Objective: Our purpose was to describe the outcome of the Rastelli repair i
n D-transposition of the great arteries and to determine the risk factors a
ssociated with unfavorable events.
Methods: From March 1973 to April 1998, 101 patients with D-transposition o
f the great arteries and ventricular septal defect underwent a Rastelli typ
e of repair. Median age and weight were 3.1 years (10th to 90th percentiles
0.3-9.9 years) and 12.8 kg (5.9-28.2), Pulmonary stenosis was present in 7
3 patients and pulmonary atresia in 18; 10 patients had no left ventricular
outflow tract obstruction,
Results: There were 7 early deaths: (7%) and no operative deaths in the las
t 7 years of the study. Risk factors for early death, by univariable analys
is, included straddling tricuspid valve (P =.04) and longer aortic crosscla
mping times (P =.04). At a median follow-up of 8.5 years, there: were 17 la
te deaths and 1 patient had undergone heart transplantation. Forty-four pat
ients had reoperations for conduit stenosis, 11 for left ventricular outflo
w tract obstruction, and 28 had interventional catheterization to relieve c
onduit stenosis. Nine patients had late arrhythmias, and there were 5 sudde
n deaths. Overall freedom from death or transplantation (Kaplan-Meier) was
82%, 80%, 68%, and 52% at 5, 10, 15, and 20 years, respectively. Freedom fr
om death or reintervention (catheterization or surgical treatment) was 53%,
24%, and 21% at 5, 10, and 15 years of follow-up, respectively.
Conclusions: The Rastelli repair can be performed with low early mortality.
However, substantial late morbidity and mortality are associated with cond
uit obstruction, left ventricular outflow tract obstruction, and arrhythmia
.