The vast majority of the patients seemed to live normal lives 20-37 years after Tetralogy of Fallot repair. Late deaths were cardiac in origin, including sudden death from arrhythmias. The number of late reoperation has been low. Considering the natural history of the disease, Fallot repair has proven to be a beneficial procedure even including the very early experience short after introduction of open heart surgery.

A clinical follow-up study was performed in 11 unoperated patients with symptoms due to vascular ring. The age at diagnosis was 2-48 months (mean 13 months); 7 of the patients were less than 12 months old. The follow-up time varied between 1 and 22 years (median 7 years). Nine patients were entirely free of symptoms, which they had outgrown in the course of 1-2 years after the diagnosis was made; none of these 9 had symptoms after the age of 4 years. Two patients had symptoms due to either oesophageal compression or to severe associated neuromuscular lesions; the follow-up time was 3 and 1 years, respectively. Our findings lead us to support a conservative attitude regarding surgery, at least if only mild symptoms are present, If the patients can be satisfactorily treated medically, they seem to outgrow their symptoms in early childhood.

No abstract

As development of right ventricular (RV) failure is a potential risk after Mustard operation for transposition of the great arteries, 17 patients were reexamined 5-13 years postoperatively. Comparisons were made with healthy controls. There were no clinical signs of heart failure. Echocardiographically determined RV end-diastolic diameter was increased to 2.5 +/- 0.8 cm (controls: 1.5 +/- 0.4 cm, p less than 0.001). Comparison of RV systolic time intervals (STI) in patients with normal left ventricular (LV) STI revealed decreased RV function, with RPEPI 165 +/- 19 msec (controls 126 +/- 12, p less than 0.001) and RPEP/RVET 0.484 +/- 0.096 (controls 0.284 +/- 0.045, p less than 0.001). Nuclear angiography demonstrated decreased RV ejection fraction (EF), viz. 42.8 +/- 6.6% (normal RV 53 +/- 6%, LV 68 +/- 9%, p less than 0.001). Only two patients showed normal (5%) rise in RV-EF during exercise. There was no evidence of deterioration with passage of time. The results do not justify use of anatomic repair at our center, since the perioperative mortality might then be higher than in the Mustard or Senning procedures.

No abstract