Pediatric Cardiology最新文献

Describe the long-term presence, predictors, and time course of postoperative dysphagia in selected congenital heart disease (CHD) patients following cardiac surgery. Retrospective study of selected CHD patients who underwent cardiac surgery that is at high risk for dysphagia and vocal fold mobility impairment (VFMI) and underwent speech pathology assessment from 2019 to 2024. Demographics, clinical history, VFMI, dysphagia severity and feeding modality were assessed. There were 322 mostly infant patients; median age was 0.64 (IQR0.19-6.9) months. Most patients were male (177,55%) and 119 (37%) had single ventricle (SV) disease. A full PO diet was maintained in 105 (32.6%) patients throughout follow-up. One hundred and ten (34.2%) patients improved from tube feeding (TF) at initial discharge to a full PO diet in a median 10.4 months (IQR4.4-23.2), whereas 107 (33.2%) remained on TFs with severe dysphagia at the end of follow-up (median 7.2, IQR1.5-17.3 months). VFMI was present in 83 patients postoperatively. VFMI resolved in 35 (38.8%) patients in a median 4.8 months (IQR2.4-8.2). VFMI and dysphagia recovery were not associated. However, the time to VFMI recovery and time to dysphagia resolution were correlated (r = 0.77, p = 0.0001). Patients with a genetic syndrome, Blalock-Thomas-Taussig shunt and/or ventricular assist device use were more likely to require persistent TF. Patients who had coarctation/aortic arch repair or SV disease were less likely to require TF at the end of follow-up. Dysphagia necessitating tube feeding persists for several months after VFMI resolves. This study will help set prognostic expectations for caregivers.

In ventricular septal defects (VSDs) close to aortic valve, mostly surgical closure is preferred. Currently, transcatheter method of device closure is done in selective cases with satisfactory results. Perimembranous with outflow extension VSDs having mild aortic valve prolapse with trivial to mild aortic regurgitation (AR) were selected. VSD devices were deployed through antegrade route with our modification of standard arteriovenous (A-V) loop technique to prevent aortic valve compression and progression of AR. Among 55 cases of perimembranous VSDs having outlet extension with aortic valve prolapse, 44 (80%) cases had successful closure with our modified technique and 11 (20%) underwent surgical closure as device closure was not amenable. At 1 year follow-up, the VSD closure rate was 91% with trivial and mild AR persisting in 24.4% and 5% of cases, respectively. Satisfactory closure of VSDs close to aortic valve is possible with our modified A-V loop technique.

The dynamic, multiplanar nature of the atrial septum can make ASDs challenging to characterize with 2D imaging. 3D imaging instantly profiles defect(s) in a single, en face view. 2D ICE-guided ASD device closure in the pediatric population has been reported, but 3D ICE-guided closure has not, and the correlation of 3D ICE measurements with fluoroscopic balloon-sizing diameter (BSD) remains unstudied. We describe our initial experience with 3D ICE in the periprocedural evaluation of percutaneous ASD closure. Consecutive patients undergoing percutaneous ASD closure at our institution from July 2022 to July 2023 were included. BSD was measured by the primary interventionalist, and non-invasive ASD dimensions were measured by two investigators intra-procedurally. Correlations were assessed between maximal BSD versus maximal ASD diameter by 2D (TEE, ICE) and 3D modalities (TEE, ICE). Fifteen patients underwent percutaneous ASD closure, and 11/15 (73%) underwent ICE (2D and 3D). Three cases omitted ICE due to equipment unavailability, and one due to inadequate imaging windows related to spinal rods. 10 of 11 patients also underwent TEE, while one had ASD closure by fluoroscopy and ICE alone due to provider preference. Six of ten TEEs (60%) included 3D imaging based on weight cut-off. The median age of ICE patients was 6 years (IQR: 7.5), with a median weight of 20.5 kg (IQR: 35.8). 3D ICE was attempted in all; image quality assessed as 'fair' or 'good' by two reviewers in 8/11 patients. Intraprocedural imaging correlated strongly with BSD [3D ICE vs. BSD (n = 7): ICC = 0.915, p < 0.001)]. Median pre-device deployment evaluation time by ICE was 14 min (IQR: 8.25), and 5 min (IQR: 3.0) post-device deployment. 3D ICE is a viable adjunctive imaging tool for guiding ASD closure procedures within the pediatric cohort, offering safety, efficiency, and congruence with established imaging modalities. Maximal ASD dimensions by 3D ICE correlate well with BSD.

