Dilatation In Children Research Articles

New systemic inflammatory indices as predictors of ascending aortic dilation in children with bicuspid aortic valve: A retrospective cross-sectional study.

Systemic inflammatory indices, originally developed to predict the prognosis of cancer patients, have found increasing application in various medical areas, including cardiovascular research. This study aimed to investigate the relationship between ascending aortic dilatation in bicuspid aortic valve patients and systemic inflammatory indices. This retrospective cross-sectional study included 122 patients with bicuspid aortic valves. These patients were divided into 2 groups based on the presence or absence of dilatation according to ascending aorta z-scores. Complete blood counts were analyzed, focusing on leukocyte, neutrophil, lymphocyte, monocyte, and platelet counts. Additionally, systemic inflammatory indices including neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio (PLR), Systemic Immune-Inflammation Index (SII), Systemic Inflammatory Response Index (SIRI), and monocyte-to-lymphocyte ratio (MLR) and pan-immune-inflammation value (PIV) were calculated from these parameters. MLR, SIRI, and PIV demonstrated acceptable diagnostic power in detecting ascending aortic dilatation in bicuspid aortic valve patients, with area under the curve (AUC) values of 0.709, 0.741, and 0.779, respectively. PLR and SII exhibited fair diagnostic power, with AUC values of 0.673 and 0.688, respectively. According to the receiver operating characteristic analysis, PIV had the highest AUC value of 0.779 (95% confidence interval [CI] = 0.69-0.86), with a sensitivity of 70.9% and specificity of 70.8% at a cutoff value of 224.93. A relationship exists between PLR, MLR, SII, SIRI, PIV, and ascending aorta dilatation in pediatric patients with bicuspid aortic valves. These findings suggest that inflammation may play a role in the dilatation of the ascending aorta in bicuspid aortic valve patients.

Circulating Matrix Metalloproteinases for Prediction of Aortic Dilatation in Children with Bicuspid Aortic Valve: A Single-Center, Observational Study

Circulating biomarkers have been proposed for early identification of aortic dilatation progression associated with bicuspid aortic valve (BAV), but matrix metalloproteinases (MMPs) are distinguished as signatures of increased extracellular matrix degradation, a landmark of aneurysm formation. The current study aims to identify the role of MMP-1, MMP-2, MMP-9, and the MMP inhibitor, TIMP-1, in identifying aortic dilation in children with BAV. We conducted a study on 73 children divided into two study groups, depending on the presence of aortic dilatation (group 1–43 BAV controls and group 2–30 children with BAV and aortic dilatation). Each patient underwent a cardiac ultrasound and, in each case, serum MMP-1, MMP-2, MMP-9, and TIMP-1 were quantified using xMAP technology. Comparison of the MMPs between the two study groups revealed significantly higher values only in the case of TIMP-1, among BAV controls. Moreover, the same TIMP-1 inversely correlated with aortic annulus absolute size and z score, as well as with ascending aorta z score. No particular correlation between the aortic phenotype and the presence of aortic dilatation was found. Future longitudinal research starting at pediatric ages could show the significance of MMPs screening in BAV individuals as predictors of aortic aneurysm formation.

Clinical study on the effect of different administration methods of compound topicamide on pupil dilation in children.

Clinical study on the effect of different administration methods of compound topicamide on pupil dilation in children.

Characteristics and outcomes of minimally invasive surgery for congenital biliary dilatation in children aged

We evaluated the minimally invasive surgery for congenital biliary dilatation (CBD) in adults and children and analyzed the surgical outcomes, especially in children aged <6 years. Characteristics and surgical outcomes of patients with CBD who underwent minimally invasive surgery at our hospital between 2013 and 2023 were retrospectively reviewed. Overall, 129 patients (89 children aged <6 years, 9 children aged between 6 and 18 years, and 21 adults) were included in this study. Children exhibited more protein plug presence and abnormal biochemical data than adults. Incidence of postoperative pancreatic fistulas was highest in the adult group (3.4%, 11%, and 33%, respectively, p < .01). In children aged <6 years, postoperative bile leakage and pancreatic fistulas occurred in 9.0% and 3.4% of patients, respectively. Multivariate logistic regression analysis revealed that the Todani IVA was associated with a decrease in postoperative bile leakage (odds ratio: -1.7; 95% confidence interval: (-3.3)-(-0.22), p = .03). Adults with CBD required prolonged operative times and had more short-term complications than children with CBD. In children aged <6 years, minimally invasive surgery for CBD can be safely performed; however, a small diameter of the bile duct may be associated with bile leakage.

