Congenital Cardiac Anatomy and Operative Correction
There are numerous congenital defects that may present with human hearts, and many typically require surgical intervention. The primary goal of this chapter is to briefly define such abnormalities and introduce the reader to the various classification sch
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Charles Shepard, Robroy McIver, and James D. St. Louis
Abstract
There are numerous congenital defects that may present with human hearts, and many typically require surgical intervention. The primary goal of this chapter is to briefly define such abnormalities and introduce the reader to the various classification schemes that have been used to describe their relative anatomical and functional features. The chapter will also highlight the more common surgical procedures utilized to treat congenital cardiac lesions. Keywords
Septal defect • Aortopulmonary window defect • Coarctation of the aorta • Interrupted aortic arch • Tetralogy of Fallot • Atresia • Ebstein’s anomaly • Transposition of the great vessels • Total anomalous pulmonary venous connection • Persistent truncus arteriosus • Cardiopulmonary bypass
10.1
Introduction
The spectrum of congenital heart disease is enormously diverse, yet affects a relatively small portion of the human population [1]. Disease severity ranges from benign to lethal, and many lesions require intervention to allow survival or enhance life expectancy. Several nomenclature classifications have evolved to describe abnormalities of the cardiovascular system, with the Van Praagh and Anderson/Edwards systems being the most prominent. Richard Van Praagh presented a system based on the segmental anatomy of the developing heart. His generalized
C. Shepard, MD Division of Pediatric Cardiology, University of Minnesota Children’s Hospital, Minneapolis, MN, USA R. McIver, MD Division of Pediatric Cardiac Surgery, University of Minnesota Children’s Hospital, Minneapolis, MN, USA J.D. St. Louis, MD (*) University of Missouri-Kansas City, School of Medicine, Children’s Mercy Hospital and Clinics, Kansas City, MO, USA e-mail: [email protected]
theory states that, by understanding the anatomical position of the cardiac segments, the majority of cardiac defects may be accurately described. The three segments which Van Praagh described consist of the atria, the ventricle, and the great vessels. These segments may be described by delineating their relative positional relationships. The visceroatrial situs is defined as the relative position of the right and left atria to the sidedness of the abdominal (visceral) organs; the term situs means position or location. The bulb ventricular loop is described as the orientation of the right and left ventricles to the great vessels (pulmonary artery and aorta). These segments are referenced in sequence, with each being designated by a letter. The connection of the visceral venous vessels (the superior and inferior vena cavae) and the atrial body is termed the visceroatrial situs and is described by the letters: S, situs solitus; I, situs inversus; and A, situs ambiguous. When the visceral situs is normal (situs solitus), the stomach and spleen lie to the left, the right lobe of the liver is larger than the left, and the appendix is right sided. In situs inversus, the position of the abdominal organs is reversed, with the stomach and spleen lying on t
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