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The Annals of Thoracic Surgery, Vol 54, 1025-1029, Copyright © 1992 by The Society of Thoracic Surgeons
WI Norwood Jr, ML Jacobs and JD Murphy
Since 1985, 354 neonates have undergone palliative reconstruction for
hypoplastic left heart syndrome with 109 early deaths and 12 late deaths.
Of the survivors, before 1989, 77 patients underwent a subsequent modified
Fontan operation, consisting of baffling the atrial septal defect to the
tricuspid valve (initial 25 patients) or intraatrial baffling of the
inferior vena cava to the pulmonary arteries and superior vena cava (52
patients). There were 17 early deaths and three late deaths. Major serous
effusions developed in 42 patients (54%) after Fontan operation. Since
1989, a staged approach to Fontan's operation was undertaken in an effort
to reduce the volume load of the right ventricle as early as possible, to
minimize the impact of rapid changes in ventricular geometry and diastolic
function that can accompany a primary Fontan operation, and to reduce
effusive complications. Thus, at a mean age of 6 months, 121 patients have
undergone closure of aortopulmonary shunt, augmentation of central
pulmonary arteries, and association of the superior vena cava with the
branch pulmonary arteries (hemi-Fontan procedure). Of these, 61 patients
have already undergone completion of the Fontan procedure with six early
deaths and three late deaths. Major serous effusions developed in 28
patients (46%) with the staged Fontan. For perspective, the contemporary
experience since January 1991 consists of 58 neonates who have undergone
initial palliation with 11 deaths (19%), 17 patients who have undergone the
hemi-Fontan procedure with one death (6%), and 21 patients who have
undergone completion of the Fontan operation with one death (5%).(ABSTRACT
TRUNCATED AT 250 WORDS)
ARTICLES
Fontan procedure for hypoplastic left heart syndrome
Children's Hospital of Philadelphia, Pennsylvania 19104.
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