A nomogram prediction of coronary artery dilation in Kawasaki diseases based on mtDNA copy number.

The level of mitochondrial DNA copy number (mtDNA-CN) in peripheral blood cells had been identified to be involved in several immune and cardiovascular diseases. Thus, the aim of this study is to evaluate the levels of mtDNA-CN in Kawasaki disease (KD) and to construct a nomogram prediction for coronary artery lesions in children with KD. One hundred and forty-four children with KD diagnosed from March 2020 to March 2022 were involved in the study. The clinical features and laboratory test parameters of these children were assessed between the KD and normal groups. Univariable and multivariable analyses were performed sequentially to identify the essential risk factors. Subsequently, a nomogram prediction was constructed. A total of 274 children were included in the analysis. Of these, 144 (52.6%) represented the KD group. Peripheral blood DNA mtDNA qPCR showed that the -log value of mtDNA-CN in the KD group (6.67 ± 0.34) was significantly higher than that in the healthy group (6.40 ± 0.18) (P<0.001). The area under the ROC curve for mtDNA-CN in distinguishing KD was 0.757. MtDNA-CN (OR = 13.203, P = 0.009, 95% CI 1.888-92.305), RBC (OR = 5.135, P = 0.014, 95% CI 1.394-18.919), and PA (OR = 0.959, P = 0.014, 95% CI 0.927-0.991) were identified as independent risk factors for coronary artery dilation in children with KD. Finally, the nomogram predictive was established based on the results of multivariable analysis, demonstrating the satisfied prediction and calibration values. The results of this study revealed that mtDNA-CN could be used as a biomarker in predicting the development of KD. Furthermore, the higher the mtDNA-CN was significantly associated with coronary artery dilation in KD.

Comparison of Risk-Scoring Systems in Predicting Kawasaki Disease Associated Coronary Artery Dilation in a North American Cohort.

Scoring systems used to predict morbidity in children with Kawasaki disease (KD) have been developed and validated in Asian populations. The objective of this study was to assess their utility in predicting the development of coronary artery dilation in children with KD in North America. This was a secondary analysis of a National Institutes of Health / National Heart, Lung, and Blood Institute (NIH/NHLBI) Pediatric Heart Network public use dataset from a multicenter, randomized controlled trial of pulse steroid therapy in KD in a North American cohort. The primary outcome of interest was development of coronary artery dilation. The Harada, Kobayashi, Egami, and Sano scoring systems, originally developed to predict risk of intravenous immunoglobulin (IVIG) resistance in Kawasaki patients in Japan, were applied to this cohort. Subjects were kept in the analysis only if there were complete data for every element of each scoring system-Harada (n = 132), Kobayashi (n = 88), Egami (n = 139), and Sano (n = 82). Patients classified as high-risk by the Harada score were more likely to have significant coronary artery dilation (p = 0.042), were more likely to require IVIG retreatment (p = 0.002), and were more likely to require hospital readmission (p < 0.001). The Egami, Kobayashi, and Sano scores were not predictive for any measured outcome. The Harada score can be useful in identifying KD patients at risk for developing coronary artery dilation and IVIG resistance. The Harada score has demonstrated higher sensitivity but lower specificity, making it a valuable screening tool that may benefit from supplementary diagnostic methods.

