Mouse embryos lacking the retinoic acid (RA) receptors RARalpha1 and RARbeta suffer from a failure to properly septate (divide) the early outflow tract of the heart into distinct aortic and pulmonary channels, a phenotype termed persistent truncus arteriosus. This phenotype is associated with a failure in the development of the cardiac neural crest cell lineage, which normally forms the aortico...

A ORTOPUIiMONARY FENESTRATION or partial persistent truncus arteriosus is undoubtedly not as rare a congtenital lesion as earlier statistics have suggested. This defeet is also called aortic septal defect or aortopulmonary winidow. In 1949 Perelman and Putscharl were ab)le to assemble only 14 cases from the literature. Skall-Jensen2 reported 64 additioiial patients collected between 1949 and 19...

Previous work showed that in the adult sturgeon an intrapericardial, nonmyocardial segment is interposed between the conus arteriosus of the heart and the ventral aorta. The present report illustrates the ontogeny of this intermediate segment in Acipenser naccarii. The sample studied consisted of 178 alevins between 1 and 24 days posthatching. They were examined using light and electron microsc...

Objective: One and one half ventricle repair is a surgical option for congenital cardiac anomalies characterised by right ventricle (RV) hypoplasia and/or dysplasia. Methods: From March 1994 to March 2001, eight patients (mean age 9.1 years, range 7 months to 35 years) with hypoplastic and/or dysplastic RV underwent correction of their intracardiac anomaly in association with a BCPS (one and on...

OBJECTIVE This study aims to analyse long-term results after correction of truncus arteriosus in all patients operated in one institution over 28 years. METHODS Between 1981 and 2009, 83 patients, median age 54 days, underwent repair of truncus arteriosus. Interrupted aortic arch was present in 14 (17%), severe truncal valve insufficiency in 10 (12%) and non-confluent pulmonary arteries in fi...

Vascular ring caused by double aortic arch most commonly occurs as an isolated anomaly (Keith, Rowe, and Vlad, I967). However, its association with pulmonary stenosis (Bahnson and Blalock, I950), tetralogy of Fallot (Griswold and Young, I949; Stewart, Kincaid, and Edwards, I964), truncus arteriosus (Kerwin, I936), ventricular septal defect and left superior vena cava (Lincoln et al., I969), and...