Congenital cardiac malformations in Iceland from 1990 through 1999
Abstract
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.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
The CC rates of cardiac anomalies are slightly higher than the rate reported among all births.
“The incidence of cardiac anomalies in the letrozole group was slightly lower than the rate of congenital cardiac anomalies reported among all births (0.4 -1.2%) (13)(14)(15)(16), and the CC rates are slightly higher.”
From Congenital malformations among 911 newborns conceived after infertility treatment with letrozole or clomiphene citrate (2006) · cites this paper as [13]
The incidence of cardiac anomalies in the letrozole group was slightly lower than the rate of congenital cardiac anomalies reported among all births (0.4-1.2%).
“The incidence of cardiac anomalies in the letrozole group was slightly lower than the rate of congenital cardiac anomalies reported among all births (0.4 -1.2%) (13)(14)(15)(16), and the CC rates are slightly higher.”
From Congenital malformations among 911 newborns conceived after infertility treatment with letrozole or clomiphene citrate (2006) · cites this paper as [13]
Cites
Incidence of congenital heart disease: I. Postnatal incidence
Hoffman, J. I. E. (1995) · Pediatric Cardiology · doi:10.1007/bf00801907 · Cited by 786 · see citing works
Šamánek, M., & Voříšková, M. (1999) · Pediatric Cardiology · doi:10.1007/s002469900502 · Cited by 401 · see citing works
Šamánek, M., Slavík, Z., Zbořilová, B., Hroboňová, V., Voříšková, M., & Skovranek, J. (1989) · Pediatric Cardiology · doi:10.1007/bf02083294 · Cited by 303 · see citing works
Rapidity of progression of aortic stenosis in patients with congenital bicuspid aortic valves
Beppu, S., Suzuki, S., Matsuda, H., Ohmori, F., Nagata, S., & Miyatake, K. (1993) · The American Journal of Cardiology · doi:10.1016/0002-9149(93)90799-i · Cited by 234 · see citing works
Moller, J. H., Taubert, K. A., Allen, H. D., et al. (1994) · Circulation · doi:10.1161/01.cir.89.2.923 · Cited by 168 · see citing works
Syndromes and malformations associated with congenital heart disease in a population-based study
Grech, V., & Gatt, M. (1999) · International Journal of Cardiology · doi:10.1016/s0167-5273(98)00354-4 · Cited by 92 · see citing works
Increased Prevalence of Ventricular Septal Defect: Epidemic or Improved Diagnosis
Martin, G. R., Perry, L. W., & Ferencz, C. (1989) · PEDIATRICS · doi:10.1542/peds.83.2.200 · Cited by 84 · see citing works
Epidemiology of major congenital heart defects in Sweden, 1981-1986.
Pradat, P. F. (1992) · Journal of Epidemiology & Community Health · doi:10.1136/jech.46.3.211 · Cited by 81 · see citing works
Ethnicity and socioeconomic status: Impact on the diagnosis of congenital heart disease
Fixler, D. E., Pastor, P., Sigman, E. S., & Eifler, C. W. (1993) · Journal of the American College of Cardiology · doi:10.1016/0735-1097(93)90393-f · Cited by 70 · see citing works
Ethnocultural Variations in the Prevalence and Impact of Childhood Chronic Conditions
Newacheck, P. W., Stoddard, J. J., & McManus, M. (1993) · PEDIATRICS · doi:10.1542/peds.91.5.1031 · Cited by 68 · see citing works
Early clinical screening of neonates for congenital heart defects: the cases we miss
Meberg, A., Otterstad, J. E., Frøland, G., Hals, J., & Sörland, S. (1999) · Cardiology in the Young · doi:10.1017/s1047951100008398 · Cited by 63 · see citing works
Incidence of congenital heart disease in Qatari children
Robida, A., Folger, G. M., & Hajar, H. A. (1997) · International Journal of Cardiology · doi:10.1016/s0167-5273(97)00067-3 · Cited by 62 · see citing works
Bosi, G., Scorrano, M., Tosato, G., et al. (1999) · Cardiology in the Young · doi:10.1017/s1047951100004960 · Cited by 34 · see citing works
What Is the Prevalence of Congenital Heart Diseases?
Abdulla, R. (1997) · Pediatric Cardiology · doi:10.1007/s002469900172 · Cited by 20 · see citing works
Poverty and Cardiac Disease in Children
Allen, H. D. (1991) · Archives of Pediatrics and Adolescent Medicine · doi:10.1001/archpedi.1991.02160050076021 · Cited by 18 · see citing works
Incidence of cardiovascular malformation in the northeast Tennessee and southwest Virginia area
Anand, R., & Mehta, A. V. (1996) · The American Journal of Cardiology · doi:10.1016/s0002-9149(96)90536-6 · Cited by 2 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
Stout, K. K., Daniels, C. J., Aboulhosn, J. A., Bozkurt, B., Broberg, C. S., Colman, J. M., Crumb, S. R., Dearani, J. A., Fuller, S., Gurvitz, M., et al. (2019) · Circulation · doi:10.1161/cir.0000000000000603 · Cited by 1,602 · see citing works
Tulandi, T., Martin, J., Al-Fadhli, R., Kabli, N., Forman, R., Hitkari, J., Librach, C., Greenblatt, E., & Casper, R. F. (2006) · Fertility and Sterility · doi:10.1016/j.fertnstert.2006.03.014 · Cited by 370 · see citing works
- Authors
- Stephensen, S. S., Sigfusson, G., Eiriksson, H., Sverrisson, J. T., Torfason, B., Haraldsson, A., & Helgason, H.
- Affiliations
- University of Iceland (IS)
- Venue
- Cardiology in the Young
- Publisher
- Cambridge University Press (CUP)
- Published
- 2004-08
- Volume
- 14
- Issue
- 4
- Pages
- 396-401
- ISSN
- 1047-9511 (ISSN-L)
- 1467-1107
- OpenAlex
- W2143274002
- Cited by
- 58
- References
- 16
- Citations by year
- 2026: 2 · 2025: 1 · 2024: 3 · 2023: 4 · 2022: 3 · 2021: 5 · 2020: 2 · 2019: 6 · 2018: 2 · 2017: 1 · 2016: 2 · 2015: 1 · 2014: 3 · 2013: 5 · 2012: 5
- FWCI
- 0.96
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Cardiology in the Young (publishedVersion)
- PubMed (publishedVersion)
- Hirsla (Landspítali University Hospital) (submittedVersion)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Congenital heart defects research
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cardiovascular and Diving-Related Complications
- Keywords
- congenital heart disease, incidence, ventricular septal defect, atrial septal defect, patent ductus arteriosus, pulmonary valve stenosis, bicuspid aortic valve, coarctation of the aorta, tetralogy of Fallot, Down syndrome, extracardiac anomalies, Iceland, age at diagnosis
- MeSH terms
- Electrocardiography, Female, Cardiac Catheterization, Heart Defects, Congenital, Cardiac Surgical Procedures, Humans, Iceland, Infant, Newborn, Male, Registries, Retrospective Studies, Severity of Illness Index, Echocardiography, Doppler, Incidence, Survival Rate, Coronary Angiography, Sex Distribution, Risk Assessment
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
