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Chapter Summary

This chapter describes the anatomy, physiology, medical and surgical therapies, and outcomes of some of the congenital heart diseases that result in cyanosis at presentation. Lesions discussed in detail include transposition of the great arteries (d-TGA) as well as congenitally corrected d-TGA, anomalies of pulmonary venous return, and truncus arteriosus (see Fuster and Hurst’s Central Illustration). Each of these lesions, while resulting from vastly different aberrations of embryonic cardiac development, culminates in mixed pulmonary and systemic venous return to the heart. Depending on associated cardiac lesions, these forms of cardiac disease may be incompatible with life in the neonatal period or, at best, severely life-limiting in their unrepaired form. Advances in the field of cardiothoracic surgery have allowed the immediacy of these threats to survival to be assuaged. However, residual lesions are nearly universal and determine the future course and outcomes over an individual’s lifespan. The population of adults born with cyanotic congenital heart disease is increasing, and knowledge of the sequelae of residual lesions is necessary in order to anticipate and mitigate complications.

eFig 70-01 Chapter 70: Complex Cyanotic Congenital Heart Disease: The “Mixing” Lesions



Transposition of the great arteries (d-TGA) is a congenital cardiac malformation characterized by atrioventricular (AV) concordance and ventriculoarterial (VA) discordance. The malformation was first described by Matthew Baillie in 1788.1 Baillie reported the findings of an infant’s autopsy with d-TGA in his book The Morbid Anatomy of some of the Most Important Parts of the Human Body. However, it was John Richard Farre, in 1814, who used for the first time the term transposition of the great arteries in his book Pathological Researches on Malformations of the Human Heart.2 Farre mentioned not only the term transposition, but also listed symptoms of several cases, including the first one that Baillie had mentioned.

In absence of a ventricular septal defect (VSD), d-TGA is not compatible with life and in the 1950s, the neonatal mortality of this condition was nearly 90%. In 1948, Alfred Blalock and his resident Rollins Hanlon developed the Blalock–Hanlon procedure, an atrial septectomy to improve cardiac mixing. In 1966, the balloon atrial septostomy was described and the Blalock–Hanlon septectomy became redundant. Before the first successful repair of d-TGA in 1957, other procedures such as the Baffes procedure (translocation of the inferior vena cava to the left atrium and the right pulmonary veins to the right atrium), were successfully used to palliate patients with d-TGA.3 In 1957, Ake Senning performed the first successful atrial switch procedure using flaps from the right atrial tissue and the interatrial septum.4 Some years later, in 1964, William Mustard described the Mustard procedure,5 an alternative technique in which the atrial septum ...

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