Pediatric Cardiology最新文献

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.

Protein-losing enteropathy (PLE) is a serious complication after the Fontan operation with limited treatment options. This phase 2, multi-center, open-label trial evaluated the efficacy and safety of Camostat Mesylate (CM), a serine protease inhibitor, as adjunctive therapy for PLE. Nineteen patients aged 4 years and older with PLE after the Fontan operation were enrolled. CM was administered for six months in addition to their individualized conventional treatments. Assessments were made at 1, 3, and 6 months of CM administration, and at one month after CM discontinuation. Outcomes evaluated were the changes in serum albumin level, stool alpha-1 antitrypsin, and clinical symptoms such as, diarrhea, edema, weight change, and ascites. Of the 19 patients enrolled, 4 voluntarily withdrew consent, and the data from the 15 patients who completed the study were analyzed. Their median age was 15.0 years (interquartile range, 12.0-21.5) and the median time between the Fontan surgery and PLE diagnosis was 2.4 years. Serum albumin levels increased from 2.2 to 2.5 g/dL (p = 0.183), while stool alpha-1 antitrypsin levels significantly decreased from 215.6 to 75.5 mg/dL (p = 0.016) over six months. Patients with baseline diarrhea showed notable improvements: serum albumin increased from 1.8 to 2.4 g/dL (p = 0.138) and stool alpha-1 antitrypsin decreased from 220.3 to 75.5 mg/dL (p = 0.075) over 6 months. No serious adverse events occurred. CM demonstrated significant reductions in gastrointestinal protein losses, particularly in patients with baseline diarrhea. Trial registration NCT05474664.

Fetal cardiology has grown into a robust pediatric cardiology subspecialty in the last two decades, with many congenital heart centers having a dedicated fetal cardiac program. Despite the subspeciality's clear maturation, there are no multicenter data to describe volume, practice patterns, program structures, resources, or trends. The Fetal Heart Society sought to address this deficiency by conducting an international survey of fetal cardiac programs. A survey was distributed internationally to fetal cardiac programs. One response per institution or clinical practice was collected. Respondents were asked to provide data from the 2022 calendar year. Ninety-five programs responded, with the majority representing the United States of America (n = 75, 79%) or Canada (n = 11, 12%). Most responding programs (88%) had an academic or university affiliation, and 69% were from independent children's hospitals. The median number of fetal echocardiographic studies performed annually per program was 1,000 (IQR 580-2,100). There was a median of 5 (IQR 3-7) fetal cardiologists and 5 (IQR 3-7) sonographers per program. Each fetal cardiologist interpreted an estimated median of 6 (IQR 5-8) fetal studies in a full day shift. The most common duration allocated for each fetal echocardiographic study was 45-59 min for the initial study (51%) and 45-59 min for the follow-up study (42%). 79% of programs had a fetal cardiac nurse coordinator. An independent fetal database was maintained at 78% of programs. Less than half of programs (46/95) had a formal quality improvement (QI) initiative, with only 22 programs participating in national-level QI metrics. The most frequently reported barrier to having a fetal cardiac QI program was a lack of human resources (60%), followed by a lack of institutional support/incentive (41%). Programs were more likely to have a formal fetal cardiology QI program if they had a fetal cardiac coordinator (p = 0.0029) if they had a formal fetal database (p = 0.003), or if they were a larger volume program (p = 0.026). Certain subspecialties were available at most programs, including neonatology (93%), maternal-fetal medicine (88%), genetic counseling (88%), and social work (79%). However, psychology (38%) and psychiatry (16%) services to address parental mental health issues were not as commonly available. These survey data provide a novel and comprehensive view of fetal cardiology programs with information useful for internal benchmarking, quality improvement initiatives, resource allocation, and identifying unmet needs.

The aim of this study was to assess the current status about quality grading, resources, and training in pediatric echocardiography, to define gaps in this field, and to develop potential strategies for quality improvement. A structured questionnaire was sent out to pediatric cardiologists within the Association for European Pediatric and Congenital Cardiology (AEPC). The questionnaire contained questions regarding assessment of quality, training and feedback in the field of pediatric echocardiography. Thirty-one European pediatric cardiologists from 17 countries participated. Most participants agreed (n = 28, 90%) that it is important to have standards for echocardiography quality grading for trainees. Objective instruments, however, are largely not available. Among a list of criteria on how to grade quality, quantitative or qualitative criteria with additional formative feedback was ranked highest by the respondents (53%). Although the correct diagnosis, followed by the correct use of two-dimensional (2D) imaging and the correct use of color Doppler across all valves and septae were listed as most important when performing transthoracic echocardiography, a matrix of the eight most important parameters was designed. The results show that quality grading in pediatric echocardiography varies highly among European centers. The matrix provided is a visual instrument whereby trainees can gauge the evolution in their skill as echocardiographers.

