BACKGROUND Infants with functional single ventricle have a high risk of death during the early years of life. Studies have reported improvement in postoperative survival, but they do not include preoperative deaths or those occurring before transfer. The purpose of this population-based study was to estimate 5-year survival in infants with functional single ventricle, to define factors associat...

Critical pulmonary stenosis or atresia with intact ventricular septum represents a congenital cardiac lesion for which the long-term prognosis appears to depend partly on the size of the right ventricle. Thus, the capability of noninvasive assessment of right ventricular size to predict operative outcome was examined in 15 infants (aged 1 to 30 days, mean 5.6) with severe right ventricular outf...

A maternally inherited 828 kb microdeletion of 2q37.3 manifested in a 3-week-old premature boy as left juxtaposition the atrial appendages associated with tricuspid atresia, double outlet infundibulum, subvalvar pulmonary large secundum septal defect, and right aortic arch mirror-image branching, consistent developmental arrest early heart looping. To best our knowledge, no previous 2q37 deleti...

In muscular pulmonary atresia, major aortopulmonary collateral arteries are characteristic of pulmonary atresia with ventricular septal defect and are rarely seen in pulmonary atresia with intact ventricular septum. Two unusual cases of muscular pulmonary atresia are reported, one with an intact septum and one with a perimembranous ventricular septal defect, closed in utero by aneurysmal tricus...

MOST PATIENTS with cyanotic congenital heart disease show right axis deviation and right ventricular hypertrophy of more or lesser degree in the electrocardiogram. Left axis deviation by contrast has long been recognized as the cornerstone of the diagnosis of tricuspid atresia.1 Although occasionally the A QRS is between 0° and +90° or may deviate slightly to the right in this condition, anatom...

We present the first reported case of truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage into a retro-aortic innominate vein in a neonate in whom successful palliation was performed using a two-staged procedure. An early repair after presentation along with a re-routing of anomalous pulmonary venous drainage at the time of the second stage should be undertaken...

A 31-year old woman with tricuspid atresia, after Bjork Fontan modification and reoperation due to homograft narrowing, was evaluated because of progressive exercise intolerance; she was planning a pregnancy. The right atrium (RA) to right ventricle (RV) homograft narrowing and calcification with significant reversal flow were found. Successful reoperation was performed-calcified conduit was ex...

A 17 month old girl with tricuspid atresia, ventricular septal defect, subvalvar and valvar pulmonary stenosis, and increasing cyanosis had angiographic evidence of proximal stenosis of a right modified (5 mm diameter) Blalock-Taussig shunt. She underwent palliative balloon angioplasty with a 6 mm Schneider balloon catheter. Successful dilatation was achieved without complication. Four months l...