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

Background: The 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 Results: From 2011 to 2019, the JNCVD-ACHD registered 54 institutions providing specialized care for ACHD patients in 32 of the 47 prefectures in Japan.

Electrocardiographic Diagnosis of Hypertrophic Cardiomyopathy in the Pre- and Post-Diagnostic Phases in Children and Adolescents.

Background: The usefulness of electrocardiographic (ECG) voltage criteria for diagnosing hypertrophic cardiomyopathy (HCM) in pediatric patients is poorly defined.

Methods and results: ECGs at the 1st grade (mean [±SD] age 6.6±0.

Direct oral anticoagulant use and outcomes in adult patients with Fontan circulation: A multicenter retrospective cohort study.

Background: Many adult patients with Fontan circulation are treated with antithrombotic agents, including direct oral anticoagulants (DOACs). However, few studies have investigated the efficacy, feasibility, and safety of DOACs in adult patients with Fontan circulation.

Methods And Results: In this retrospective cohort study, clinical records of 139 adult patients with Fontan circulation (70 females, 50.

Sivelestat sodium hydrate treatment for refractory Kawasaki disease.

Background: There is still no definite treatment for refractory Kawasaki disease (KD). In this pilot study, we evaluated the safety and efficacy of a new protocol consisting of sivelestat sodium hydrate (SSH) combined with additional i.v.

Increased risk of thromboembolic events in adult congenital heart disease patients with atrial tachyarrhythmias.

Background: Atrial tachyarrhythmias are a major morbidity in patients with adult congenital heart disease (ACHD). However, few studies have investigated risk stratification of thromboembolic events in ACHD patients with atrial tachyarrhythmias.

Methods And Results: This retrospective cohort study reviewed the clinical records of 2314 ACHD patients from 1977 to 2014.

Coronary ostium occlusion by coronary cusp displacement in Williams syndrome.

Williams syndrome is a contiguous gene deletion syndrome resulting from a heterozygous deletion on chromosome 7q11.23, and is characterized by distinctive facial features and supravalvular aortic stenosis (SVAS). This syndrome rarely presents unpredictable cardiac death, and yet, as illustrated in the present case, it is still not possible to predict it, even on close monitoring.