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Twenty-six patients with atrioventricular discordance and a variety of ventriculo-arterial connections have had appropriate intracardiac repairs. Seven of 26 patients (26.9%) died early postoperatively, but only two (11.1%) of 18 patients operated upon since May 1972 have died. Ten patients (40%) developed permanent complete heart block. Two patients died late postoperatively; 16 (84.1% of those surviving the early postoperative period) were in NYHA Class I or II at late follow-up. Seventeen of the patients had "corrected transposition." Six developed tricuspid incompetence with regurgitation into the left atrium after repair. Two patients with atrioventricular discordance and double outlet right ventricle survived operation and are asymptomatic but have permanent pacemakers. One patient died after repair of double outlet left ventricle with atrioventricular discordance. Three of four patients with atrioventricular discordance and "pseudotruncus arteriosus" survived repair with valved external conduits and are well. One of two patients is well after repair of "isolated ventricular inversion" and ventricular septal defect.

Intracardiac repair of cardiac malformations with atrioventricular discordance.