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J Thorac Cardiovasc Surg 2003;125:S82-S84
© 2003 The American Association for Thoracic Surgery
Editorials |
From the Department of Surgery, St Christopher's Hospital for Children, Philadelphia, Pa.
Received for publication July 24, 2000. Accepted for publication July 28, 2000. Address for reprints: Marshall L. Jacobs, MD, Department of Surgery, St Christopher's Hospital for Children, Erie Ave at Front St, Philadelphia, PA 19134.
| The first 300 words of the full text of this article appear below. |
Hypoplastic left heart syndrome (HLHS) is the most frequently encountered of the various congenital heart malformations characterized by a single well-developed ventricle. The introduction more than three decades ago by Fontan and Kreutzer of operative strategies to separate the systemic and pulmonary circulations in patients with tricuspid atresia led to the recognition that all single-ventricle anomalies could be managed in a similar fashion, at least in theory. The unique challenge of aortic atresia-HLHS was that the presence of systemic outflow obstruction and thus ductal dependency of the systemic circulation resulted in rapid and predictable hemodynamic deterioration of neonates with this condition and demanded an operative strategy considerably more ambitious than merely regulating pulmonary blood flow by means of either a pulmonary artery band or a systemic-pulmonary shunt. The availability in the 1970s of prostaglandins to ensure patency of the ductus arteriosus was of major importance in the overall management of neonates with HLHS.
During the same era, numerous investigators and surgical teams experimented with a host of innovative and imaginative strategies to stabilize the circulation of neonates with aortic atresia and related malformations. In addition to the magnitude of the overall challenge, two things became apparent: (1) separation of the systemic and pulmonary circulations by atriopulmonary or cavopulmonary connection could not be achieved successfully in young infants because of the prohibitively high pulmonary vascular resistance and (2) palliative operative strategies that did not preserve the architecture and integrity of the pulmonary arteries and their distal vascular bed only postponed by months the inevitable death that previously had typically occurred during the first few weeks of life.
From these early lessons, Norwood distilled the essential features of successful neonatal palliation of aortic atresia and other forms of HLHS: (1) association of the aorta directly with the right ventricle in a fashion
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