Recently, heterozygous mutations of human NKX2.5 were identified in patients with congenital heart disease. 2 The most common phenotypes were progressive atrioventricular conduction delays (AV block) and secundum atrial septal defect (ASD), but other anatomical abnormalities, such as ventricular septal defect, tetralogy of Fallot, or tricuspid valve abnormalities, including Ebstein’s anomaly, a...

YSTEMIC venous anomalies, such as persistent left superior vena cava and absent inferior vena cava, are not so rare in various congenital heart diseases.1) Preoperative identification of these anomalous systemic venous connections is clinically important, because their presence necessitates modified technique for venous cannulation of cardiopulmonary bypass. In cases of persistent left superior...

BACKGROUND Adults with an atrial septal defect (ASD) frequently develop late atrial arrhythmias. We sought to characterize the pattern and persistence of atrial electrical remodeling caused by chronic right atrial (RA) stretch in this group. METHODS AND RESULTS Thirteen ASD patients without atrial arrhythmia (42+/-10 years old; RA volume, 65+/-16 mL) and 17 normal control subjects (44+/-11 ye...

Cor triatriatum dexter is a malformation resulting from lack of normal regression of the embryonic right valve of the sinus venosus. In this situation, the right atrium is divided by a membrane into two chambers. Two-dimensional echocardiography was used in the antemortem diagnosis of this rare cardiac anomaly in a neonate. Associated cardiac lesions were also documented. The patient died, and ...

BACKGROUND There are currently no standardized three-dimensional (3D) transesophageal echocardiographic (TEE) views of the interatrial septum and atrial septal defects (ASDs). Without a standardized approach, it is difficult to ascertain the important anatomic relationships (such as the location of the aortic rim of an ASD), to perform relevant measurements (such as the size of an ASD or the si...