Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort

Objective: Patients with repaired tetralogy of Fallot (TOF) experience increased rates of mortality and morbidity in adulthood. This study was designed to identify risk factors for death and ventricular tachycardia (VT) in a large contemporary cohort of patients with repaired TOF. Methods: Subjects with repaired TOF from four large congenital heart centres in the USA, Canada and Europe were enrolled. Clinical, ECG, exercise, cardiac magnetic resonance (CMR) and outcome data were analysed. Results: Of the 873 patients (median age 24.4 years), 32 (3.7%) reached the primary outcome (28 deaths, 4 sustained VT; median age at outcome 38 years; median time from CMR to outcome 1.9 years). Cox proportional-hazards regression identified RV mass-to-volume ratio ≥0.3 g/mL (HR, 5.04; 95% CI 2.3 to 11.0; p<0.001), LV EF z score<−2.0 (HR, 3.34; 95% CI 1.59 to 7.01; p=0.001), and history of atrial tachyarrhythmia (HR, 3.65; 95% CI 1.75 to 7.62; p=0.001) as outcome predictors. RV dysfunction was predictive of the outcome similar to LV dysfunction. In subgroup analysis of 315 subjects with echocardiographic assessment of RV systolic pressure, higher pressure (HR 1.39; 95% CI 1.19 to 1.62; p<0.001) was associated with death and sustained VT independent of RV hypertrophy and LV dysfunction. Conclusions: RV hypertrophy, ventricular dysfunction and atrial tachyarrhythmias are predictive of death and sustained VT in adults with repaired TOF. These findings may inform risk stratification and the design of future therapeutic trials

〔資 料〕 保護者が認知する子どもの個性と習い事との関連

The variety of extracurricular lessons available to children compared to what was available to those children’s parents has increased. Parents need more information to help them select the lessons for their children that will be most effective in stimulating the development of their children. The present study aims to obtain fundamental data to be used in the creation of an information tool that will help parents select the best lessons for their children. Specifically, an online questionnaire was distributed to parents whose children have taken lessons in order to understand the association between psychological factors in their children and the types of lessons that their children have been taking.A total of 1,217 responses were obtained. Of them, 772 respondents （662 parents of elementary school children and 110 parents of preschool children） who had been taking a lesson for at least 13 months were included in the analyses. Swimming was the most common lesson taken by elementary school children, followed by piano. Regarding psychological factors, the level of multiple intelligences, particularly interpersonal intelligence, was rated higher by parents whose children were taking piano as compared with those whose children were participating in swimming

Status of adult outpatients with congenital heart disease in Japan: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease Registry

BackgroundThe Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease (JNCVD-ACHD) was founded in 2011 for the lifelong care of adult patients with congenital heart disease (ACHD patients). This network maintains the first Japanese ACHD registry.Methods and resultsFrom 2011 to 2019, the JNCVD-ACHD registered 54 institutions providing specialized care for ACHD patients in 32 of the 47 prefectures in Japan. The registry collected data on the disease profile for 24,048 patients from 50 institutions and the patient characteristics for 9743 patients from 24 institutions. The most common ACHDs were atrial septal defect (20.5 %), ventricular septal defect (20.5 %), tetralogy of Fallot (12.9 %), and univentricular heart (UVH)/single ventricle (SV; 6.6 %). ACHD patients without biventricular repair accounted for 37.0 % of the population. Also examined were the serious anatomical and/or pathophysiological disorders such as pulmonary arterial hypertension (3.0 %) including Eisenmenger syndrome (1.2 %), systemic right ventricle under biventricular circulation (sRV-2VC; 2.8 %), and Fontan physiology (6.0 %). The sRV-2VC cases comprised congenitally corrected transposition of the great arteries without anatomical repair (61.9 %) and transposition of the great arteries with atrial switching surgery (38.1 %). The primary etiology (86.4 %) for Fontan physiology was UVH/SV. In addition, developmental/chromosomal/genetic disorders were heterotaxy syndromes (asplenia, 0.9 %; polysplenia, 0.7 %), trisomy 21 (4.0 %), 22q11.2 deletion (0.9 %), Turner syndrome (0.2 %), and Marfan syndrome (1.1 %).ConclusionsAlthough the specific management of ACHD has systematically progressed in Japan, this approach is still evolving. For ideal ACHD care, the prospective goals for the JNCVD-ACHD are to create local networks and provide a resource for multicenter clinical trials to support evidence-based practice

