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The Journal of Thoracic and Cardiovascular Surgery, Vol 96, 864-877, Copyright © 1988 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
JE Sell, RA Jonas, JE Mayer, EH Blackstone, JW Kirklin and AR Castaneda
Seventy-one patients with interrupted arch entered the Boston Children's
Hospital between Jan. 1, 1974, and Jan. 1, 1987, of whom 63 underwent an
operation. Type B was the most prevalent form of interrupted arch, and
ventricular septal defect alone was the commonest coexisting cardiac
anomaly. Among the 63, the 30-day and the 1-, 5-, and 10-year survival
rates were 61%, 52%, 48%, and 47%, respectively. The mortality rate
declined strikingly during the experience, and by multivariate analysis in
patients with coexisting ventricular septal defect operated on in 1986, the
probability of death within 2 weeks of repair was only 7%. Also,
preoperative therapy became progressively more intense and more prolonged.
The complication of left ventricular outflow tract obstruction developed in
eight of the 33 patients undergoing repair of interrupted arch and of
isolated ventricular septal defect. The time-related freedom from this
complication was 97%, 78%, and 58% at 1 month, 1 year, and 3 years,
respectively. Seven of the eight patients underwent a surgical procedure
directed against the left ventricular outflow tract obstruction, and all
have survived. Recurrent or persistent aortic arch obstruction became
evident after repair in 15 patients and appeared more frequently and
earlier after direct anastomosis than after tube graft repair. All patients
had either reoperation or balloon dilation, but all were alive at follow-
up. Most surviving patients are active and without symptoms. Inferences: An
aggressive surgical program can result in survival and a good clinical
state for at least 10 years after birth of over 40% of patients born with
interrupted arch. Multiple anatomic bases account for the development of
left ventricular outflow tract obstruction in about 50% of the patients
undergoing repair of interrupted arch with coexisting ventricular septal
defect. Repair by direct anastomosis combined with repair of the coexisting
defect whenever possible is optimal therapy.
ARTICLES
The results of a surgical program for interrupted aortic arch
Boston Children's Hospital, MA 02115.
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