The Mustard procedure in infants (less than 100 days of age). Ten-year follow-up

Surgical treatment of transposition of the great arteries and intact ventricular septum has changed markedly in the past 10 years. However, long-term follow-up on new approaches is not available. In a unique group of patients, operated on in the first 100 days of life with the Mustard procedure, such follow-up is available, and the results of these true infant repairs represent a benchmark against which subsequent techniques applied to infants should be compared. During the period of 1975 to 1980, 36 infants, less than 100 days of age, who had transposition of the great arteries and intact ventricular septum, underwent Mustard repair at the University of California, San Francisco. Ages ranged from 4 to 98 days (mean 46 days) and weights from 2.3 to 6.6 kg (mean 3.5 kg). There were no early deaths, and late follow-up was available from 8 to 13 years (mean 10 years). The late survival rate was 97% (mean 10 years). There was a 62% rhythm disturbance-free survival rate, 89% reoperation-free survival rate, and 91% pacemaker-free survival rate. Echocardiographic evaluation revealed obstruction of the superior vena cava in eight patients, tricuspid insufficiency in four, right ventricular dysfunction in two, and left ventricular outflow tract obstruction in three. The Mustard procedure, performed in the first 100 days of life, results in a high rate of survival (early 100% and late 97%, at a mean of 10 years) and a low incidence of late complications against which other techniques of infant repair should be compared.