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The Italian Multicentric Study on Epidemiology of Congenital Heart Disease: first step of the analysis

Published online by Cambridge University Press:  19 August 2008

Giuliano Bosi ,
Marco Scorrano ,
Giovanna Tosato ,
Elena Forini  and
Roxana Chakrokh
Giuliano Bosi*
Affiliation:
Section of Pediatrics, Department Med. Clin. Experiment, University of Ferrara, Italy
Marco Scorrano
Affiliation:
Section of Pediatrics, Department Med. Clin. Experiment, University of Ferrara, Italy
Giovanna Tosato
Affiliation:
Section of Pediatrics, Department Med. Clin. Experiment, University of Ferrara, Italy
Elena Forini
Affiliation:
Section of Pediatrics, Department Med. Clin. Experiment, University of Ferrara, Italy
Roxana Chakrokh
Affiliation:
Pediatric Cardiology Unit, University of Ferrara, Italy
*
G. Bosi MD, Pediatric Cardiology Unit, Dpt. Med. Clin. Experiment., University of Ferrara, Via Savonarola 9, 44100 Ferrara, Italy. Tel: 0039 0532 295389; Fax: 0039 0532 202103; E-Mail: bsg@dns.unife.it
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Abstract

We present the aims, methodology and initial results from the Italian Multicentric Study for the registration and follow-up of congenital heart disease. The general aims are to measure the prevalence of congenital heart disease in different geographic areas of Italy, and to assess the survival and outcome of affected babies. During the years 1992 and 1993, eighteen centers for Pediatric Cardiology spread all over the Country enrolled 1445 new babies with congenital cardiac malformations from a population of 341, 647 surveyed livebirths. The new cases were registered using the same methodologic criterions of the EUROCAT study in order to evaluate differences and/or similarities between the studies. The prevalence varied between 1.8‰ and 8.1‰; the average being 4.6%. The large range in prevalence is presumed to be related to different customs and hierarchies in flow and referral of patients. We provide total prevalence of individual lesions, and distribution of sentinel cardiac anomalies, in the Italian study and compare them with EUROCAT. Isolated ventricular septal defect is the most common lesion (39%); followed by atrial septal defect (7.5%); pulmonary valvar stenosis (7.3%); atrioventricular septal defects (5.4%); patency of the arterial duct (3.8%); complete transposition (3.7%); tetralogy of Fallot (3.3%); aortic coarctation (2.4%); aortic valvar stenosis (2.2%); and left heart hypoplasia (1.8%). The echographic stratification of ventricular and atrial septal defects, by location and size, was in keeping with the findings of the EUROCAT study. Because of the recent widespread availability of color-Doppler tecniques, the stratification of aortic and pulmonary valvar stenosis was an innovative approach in our study. Among the complex cardiovascular anomalies, double inlet ventricle and pulmonary atresia had a proportion of about 2% each; with double outlet right ventricle, common arterial trunk, Ebstein's malformation, tricuspid atresia, interrupted aortic arch and totally anomalous pulmonary venous connection having a proportion ranging from 0.5 to 0.8%. We discuss clinical features, such as frequency of extracardiac anomalies and familial aggregation of congenital heart disease, in comparison with the EUROCAT data.

Keywords

congenital heart diseaseepidemiologyitalian multicentric study
Type
Original Articles
Information
Cardiology in the Young , Volume 9 , Issue 3 , May 1999 , pp. 291 - 299
Copyright
Copyright © Cambridge University Press 1999

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References

1.Mitchell, SC, Korones, SB, Berendes, NW. Congenital heart disease in 56.109 births: incidence and natural history. Circulation 1971; 37: 323–332.CrossRefGoogle Scholar
2.Hoffman, JIE. Incidence, mortality and natural history. In: Anderson, RH, Maccartney, FJ, Shineborne, et al. , eds Pediatric Cardiology. London: Churchill Livingstone; 1987: 3–14.Google Scholar
3.Ferencz, C, Correa-Villasenor, A. Epidemiology of cardiovascular malformations: the state of the art. Cardiol Young 1991; 1: 264–284.CrossRefGoogle Scholar
4.De Walls, PH, Weatherall, JAC, Lechat, ME, eds. Registration of congenital anomalies in Eurocat centers 1979–1988. Bruxelles: Cabay, 1985.Google Scholar
5.Eurocat Working Group, ed. Eurocat Report 4. Surveillance of congenital anomalies 1980–1988. Bruxelles: Department of Epidemiology. Catholic University of Louvain, 1991.Google Scholar
6.Garani, GP, Astolfi, G, Cocchi, G, Magnani, C, Milan, M, Calzolari, E. Congenital hearts defects in Emilia Romagna, Italy: epidemiological study in 267.127 consecutive births. Med. Genetik (A) 1995; 2: 177.Google Scholar
7.Pexieder, T, Bloch, D, Oestreicher, D, Swiss Paediatric Cardiologists Group. Analysis of congenital heart disease in 1984 birth cohort. Eur J Epidemiol 1986; 2: 330.Google Scholar
8.Stocker, F, Arbenz, U, Desbaillets, P. Vorkommen und Verlauf angeborener Herzfeheler im ersten Lebensjahr in der Schweiz. Paediat Fortbild Praxis 1980; 31: 90–100.Google Scholar
9.Pexieder, T, De Walls, PH, Bosi, G, Stoll, C, Gallez, A, Houston, A, Vliers, A, Wilkinson, J. Preliminary results of the sub-project for the registration and follow-up of congenital heart disease. In: De Walls, PH, Lechat, ME eds. Eurocat Report (2): Surveillance of congenital anomalies (years 1980–84). 1987; 211–227.Google Scholar
10.Pexieder, T, Bloch, D, EUROCAT Working Party on Congenital Heart Disease. EUROCAT Subproject on epidemiology of congenital heart disease: First analysis of the completed study. In: Clark, EB, Markwald, RR, Takao, A eds: Developmental Mechanisms of Heart Disease. Futura Publishing Co., Armonk, NY 1995; 655–678.Google Scholar
11.Ferencz, C. On the birth prevalence of congenital heart disease. J Am Coll Cardiol. 1990; 16: 1701–1702.CrossRefGoogle ScholarPubMed
12.Mayberry, JC, Scott, WA, Goldberg, SH. Increased birth prevalence of cardiac defects in Yuma, Arizona. J Am Coll Cardiol 1990; 16: 1696–1700.CrossRefGoogle ScholarPubMed
13.Ferencz, C, Rubin, JD, Loffredo, CA, Magee, CA, eds. Epidemiology of congenital heart disease. The Baltimore-Washington Infant Study: Perspectives in Pediatric Cardiology, Vol 4. Futura Publishing Co., Mount Kisco, 1993.Google Scholar
14.Pexieder, T, Bloch, D, Ferencz, C, Eurocat and BWIS. Standard definitions of cardiovascular malformations (CVM) promote accuracy of regional comparisons. Circulation 1991; 84: II385.Google Scholar
15.Fixler, DE, Pastor, P, Chamberlin, M, Sigman, E, Clayton, WE. Trends in congenital heart disease in Dallas county births. Circulation 1990; 81: 137–142.CrossRefGoogle ScholarPubMed
16.Laursen, HB. Some epidemiological aspects of congenital heart disease in Denmark. Acta Paediatr Scand 1980; 69: 619–624.CrossRefGoogle ScholarPubMed
17.Dickinson, DF, Arnold, R, Wilkinson, JL. Congenital heart disease among 160480 liveborn children in Liverpool 1960–1969. Br Heart J 1981; 46: 55–62.CrossRefGoogle Scholar
18.Carlgren, LE, Ericson, A, Kallen, B. Monitoring of congenital cardiac defects. Pediatr Cardiol 1987; 8: 247–256.CrossRefGoogle ScholarPubMed
19.Grabitz, RG, Joffres, MR, Collins-Nakai, RL. Congenital heart disease: incidence in the first year of life. Am J Epidemiol 1988; 128: 381–388.CrossRefGoogle ScholarPubMed
20.Stoll, C, Alembik, A, Roth, MP, Dott, B, De Geeter, B. Risk factors in congenital heart disease. Eur J Epidemiol 1989; 5: 382–391.CrossRefGoogle ScholarPubMed
21.Pradat, P. Epidemiology of major congenital heart defects in Sweden 1981–1986. Epidemiol Community Health 1992; 46: 211–215.CrossRefGoogle Scholar
22.Fyler, DC. Report of the New England regional infant cardiac program. Pediatrics. 1980; 65(suppl): 376–461.Google Scholar
23.Kenna, AO, Smithells, BW, Fielding, DWCongenital heart disease in Liverpool 1960–69. Quart J Med. 1978; 43: 17–44.Google Scholar
24.Samanek, M, Slavik, Z, Zborilova, B, Hrobonova, V, Voriskova, M, Skovranek, J. Prevalence, treatment, and outcome of heart disease in live-born children: a prospective analysis of 91.823 live-born children. Pediatr Cardiol 1989; 10: 205–211.CrossRefGoogle ScholarPubMed
25.Manetti, A, Pollini, I, Cecchi, F, de Simone, L, Cianciulli, D, Carbone, C, Romanelli, A, Bianchi, F, Dolara, A. Epidemiologia delle malformazioni cardiovascolari. III. Prevalenza e decorso in 46.895 nati vivi alia Maternità di Careggi, Firenze, nel periodo 1975–1984. G Ital Cardiol 1993; 23: 145–152.Google Scholar
26.McNamara, DG, Latson, LA. Long-term follow-up of patients with malformations for which defeinitive surgical repair has beeen available for 25 years or more. Am J Cardiol 1982; 50: 560–568.CrossRefGoogle ScholarPubMed
27.Layde, PM, Dooley, K, Erickson, JD, Edmonds, LD. Is there an epidemic of ventricular septal defects in the usa? Lancet 1980; I: 407–408.CrossRefGoogle Scholar
28.Mol, DG, Guntherot, WB. Spontaneous closure of uncomplicated ventricular septal defect. Am J Cardiol 1987; 60: 674–678.Google Scholar
29.Moller, JH, Anderson, RC. 1000 consecutive children with a cardiac malformation with 26-year to 37-year follow-up. Am J Cardiol 1992; 70: 661–667.CrossRefGoogle Scholar
30.Gersony, WM, Hayes, CJ, Driscoll, DJ, Keane, JF, O'Fallon, WM, Pieroni, DR, Wolfe, RR, Weidman, WH. Second natural history study of congenital heart defects. Quality of life of patients with aortic stenolsis, pulmonary stenosis, or ventricular septal defect. Circulation 1993; 87(2 Suppl.): 152–165.Google ScholarPubMed
31.Kitada, M, Uheda, K, Nakagawa, T, Yamaguchi, Y. Follow-up study into early adulthood of patients with congenital heart disease. Jpn Cir 1987; 51: 1409–1414.CrossRefGoogle Scholar
32.Campbell, M. Incidence of cardiac malformations at birth and later and neonatal mortality. Br Heart J 1973; 35: 189–200.CrossRefGoogle ScholarPubMed
33.Radzik, D, van Doesburg, N, Fournier, A, Ducharme, G, Marchand, T, Davignon, A. Predictive factors for spontaneous closure of atrial septal defect diagnosed during the first 3 months of life. XXVIII Annual General Meeting of the Association of European Paediatric Cardiologists (A) 1992; Berlin, Germany,April 22–26.CrossRefGoogle Scholar
34.Kramer, HH, Majewski, F, Trampisch, HJ, Rammos, S, Bourgeois, M. Malformation patterns in children with congenital heart disease. Am J Dis child. 1987; 141: 789–795.Google ScholarPubMed
35.Sancez Cascos, A. Malformationes congenitos associadas a los cardiopatias congenitos. 1987; 180: 253–255.Google Scholar
36.Landtman, B. Clinical and morphological studies in congenital heart disease: a review of 777 cases. Acta Paed Scand 1971; 213: 1–27.Google ScholarPubMed
37.Khoury, MJ, Erickson, JD. Improved ascertainment of cardiovascular malformations in infants with Down's syndrome. Implication for the interpretation of increasing rates of cardiovascular malformations in surveillance systems. Am J Epidemiol 1992; 136: 1457–1464.CrossRefGoogle ScholarPubMed
38.Stocker, FP. Angeborene Herzfehler: was geschieht im Kindesalter? Schweiz Med Wochenschr. 1993; 123: 2037–2041.Google Scholar
39.Ferencz, C, Boughman, JA, Neil, CA, Brenner, Ji, Perry, LWand The Baltimore-Washington Infant Stusy Group. Epidemiology of cardiovascular malformations: questions of inheritance. J Am Coll Card 1989; 14: 756–763.CrossRefGoogle ScholarPubMed
40.Ferencz, C, Loffredo, CA, Correa-Villasenor, A, Wilson, PD eds. Genetics and environmental risk factors of major cardiovascular malformations. The Baltimore Washington Infant Study, 1981–89: Perspectives in Pediatric Cardiology, Vol 5: Futura Publishing Co., 1997.Google Scholar