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Ann Thorac Surg 2002;73:339-340
© 2002 The Society of Thoracic Surgeons


Review

Invited commentary

Marshall L. Jacobs, MDa

a St. Christopher’s Hospital for Children, Philadelphia, PA 19134, USA


    Introduction
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 Introduction
 
Two decades ago, a number of innovative surgeons attempted a variety of palliative operations with the shared goal of stabilizing the fragile circulation of neonates with hypoplastic left heart syndrome. In that early surgical experience, postoperative survival was a rare event. The approach advocated in the early 1980s by Norwood was unique not only with respect to the achievement of intermediate survival, but because the initial palliative surgery was planned and executed in the context of the very specific and ambitious goal of eventually accomplishing a modification of Fontan’s operation. Thus, its essential ingredients included establishment of a permanent communication between the right ventricle and the aorta while preserving the potential for aortic growth, limitation of pulmonary blood flow to attenuate pulmonary vascular changes caused by elevated pulmonary blood flow and pressure, and creation of a satisfactory interatrial communication to ensure unobstructed pulmonary venous return to the systemic right ventricle. Reports of success using this strategy were met with an unusual degree of cynicism and skepticism, and individuals in the disciplines of pediatric cardiology and congenital heart surgery continued to promulgate scholarly reports advancing arguments why some, or many, or all neonates with hypoplastic left heart syndrome could not or should not be subjected to such a treatment strategy. These arguments continued, though less voiciferously, when other surgeons besides Norwood reported similarly encouraging results with this strategy, and as importantly acknowledged and described the "learning curve" phenomenon which yielded steadily improving outcomes. Those arguments are less often heard in the current era, and emphasis has shifted to ways of refining treatment strategies to further reduce morbidity or mortality. The common basis for all such innovations is an understanding of the fundamental goals of balancing systemic and pulmonary blood flow, and optimizing systemic oxygen delivery.

Mastery of the challenge of surgical palliation of hypoplastic left heart syndrome involves much more than the experience required to appreciate and avoid the pitfalls inherent in a technically demanding but physiologically sound and reproducible surgical exercise. As importantly, it requires a multi-disciplinary team approach that is based upon an understanding of the dynamic and fragile physiology of the neonate with a single ventricle with duct-dependent systemic circulation preoperatively, and shunt dependent pulmonary circulation postoperatively. The preceding review by Pearl and colleagues clearly summarizes the lessons learned over the past two decades and highlights as well some of the more contemporary and innovative strategies designed to minimize the morbidity associated with operative and perioperative care of neonates with hypoplastic left heart syndrome. As such, it is of considerable value not only to congenital heart surgeons, but to our colleagues in pediatric cardiology and critical care as well.





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