#1607 Prevalence and risk factors of aortic root dilatation in children with X-linked Alport syndrome

Abstract Background and Aims Aortic dilatation in X-linked Alport syndrome (XLAS) is considered a specific manifestation. On the other hand, all patients with CKD have been shown to have an increased risk of aortopathies. The aim was to define prevalence and risk factors for aortic dilatation in children with XLAS. Method Retrospective cross-section single center study included XLAS’ children (n = 98,64 M) and patients with CACUT (comparison group, n = 26,12 M). The aorta was measured at level of the sinus of Valsalva (SoV), aortic dilatation was determined by z-score &amp;gt;2 for BSA. Body mass index (BMI, kg/m2), z-scores of mean systolic blood pressure (SBP), mean diastolic blood pressure (DBP), mean blood pressure (MBP), proteinuria (Pr, mg/m2/day), eGFR (ml/min/1.73 m2), z-scores of left ventricular mass index (LVMI), left ventricular end diastolic diameter (LVEDD), relative wall thickness of the left ventricle (RWT, N &amp;lt; 0,42) data were obtained. Results There were no difference in age (11,12 ± 3,75 vs 10,87 ± 2,34 yrs; p = 0,78), gender (males 0,55 vs 0,46; p = 0,45), BMI (18,7 ± 3,7 vs 19,1 ± 4,7; p = 0,78), eGFR (103,1 ± 16,9 vs 90,5 ± 11,4; p = 0,50), prevalence of hypertension (0,11 vs 0,19; p = 0,21) between the groups. The prevalence of SoV dilatation did not differ between XLAS and CACUT (0,06 vs 0,08; p&amp;gt;0,18). SoV dilatation was associated with male gender (β1 = 0,31, p = 0,02; β2 = 0,34, p = 0,02), LVMI (β1 = 0,26, p = 0,02; β2 = 0,24, p = 0,02) in both groups, with BMI (β1 = -0,27, p = 0,03), LVEDD (β1 = 0,21, p = 0,04) in children with XLAS. No statistically significant effect of SBP, DBP, MBP, Pr, eGFR on SoV dilatation has been demonstrated. Conclusion The prevalence of SoV dilatation in children with XLAS was higher than in general population, but comparable to children with CACUT. The male gender, low BMI, LVMI and LVEDD were associated with SoV dilatation in XLAS. We did not show the relationship between blood pressure load, proteinuria, eGFR and SoV dilatation in XLAS.

Clinical significance of incidental common bile duct dilatation in children: A 10-year single medical center experience

BackgroundWith the widespread use of abdominal ultrasonography (US), incidental detection of common bile duct (CBD) dilatation is common in pediatric populations. This study investigated the causes and clinical significance of CBD dilatation in children without biliary symptoms, jaundice, or causative lesions in US. MethodsWe retrospectively reviewed pediatric patients with CBD dilatation from July 2013 to June 2023. All cases were detected via abdominal US. We analyzed the patients’ clinical manifestations, laboratory data, diagnosis, underlying diseases, and clinical course. ResultsIn a total of 687 patients enrolled, 338 met inclusion criteria (90 in hepatobiliary, 248 in CBD dilatation group). Of 128 patients with incidental CBD dilatation who underwent regular US examinations, 91 (71.1%) experienced resolution during follow-up. The proportion of patients with intrahepatic duct dilatation was significantly higher in the non-resolution group (p = 0.038). General health examination group had significant smaller CBD diameter compared to the gastrointestinal and infection groups. Correlation analysis found starting point of resolution decline at 3.24 mm (all-inclusive) and 2.51 mm (infant group) CBD diameter. ConclusionsMost children with incidental CBD dilatation did not have abnormal hepatobiliary function or other sonographic abnormalities. They usually remained asymptomatic and experienced uneventful clinical courses.

