Late failure of systemic ventricle after atrial correction for transposition of great arteries

The incidence of late systemic ventricular failure (SVF) was determined in 220 early survivors of atrial correction for transposition of great arteries (TGA), operated upon between 1964 and 1985. SVF was defined as either severe heart failure (NYHA class III or IV) or death due to the same cause, in the absence of other haemodynamic disturbances. The actuarial survival rate was 96% after 1, 94% after 5 and 87% after 10 years. SVF occurred in 16/220 patients (7.2%). SVF was more common in complex TGA (TGA + VSD or PS or both) with 11/99 patients (11%) than in simple TGA with 5/121 patients (4.1%), P less than 0.01. Actuarial freedom from SVF was 97% at 5, and 92.5% at 10 years. SVF caused 10/25 (40%) late deaths. SVF occurred from the 1st postoperative year up to 20 years after correction (average interval 6.3 years). In 1 of 2 patients a systemic atrioventricular (AV) valve annuloplasty did not improve SVF. Heart transplantation was performed in 2 patients and 1 is on the waiting list. SVF is a rare late complication of atrial correction for TGA with a constant incidence in the late course. SVF is more common in complex TGA.