migration of cardiac neural crest cells into the pharyngeal arches and the pharyngeal and splanchnic mesoderm contributes to the development of the cardiac outflow tract. the adrenal cortex is derived from the splanchnic mesoderm. neuroblastoma is more prevalent in patients with congenital heart disease than in the general population, because both originate from embryonal neural crest-derived c...

Primary cardiac arrhythmias are often caused by defects, predominantly in the genes responsible for generation of cardiac electrical potential, i.e., cardiac rhythm generation. Due to the variability in underlying genetic defects, type, and location of the mutations and putative modifiers, clinical phenotypes could be moderate to severe, even absent in many individuals. Clinical presentation an...

T HE PROBABILITY that postnatal spontaneous closure of ventricular septal defect occurs with some frequency has been evident to clinicians for a number of years. This postulate is based upon the presence of characteristic murmurs in childhood which disappear with the growth and development of the patient.1-7 Convincing evidence of spontaneous functional closure of ventricular septal defects is ...

introduction: orofacial clefts are among the most common congenital anomalies. patients presenting with orofacial clefts often require surgery or other complex procedures. a cleft lip or palate can be a single anomaly or a part of multiple congenital anomalies. the reported prevalence of cleft disease and associated anomalies varies widely across the literature, and is dependent on the diagnost...

Many patients with congenital heart disease and systemic-to-pulmonary shunts develop pulmonary arterial hypertension (PAH), particularly if the cardiac defect is left unrepaired. A persistent increase in pulmonary blood flow may lead to obstructive arteriopathy and increased pulmonary vascular resistance, a condition that can lead to reversal of shunt and cyanosis (Eisenmenger syndrome). Cardia...

IN A PREVIOUS study' cardiac output, intracardiac and intravascular pressures, levels of gas in blood, and acid-base profiles were observed for several days following repair of atrial septal defect and similar lesions. This is a report of similar observations following closure of ventricular septal defect. As in the previous study, the greatest deviations from normal in cardiac function were ob...

We investigated regulation of cardiac adenylate cyclase in 29-d-old BIO 14.6 Syrian hamsters, which inherit cardiomyopathy as an autosomal recessive trait. Pharmacologic stimulation of adenylate cyclase in cardiac membranes with isoproterenol, fluoride ion, guanine nucleotide, forskolin, and manganous ion indicated that there was defective coupling of the guanine nucleotide-binding protein that...

Congenital absence of pulmonary valve leaflets is an uncommon condition usually associated with ventricular septal defect and an obstructive pulmonary valve ring. Twenty-one patients with these malformations are described. Twenty had an associated ventricular septal defect with ventriculoarterial concordance, and one also had transposition of the great arteries, ventricular septal defect, and o...

This report describes the ability of computed tomography angiography (CTA) imaging of the heart to visualize an acquired shunt between the left ventricular outflow tract (LVOT) and the right atrium (RA) (Gerbode defect). Previously, transesophageal echocardiography (TEE) has been the mainstay of diagnosis. To the best of our knowledge, the use of cardiac CTA imaging to visualize and diagnose th...