Aortic Dilatation in Children and Young People With ADPKD

IntroductionAortic root dilatation is a reported cardiovascular sequela seen in children and young people (CYP) with chronic kidney disease (CKD), but has yet to be described in those with Autosomal dominant polycystic kidney disease (ADPKD). MethodsSingle centre, cross-sectional study in a dedicated ADPKD clinic. Echocardiograms were evaluated for the presence of dilatation [defined by a z-score ≥2 (≥99th percentile) standard deviations from the mean] at four standardised locations: the aortic valve annulus, Sinuses of Valsalva (SoV), sinotubular junction (STJ) and the ascending aorta. Measurements were compared with a control group to assess prevalence, severity and determinants of aortic dilatation. Results97 children, median age [IQR] of 9.3 [6.1, 13.6] years were compared with 19 controls without ADPKD or other CKD. The prevalence of dilatation ranged from 5.2-17% in ADPKD, depending on anatomical location with no aortic dilatation identified in the control group. In multivariable regression, aortic root dilatation was strongly and positively associated with cyst burden at the aortic valve annulus and SoV (β= 0.42 and β=0.39, both p<0.001), with age at SoV (β= -0.26, p=0.02), SBP z-score at SoV (β= -0.20, p=0.04) and LVMI at SoV and STJ (β= 0.24, p=0.02 and β= 0.25, p=0.03 respectively) following adjustment for age, sex, BMI z-score, eGFR, SBP z-score and LVMI. ConclusionsOur data suggests increased prevalence of aortic root and ascending aortic dilatation in CYP with ADPKD compared with controls. Further studies are needed to understand the pathogenesis and its contribution to the high cardiovascular morbidity in ADPKD.

Predictor of mitral valve regurgitation severity and left ventricular dilatation using amino-terminal pro-brain natriuretic peptide marker in pediatric rheumatic heart disease.

Early rheumatic heart disease (RHD) is characterized by valve regurgitation, leading to ventricular distention and possible elaboration of amino-terminal pro-brain natriuretic peptide (NT-proBNP). Thirty-one children with RHD were entered in the study. Transthoracic echocardiography was performed in all patients to assess the severity of the valve disease and cardiac function, thus will be classified into three groups: mild, moderate, and severe mitral valve regurgitation (MR), with and without left ventricular (LV) dilatation. At the time of echo, venous blood samples were drawn; thus, NT-proBNP levels were measured using sandwich immunoassay. The median NT-proBNP levels in mild, moderate, and severe MR were 32.34, 120.75, and 7094 pg/ml, respectively. The median NT-proBNP levels in patients with and without LV dilatation were 3045 and 30.82 pg/ml. There was a significant correlation between the severity of MR and NT-proBNP levels (P < 0.001), thus a significant correlation between LV dilatation and NT-proBNP levels (P = 0.013). A cutoff value of 2598.50 pg/ml was obtained with a sensitivity value of 90% and a specificity of 90.5% for NT-proBNP levels against severe MR. A cutoff value of 199.35 pg/ml was obtained with a sensitivity value of 73.3% and a specificity of 75% for NT-proBNP levels on LV dilatation. A cutoff value of 2598.50 pg/ml was obtained with a sensitivity value of 85.7% and a specificity of 79.2% for NT-proBNP levels against severe MR with LV dilatation. There was a significant relationship between NT-proBNP levels and the severity of MR and LV dilatation in children with RHD.

Discussion to: Influence of preoperative aortic regurgitation on long-term autograft durability and dilatation in children and adolescents undergoing the Ross procedure

Discussion to: Influence of preoperative aortic regurgitation on long-term autograft durability and dilatation in children and adolescents undergoing the Ross procedure

Clinical analysis of endoscopic esophageal dilation for the treatment of corrosive esophageal strictures in children

To investigate the clinical application of endoscopic esophageal dilation in the treatment of corrosive esophageal strictures in children. A retrospective analysis was performed on the clinical data of 15 children with corrosive esophageal strictures who underwent endoscopic esophageal dilation in Children's Hospital, Zhejiang University School of Medicine. The clinical features, treatment modality of endoscopic esophageal dilation, number of dilations, complications, and prognosis were reviewed. A total of 96 esophageal dilations were performed in the 15 children with corrosive esophageal strictures, with a median of 6 dilations per child. Among them, 9 children (60%) underwent 6 or more dilations. The children with a stricture length of >3 cm had a significantly higher number of dilations than those with a stricture length of ≤3 cm (P<0.05). The children with strictures in a single segment had a significantly better treatment outcome than those with strictures in multiple segments (P=0.005). No complication was observed during all sessions of dilation. The overall effective rate (including significant improvement and improvement) of endoscopic esophageal dilation treatment was 87%, with 2 cases of failure. Endoscopic esophageal dilation is an effective and relatively safe treatment method for corrosive esophageal strictures in children, and children with strictures in a single segment tend to have a better treatment outcome than those with strictures in multiple segments.

