Nýgengi og greining meðfæddra hjartagalla á Íslandi

Abstract

The purpose of our study was to investigate the incidence of congenital heart defects in Iceland, the distribution of specific defects, the age at diagnosis and causes of death. The study deals with children born in Iceland from 1985-1989. We divided the patients in two groups; patients with minor heart defects and patients with major defects, the second group being patients who needed treatment for their cardiac disease or are likely to need treatment. There were 215 children found to have congenital heart defects, 1.1% of cildren born. Of these 99 had major cardiac defects. Of the 116 patients with minor cardiac defects 94 had small ventricular septal defect (VSD). Of the 99 children with major cardiac defects 25 had VSD, 16 had atrial septal defect (ASD), 15 had patent ductus arteriosus, 7 had transposition of the great arteries, 6 had tetralogy of Fallot, 8 had aortic stenosis. Other defects were less frequent. Two patients born in 1985-1986 had ASD but 14 born in 1987-1989. Twenty three of the patients had other extracardiac congenital anomalies, of those 12 with chromosomal abnormalities 9 of whom had Down syndrome. About 47% of the patients with major cardiac anomalies were diagnosed before discharge from the delivery institution. Of the other 17% were referred from routine well baby care, 18% were referred from doctors' offices, and 18% were diagnosed after hospital admission for what was felt to be a noncardiac problem. Seventeen patients with major cardiac defects died. The cause of death was the cardiac condition in most of the patients. The 80 survivors are all in good health. We conclude that the incidence of congenital heart defects in Iceland is similar although somewhat higher in Iceland compared to other population studies. ASD is more common in the latter part of our observation period suggesting that there are probably some undiagnosed patients in the group of children born 1985-1986. All the children with minor cardiac defects are in good health and all the survivors of major cardiac defect are in good health.

Tilgangur rannsóknarinnar var að athuga nýgengi meðfæddra hjartagalla á Íslandi, dreifingu einstakra galla, greiningu og afdrif barna með hjartagalla. Rannsóknin nær til barna sem fædd eru á Íslandi á árunum 1985-1989. Alls greindust 215 börn með hjartagalla eða 1,1% fæddra. Þar af voru 99 börn með alvarlegan hjartagalla eða 0,47% fæddra. Af þeim sem voru með vægan hjartagalla (116 börn) voru 94 með lítið op á milli slegla (VSD). Af börnum með alvarlegan hjartagalla voru 25 með VSD, 16 með op á milli gátta, 15 með opna fósturæð, átta með þrengsli í ósæð, sjö með slagæðavíxl, sex með Fallots tetralogíu, en aðrir gallar komu sjaldnar fyrir. Tuttugu og þrjú börn sem höfðu alvarlegan hjartagalla höfðu einnig aðra fæðingargalla, þar af voru 12 börn með ýmsa litningagalla (Downs-heilkenni, níu börn). Helmingur barnanna með alvarlegan hjartagalla greindust fyrir brottför af fæðingarstofnun (47%). Hin börnin greindust eftir komu í ungbarnaeftirlit (17%), eftir komu á stofu hjá lækni (18%) og við innlögn á sjúkrahús (18%). Sautján börn, af 99 með alvarlegan hjartagalla, létust en 80 eru við góða heilsu. Við ályktum að nýgengi meðfæddra hjartagalla sé svipað hérlendis og víða erlendis. Þó finnast hér heldur fleiri börn með op á milli gátta og opna fósturæð. Nýgengi opa á milli gátta hefur farið vaxandi á tímabilinu. Börnum með alvarlega hjartagalla farnast að jafnaði mjög vel eftir aðgerð.