Late complications and qualify of life after atrial correction of transposition of the great arteries in 12 to 18 year follow-up

Background Most adolescents and young adults born with transposition of the great arteries and survivors of Mustard operation are alive today and are leading ordinary lives. How far is the quality of their life influenced by residual findings? Methods. We have analysed the oldest group of 177 children operated on with the Mustard procedure during 1979-1984 from our total experience of 609 co rrections of transposition of the great arteries until 1996. In the long-te rm follow-up 12 to 18 years after Mustard operation the clinical investigat ion, 24 hours ECG Holter monitoring, 2D and colour flow Doppler echocardiog raphy, radionuclide angiocardiography and exercise testing were performed. Results. Basic sinus rhythm had 61% of 137 survivors, sinus node dysfunctio n had 51%. All but 4 patients needed no antiarrhythmic therapy. There were 10 sudden deaths. Severe tricuspid valve regurgitation developed in 15.9% o f children, and decreased to 6% after intensive medical treatment. The righ t ventricular systolic dysfunction was found on radionuclide ventriculograp hy in 8% of children after surgery. The left ventricular ejection fraction was subnormal in 10% and left ventricular diastolic function expressed by r apid filling fraction was subnormal in 80% of patients and may cause inabil ity to increase cardiac output at exercise The obstruction of caval veins w as found in. 9%. There were neurological complications in 13.3% (10% alread y preoperatively) but severe brain damage was found in 4.4% only. Conclusions. Exercise tolerance with the maximum oxygen consumption over 30 ml/kg/min in 96% of investigated children suggests the good ability and fu ll efficiency for recreation sports. Most survivors (84%) are reported to l ead ordinary lives and function in NYHA class I.