The impact of maternal diabetes on the fetal heart is complex and not fully understood. The study aimed to investigate how different types and stages of maternal diabetes affect fetal heart function and ventricular remodeling during prenatal and postnatal periods. The study included 45 pregnant women with diabetes and 15 with uncomplicated pregnancies. Fetal echocardiograms were performed between 28 and 32 weeks of gestation using different techniques to assess cardiac function. Measurements of ventricular transverse and longitudinal diameters, as well as the sphericity index, were determined. Postnatal follow-up echocardiograms were also performed. The diabetic pregnant group was divided into three subgroups: 15 with GDM and 30 with PGD, including 15 wellcontrolled and 15 uncontrolled cases. The E and E/A ratio values for the mitral and tricuspid valves were significantly lower in fetuses and neonates affected by diabetes compared to the healthy group. The Tei-Doppler index for the left ventricle was significantly higher in pregnancies affected by diabetes, affecting both fetuses and neonates. The right ventricle exhibited significant differences in all groups of diabetic pregnancies in fetuses and postnatally in cases of pregestational diabetes. The sphericity index differed significantly in all fetal diabetic groups and in neonates compared to controls, except for the LV in the GDM cohort. Postnatally, the sphericity index of the RV increased compared to fetal values, while the LV sphericity index remained unchanged. Maternal diabetes mellitus affects fetal cardiac function and structure, which may continue into the early days of life.

Background: A large non-restricted left-to-right shunt across a patent ductus arteriosus (PDA) with the resultant volume and pressure load may impact right ventricle (RV) function. We aimed to compare RV function on echocardiogram before and soon after transcatheter closure of PDA in preterm neonates.

Method: This single-center retrospective study included preterm infants ≤ 2 kg who underwent PDA transcatheter closure. Echocardiograms before and within 24 h after transcatheter closure were analyzed by a single reader for i) Tricuspid Annular Plane systolic excursion (TAPSE), ii) RV Velocity Time Integral (VTI) outflow, iii) RV Fractional area change (FAC), iv) Systolic-to-diastolic time (SD) ratio from the tricuspid valve regurgitation, and v) Stroke distance, the product of VTI and heart rate. Paired t test was used to compare parameters before and after device closure.

Results: Our cohort (n = 97), 54.6% of whom were males, had a mean (Standard Deviation) gestational age of 24.9 (1.9) weeks and birth weight of 742 (244) grams. The median (IQR) age at procedure was 29 (21-45) days. We found evidence of RV dysfunction (abnormal TAPSE in 14%, FAC in 66.5% and SD in 100%) at baseline with significant improvement 24 h after device closure in RV VTI, FAC, SD, stroke distance, and myocardial performance index.

Conclusion: Among preterm infants with a PDA, transcatheter closure was associated with significant short-term improvement in RV systolic function. These data provide novel intriguing insights into the potential benefit of unloading of the RV through device closure in this population.

ECG criteria for diagnosing chamber enlargement has poor predictive value. Elevated voltages in mid-precordial leads may have clinical utility and we hypothesized that ECGs with a single mid-precordial voltage ≥ 60 mV correlate with congenital heart disease (CHD) in a pediatric cohort. This was a retrospective analysis of pediatric ECGs at the University of Minnesota from 2006 to 2021. Included patients had a single mid-precordial lead (V2-V5) QRS voltage ≥ 60 mV and an echocardiogram within 1 month. ECG parameters including rhythm, atrial enlargement, axis deviation, hypertrophy criteria and echocardiogram findings were evaluated. Of 122 patients (mean 1.6 ± 2.5 years, 62% male), seventeen (14%) (mean 2.5 ± 3.0 years) had normal anatomy and 105 (86%) (mean 1.5 ± 2.4 years) had CHD. Mean maximum QRS voltage was 75.9 ± 13.5 mV with a significantly higher mean maximum voltage in the CHD versus control group (77.0 ± 13.7 vs 69.2 ± 9.7 mV, p = 0.012). A receiver operating characteristic curve for maximum QRS voltage had an AUC of 0.691 (95% CI 0.546-0.835). 66.5 mV had the best sensitivity (76%) and specificity (59%) combination and a value of 92.5 mV had a specificity of 100% for CHD. A QRS voltage of 60 mV in mid-precordial leads was associated with CHD in a cohort of pediatric patients. Echocardiograms remain reasonable in these patients though larger cohort studies are needed to develop ideal cutoffs.

Fulminant presentations of acute myocarditis can predict cardiac functional and patient outcomes. This longitudinal study compared pediatric cohorts with fulminant Multisystem Inflammatory Syndrome-related myocarditis (MISCM) vs non-COVID-19 viral myocarditis (VM) to test the hypothesis that the adverse left ventricular (LV) remodeling rather than the phenotype of presentation predicts clinical outcomes. This is a retrospective analysis of 54 children with MISCM (age 6 ± 4 years, weight 32.5 ± 24.6 kg, male 44%) and 26 children with VM (age 3.8 ± 4.8 years, weight 17.7 ± 17.7 kg, male 56%) on hospitalization and one-year follow-up. VM patients exhibited acute LV remodeling, but MISC patients did not (LV end-diastolic dimension z score 2.05 ± 2.50 vs 0.04 ± 1.10, p = 0.00). Compared to the MISCM, VM patients had severe LV systolic and diastolic dysfunction (ejection fraction 54.6% vs 39.9%, four-chamber longitudinal strain - 15.6% vs - 8.7%, and left atrial strain 25.5% vs 13.9% p = 0.000), increased need for mechanical circulatory support (39% vs 7%), 2 mortalities, one cardiac transplant, and stage C heart failure in the 17 survivors at discharge. Ejection fraction normalized but abnormal segmental four-chamber longitudinal strain persisted in both cohorts with most VM patients remaining on anti-failure treatment at one-year follow-up. Inflammation-mediated acute LV remodeling rather than the phenotype of presentation may determine LV function and patient outcomes. Non-invasive imaging can play a useful role in the assessment of the mechanism of LV remodeling and defining the trajectory of LV function and cardiac outcomes.

