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...

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital anomaly characterized by complete or partial aplasia of the uterus and upper part vagina. It reported to be associated with cardiovascular disorders including atrial septal defect, anomalous pulmonary venous return, aortopulmonary window, valve stenosis, mitral prolapse, tetralogy Fallot, truncus arteriosus, patent ductus arter...

Echocardiography was performed in 62 patients--20 with truncus arteriosus, 14 with tetralogy of Fallot, and 28 with pulmonary atresia with ventricular septal defect. Features common in all three groups were: large single systemic arterial trunk overriding the ventricular septum, mitral-semilunar continuity, large right ventricular dimension, and normal septal motion. Indocyanine green contrast ...

BACKGROUND Great arteries in congenital heart disease (CHD) may dilate, become aneurysmal, or rupture. Little is known about medial abnormalities in these arterial walls. Accordingly, we studied 18 types of CHD in patients from neonates to older adults. METHODS AND RESULTS Intraoperative biopsies from ascending aorta, paracoarctation aorta, truncus arteriosus, and pulmonary trunk in 86 patien...

INTRODUCTION Single ventricle and truncus arteriosus are both rare congenital cardiac syndromes with limited survival. Their occurrence together is extremely uncommon and prolonged survival is exceptionally rare. We present the case of a patient who had both of these defects with survival to age 45. CASE PRESENTATION We describe the vase of a 45-year-old man with the unusual occurrence of two...

Originating as a single vessel emerging from the embryonic heart, the truncus arteriosus must septate and remodel into the aorta and pulmonary artery to support postnatal life. Defective remodeling or septation leads to abnormalities collectively known as conotruncal defects, which are associated with significant mortality and morbidity. Multiple populations of cells must interact to coordinate...