T. Gilljam, TRANSPOSITION OF THE GREAT-ARTERIES IN WESTERN SWEDEN 1964-83 - INCIDENCE, SURVIVAL, COMPLICATIONS AND MODES OF DEATH, Acta paediatrica, 85(7), 1996, pp. 825-831
The records of all children with simple (n = 73) and complex (ii = 35)
transposition of the great arteries who were born in western Sweden d
uring 1964-53 were studied. The incidence was 0.24/1000 births. The pr
eoperative mortality decreased from 100%, in 1964, before palliation w
as introduced, to 16% at the end of the study period. Sixty-six underw
ent corrective procedures: 37 Mustard, 21 Senning, 3 arterial switch a
nd 5 Rastelli operations. The perioperative mortality, which was 26% i
n simple transposition and 75% in complex, did not decrease significan
tly during the study period. Of 39 hospital survivors, eight Mustard a
nd two Senning patients died late of baffle complications (I? = 2), su
dden death (n = 6), pulmonary vascular disease (n = 1) and systemic ve
ntricle dysfunction (II = 1). The cumulative 20-year survival was 32%
in simple transposition and 17% in complex. In a pioneer population wi
th transposition, long-term survival was unsatisfactory. This was caus
ed by continuing high perioperative mortality and late deaths.