Dysbiosis of gut microbiota in patients with protein-losing enteropathy after the Fontan procedure.

BACKGROUND: There is a lack of predictive biomarkers for the onset or activity of protein-losing enteropathy (PLE), a Fontan procedure-associated complication. Here, we aimed to identify the gut microbiota composition of patients with active PLE and investigate its relationship with PLE activity. METHODS: This multicenter case-control study involved patients who developed PLE (n = 16) after the Fontan procedure and those who did not (non-PLE; n = 20). Patients with PLE who maintained a serum albumin level of ≥3 g/dL for >1 year were included in the remissive-stage-PLE group (n = 9) and those who did not maintain this level were included in the active-PLE group (n = 7). 16S rRNA gene sequencing analysis of fecal samples was performed using QIIME2 pipeline. Alpha (Shannon and Faith's phylogenetic diversity indices) and beta diversity was assessed using principal coordinate analysis based on unweighted UniFrac distances. RESULTS: Shannon and Faith's phylogenetic diversity indices were lower in the active-PLE group than in the remissive-stage- (q = 0.028 and 0.025, respectively) and non-PLE (q = 0.028 and 0.017, respectively) groups. Analysis of beta diversity revealed a difference in the microbiota composition between the active-PLE and the other two groups. Linear discriminant effect size analysis demonstrated differences in the relative abundance of Bifidobacterium and Granulicatella spp., and Ruminococcus torques between patients with active- and those with remissive-stage-PLE. CONCLUSIONS: Gut microbiota dysbiosis was observed in patients with active PLE. Changes in the bacterial composition of the gut microbiota and decreased diversity may be associated with the severity of PLE.

Case report: High-dose epoprostenol therapy in pediatric patients with pulmonary hypertension and developmental lung disease.

Pulmonary hypertension (PH) with developmental lung disease is a life-threatening disease and accounts for 10%-12% of pediatric PH patients. Administration of specific pulmonary vasodilators to pediatric PH patients has brought about improvement of their long-term prognosis. Intravenous epoprostenol therapy is a gold standard therapy for severe idiopathic pulmonary arterial hypertension (IPAH), but there are few reports demonstrating the efficacy of epoprostenol for pediatric PH patients with developmental lung disease, especially when treating with high doses of epoprostenol. Two cases of pediatric PH patients with alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) and congenital diaphragmatic hernia (CDH) with bronchopulmonary dysplasia (BPD), respectively, treated with epoprostenol above 100 ng/kg/min are presented. In these two cases, severe PH was improved significantly by an aggressive increase of the epoprostenol infusion rate with administration of oral pulmonary vasodilators and appropriate respiratory management, without any significant adverse effects. High-dose epoprostenol therapy may be one of the therapeutic options in pediatric PH patients with developmental lung disease.

Ultrasound-guided femoral vascular access in pediatric cardiac catheterization.

BACKGROUND: In pediatric catheterization, the palpation and landmark (PL) technique is widely used for femoral arterial and venous (FAV) cannulation. Over the past decade, the ultrasound-guided (US) technique has replaced the PL technique. This study aimed to assess the clinical impact of application of the US technique on the success rate and completion time of FAV cannulation during cardiac catheterization in children. METHODS: This is a retrospective observational study of consecutive pediatric patients who underwent cardiac catheterization in a tertiary care children's hospital from April 2016 to March 2022. The association between FAV cannulation success rate and the US technique was analyzed using multiple logistic regression analysis by adjusting for potential confounders, including patient and operator characteristics and procedural details. RESULTS: A total of 749 patients (PL, 378; US, 371) were analyzed. The odds ratio (OR) of the US technique success rate for FAV cannulation was 2.03, 95% confidence interval (CI), 1.10-3.73; p = 0.02. The OR of the cannulation success rate of children aged <1 year was 0.16 (95% CI, 0.03-0.97; p = 0.046). CONCLUSIONS: The US-guided technique was associated with an increased success rate of FAV cannulation, compared with the PL technique. Moreover, age < 1 year was an independent factor associated with a lower success rate of FAV cannulation. The US-guided technique might be an effective procedure in FAV cannulation during cardiac catheterization in children.

Conception by assisted reproductive technology in infants with critical congenital heart disease in Japan.

RESEARCH QUESTION: What is the proportion of infants born as a result of assisted reproductive technology ART across different types of neonatal critical congenital heart disease (CCHD) in a Japanese population? DESIGN: A retrospective analysis of 418 consecutive infants with CCHD that required catheter treatment or surgery within the first 28 days of life or ductal-dependent lesions, in two paediatric centres in Japan, between January 2014 and December 2019. The proportion of ART in infants with each type of CCHD was evaluated. The proportion of ART in infants with univentricular heart defect (UVH) compared with those with biventricular heart defect (BVH) was evaluated. RESULTS: The study group included 229 boys and 189 girls, with a gestational age of 38 ± 2 weeks. Overall, 61 infants (14.6%) were conceived by fertility treatment with 46 (11.0%) conceived by ART. Univentricular heart defect and BVH were identified in 111 infants (26.6%) and 307 infants (73.4%), respectively. The proportion of infants conceived by ART was significantly higher in UVH (16.2%) than in BVH (9.1%) (OR 2.28, 95% CI 1.11 to 4.68, P = 0.025), regardless of maternal age and maternal history of miscarriage. CONCLUSIONS: The proportion of ART in infants with CCHD, especially UVH, was high. These findings could form the basis of a rationale for carrying out fetal echocardiography in fetuses conceived by ART.

