EIF2AK4 = 2, ENG = 1, KDR = 3, SMAD1 = 1, SMAD9 = 1, TET2 = 3). Five of the patients with the mutation were under the age of 18, and 12 patients were adults. The most common CHD in patients with detected variants was VSD. PAH-related genetic variants were not uncommon in APAH-CHD patients. Our study identified 12 novel variants that may help to understand the genetic basis of APAH-CHD. Trial Registration The study has been registered on ClinicalTrials.gov with the identification number NCT05550389.

We sought to determine the impact of the COVID-19 on prenatal diagnosis (PND) of clinically significant congenital heart disease (CHD) and the role of socioeconomic status (SES), complexity of diagnosis, and proximity to advance testing. This single-center retrospective study evaluated 2 eras of infants (COVID (born July 1, 2020-July 31, 2023) and pre-COVID (born June 1, 2017-July 1, 2020) who had cardiac surgery in the first year of life. 512 infants, 292 in pre-COVID era and 220 in COVID era with no significant difference in the rate of prenatal care (PNC) or PND in the COVID era (88%/42%) versus pre-COVID era (93%/48%) (χ² = 3.22, p = 0.07, χ² = 1.9, p = 0.17). Distance from advanced testing had no influence on PND in the COVID era [55% close versus 53% further away (χ² = 2, p = 0.65)]. When evaluating SES with income per zip code, the higher SES group had increased PND during the pandemic compared to both pre-COVID era and low SES group. However, social deprivation index (SDI) based on zip code showed the higher SES group had a decrease in PND rates. Both metrics showed no change in PND in the lower SES group during COVID. COVID-19 had no significant change in the PND of clinically significant CHD during the pandemic. The differing SES results using income versus SDI of patient zip codes suggest that barriers to PND is multifactorial. The discrepancy in PND reflects poor referral rates to advanced testing, highlighting the importance of educating frontline healthcare professionals to improve outcomes.

We share a single-center experience of transcatheter pulmonary valve replacement (PVR) with the Venus-P valve, discussing procedural insights, considerations for valve sizing in relation to valve expansion and geometry, and associated outcomes. Retrospective review of 42 consecutive cases from January 2017 to March 2024. The median age and weight of the cohort were 39.6 (27.3, 58.6) years and 69.8 (59.8, 85.3) kg and the Venus-P valve was successfully implanted in 98%. Severe adverse events included valve migration requiring surgical explant and PVR (n = 1), sustained ventricular arrhythmia (n = 1), and endocarditis (n = 1). At mean follow-up of 20.3 ± 19.6 months, there was no deterioration in valve performance and statistically significant improvement in NYHA class, right ventricular volumes, biventricular stroke volume, and LV ejection fraction. Cinefluoroscopic measurements of the deployed valves showed highest expansion and lowest eccentricity at valve coaptation level and greater frame deformation at the inflow compared to outflow flare. Supra-annular (n = 9) implant showed a higher expansion of the straight valve section compared to transannular (n = 32) deployment, related to lesser degree of valve oversizing and tissue characteristics at implant site. Transcatheter PVR with the Venus-P valve can be safely performed in a broad range of anatomies with clinical benefit at medium-term follow-up. Valve sizing focused on adequate deformation of inflow and outflow flares at implant location with minimal constrain at the valve portion and supra-annular positioning, may result in a higher degree of valve expansion and circularity. Ongoing follow-up of this cohort will provide important insights into valve longevity and long-term outcomes.

Bioelectrical impedance analysis (BIA) is a noninvasive tool that can estimate volume status using fluid compartment ratios. Previous studies have demonstrated that BIA can be used to help manage heart failure using the edema index (EI), which is the ratio of extracellular water (ECW) to total body water (TBW). This study set out to better define the relationship between BIA fluid compartment estimations and invasive hemodynamic measurements, in the context of pediatric and congenital heart disease. 52 individuals underwent 59 elective catheterizations and BIA. Data from the BIAs were compared with the hemodynamic catheterization data. The median age at the time of catheterization was 16.6 [13.5, 19.6] years (63% < 18 years-old), and 29% were female. In multivariable analysis, EI (β = 103.5 ± 47.9, p = 0.04), body mass index (BMI) (β = 0.16 ± 0.07, p = 0.02), and current Fontan circulation (β = 3.06 ± 0.96, p = 0.002) were statistically significant predictors of pulmonary capillary wedge pressure (PCWP). Individuals with an EI ≥ 0.39 had a statistically significant higher PCWP compared to those with an EI < 0.39 (12 [11, 17] vs. 10 [8, 12], = 0.05), with an area under the curve (AUC) of 0.76 (95% CI 0.65, 0.87). There was no statistically significant mean difference between the pre-catheterization and either post-catheterization EI (- 0.0001 mean difference (0% change), p = 0.92), or body fat mass (BFM) (+ 0.035 mean difference (0.2% change), p = 0.81). This study suggests that BIA, and more specifically EI, can noninvasively provide valuable information about hemodynamic data. BIA provides a quick, easy, non-invasive method for assessing and managing the volume status in individuals with congenital heart disease.

Exertional chest pain in the setting of a structurally normal heart and no known arrhythmia are rarely thought to be related to cardiac etiology. Common non-cardiac etiologies of chest pain in the pediatric population include the following: lung infections, musculoskeletal, asthma, acid reflux, and anxiety. However, common cardiac etiologies that can cause exertional chest pain typically include the following: congenital heart disease, coronary anomalies, myocardial infections, left ventricular outflow obstruction, illicit drug use, and prothrombotic conditions. Rarely, pediatric patients present with an isolated finding of left main coronary artery ostial stenosis requiring intervention. Many of the published reports of left main ostial stenosis are in connection with other congenital heart disease, inflammation, or connective tissue disorders who have undergone percutaneous intervention. This report describes an 8-year-old patient with exertional chest pain for 5 years found to have isolated left ostial stenosis requiring coronary artery bypass grafting at the age of 13.

Infants and children with congenital heart disease (CHD) often require supplemental nutrition via tube feeding before and after surgery. Tube feeding may be required due to poor weight gain, inadequate oral-motor skills, and/or reduced endurance for oral feeding. Our team has described a successful approach to weaning this population (Horsley et al. (2022) Pediatr Cardiol 43:1429-1437). A subgroup of tube-fed patients with CHD who demonstrate oral feeding aversion (OA) presents unique challenges to the tube weaning process. We discuss our team's experience with tube weaning orally averse children with CHD. Of 36 patients enrolled in the Cincinnati Children's Heart Institute Cardiology Feeding Tube Wean Clinic, 11 (31%) were determined to have oral feeding aversion (OA) by a speech-language pathologist (SLP). Descriptive data comparing the OA group to the non-averse group was gathered prior to and during the wean. Both groups had the ability to tube wean successfully, although the OA group had lower median oral intake (9 vs 24%) and higher age at start of the wean (9 vs 4 months). Those with OA also had a longer median duration of wean in days (17 vs 12 days) and higher likelihood of return to tube feeds within six-month post-wean (22 vs 0%). Additionally, the OA group had a higher percentage of genetic syndromes (36 vs 16%), although this was not found to be statistically significant in this study. Children with OA present with unique challenges for tube weaning. The results of this study show that weaning children with CHD and OA is possible with a multidisciplinary team who is knowledgeable about this population.