Comparison between transcatheter versus surgical intervention for pediatric aortic valvular stenosis: a multicenter study in Japan.

It is controversial whether children with isolated aortic valvular stenosis (vAS) initially undergo transcatheter or surgical aortic valvuloplasty (BAV or SAV). This multicenter retrospective case-control study aimed to explore outcomes after BAV or SAV for pediatric vAS. We studied children (aged < 15 years) with vAS treated at 4 tertiary congenital heart centers, and compared the rates of survival, reintervention, and valve replacement between patients with BAV and SAV. A total of 73 subjects (BAV: N = 52, SAV: N = 21) were studied. Age and aortic annulus z-score at the first presentation were 85 (26-530) days and - 0.45 (- 1.51-0.59), respectively. During the follow-up period of 121 (47-185) months, rates of 10-year survival (BAV: 88% vs. SAV: 92%, P = 0.477), reintervention (BAV: 58% vs. SAV: 31%, P = 0.626), and prosthetic/autograft valve replacement (BAV: 21% vs. SAV: 19%, P = 0.563) did not differ between the groups. Freedom from reintervention rate significantly correlated with aortic annulus z-score (hazard ratio [HR] 0.66, 95% confidence interval [CI] 0.49-0.88, P = 0.005), and freedom from prosthetic/autograft valve replacement rate significantly correlated to the degree of aortic regurgitation after the first intervention (HR: 4.58, 95% CI 1.19-17.71, P = 0.027). Propensity score-matched analysis (N = 16) did not show the differences in survival and reintervention rates between the groups. Long-term survival was acceptable, and the rates of freedom from reintervention and prosthetic/autograft valve replacement were comparable between children with vAS who underwent BAV and SAV.

Corrigendum: Use of the index of pulmonary vascular disease for predicting longterm outcome of pulmonary arterial hypertension associated with congenital heart disease.

[This corrects the article DOI: 10.3389/fcvm.2023.1212882.].

Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease.

Aims: Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH. Methods: This retrospective study examined lung pathology images of 764 patients with CHD-PAH aged <20 years whose lung specimens were submitted to the Japanese Research Institute of Pulmonary Vasculature for pulmonary pathological review between 2001 and 2020. Clinical information was collected retrospectively by each attending physician. The primary endpoint was cardiovascular death. Results: The 5-year, 10-year, 15-year, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with an IPVD of ≥2.0 had significantly poorer survival than the group with an IPVD <2.0 (P = .037). The Cox proportional hazards model adjusted for the presence of congenital anomaly syndromes associated with pulmonary hypertension, and age at lung biopsy showed similar results (hazard ratio 4.46; 95% confidence interval: 1.45-13.73; P = .009). Conclusions: The IPVD scoring system is useful for predicting the long-term outcome of CHD-PAH. For patients with an IPVD of ≥2.0, treatment strategies, including choosing palliative procedures such as pulmonary artery banding to restrict pulmonary blood flow and postponement of intracardiac repair, should be more carefully considered.

Sudden out-of-hospital cardiac arrest in pediatric patients in Kyushu area in Japan.

BACKGROUND: It is well-known that a neurologically favorable outcome of out-of-hospital cardiac arrest (OHCA) is associated with the presence of bystander-initiated cardiopulmonary resuscitation (bystander CPR) and use of an automated external defibrillator. However, little is known about the effect of the presence of pre-existing conditions, prior activity, and locations on the outcome of pediatric OHCA. METHODS: We analyzed the data from questionnaires about pediatric patients with OHCA aged from 3 days to 19 years in the Kyushu area in Japan between 2012 and 2016. RESULTS: A total of 594 OHCA cases were collected. The numbers of OHCA cases and the rate of 1 month survival with a favorable neurological outcome during sleeping, swimming / bathing, and exercise were 192 (1.0%), 83 (32.5%), and 44 (65.9%), respectively. When an OHCA occurred at school (n = 56), 88% of children / adolescents received bystander CPR, but when it occurred at home (n = 390), 15% received bystander CPR. Cardiovascular (n = 61), suicide (n = 61), and neurological / neuromuscular (n = 44) diseases were three major pre-existing conditions. The OHCA of cardiovascular disease was associated with exercise (24/61) and mainly occurred at school (22/61). The OHCA of neurological / neuromuscular disease was associated with swimming/bathing (15/44) and mainly occurred during bathing at home (12/44). Multivariate regression analysis showed that the presence of bystander CPR (P < 0.001) and occurrence of OHCA at school (P < 0.001) were independently predictive of a favorable outcome in pediatric OHCA. CONCLUSION: The outcome was different among pre-existing conditions, prior activity, and location of OHCA. These findings might be useful for preventing OHCA and improving the outcome of pediatric OHCA.

Complete absence of bile and pancreatic ducts in a newborn: a new entity of congenital anomaly in hepato-pancreatic development.

We examined a newborn who had no bile and pancreatic ducts. Hydrops was evident after 29 weeks of gestation and she died shortly after birth, weighing 1,368 g. One of her siblings had died of hydrops at about six months of gestation, and there were two more miscarriages of unknown cause. At autopsy on the newborn, the liver had an abnormally round shape and the pancreas was not in the normal position. There was an ectopic small pancreas with normally developed islets. Histological analysis revealed the complete absence of extra- and intra-hepatic bile and pancreatic ducts. Immunostaining of these tissues showed no positive bile duct marker staining using epithelial membrane antigen and cytokeratin 19 in the liver. Albumin and alpha-fetoprotein staining was positive in the liver, and insulin and glucagon staining was positive in the remaining islets. Thus, this case is characterized by complete absence of bile and pancreatic ducts. These findings suggest the existence of a gene linked to the development of bile and pancreatic ducts.