Coronary anomalies in single ventricles: Insights from selective angiographic assessment.

Background: Adults with congenital heart disease have a higher risk of coronary artery disease compared to the general population. However, there is limited information on coronary artery distribution in patients with a single ventricle (SV), which is important in understanding potential cardiovascular events. This study aimed to evaluate coronary artery morphology and anomalies in patients with SV based on selective coronary angiography (CAG).

Identification of Rare Variants in Patients With Williams Syndrome and Severe Peripheral Pulmonary Stenosis.

Background: Peripheral pulmonary stenosis (PPS) is a condition characterized by the narrowing of the pulmonary arteries, which impairs blood flow to the lung. The mechanisms underlying PPS pathogenesis remain unclear. Thus, the aim of this study was to investigate the genetic background of patients with severe PPS to elucidate the pathogenesis of this condition.

Utility of urinary liver-type fatty acid-binding protein as a prognostic marker in adult congenital heart patients hospitalized for acute heart failure.

Progression to acute kidney injury (AKI) under treatment in adult congenital heart disease (ACHD) patients with heart failure is associated with poor prognosis, early detection and interventions are necessary. We aimed to explore the utility of urinary liver-type fatty acid binding protein (L-FABP) in ACHD patients hospitalized for acute decompensated heart failure (ADHF). We prospectively evaluated hemodynamic, biochemical data, and urinary biomarkers including urinary L-FABP in ACHD patients hospitalized in our institution from June 2019 to March 2022.

Platelet volume indices correlate to severity of heart failure and have prognostic value for both cardiac and thrombotic events in patients with congenital heart disease.

Patients with heart failure (HF) reportedly have activated platelets with increased platelet distribution width (PDW) and mean platelet volume (MPV), which lead to thrombotic events. These platelet indices are easily measured by routine blood tests and have been proposed as potential markers of cardiac events. We performed the present study to clarify whether platelet indices correlate to the severity of HF and to the prognosis of patients with congenital heart disease (CHD).

Management of pregnancy and delivery in women with transposition of the great arteries after atrial switch operation: A 16-year single-center experience.

Aim: This study aimed to summarize cases of successful pregnancy and delivery in patients with transposition of the great arteries (TGA) after atrial switch operation (ASO), to provide management, clinical experience, and maternal and fetal outcomes.

Methods: During a 16-year period (2004-2019), we experienced 30 pregnancies in 15 patients after ASO at our institution. We retrospectively reviewed the medical records of the patients.

Right arch with prostaglandin-dependent coarctation and aberrant left carotid artery in a 22q11.2 deletion infant.

Right aortic arch in association with coarctation of the aorta and vascular ring is a rare anatomy. We present an infant with chromosome 22q11.2 deletion, who had the right aortic arch with retroesophageal aberrant left subclavian artery and left internal carotid artery.

Predictors of neo-aortic valve regurgitation in the long term after arterial switch operation.

Although arterial switch operations (ASOs) have been performed globally to repair d-transposition of the great arteries (d-TGA) in neonates and infants, few studies have been reported regarding the influence of the hemodynamics of patients with d-TGA who have undergone ASO on the development of neo-aortic valve regurgitation (AR). We aimed to investigate the relationship between the hemodynamics and development of AR after ASO in patients with d-TGA by catheter evaluation. This observational study screened 114 consecutive patients who underwent ASO for d-TGA or Taussig-Bing anomaly and who subsequently underwent catheter evaluations in our institution.

Skeletal muscle index determined by bioelectrical impedance analysis is a determinant of exercise capacity and a prognostic predictor in patients with congenital heart disease.

Background: Patients with congenital heart disease (CHD) reportedly have reduced exercise capacity. Underlying cardiac anatomy and a sedentary lifestyle are thought to be associated with exercise impairment. However, little has been reported regarding the relationship between quantitative body composition and exercise capacity.

Clinical Significance of Central Venous Pressure During Exercise After Fontan Procedure.

Various late complications are associated with the Fontan procedure. In patients who undergo the Fontan procedure, the central venous pressure (CVP) tends to be higher than normal. However, the relationship between CVP (at rest and during exercise) and late complications associated with the Fontan procedure is unknown.

Prevalence and risk factors of sleep apnea in adult patients with congenital heart disease.

Background: Although sleep apnea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with congenital heart disease are limited.

Methods: In this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia.

Prevalence and risk factors of sleep apnoea in adult patients with CHD.

Background: Although sleep apnoea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with CHD are limited.

Methods: In this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia.