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Epidemiology of congenital heart disease in Merseyside—1979 to 1988

Published online by Cambridge University Press:  19 August 2008

Mark Jackson
Affiliation:
Royal Liverpool Children‘s Hospital, Alder Hey, Liverpool
Kevin P. Walsh
Affiliation:
Institute of Child Health, University of Liverpool and the Cardiac Unit, Alder Hey, Liverpool
Ian Peart
Affiliation:
Institute of Child Health, University of Liverpool and the Cardiac Unit, Alder Hey, Liverpool
Robert Arnold*
Affiliation:
Institute of Child Health, University of Liverpool and the Cardiac Unit, Alder Hey, Liverpool
*
Dr. Mark Jackson, The Cardiothoracic Centre Liverpool NHS Trust, Thomas Drive, Liverpool, L14 3PE, United Kingdom. Tel. 0151 254 7937; Fax.0151 2547935.

Abstract

All infants and children born alive with documented congenital heart disease (excluding those weighing less than 2500 g at birth with a diagnosis of patent arterial duct) within the county of Merseyside between 1979 and 1988 were identified from the combined records of the Liverpool Congenital Malformations Registry and the Cardiac Unit, Royal Liverpool Children‘s Hospital Alder Hey, Liverpool. In total, 1543 cases were disclosed from a live-birth population of 203,880 providing a prevalence rate at birth of 7.57 (95% confidence interval: 7.20–7.96) cases per 1000 livebirths. The overall crude death rate was 18.5% (95% confidence interval: 16.4–20.3). An additional 240 (15.6% [95% confidence interval: 13.8–17.5%]) cases of heart disease not identified by the registry were disclosed from the records of the cardiac unit, demonstrating the unreliability of rates published by monitoring bodies. The annual prevalence rate at birth varied from 6.3 to 9.0 per 1000 livebirths, and seemed to be associated with the impact of new imaging technology. Regional variation was marked (5.3 to 8.9 per 1000 livebirths) and unaccountable in terms of potential morphogenetic mechanism, suggesting inconsistencies in referral of cases. Exclusion of regions of poor ascertainment in the calculation of a revised figure for the prevalence rate at birth suggests that as many as 89 in every 10,000 infants born alive may have structural cardiac anomalies. In conclusion, annual and regional variation of ascertainment in registry studies remains the enemy of an accurate description of the true prevalence rate at birth for congenital heart disease.

Type
Original Manuscripts
Copyright
Copyright © Cambridge University Press 1996

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References

1. Office of Population Censuses and Surveys. Congenital malformation statistics notifications. Series MB3 No. 3, 1987. Her Majesty‘s Stationary Office, London.Google Scholar
2.Clark, EB, Takao, A. Overview: A focus for research in cardiovascular development. In: Clark, EB, Takao, A (eds). Developmental Cardiology: Morphogenesis and Function. Futura Publishing Company Inc., New York, 1990, pp 312.Google Scholar
3.Kenna, AP, Smithells, RW, Fielding, DW.Congenital heart disease in Liverpool: 1960–69. Quart J Med 1975; 173: 1744.Google Scholar
4.Bound, JP, Logan, FWE.Incidence of congenital heart disease in Blackpool 1957–1971. Br Heart J 1977; 39: 445450.CrossRefGoogle ScholarPubMed
5.Samanek, M, Slavik, Z, Krejcir, M.Annual and seasonal variations in the prevalence of congenital cardiac malformations in live-born infants. Cardiol Young 1992; 2: 152157.CrossRefGoogle Scholar
6.Vesterby, A, Nielsen, K, Borg, L, Paulsen, S, Baandrup, U.Congenital heart malformations in Jutland, Denmark: A three year necropsy study in children aged 0–14 years. Br Heart J 1987; 58: 653658.CrossRefGoogle Scholar
7.Lien, W-P, Chen, J-J, Chen, J-H, Lin, J-L, Hsieh, Y-Y, Wu, T-L, Chu, S-H, Hung, C-R.Frequency of various congenital heart diseases in Chinese adults: Analysis of 926 consecutive patients over 13 years of age. Am J Cardiol 1986; 57: 840844.Google ScholarPubMed
8.Hoffman, JIE. Incidence, mortality and natural history. In: Anderson, RH, Macartney, FJ, Shinebourne, EA, Tynan, M (eds). Paediatric Cardiology. Churchill Livingstone, Edinburgh, 1987, pp 314.Google Scholar
9.Clark, EB. Growth, morphogenesis and function: The dynamics of cardiovascular development. In: Moller, JM, Neal, WA, Lock, JA (eds). Fetal, Neonatal and Infant Heart Disease. Appleton-Century-Crofts, New York, 1989, pp 114.Google Scholar
10.Rose, V, Clark, EB. Etiology of Congenital Heart Disease. In: Freedom, RM, Benson, LN, Smallhorn, JF (eds). Neonatal Heart Disease. Springer-Verlag, London, 1992, pp 317.CrossRefGoogle Scholar
11.Owens, JR, Simpkin, JMMcGuinness, L, Harris, F.The Liverpool Congenital Malformations Registry. Paediatric and Perinatal Epidemiology 1988; 2: 240252.CrossRefGoogle ScholarPubMed
12.British Paediatric Association Classification of Diseases. The British Paediatric Association, London, 1979.Google Scholar
13.Gittenberger-De-Groot, A.Persistent ductus arteriosus: most probably a primary congenital malformation. Br Heart J 1977; 39: 610618.CrossRefGoogle ScholarPubMed
14.Armitage, P, Berry, G.Statistical Methods in Medical Research, second edition. Blackwell Scientific Publications, Oxford, 1987.Google Scholar
15.Kaplan, EL, Meier, P.Nonparametric estimation from incomplete observations. J Am Stat Assoc 1958; 53: 457481.CrossRefGoogle Scholar
16.Hoffman, JIE.Natural history of congenital heart disease. Problems in its assessment with special reference of ventricular septal defects. Circulation 1968; 37: 97125.CrossRefGoogle ScholarPubMed
17.Hoffman, JIE, Christiansen, R.Congenital heart disease in a cohort of 19,502 births with long-term follow-up. Am J Cardiol 1978; 42: 641647.CrossRefGoogle Scholar
18.Ferencz, C, Rubin, JD, McCarter, RJ, Brenner, JI, Neill, CA, Perry, LW, Hepner, SI, Downing, JW.Congenital heart disease: Prevalence at livebirth. The Baltimore-Washington Infant Study. Am J Epidemiol 1985; 121: 3136.CrossRefGoogle ScholarPubMed
19.Carlgren, LE, Ericson, A, Kallen, B.Monitoring of congenital cardiac defects. Pediatr Cardiol 1987; 8: 247256.CrossRefGoogle ScholarPubMed
20. Eurocat Working Group. Eurocat report 3. Surveillance of congenital anomalies years 1980–1986. University ofLouvain, Brussels, 1989.Google Scholar
21.Martin, GR, Perry, LW, Ferencz, C.Increased prevalence of ventricular septal defect: Epidemic or improved diagnosis. Pediatrics 1989; 83: 200203.CrossRefGoogle ScholarPubMed
22.Rose, V, Boyd, ARJ, Ashton, TE.Incidence of heart disease in the city of Toronto. Canadian Med J 1964; 91: 95100.Google ScholarPubMed
23.Dickinson, DF, Arnold, R, Wilkinson, JL.Congenital heart disease among 160,480 liveborn children in Liverpool 1960 to 1969. Implications for surgical treatment. Br Heart J 1981; 46: 5562.CrossRefGoogle Scholar
24.Kitchiner, DJ, Jackson, M, Walsh, K, Peart, I, Arnold, R.Incidence and prognosis of congenital aortic valve stenosis in Liverpool (1960–1990). Br Heart J 1993; 69: 7179.CrossRefGoogle ScholarPubMed