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Vascular Rings, Pulmonary Arterial Sling, and Related Conditions

Introduction The aortic arch, the arterial duct, and the right and left pulmonary arteries have a close spatial relationship with the major airways and the esophagus. Due to this close proximity, abnormalities in the size, position, and/or pattern of branching pattern of these structures may cause obstruction to the trachea, bronchi, or esophagus. Many of the abnormalities of position and branching, along with the so-called pulmonary…

Congenital Coronary Anomalies

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 origin and course. Anatomy and Development Even in the normally structured heart, the coronary arteries can have multiple anomalous origins and epicardial courses. However, such lesions…

Coarctation and Interrupted Aortic Arch

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 heart, coarctation most usually described an area of narrowing of the thoracic aorta in the region of the insertion of the arterial duct, with or without…

Congenital Anomalies of the Aortic Valve and Left Ventricular Outflow Tract

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 ventricle, of course, although usually an aortic valve, can be a pulmonary valve when the ventriculoarterial connections are discordant. From the morphologic stance, the same lesions…

Pulmonary Atresia With Intact Ventricular Septum

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 univentricular atrioventricular connections such as double-inlet left ventricle (LV) or tricuspid atresia. The ventricle can be hypoplastic when the ventricular septum is intact and there is…

Pulmonary Stenosis

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 Associated Anomalies Pulmonary stenosis is the third most common congenital cardiac malformation. In a large prospective study of all live-born infants that also included data from…

Arterial Duct: Its Persistence and Its Patency

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 blood was aerated in the placenta and that blood was diverted away from the liver by a short vessel connecting the portal to the inferior caval…

Common Arterial Trunk

Introduction Common arterial trunk is an uncommon congenital cardiac lesion. The essential anatomic characteristic is the presence of a common ventriculoarterial junction that gives rise to an arterial trunk. This, in turn, gives rise to the systemic, pulmonary, and coronary circulations. In this chapter, we examine some of the details of this anatomic arrangement, the methods whereby the condition is diagnosed, and the techniques for its…

Double-Outlet Ventricle

Introduction Double-outlet ventricle is no more than an abnormal ventriculoarterial connection. Therefore the feature may occur with each atrial arrangement, any atrioventricular connection, and all possible variations of ventricular morphology. The morphologic arrangement can then be further complicated by associated defects, some common and others rare. The clinical picture is as inconstant as the anatomic permutations and associations suggest. This chapter reviews the various combinations in…

Congenitally Corrected Transposition

Introduction The essence of congenitally corrected transposition is the presence of discordant connections at both atrioventricular (AV) and ventriculoarterial (VA) junctions. This segmental combination (like transposition itself, Chapter 37 ), can be found in patients with either usual or mirror-imaged atrial arrangement but not in the presence of isomeric atrial appendages. Strictly speaking, congenitally corrected transposition exists only when the VA connections are also discordant. Discordant…

Transposition

Introduction The congenital malformation characterized by origin of the arterial trunks from morphologically inappropriate ventricles has probably been the source of as much confusion and controversy as any other single topic in pediatric cardiology. When Matthew Baillie described the first case, he had no problems with nomenclature, describing the entity in accurate fashion as a “singular malformation.” Since then, the entity has most usually been described…

Tetralogy of Fallot With Pulmonary Atresia

Introduction: Morphology This chapter deals with one of the most complex—and difficult to treat surgically—of all congenital cardiac malformations: tetralogy of Fallot with pulmonary atresia. We focus particularly on tetralogy of Fallot with pulmonary atresia (TOF/PA) and major aortopulmonary collateral arteries (MAPCAs). MAPCAs are defined as systemic arterial vessels that terminate in the lung parenchyma. These vessels form during embryologic development and persist until birth and…

Tetralogy of Fallot With Pulmonary Stenosis

Introduction Lev and Eckner made the appropriate statement that no two cases of tetralogy are exactly the same. Although that is fundamentally true, the characteristic anatomy permits its instant recognition as a phenotypic entity. It is appropriate that we continue to use the term named in honor of Arthur Louis Etienne Fallot, who described the combination of an interventricular communication, biventricular origin of the aorta, muscular…

Diseases of the Mitral Valve

The scope of this chapter is restricted to congenital lesions of the mitral valve. Anomalies of the mitral valve may also be caused by (or associated with) other congenital heart conditions or general diseases. See Table 34.1 and the chapters related to these topics for more details regarding these conditions. Table 34.1 Diseases of the Mitral Valve Lesions/Mechanisms Potential Effects Chapters CAUSES OF MITRAL VALVE LESIONS…

Diseases of the Tricuspid Valve

Introduction The most common congenital malformations afflicting the tricuspid valve are Ebstein malformation and tricuspid valvar dysplasia. These lesions are considered in this chapter, together with a brief look at acquired malformations. Tricuspid valvar abnormalities, or abnormalities of the morphologically right atrioventricular valve, are also often associated with atrioventricular septal defects with common atrioventricular junction (see Chapter 31 ), ventricular septal defect with straddling valve (see…

Ventricular Septal Defect

Introduction Holes between the ventricles can occur as isolated anomalies but are also seen in association with many other defects. For example, they are found as integral parts of entities such as the tetralogy of Fallot, double-outlet ventricles, and most cases of common arterial trunk. They are also frequently encountered in association with transposition and congenitally corrected transposition. Although fundamentally different from ventricular septal defects, atrioventricular…

Atrioventricular Septal Defects

There is a group of lesions unified by the anatomic hallmark of a common atrioventricular junction coexisting with deficient atrioventricular septation. The key to their differentiation from other potentially related defects is the presence of the common junction, including the arrangement of the fibrous skeleton of the heart, which is fundamentally different from the morphology found in the normal heart. In clinical terms, it is probably…

Division of Atrial Chambers (“Cor Triatriatum”)

Introduction Division, or partitioning, of one of the atrial chambers is a rare malformation. Severe symptoms, or even death, may result if this condition is untreated. When recognized and treated surgically, life expectancy should be normal. Either the morphologically right or the morphologically left atrium can be divided by fibromuscular partitions. Clinically significant division of the morphologically left atrium is by far the most common type,…

Interatrial Communications

Introduction Channels permitting shunting between the atrial chambers are important congenital cardiac anomalies because they are relatively common and are more nearly completely correctable than most other cardiac malformations, even more so in the era of catheter intervention. It may be difficult, nonetheless, to diagnose the presence of such communications, and often there is no suspicion of cardiac disease during infancy and early childhood. Moreover, the…

Pulmonary Venous Abnormalities

Introduction This chapter considers abnormalities of the pulmonary veins, in particular their anomalous connections to the systemic venous system. The variant of partially anomalous pulmonary venous connection associated with the sinus venosus interatrial communication is described in detail. The anomalous pulmonary venous connections so commonly associated with isomerism of the atrial appendages are mentioned in this chapter but are discussed more fully in Chapter 26 ,…