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Congenital cardiac malformations in Iceland from 1990 through 1999
Sigurdur Sverrir Stephensen1, Gunnlaugur Sigfusson, Herbert Eiriksson
1Children's Hospital, University Hospital of Iceland, Hringbraut, Reykjavik, Iceland.
Insights
The incidence of congenital heart defects in Iceland was 1.7% between 1990-1999. Improved access to pediatric cardiologists likely explains the increased diagnosis rates for these cardiac malformations.
Area of Science:
- Cardiology
- Pediatrics
- Public Health
Background:
- Congenital heart malformations affect approximately 1% of live births.
- Understanding the incidence and types of these defects is crucial for public health initiatives.
Purpose of the Study:
- To determine the incidence of congenital cardiac malformations in children born in Iceland from 1990 to 1999.
- To analyze the distribution, diagnosis, and characteristics of these defects within the Icelandic population.
Main Methods:
- Retrospective analysis of medical records from national hospitals, a private pediatric cardiology clinic, echocardiography databases, autopsy reports, and death certificates.
- Inclusion of data from 44,013 live births between 1990 and 1999.
- Investigation of specific malformation types, age at diagnosis, referral sources, and treatment outcomes.
Main Results:
- A total of 740 cases of congenital cardiac malformations were identified, representing an incidence of 1.7%.
- Ventricular septal defect was the most common (45.7%), followed by atrial septal defect (12.2%) and patent ductus arteriosus (11.5%).
- Extracardiac anomalies were present in 12.0% of cases, with chromosomal defects, including Down's syndrome, noted in a subset.
Conclusions:
- The annual incidence of diagnosed congenital cardiac malformations increased during the study period, particularly for minor defects.
- The observed incidence of 1.7% is higher than previous studies, potentially due to excellent access to specialized pediatric cardiology care in Iceland.
- Diagnosis, registration, and follow-up are centralized, suggesting improved detection and reporting contribute to the higher incidence.
Introduction And 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.
Materials And 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.
Discussion:
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.
