The aim of the study was to report the incidence and causes of preoperative
deaths in isolated transposition of the great vessels and to describe the
clinical findings in these neonates.
Patients and methods. - In five French centers of pediatric cardiology, dat
a of all the neonates with isolated transposition of the great vessels who
died before arterial switch operations between January 1986 and June 1996 w
ere obtained from reviewing hospital files, echocardiography records and au
topsy reports.
Results. - Among 199 neonates with transposition of the great vessels, 20 (
9.9%) died before surgery. The death was related to intracranial haemorrhag
e in one premature neonate, severe and early hypoxemia in 13 full-term pati
ents (group A) and later sudden collapse in six patients (group B). In grou
p A, the symptoms occurred within 20 minutes after the birth and included c
yanosis (n = 12), acute respiratory distress (n = 8), and shock (n = 4). De
spite assisted ventilation (n = 13), bicarbonate infusion (n = 12), prostag
landin E-1 (n = 7), inotropic drugs (n = 5) and balloon atrioseptostomy (n
= 7), death occurred at the median age of five hours. The patent foramen ov
ale was absent or tiny in ten patients, normal in one patient and not speci
fied in two patients. The ductus arteriosus was patent in ten patients and
not specified in three patients, In group B, the neonates were initially in
a good hemodynamic condition. Unexplained death occurred between two and f
ive days after the birth: one infant with a large patent foramen ovale did
not receive prostaglandin E,, four patients died a few hours after an angio
graphic study or a ballon atrioseptostomy was performed in a catheterizatio
n laboratory and one child suffered from a cerebral anoxia due to a tighten
ed cord.
Conclusion. - We conclude that the high preoperative mortality rate in isol
ated transposition of the great vessels is mainly due to absent or small at
rial shunt. These findings suggest that only prenatal diagnosis of transpos
ition of the great vessels with immediate balloon atrioseptostomy could avo
id a fatal outcome. (C) 2000 Editions scientifiques et medicales Elsevier S
AS.