Efficacy of bougie versus balloon dilatation in children with benign esophageal stricture: a propensity score–matched retrospective cohort study

Background and AimsEndoscopic dilatation is the primary management of esophageal strictures in children. However, pediatric data on which of the two endoscopic techniques, bougie or balloon, is better, and the effect of endoscopic dilatation on the growth of children is lacking. We aimed to evaluate (i) whether bougie or balloon dilatation is superior in efficacy and safety for short esophageal strictures in children and (ii) the impact of endoscopic dilatation on their growth. MethodsWe performed endoscopic dilatation every 2 to 3 weekly using either a bougie or balloon and considered it adequate if we could dilate the esophageal lumen to 15 mm (11mm in < 2 years, 12mm in 2-5years of age) with complete relief of symptoms for >2 months. ResultsTwo-fifty-nine children were enrolled, with 2580 dilatations performed during the study. We performed a comparative analysis on 77 children where an exclusive bougie versus balloon dilatation was carried out for short strictures (defined as stricture length <5cm). Both bougie and balloon showed similar efficacy [sessions for adequate dilatation: 5 (2.5-7.5) vs. 4 (2-6), p=0.40] and safety [perforation rate; 0.54% vs. 0.35%, p=0.591] for short strictures. On follow-up (median of 17 months), there was a significant improvement in both weight and height z-scores in children with successful dilatation. ConclusionsEndoscopic bougie and balloon dilatations were safe and effective, with no significant difference for short strictures. Successful dilatation resulted in significant improvement in growth on follow-up.

Cutoffs and Characteristics of Abnormal Bowel Dilatation in Pediatric Short Bowel Syndrome.

Although excessive intestinal dilatation associates with worsened outcomes in pediatric short bowel syndrome (SBS), little is known about the natural history and definition of pathological dilatation. We addressed fore-, mid-, and hind-gut dilatation in children with SBS, who had not undergone autologous intestinal reconstructive (AIR) surgery, in relation to controls. SBS children without history of AIR surgery (n = 59) and age-matched controls without any disclosed intestinal pathology (n = 140) were included. Maximum diameter of duodenum, small bowel (SB), and colon were measured in each intestinal contrast series during 2002 to 2020 and expressed as diameter ratio (DR) to L5 vertebrae height. Predictive ability of DR for weaning off parenteral nutrition (PN) was analyzed with Cox proportional hazards regression models using multiple cutoffs. Duodenum (DDR), SB (SBDR), and colon (CDR) DR were 53%, 183%, and 23% higher in SBS patients compared to controls ( P < 0.01 for all). The maximal DDR and SBDR measured during follow-up is associated with current PN dependence and young age. DDR correlated with SBDR ( r = 0.586, P < 0.01). Patients with maximal DDR less than 1.5, which was also the 99th percentile for controls, were 2.5-fold more likely to wean off PN ( P = 0.005), whereas SBDR and CDR were not predictive for PN duration. All segments of remaining bowel, especially SB, dilate above normal levels in children with SBS. In SBS children without AIR surgery, PN dependence and young age is associated with duodenal and small intestinal dilatation, while duodenal dilatation also predicted prolonged PN.

Competitive Sports Participation is Associated with Decreased Risk of Obesity in Children with Bicuspid Aortic Valve.

Individuals with bicuspid aortic valve (BAV) have historically been advised to avoid contact sports and isometric exercise for risk of increasing aortic dilation and valve disease. There is mounting evidence that current sports participation guidelines qualify children for a high rate of sports exclusion, and that this population is at increased risk of obesity. The primary aim of this study was to evaluate relationship between sports participation and aortic dilation in children with bicuspid aortic valve and secondarily the relationship between competitive sports participation and obesity. We performed a review of children between the ages of 8 and 17years with isolated BAV followed at Oregon Health & Science University. We excluded those with other congenital heart diseases, genetic conditions, prior cardiac intervention, and inability to ambulate independently. Parents completed a phone survey detailing their child's level of daily activity and participation in competitive sports. Demographic information and most recent echo findings were collected from the electronic medical record. We found no difference between the aortic diameters of athletes vs non-athletes. We also found that sports participation and daily activity were both associated with a decreased likelihood of obesity (OR 0.24, 95% CI 0.078-0.73 and OR 0.24, 95% CI 0.081-0.71 respectively). In summary, in our sample population, competitive sports participation was associated with a decreased risk of obesity. Competitive sports participation does not appear to be associated with an increased risk of aortic dilation in our population.