The Impact of the Pulmonary Artery Index and Aortopulmonary Collateral Artery Coil Embolization on Intractable Pleural Effusions After a Fontan Surgery.

An intractable pleural effusion is a common comorbidity of a Fontan operation, occasionally leading to undesirable outcomes. The preventive effect of aortopulmonary collateral (APC) coil embolization against a pleural effusion before a Fontan operation is still controversial.This is a retrospective single-center study; among 227 Fontan cases, 57 cases with complete MRI data were analyzed at first. Factors associated with the duration of pleural drainage (median: 6 (2-41) days) and that of postoperative hospital stay (median: 25 (14-91) days) were analyzed using a multiple regression analysis. The pulmonary artery index (PAI; Nakata index) was associated with both the pleural drainage duration (P < 0.05, r2 = 0.17) and postoperative hospital stay (P < 0.05, r2 = 0.10).Thereafter, all the 227 patients were classified into the following three groups: Group A (12 patients in whom the embolization was performed within 30 days before the Fontan surgery), Group B (131 patients in whom the embolization was performed more than 30 days before the Fontan surgery), and Group C (84 patients in whom the embolization was not performed). Patients in Group A were found to be associated with the shortest length of both periods (P < 0.05).Lower PAI values were related to a prolonged pleural drainage duration and postoperative hospital stay. APC coil embolizations may reduce the risk if they are performed shortly (less than 30 days) before the operation.

Valvular changes after aortic valve neo-cuspidization in children: A case series.

BACKGROUND: Aortic valve neo-cuspidization (AVNeo), a procedure wherein the aortic valve is reconstructed utilizing an autologous pericardium, has recently been more commonly performed in children. However, the postoperative morphological changes in the aortic valve of pediatric patients remain unknown. The current study aimed to describe the intraoperative and postoperative findings of aortic regurgitation (AR) and stenosis (AS) after AVNeo in children. METHODS: This case series describes the morphological changes in AR and AS, and their severity, between the perioperative period and 3 months postoperative period after AVNeo in children (<18 years) who underwent AVNeo between April 2016 and March 2020. Data were collected at two measurement points: (i) intraoperative transesophageal echocardiography after weaning from cardiopulmonary bypass (io-TEE); (ii) postoperative transthoracic echocardiography 3 months after the procedure (po-TTE). RESULTS: Seven patients were included in this case series. The number of postoperative AR sites and the ratio of AR jet area to the left ventricular outflow tract area showed a tendency to decrease between io-TEE and po-TTE. All AR sites were integrated during the postoperative period. One patient identified developed intraoperative AS, which maintained its severity after AVNeo. CONCLUSIONS: Most cases exhibited spontaneous improvement in AR, while one developed postoperative AS. Further prospective investigation is, therefore, needed to explore surgical outcomes following AVNeo among children.

Comparison of Clinical Characteristics of Human Metapneumovirus and Respiratory Syncytial Virus Infections in Hospitalized Young Children.

Human metapneumovirus (HMPV) and respiratory syncytial virus (RSV) are the leading causes of acute respiratory tract infection in children, and clinical manifestations of these virus infections are considered similar. To investigate the differences in clinical characteristics between HMPV and RSV infections in young children, we prospectively enrolled children ＜ 3 years old who required hospitalization with acute respiratory tract infection due to HMPV or RSV at 10 hospitals in Japan. We enrolled 48 children with HMPV infection and 141 with RSV infection. Patients with HMPV infection were older than those with RSV infection. High-grade fever was more frequently observed in patients with HMPV infection, whereas no significant differences in respiratory symptoms were apparent. Abnormal serum lactate dehydrogenase values and consolidation shadows on chest X-ray were more frequently observed in patients with HMPV infection. During hospitalization, nasal mucus suction was more frequently required in patients with RSV infection. On the other hand, ß2-adrenergic agonists, corticosteroids, and leukotriene receptor antagonists were more frequently used in patients with HMPV infection. These findings suggest that HMPV and RSV infections show similar respiratory symptoms, but HMPV infection is more likely to lead to the development of pneumonia, at least among hospitalized young children.

[Acute type a aortic dissection operated in childhood; report of a case].

A 12-year-old boy without any previous history and risk factors of cardiovascular disease presented to the emergency room with persisting general fatigue, bilateral shoulder pain and facial pallor. He was diagnosed as acute type A aortic dissection with cardiac tamponade by ultrasonic cardiogram (UCG) and computed tomography (CT) imaging, and the emergency surgery was indicated. He underwent hemiarch replacement because his aorta diameter was quite small but grafting as a large vascular prosthesis as possible was necessary in consideration of the growth. His postoperative course was uneventful and good, and he was discharged from our hospital in day 21 postoperatively. Acute aortic dissection in childhood is very rare but life-threatening. We should consider the particularity of children and make early diagnosis and treatment appropriately.