Detailed characterization of myocardial deformation, ventricular shape, outflow tract size, inflow Doppler patterns, cerebroplacental circulation, and cardiac output of fetuses with suspected coarctation of the aorta (COA) and a control group to gain further insights into differences between these groups. Expectant women were prospectively recruited for assessment during the third trimester of pregnancy and a comparison of echocardiographic characteristics and fetoplacental circulation according to postnatal diagnosis of either confirmed COA (c-COA), false-positive COA (fp-COA), and a control population. There were 42 fetuses recruited with suspected COA of whom 20/42 (48%) had c-COA. Fetuses with c-COA demonstrated lower (less negative) LV global longitudinal strain (LV-GLS) compared to controls (- 20.2% ± 4.3 vs. - 23.1% ± 2.7, p = 0.01) and a non-significant trend to lower strain in the fp-COA group (LV-GLS: - 20.7% ± 5.0, p = 0.053) compared to controls. RV-GLS was significantly reduced in the fp-COA group compared to the c-COA and control groups (fp-COA: - 19.8% ± 4.5, c-COA: - 23.1% ± 4.4, control: - 23.5% ± 3.6, p = 0.04). C-COA and fp-COA had a less spherical (narrower) LV, shorter LV, and a more spherical RV compared to controls. The arterial duct diameter was larger in c-COA compared to fp-COA and controls. When analyzed according to diagnostic group, determinants of GLS and sphericity index differed between groups. For c-COA, there was correlation between LV-GLS and RV-GLS (r = - 0.51, p = 0.021). Determinants of LV-GLS in fp-COA were distal transverse aortic arch z-score and umbilical artery PI (p = 0.026, p = 0.037 respectively). The only determinant of RV-GLS in the FP-COA was arterial duct z-score (r = - 0.51, p = 0.019). There are measurable differences in functional parameters between c-COA, fp-COA, and controls. The hemodynamic characteristics of c-COA and fp-COA merit further study and should include study of the RV and cerebroplacental evaluation.

This study aimed to evaluate the nationwide 30-year survival among Danish univentricular heart patients who underwent Fontan completion between 1977 and 2023. Secondary objectives included assessment of the impact of era, Fontan type, and ventricular dominance on survival. Finally, clinical performance and the prevalence of select morbidity were described for survivors. The Danish Fontan cohort of 301 patients was identified using surgical registries and digitalized health records. Information on mortality, morbidity, and clinical performance was collected from digital medical records using national personal identification numbers. Survival analysis was conducted using Kaplan-Meier estimates, and morbidity outcomes were compared for three consecutive age groups (< 18 years, 18-29 years, and ≥ 30 years). The 30-year survival rate after Fontan completion was 87%, while freedom from death, transplant, or Fontan takedown was 79%. Improved outcomes were observed for patients undergoing Fontan completion after 1997 and those with a non-hypoplastic left heart syndrome (HLHS) diagnosis. Morbidity, including ventricular dysfunction, arrhythmia, significant atrioventricular valve regurgitation, and protein-losing enteropathy, was present in 38% of survivors and increased with age. The proportion of (self-reported) physically active individuals and their %pred VO2peak declined with age, with those aged ≥ 30 years demonstrating the highest prevalence of Fontan-related complications (56%) and the lowest exercise capacity (51%pred VO2peak). In conclusion, long-term survival after Fontan completion was 87%. Survival has improved in the later era and was superior for those with a non-HLHS diagnosis. The prevalence of select morbidity was substantial at 38% while also displaying an age-related increase.