Influence of preoperative aortic regurgitation on long-term autograft durability and dilatation in children and adolescents undergoing the Ross procedure

ObjectivePrimary aortic insufficiency (AI) is a risk factor for autograft reintervention in adults undergoing the Ross procedure. We sought to examine the influence of preoperative AI on autograft durability in children and adolescents. MethodsFrom 1993 to 2020, 125 consecutive patients between ages 1 and 18 underwent a Ross procedure. The autograft was implanted using a full-root technique in 123 (98.4%) and included in a polyethelene terephthalate graft in 2 (1.6%). Patients with aortic stenosis (aortic stenosis group) (n = 85) were retrospectively compared with those with AI or mixed disease (AI group) (n = 40). Median length of follow-up was 8.2 years (interquartile range, 3.3-15.4 years). The primary end point was the incidence of severe AI or autograft reintervention. Secondary end points included changes in autograft dimensions analyzed using mixed-effect models. ResultsThe incidence of severe AI or autograft reintervention was 39.0% ± 13.0% in the AI group and 8.8% ± 4.4% in the aortic stenosis group at 15 years (P = .02). Annulus z scores increased in both aortic stenosis and AI groups over time (P < .001). However, the annulus dilated at a faster rate in the AI group (absolute difference, 3.8 ± 2.0 vs 2.5 ± 1.7; P = .03). Sinus of Valsalva z scores increased in both groups as well (P < .001), but at similar rates over time (P = .11). ConclusionsChildren and adolescents with AI undergoing the Ross procedure have higher rates of autograft failure. Patients with preoperative AI have more pronounced dilatation at the annulus. Akin to adults, a surgical aortic annulus stabilization technique that modulates growth is needed in children.

Esophageal dilation through bouginage or balloon catheters in children, as the treatment of benign esophageal strictures: results, considering the etiology, and the methods.

Corrosive substance ingestion, history of esophageal surgery, and reflux esophagitis are the main causes of benign esophageal strictures in children. Esophageal dilation is the first treatment option. Bougies and balloons are the most frequently used dilation tools. The literature record on esophageal dilation methods and their results is mostly composed of data gathered from adults, who differ from children in many terms, including etiology, indications, and results. This study aims to evaluate esophagial dilation in children; comparing the two mentioned modalities; and considering the impact of different diseases on dilation success. The benign esophageal stricture cases who had undergone esophageal dilation between 2001 and 2009, at two tertiary health-care centers of a university were evaluated retrospectively with regard to stricture etiology, treatment methods, and their results. In addition, balloon and bougie dilations were compared. Fifty-four cases were dilated in 447 sessions. The strictures were due to corrosive ingestion or anastomoses in 72.2% of the cases. Of the dilation sessions, 52.6% were performed with Savary-Gilliard bougies, and the rest with balloon dilators. No guidewire was needed in 53.2% of the bougie sessions. Fluoroscopy was used during balloon dilation sessions as a routine part of the method, while it was needed only to check the guide location when needed during the bougie dilation sessions. The complication rates of balloon and bougie dilation sessions were 2.4% and 2.1%, respectively. The mean session length was 26.2±11.8 and 42.6±13.7 min, for bougie and balloon, respectively. Success rate was 93.7% for the balloon, while 98.2% of the bougie sessions. Balloon catheters used were disposable. Savary-Gilliard bougies have advantages over balloon catheters with less need of fluoroscopy, shorter duration of sessions, and lower cost. Both methods are equivalently safe with close complication rates.

Retracted: Carotid Intima-Media Thickness and Ankle-Brachial Index and Their Correlation with Coronary Artery Dilatation in Children with Kawasaki Disease.

[This retracts the article DOI: 10.1155/2021/7372424.].

Factors influencing outcomes of esophageal balloon dilatation for anastomosis site stenosis after esophageal atresia surgery.

