Double-outlet right ventricle. Surgical results, 1970-1980.

Between Jan. 1, 1970, and Jan. 1, 1980, 62 consecutive patients underwent repair of classic double-outlet right ventricle (DORV). Patients with subpulmonic ventricular septal defect (VSD), complete atrioventricular canal, atrioventricular discordance, and univentricular heart were excluded. One hundred six associated defects were present in 54 patients. Forty-six patients had pulmonary stenosis. The 36 male and 26 female patients ranged in age from 8 months to 37 years (median age 9 years). The early mortality was 11% for those with pulmonary stenosis, 25% for those without pulmonary stenosis, and 15% for the entire series. The risk of mortality was related to the age of the patient at operation. Causes of early deaths included low cardiac output (three patients), high residual right ventricular pressure (three patients), anomalous coronary artery injury (one patient), infection (one patient), and hemorrhage (one patient). Eleven late deaths occurred among the 53 operative survivors (21%). Ten (91%) of the late deaths were attributed to arrhythmia. All except one of the long-term survivors are in Functional Class I or II. Although the operative mortality for the repair of DORV continues to decrease, the late mortality is of concern, and the problem of late arrhythmia necessitates further study and analysis.