The Mustard operation corrects the effects of congenital transposition of the great arteries by creating an intraarterial baffle to direct pulmonary venous blood to the tricuspid orifice and systemic venous blood to the mitral orifice. To identify the long-term effects of this procedure, we followed 372 patients with complete transposition of the great arteries who survived the Mustard operation for at least three months. The mean follow-up period was 4.5 years (range, 0.4 to 15.9); the mean age at operation was 2.0 years. Mean resting heart rates were consistently lower than those for age-matched normal children. Seventy-six per cent of the patients had sinus rhythm during the year of operation — a figure that decreased to 57 per cent by the end of the eighth postoperative year. Twenty-five patients died during the follow-up period, nine suddenly. Life-table analysis revealed a cumulative survival rate of 91 per cent for 11 years and 71 per cent for 15 years after the operation. No strong risk factor for sudden unexpected death was identified. This study demonstrates that extended survival among patients with transposition can be expected after the Mustard operation. However, over time there is a decreasing prevalence of normal sinus rhythm in survivors, as well as a small risk of sudden death. (N Engl J Med 1984; 310:1635–8.). THE Mustard operation1 for children with complete transposition of the great arteries has resulted in excellent short-term clinical and hemodynamic improvement; the long-term outcome, however, is unknown. Earlier reports have documented the important role of rhythm disturbances in the mortality of patients after the Mustard operation2 3 4 5 6 7 8 9 10 11; however, these studies have been limited on the one hand by the small number of patients and on the other by a relatively short period of follow-up. Therefore, to examine the long-term effects of the Mustard operation, particularly on cardiac rhythm in a larger number of patients over a longer surveillance period, eight.
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