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Introduction

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The results of the surgical treatment of congenital cardiac malformations have been transformed within the last half-century. Prior to the development of cardiopulmonary bypass, it was impossible to even contemplate the repair of major malformations, even though ingenious surgeons had successfully closed simple intracardiac lesions such as holes between the atrial chambers. Even when it proved possible for the surgeon to work within the heart in the setting of a bloodless operative field, the results of operative repair were far from perfect, and many patients died subsequent to the operative procedure. Nowadays, it is unusual for any patient with a relatively simple defect to die, and even those with “complex” lesions are now expected to survive. There have been many reasons for these truly remarkable advances, but without question one has been the knowledge of detailed intracardiac anatomy accrued within recent decades. In the past, it was often thought that, in the setting of so-called complex malformations, this anatomy was difficult to understand. It is now recognized that, although the combination of given lesions can truly be complex, the anatomy itself is relatively straightforward provided that it is approached in straightforward fashion and using a system of analysis that is simple and logical. To achieve success, therefore, the pediatric cardiac surgeon must hone not only the surgical skills but also the intellectual rigor needed to analyze the structure of the congenitally malformed heart. It would be foolish to suggest that problems do not still exist in obtaining the necessary morphologic understanding, but these problems can be minimized by using a simple philosophy combined with use of words in their vernacular sense. Too many of the problems of the past have reflected linguistic rather than scientific disagreements. It remains appropriate to remember the aphorism attributed to Rudolf Virchow, namely that “those who fail to learn from the mistakes of the past are condemned to repeat them.” In this chapter, therefore, we describe the basics of cardiac anatomy that permit the surgeon to diagnose and recognize the arrangement of the cardiac chambers during surgical procedures and at the same time appreciate the likely position of the vital conduction tissues. The concomitant advances made over recent years in diagnostic techniques are now such that the basic layout of the heart will have almost certainly been established prior to commencement of intracardiac procedures. Nonetheless, even in the most complex cases, preliminary diagnosis demands little more, in terms of morphology, than the distinction of a right atrium from a left atrium, a right ventricle from a left ventricle, and an aorta from a pulmonary trunk. It is such distinctions that provide the basis for simple sequential segmental analysis.1 For the surgeon, the anatomy of holes and blockages within the heart and great vessels are of equal or even greater significance. This morphology is addressed elsewhere in the appropriate chapters of this book; therefore, we confine ourselves to showing how the anatomy of some important defects differs ...

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