Homografts conduits has been the gold standard for right ventricle to pulmonary artery (RV-PA) conduit. Several different types of xenograft valved conduits have been used as an alternative to homografts, due to their limited availability. In this single center, retrospective study, we analyzed the outcomes of patients with bovine jugular vein conduit (Contegra) and porcine valved conduit (Biointegral) in terms of survival and reintervention rate. Between 2012 and 2023, 44 children underwent surgical repair with RV-PA conduits using Contegra (n = 20) or Biointegral (n = 24). Patients with truncus arteriosus and patients who underwent unifocalization and Ross procedures were excluded. The operations in which other RV-PA conduits such as homografts, Gore-Tex grafts with PTFE handmade valved were used, were also excluded from the study. The median age of the patients was 19 (3-60) months and 84% of the patients (n = 37) had a history of previous intervention. Hospital mortality was 4.5% (n = 2). The median length of stay in intensive care unit and hospital was 5 (2-63) and 19 (2-145) days, respectively. 36 of the patients (82%) were followed for a median of 68 (4.8-143.7) months. There was one late death in Contegra group and five late deaths in Biointegral group. Survival analysis revealed that 1, 5, and 10-year survival rates were 100%, 90%, and 90% in Contegra group and 81%, 76.2%, and 33.9% (p = 0.047) in Biointegral group, respectively. During follow-up period, 11 patients (30.5%) needed reintervention (n = 3 in Contegra; n = 8 in Biointegral group). Freedom from reintervention rates were 100%, 94.1%, and 47.1% at 1, 5, and 10 years in Contegra group and 100% and 63.3% at 1 and 5 years in Biointegral group, respectively (p = 0.024). In this study, the outcomes of Contegra conduits were statistically significantly better than Biointegral conduits. Contegra is still the most valuable alternative to homografts. We believe that the choice of conduit in the first surgery is an important decision that directly affects survival and re-intervention rates.

Children with congenital heart disease are at increased risk of neurodevelopmental impairment and those with hypoplastic left heart syndrome (HLHS) are among the highest risk group. The first stage of palliation for HLHS, typically performed in the newborn period, is either a Norwood stage I procedure (NS1P) or hybrid stage 1 procedure (HS1P). Our study sought to evaluate the neurodevelopmental outcomes of patients who undergo HS1P compared to NS1P using multicenter registry data. The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry was used to identify infants who had either NS1P or HS1P and completed the Ages and Stages Questionnaires-3 (ASQ-3) at age 6 months. Patient and clinical characteristics and ASQ-3 results were compared between HS1P and NS1P groups. A 6-month ASQ-3 was completed in 459 patients, 42 patients following HS1P and 417 following NS1P. Patients who underwent HS1P were more likely to have a birth weight less than 2.5 kg (14.6% vs. 4.2%, p = 0.01) and have a genetic or chromosomal anomaly (19% vs. 8.2%, p = 0.04). Gross motor skills were the most impaired of the measured domains for the entire cohort. There were no significant differences in impairment in any ASQ-3 domain between the groups, even when the analysis was adjusted for pre-operative mechanical ventilation, non-cardiac anomaly, and center. Despite patients who undergo HS1P representing a heterogenous group with other medical comorbidities, their early neurodevelopmental outcomes were comparable to patients who underwent NS1P.

In single-ventricle circulation, pulmonary vascular resistances (PVR) play a crucial role at various stages of surgical palliation. Increased PVR detected at cardiac catheterization represents a contraindication to Fontan completion and may lead to an early circuit failure in the postoperative period. Pulmonary vasodilator therapy (PVT) may lower PVR and enhance pre- and post-surgical outcomes in Fontan patients. This study reports the experience with the use of PVT in a tertiary center, focusing on its role in lowering PVR before Fontan procedure and assessing its impact on postoperative outcomes. We analyzed 151 pediatric patients with single-ventricle heart diseases in pre-Fontan stage at our institution from January 2014 to December 2023, collecting demographics, anatomical diagnoses, clinical history, administration of PVT, surgical complications, pre-Fontan hemodynamic parameters, duration of intubation, chest tube retention, oxygen therapy needs, and total hospitalization time. In 17 out of 18 patients (94.4%) who were previously considered unsuitable for Fontan completion, a significant decrease in PVR (p = 0.006) was observed after starting PVT, enabling surgery to be performed. Among 113 patients (74.8%) undergoing Fontan, no differences in postoperative outcomes were observed between those who received PVT in the pre-Fontan stage and those who did not. PVT was started in 50 out of 113 patients (44.2%) after surgery, primarily due to elevated pulmonary pressures on invasive monitoring; among them, 24 patients (48%) had already been on therapy prior to the operation. PVT in the postoperative period was associated with worse outcomes compared to patients not receiving therapy, likely due to the more severe conditions of treated patients. The use of PVT during the pre-Fontan stage increases the number of individuals eligible for surgical palliation, with minimal impact on postoperative outcomes.