Serum Hyaluronic Acid Concentration in Fontan Circulation: Correlation With Hepatic Function and Portal Vein Hemodynamics

Abstract Although liver fibrosis causes significant morbidity in the late postoperative period of the Fontan procedure, the diagnostic value of hyaluronic acid (HA), a serum marker of liver fibrosis, has not been established in Fontan patients. The purpose of this study was to determine whether increased serum HA concentration in Fontan patients is associated with an increase in inspiratory-to-expiratory flow rate ratio (Qin/ Qex) of the portal vein (PV), which is indicative of liver fibrosis. We retrospectively studied 28 consecutive patients with Fontan circulation who underwent cardiac catheterisation for various indications. The median age at examination was 5.5 years (range 2.2-5.6). The median HA concentration was 17.7 ng mL -1 (range 10.0-82.1), which was used to divide our 28 patients into two groups. Patients in the high-HA group had significantly greater Qin/Qex of the PV than those in the low-HA group (1.25 ± 0.12 vs. 1.12 ± 0.11, p \ 0.05). Platelet counts were significantly lower in the high-HA group (216 ± 74 vs. 294 ± 104 9 10 9 L -

Minimally invasive, pericardial implantable cardioverter-defibrillator placement in a young child

We report the successful minimally invasive placement of a pericardial implantable cardioverter-defibrillator (ICD) in a 16-kg child. A transvenous ICD dual coil was advanced through a small subxiphoid incision and screwed into the oblique sinus pericardium under fluoroscopic guidance. An additional sense-pace lead was sutured onto the right ventricular apex, and the generator was placed in the upper abdominal wall through the same incision. Threshold testing demonstrated successful defibrillation at 15 J. After implantation, the patient had two episodes of appropriate shock for ventricular fibrillation. The ICD system continues to show stable impedance at 6 months of follow-up

Tracheid Length of Sugi(Cryptomeria japonica) as an lndex of Wood Quality for Forest Tree Breeding

Eleven sugi cultivars in 20-year-old stands were sampled to quantify the tracheid lengths for juvenile wood and mature wood. Tracheid lengths of latewood were estimated from macerated tissue in every ring at the breast height of three to five trees in each cultivar. Tracheid lengths of every cultivars were initially short near the pith and increased outwards within about 15 rings from the pith, as shown in Fig.1. The result was that the tracheid length of the 16th ring was regarded as the representative value of tracheid length in mature wood. There were high correlations between tracheid lengths of juvenile wood and the tracheid length of the 16th ring from the pith, and the decision coefficients for correlations were shown in Fig.2 and Fig.3. However Figs.4 and 5 show that the plots of rings near the pith for cv. obiaka and cv. hinode deviate from the regression lines, and the tracheid lengths of these rings may be unsuitable for predicting the tracheid length in mature wood. In the 8th ring from the pith, there was no cultivar deviated markedly from the regression line of the tracheid length of the 16th ring on tracheid length of a given ring in juvenile wood as shown in Fig.9.スギ11品種の胸高部位円板を用いて, 未成熟材仮道管長から成熟材仮道管長を予測できるか否かを検討した. この検討に先立ち, 未成熟材から成熟材への移行時期を調べたところ, いずれの品種でも, 髄から15年輪目以降を成熟材とみなせた. そこで, 成熟材仮道管長として髄から16年輪目の仮道管長を採用した. 未成熟材の仮道管長と成熟材の仮道管長との聞に, 極めて高い相関関係が存在することが明らかになった. しかし, 髄近くの年輪では, 両者の関係を表す回帰直線から大きくはずれる品種が観察され, 年輪数の増加にともなう仮道管の伸長率に, 品種間で違いがみられた. このことは, 髄付近の年輪から成熟材の仮道管長を予測するとき, 重要な留意点と位置づけられねばならないであろう. なお, 髄からの年輪数が増加し, 8年輪目以降の年輪では, 回帰直線から大きくずれる品種は認められず, 成熟材の仮道管長を精度良く予測できるとみなすことができた