Esophageal balloon dilatation is an effective treatment for anastomotic strictures, but the factors affecting the outcome of dilatation remain unclear. To investigate the predictive factors of esophageal balloon dilatation outcome in children with anastomotic stricture after esophageal atresia repair. We retrospectively reviewed children with esophageal atresia who underwent esophageal balloon dilatation for postoperative strictures between August 2007 and February 2021. We investigated each child's age, weight and height; type of esophageal atresia surgery; shape, length and level of stricture; esophageal balloon dilatation balloon size; application of mitomycin; number of inflation sessions; and number of esophageal balloon dilatation sessions. The outcome of each esophageal balloon dilatation session was determined as improvement in stricture diameter between pre- and post-esophageal balloon dilatation esophagography. We used uni- and multivariate analyses with generalized estimating equations to evaluate outcome predictors. Overall, 69 children (mean age, 2.3years; 45 boys) underwent 227 esophageal balloon dilatations. In the univariate analysis, the positive effect of esophageal balloon dilatation decreased with increased age, weight, height, balloon size and number of esophageal balloon dilatation sessions. Additionally, the positive effect was decreased in cervical-level strictures and with the application of mitomycin during esophageal balloon dilatation. In the multivariate analysis, independent prognostic factors of the positive esophageal balloon dilatation effect were age (incidence rate ratio [IRR]: -0.01; 95% confidence interval [CI]: -0.01, -0.002), shape of stricture (IRR: -0.54; 95% CI: -0.91, -0.18) and number of esophageal balloon dilatation sessions (IRR, -0.10; 95% CI: -0.14, -0.18). Repeated esophageal balloon dilatation, older age and eccentric stricture shape are associated with poor response to esophageal balloon dilatation in children with anastomotic strictures after esophageal atresia repair.

Yield of echocardiographic screening of first-degree relatives of children with bicuspid aortic valve

Abstract Background Bicuspid aortic valve (BAV) is the most common congenital heart defect, affecting 0.8% of new-borns. BAV is associated with valve dysfunction, as well as an increased risk of aortopathy. The prevalence of BAV in first-degree relatives of symptomatic BAV patients, is reported to be 5–10%. First-degree relatives also have an increased risk of aortic dilatation, independently of aortic valve morphology. Purpose The purpose of the study was to determine the prevalence of BAV, associated valve dysfunction and aortopathy in first-degree relatives to children diagnosed with BAV neonatally in a population-based study. Methods Between April 2016 and October 2018 all expecting parents at three major maternity centres were offered inclusion in a large-scale population study with focus on congenital heart disease (N≥25,000). A total of 197 children, in 196 families, were diagnosed with BAV. All first-degree relatives, including half-siblings, were offered inclusion in the follow-up study with standardized transthoracic echocardiography. Adults were also examined with transoesophageal echocardiography. Aorta diameters were measured at the AV annulus, sinuses of Valsalva, sino-tubolare junction and in the proximal ascending aorta. Aortic dilatation in children were defined as any aortic root or ascending aorta diameter ≥2 standard deviations (SD) from the expected mean, calculated as z-score using formulas from the Paediatric Heart Network Echocardiogram Database. In adults, aortic dilatation was defined as aortic root and/or ascending aorta diameters indexed to body surface area (BSA) exceeding normal reference values established by the European Association of Cardiovascular Imaging. Results In total, 352 first-degree relatives (242 adults [35.3 years SD 5.5] and 110 children [4.5 years, SD 3.5] were included. BAV was diagnosed in 24 relatives (6.8%). BAV could not be conclusively ruled out in 52 relatives (14.8%), who are awaiting further examination with transoesophageal echocardiography. Dilatation of the aortic root was observed in 8 adult relatives and in 17 children (7.1%). Aortic valve regurgitation was observed in 23 relatives (6.5%). In total 58 relatives (16.2%) were diagnosed with either BAV, dilated aorta, or aortic regurgitation. Conclusion One in fifteen first-degree relatives to children diagnosed with BAV neonatally, also had BAV, corresponding to &amp;gt;8 fold increase in prevalence compared with the background population. One in six relatives had BAV, dilated aorta, or aortic regurgitation. These findings are of importance for family-screening programs of BAV. Funding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Danish Children's Heart Association