Paediatric cardiac tumours are rare. The most common tumour is a rhabdomyoma, a benign tumour of the myocardium associated with Tuberous Sclerosis Complex (TSC), a rare genetic condition caused by constitutional pathogenic variants in either the TSC1 or TSC2 genes. Although benign, complications related to obstructed flow through the heart or intractable arrhythmias occur. A 23-year retrospective study of patients referred to the National Scottish Paediatric Cardiology service with evidence of a cardiac tumour. 51 patients identified; 12 prenatally, 8 live born. Of the 47 patients born alive, 44 (93.6%) patients had a benign cardiac tumour and 3 (6.4%) a malignant tumour. Rhabdomyomas were shown to be the most common tumour type in patients with TSC (p = 0.000861) and overall. 8/44 (18%) benign tumours had a documented arrhythmia, 50% requiring treatment with beta blockade. 7 patients with rhabdomyomas received an mTOR inhibitor, 6 were recorded as TSC 2 genotype. There was significant extra cardiac symptom burden for the TSC subtypes (p = 0.00105), particularly TSC2, related to renal and neurological complications. The natural history of rhabdomyomas is slow regression and if no significant mass or rhythm disturbances in early childhood, a positive cardiovascular prognosis. Identifying cases associated with TSC is important to counsel families regarding the longer-term implications related to morbidity and mortality particularly in TSC2 associated cases, which typically have a more severe phenotype. Targeted medical therapy is indicated and shown to be effective for the treatment of benign cardiac tumours causing significant rhythm or mass effect. mTOR inhibitors should be considered in the treatment of rhabdomyomas and beta blockade for haemangiomas.

To summarize the surgical treatment experience and long-term outcomes of patients with anomalous origin of one pulmonary artery from the ascending aorta (AOPA). From December 2009 to December 2022, 76 patients undergoing surgical treatment for AOPA in our hospital were enrolled. Two different reimplantation methods were used to correct the anomaly, including direct anastomosis in 45 (group A) and angioplasty with autologous tissue in 31 patients (group B). Early and late outcomes were compared between the two groups, and the independent risk factors for aberrant pulmonary artery(aPA) restenosis were determined. The median age at repair was 90 (8-1211) days. Hospital death occurred in two patients. During the follow-up period, there was no all-cause death. One patient in group A was lost to follow-up. Freedom from postoperative aPA restenosis at 1, 5, and 10 years in A group was 92.3, 85.4, and 85.4%, respectively. Freedom from postoperative aPA restenosis at 1, 5, and 10 years in B group was 75.2, 63.3, and 57.5%, respectively (log-rank, P = 0.038). Multivariate Cox proportional hazards regression analysis showed that smaller innate Z-score of aPA and angioplasty with autologous tissue were independent risk factors for aPA restenosis. Freedom from reintervention for aPA restenosis at 1, 5, and 10 years in A group was 97.1, 93.0, and 93.0%, respectively. Freedom from reintervention for aPA restenosis at 1, 5, and 10 years in B group was 95.7, 85.3, and 77.5%, respectively (log-rank, P = 0.235). Surgical reimplantation in AOPA patients has resulted in favorable long-term outcomes. Direct anastomosis is superior to angioplasty with autologous tissue in avoiding late aPA restenosis. Intrinsic dysplasia of aPA also increases the incidence of stenosis. However, the type of reimplantation did not significantly affect late reintervention.

Partial heart transplantation is a novel approach to deliver a growing donor valve in pediatric recipients needing valve replacements. Objective data on the rate of growth of semilunar valves in patients following orthotopic heart transplantation (OHT) are necessary to set expectations for partial heart transplant semilunar valve growth. A retrospective cohort study was performed, which included twelve infants who underwent OHT and twelve controls with ventricular septal defects (VSD). Semilunar valve annulus absolute dimension over serial echocardiograms was recorded, Z-scores were calculated, and mixed-effects models were applied to the absolute dimension (mm) and scaled dimension (Boston Z-score). Aortic and pulmonary valve annuli in OHT patients grow. There is a downward trend in aortic valve annulus Z-score over time for OHT patients compared to population norms and controls with VSDs (difference in slopes: - 0.119 Z-score/y, 95% CI: [- 0.209, - 0.029], p = 0.011); there is a non-significant difference for the pulmonary valve annulus (difference in slopes: - 0.067 Z-score/y, 95% CI: [- 0.155 0.022], p = 0.140). Semilunar valves in pediatric OHT patients grow at a slower rate than controls. There was no semilunar valve obstruction in our cohort. While the described difference in valve growth may not be clinically significant for pediatric OHT recipients, these growth rates inform the anticipated growth trajectory for the partial heart transplant graft.