title: Congenital cardiac malformations in Iceland from 1990 through 1999.
abstract: BACKGROUND
  About 1% of live-born children have congenital malformations of the heart. The aim of our study was to investigate the incidence of such defects in children born in Iceland during a period of 10 years, extending from 1990 through 1999.
  
  
  METHODS
  Information about the patients was obtained from medical records from two hospitals that cover the whole country, a private clinic of pediatric cardiologists, an echocardiography database, autopsy reports, and death certificates. We investigated the distribution of specific malformations, the age at diagnosis, the symptoms leading to the diagnosis, the source of referral, and treatment and quality of life.
  
  
  RESULTS
  Between 1990 and 1999, there were 44,013 live births in Iceland, of which 740 patients were diagnosed with congenital cardiac malformations, accounting for 1.7% of the live-born children. The distribution was made up of 338 patients with ventricular septal defect (45.7%), 90 with atrial septal defect (12.2%), 85 with patency of the arterial duct (11.5%), 48 with pulmonary valvar stenosis (6.5%), 38 with a bicuspid aortic valve (5.1%), 28 with aortic coarctation (3.8%), 22 with tetralogy of Fallot (3.0%), 14 with transposed great arteries (1.9%), 11 with aortic stenosis (1.5%), 10 with atrioventricular septal defect and common atrioventricular orifice (1.4%), 9 with mitral valvar regurgitation (1.2%), 7 with sub-aortic stenosis (0.9%), and 5 with hypoplasia of the left heart (0.7%). Extracardiac anomalies were seen in 89 patients (12.0%). Chromosomal defects were seen in 36 patients, of whom 28 had Down's syndrome.
  
  
  CONCLUSIONS
  The annual incidence of diagnosis of patients with congenital cardiac malformations increased during the period of study. This was noted for minor defects, but the incidence of the major anomalies did not alter. Our observed yearly incidence, at 1.7%, was higher than noted in a previous study covering the years 1985 through 1989, and is also higher than in other population-based studies. The most likely explanation is the fact that access to pediatric cardiologists in Iceland is very good. Diagnosis, registration, and follow-up are conducted by only a few cardiologists, and take place at a single center for pediatric cardiology.
authors.affiliation: Children's Hospital, University Hospital of Iceland, Hringbraut, Reykjavik, Iceland.
authors.forename: Sigurdur Sverrir
authors.initials: SS
authors.lastname: Stephensen
authors.forename: Gunnlaugur
authors.initials: G
authors.lastname: Sigfusson
authors.forename: Herbert
authors.initials: H
authors.lastname: Eiriksson
authors.forename: Jon Thor
authors.initials: JT
authors.lastname: Sverrisson
authors.forename: Bjarni
authors.initials: B
authors.lastname: Torfason
authors.forename: Asgeir
authors.initials: A
authors.lastname: Haraldsson
authors.forename: Hrodmar
authors.initials: H
authors.lastname: Helgason
mesh_terms: D006328:Cardiac Catheterization / Q000379:methods; D006348:Cardiac Surgical Procedures / Q000379:methods; D017023:Coronary Angiography; D015150:Echocardiography, Doppler; D004562:Electrocardiography / Q000379:methods; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000453:epidemiology* / Q000601:surgery; D006801:Humans; D007055:Iceland / Q000453:epidemiology; D015994:Incidence; D007231:Infant, Newborn; D008297:Male; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D015996:Survival Rate