[Transposition of the great arteries in Iceland over a 26 year period from 1971 to 1996.].
OBJECTIVE
We reviewed our experience regarding D- and L-transposition of the great arteries (D- and L-TGA) in Iceland over a 26 year period, from 1971 to 1996. We looked at incidence, diagnosis, treatment and outcome and any changes in these parameters during the study period were noted.


METHODS
Data were obtained from hospital records which contained echocardiographic, cardiac catheterization- and autopsy reports.


RESULTS
There were 31 children diagnosed as having transposition of the great arteries during the study period, 29 had D-TGA. Follow-up period was from 11 months to 21 years (median 13 years). The incidence was 1:3681 births and male to female ratio 2.4:1. Cardiac catheterization was used as a diagnostic tool in 11 cases, but as of 1984 all diagnoses were made by echocardiography. Of the patients with D-TGA, 21 (72%) had no additional cardiac defects, however when these were present, a ventricular septal defect was most common. Twenty-six patients (84%) underwent a balloon atrial septostomy and it was successful in 24 (92%). Twenty-three of 31 patients (74%) have had cardiac surgery, all fully corrective. Of the 23 surgeries, 21 were done in London, England. Fifteen children had atrial switch repair, a Mustard operation was done twice and Senning in 13 patients. In five cases an arterial switch operation was performed and three patients had other surgeries. Half of the patients had a difficult post operative course, however there was only one death within the first month following surgery. Of the 31 children born with TGA from 1971 to 1996, eight (26%) have died and two are lost to follow-up. All the patients that died were born during the first half of the study period. Of these eight children, four died within the first four days of life. Corrective cardiac surgery had been done on two patients before death. There are 21 patients alive which updated information is available on. All are in good condition leading full active lives. Five patients are needing prescription medications, thereof two antiarrhythmics. Of the most recent echocardiograms, 16 are without hemo-dynamically significant sequela. In five cases however, echocardiography shows significant abnormalities, probably warranting intervention within the next few years.


CONCLUSIONS
The diagnosis, treatment and outcome of patients with transposition of the great arteries has improved dramatically over the years. Close follow-up of these patients with regards to sequela of cardiac surgery is mandatory, especially since the majority of this patient population has had atrial switch repair.
Eiriksson
H
H
Helgason
H
H