A newborn boy with congenital contractural arachnodactyly (CCA) was found to have an atrial septal defect, ventricular septal defect, patent ductus arteriosus, and aortic arch anomalies. These resulted in congestive failure but subsequent progressive dilatation of both great arteries and development of aortic regurgitation were associated with eventual cardiorespiratory failure and death at 11....

BACKGROUND Persistent left superior vena cava (PLSVC) is a variant of systemic venous return which is observed in 0.3% of autopsies in the general population and in 4-8% of patients with congenital heart disease. AIMS To evaluate associated cardiac, extracardiac and chromosomal anomalies in prenatally diagnosed cases of PLSVC and to review their outcome. STUDY DESIGN Retrospective comparati...

Background. In the management of hearts with deranged laterality, it is essential that the left and right atrial chambers are correctly identified. There are two major approaches, which are based on venous connections or on the morphology of the atrial appendages, and there is no consensus as to which is the most useful. We used the ivliv mouse mutant, which is known to be pertinent to this pro...

OBJECTIVES To determine whether offering cardiac screening to relatives of patients with left ventricular outflow tract obstructions (LVOTOs) would be justified. BACKGROUND LVOTOs have been recognised as a group of congenital heart diseases with 'high heritability'. One of the LVOTOs, the bicuspid aortic valve, is often asymptomatic, but has become known to be associated with sudden, unexpect...

Congenital coronary sinus anomalies are extremely rare, and they have received relatively little attention. This is probably due to the lack of both clinical symptoms and significant cardiac functional disturbance. We present two cases of a coronary sinus anomaly and briefly review the literature. Recognizing and being familiar with the variations of a congenital coronary sinus anomaly in conge...

Congenital anomalies of coronary arteries are relatively frequently found in general population (their incidence reaches 0.2-0.9%) [1,2]. Most of them are benign and do not require interventional or surgical treatment, but in some cases them can result in sudden death or myocardial infarction [3]. Coronary CT-angiography (CTA) or MR-angiography are effective imaging modalities for non-invasive ...

The clinical manifestations of coronary artery anomalies vary in severity, with some anomalies causing severe symptoms and cardiovascular sequelae and others being benign. Cardiovascular computed tomography (CT) has emerged as the standard of reference for identification and characterization of coronary artery anomalies. Therefore, it is important for the reader of cardiovascular CT images to b...

OBJECTIVE To compare the distribution of congenital anomalies within the VACTERL association (vertebral defects, anal atresia, cardiac, tracheoesophageal, renal, and limb abnormalities) between patients exposed to tumor necrosis factor-α (TNF-α) antagonist and the general population. METHODS Analysis for comparison of proportional differences to a previous publication between anomaly subgroup...

there are often duplicated ureter with types of congenital anomalies and associated clinical complications. the unilateral duplicated ureter was observed as an incidence result in usual dissection. during routine dissection of the body of an adult male with middle-aged 40-50 year old, the duality of unilateral incompletely ureter was seen in above. two branches of ureter are coalesce in 7 cm di...

anomalous origin of the left coronary artery from the pulmonary artery (alcapa) is a rare congenital cardiac malformation. we report three cases of alcapa who survived to adulthood. the first case was a 51-year-old woman who complained of typical chest pain that was diagnosed with alcapa using cardiac catheterization and coronary computed tomographic angiography (cta). the second case was a 30-...