[Tetralogy of fallot in iceland from 1968 to 2001.]

We reviewed our experience regarding tetralogy of Fallot (TOF) in Iceland over a 34 year period from 1968 to 2001. We looked at incidence, diagnosis,treatment and outcome and any changes in these parameters during the study period.

Data were obtained from hospital records containing echocardiographic, cardiac catheterization, surgical and autopsy reports.

Forty six children were diagnosed as having TOF during the study period. The incidence was 1:3209 births and male to female ratio 1.2:1. The followup period was from two months to 33 years (median 10.5 years). Thirty seven patients had classic TOF, six had TOF with pulmonal atresia and three had other anatomical variants. Six children had chromosomal abnormalities and another five had physical anomalies outside of the heart. Nine children (24.3%) with classic TOF had a systemic to pulmonary artery shunt placed. Thirty eight (82.6%) of the patients have had corrective cardiac surgery and five in addition are scheduled to undergo such procedure in the near future. Three patients died before corrective surgery. All corecctive operations were carried out abroad. Half of the patients had difficulties following surgery and two (5.3%) died in the immediate post operative period. Of the 46 children born with TOF during the study period, seven (15.2%) have died and three are lost to followup. Corrective heart surgery had been done in four of the seven patients who died. Of the 36 patients alive in whom current information is available, 32 (88.9%) are in good physical condition leading full active lives. Corrective surgery which is planned for the other four is expected to improve their condition. Of 30 patients with classic TOF, two are being treated for arrhythmia and four have had interventional cardiac catheterizations three to 24 years following corrective surgery. In 10 of 30 pateints with classic TOF the most recent echocardiogram showed significant abnormalities. Most commonly this consisted of moderate to severe enlargement of the right ventricle with significant pulmonary valve insufficiency.

TOF is a complex congenital heart defect with high incidence of coexistant chromosomal and physical abnormalities. Progress in recent years regarding surgical treatment and care of these patients in general has dramatically improved outcome.