Introduction For the anatomist and pathologist, the terms “univentricular” and “single” have proven to be among the most controversial words used to describe a congenitally malformed heart. Since the mid-1990s, light has begun to emerge at the end of the…
Introduction The various abnormal ventriculoarterial connections, such as transposition in its regular or congenitally corrected variants, double-outlet ventricles, and common arterial trunk, are all congenital malformations involving the ventricular outflow tract. They are often described in terms of “conotruncal malformations,”…
Introduction An abnormally positioned heart is not in itself a malformation of major significance. Such abnormal positioning, for example, can be secondary to a deformity or defect of the chest wall, such as pectus excavatum or carinatum. The exceptions are…
Introduction The so-called Rathke diagram, showing six pairs of arteries encircling the tracheoesophageal pedicle, is still frequently used to provide an understanding of the morphogenesis of abnormalities of the brachiocephalic arteries in human patients with congenitally malformed hearts ( Fig.…
This chapter discusses the range of congenital coronary anomalies that may be found in an otherwise structurally normal heart, first reviewing the anatomy and development of the coronary arteries before focusing on the most clinically significant anomalies of coronary artery…
Introduction Coarctation derives from the Latin term coartatio , which translated literally means “a drawing together.” Aortic coarctation, therefore, indicates a narrowing at some point along the course of the aorta. When used in the context of the congenitally malformed…
Introduction It is well recognized that obstruction within outflow tract of the morphologically left ventricle may be above the arterial valve, at the level of the valve itself, or in the subvalvar region. The arterial valve of the morphologically left…
The right ventricle (RV) can be hypoplastic in various settings. It can be small in the presence of deficient ventricular or atrioventricular septation, producing so-called left ventricular dominance. The chamber can also be small and incomplete in the setting of…
This chapter discusses pulmonary stenosis as an isolated finding. When pulmonary stenosis exists as part of a more complex anomaly, such as tetralogy of Fallot, the reader is directed to the complete descriptions found in the relevant chapters. Incidence and…
Historical Considerations When Siegal reexamined the original second century Greek text of Galen, he pointed out that Galen was familiar with many aspects of the fetal circulation even though he did not realize that blood circulated. Galen understood that fetal…