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The Journal of Thoracic and Cardiovascular Surgery, Vol 92, 6-13, Copyright © 1986 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
RA Jonas, P Lang, D Hansen, P Hickey and AR Castaneda
After Norwood's initial report of successful first-stage palliation of
hypoplastic left heart syndrome in neonates, an improved understanding of
both the pathologic anatomy and physiology of this common condition has led
to improving surgical results. Twenty-five neonates have been treated by
modifications of the Norwood procedure, and the early mortality between
January, 1984, and July, 1985, has been 24% (six deaths). Two premature
neonates weighing less than 2 kg died, as did two neonates who could not be
resuscitated with prostaglandin E1 and had cardiac arrests immediately
preoperatively. There have been four late deaths. The frequent occurrence
(80%) of a juxtaductal coarctation or coarctation shelf necessitates
alteration of surgical technique. Failure to do so will be reflected in an
early persistent postoperative metabolic acidosis with an arterial oxygen
tension greater than 35 to 40 mm Hg in the operating room or greater than
40 to 45 mm Hg in the intensive care unit. A similar acid-base derangement
will be seen if the systemic-to-pulmonary shunt is excessively large.
Careful ventilatory and pharmacologic management of the ratio of pulmonary
to systemic vascular resistance is an essential part of the perioperative
management of these neonates with two parallel competing circulations. Late
postoperative catheterization findings have emphasized the importance of a
wide atrial septectomy, because restrictive atrial septal defects have
developed in a number of children, despite partial surgical atrial
septectomy. Central pulmonary artery reconstruction must be carefully
performed to avoid distorted hypoplastic pulmonary arteries that would be
unsuitable for a second-stage modified Fontan procedure.
ARTICLES
First-stage palliation of hypoplastic left heart syndrome. The importance of coarctation and shunt size
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