Abstract

Consensus is lacking regarding the optimal operation for transposition, ventricular septal defect, and pulmonary stenosis. Between 1968 and 2012, a total of 76 patients underwent the Rastelli procedure, with 52 mid- or long-term survivors. A bracketing analysis was used to estimate the likelihood of late left ventricular outflow tract obstruction (LVOTO). Early mortality decreased over the period of study, with no hospital mortality since 2000. Among one year survivors, 10- and 20-year survival was 90% and 72%, respectively. Freedom from reoperation for LVOTO was 87% at 20 years, with no increase in risk among patients having the procedure before 5 years of age. Available late echocardiographic or catheterization data indicated mild or no LVOTO at a median of 14.3 years in a subset of 38 patients. Estimated freedom from major LVOTO at 20 years is bracketed between the estimate of 87% freedom from reoperation for LVOTO at 20 years and the 78% freedom from reoperation for LVOTO or cardiac death by 20 years. The Rastelli operation can be performed in the current era with an early mortality that approaches 0% and with 20-year survival that exceeds 70%. The late risk of important LVOTO appears to range from about 13% to 22% at 20 years, with no increase in risk among patients operated upon before the age of 5 years.

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