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An echocardiographic study of diagnostic accuracy, prediction of surgical approach, and outcome for fetuses diagnosed with discordant ventriculo-arterial connections

Published online by Cambridge University Press:  01 October 2007

Cécile J. Pascal ,
Ian Huggon ,
Gurleen K. Sharland  and
John M. Simpson
Cécile J. Pascal
Affiliation:
Service de Cardiologie Pédiatrique, Hôpital Mère et Enfants, CHU, Nantes, France
Ian Huggon
Affiliation:
Fetal Cardiology Unit, Department of Congenital Heart Disease, Evelina Children’s Hospital, London, United Kingdom
Gurleen K. Sharland
Affiliation:
Fetal Cardiology Unit, Department of Congenital Heart Disease, Evelina Children’s Hospital, London, United Kingdom
John M. Simpson*
Affiliation:
Fetal Cardiology Unit, Department of Congenital Heart Disease, Evelina Children’s Hospital, London, United Kingdom
*
Correspondence to: Dr John M. Simpson, Consultant in Fetal and Paediatric Cardiology, Fetal Cardiology Unit, Dept of Congenital Heart Disease, 6th Floor, Evelina Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, Lambeth Palace Road, London SE1 7EH, United Kingdom. Tel: +44 (0)20 7188 2308; Fax: +44 (0)20 7188 2307; E-mail: john.simpson@gstt.nhs.uk
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Abstract

Our aims were to examine the diagnostic accuracy of prenatal diagnosis of concordant atrioventricular and discordant ventriculo-arterial connections, the accuracy of predictions made concerning the postnatal surgical approach, and a description of the different subtypes related to outcome. All fetuses were evaluated at a tertiary centre for fetal cardiology between January, 1994, and December, 2003. In this period, we identified congenitally malformed hearts in 1,835 fetuses, of whom 56 (3%) met the criterions of inclusion. Of the total, 30 (54%) had an intact ventricular septum, 9 (16%) had an associated ventricular septal defect, 7 (13%) had a ventricular septal defect and pulmonary stenosis, 1 (2%) had pulmonary stenosis and an intact ventricular septum, 8 (14%) had a ventricular septal defect and aortic coarctation, and 1 (2%) had coarctation of the aorta with an intact ventricular septum. All the discordant ventriculo-arterial connections were correctly identified. For associated ventricular septal defects, the diagnostic sensitivity was 96%, with specificity of 88%, positive predictive value of 85%, and negative predictive value of 97%. For aortic coarctation, the sensitivity was 100%, specificity 96%, positive predictive value 82%, and negative predictive value 100%. The prediction of the surgical approach was accurate in 41 of 48 cases (85%). For those fetuses with pulmonary stenosis and ventricular septal defect, the ratio of the diameters of the pulmonary trunk was shown to be helpful in predicting the possibility of an arterial switch as opposed to the Rastelli type of repair. Of the 49 liveborn infants, 46 were alive at 30 days (94%, with 95% confidence intervals from 83 to 99%), and 43 at one year (88%, 95% confidence intervals from 75 to 95%). Deaths were mainly related to the anatomy of the coronary arteries, and associated cardiac lesions.

Keywords

Transposition of the great arteriesechocardiographyprenatal diagnosis
Type
Original Article
Information
Cardiology in the Young , Volume 17 , Issue 5 , October 2007 , pp. 528 - 534
Copyright
Copyright © Cambridge University Press 2007

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References

1.Becker, TA, Van Amber, R, Moller, JH, Pierpont, ME. Occurrence of cardiac malformations in relatives of children with transposition of the great arteries. Am J Hum Genet 1996; 66: 28–32.3.0.CO;2-S>CrossRefGoogle ScholarPubMed
2.Bull, C, Yates, R, Sarkar, D, Deanfield, J, de Leval, M. Scientific, ethical and logistical considerations in introducing a new operation: a retrospective cohort study from a pædiatric cardiac surgery. BMJ 2000; 320: 1168–1173.CrossRefGoogle ScholarPubMed
3.Fraisse, A, Skender, N, Nassi, C, Gaudart, J, Paut, O, Camboulives, J. Causes de mortalité préopératoire de la transposition des gros vaisseaux, à propos de deux cas. Arch Mal Cœur Vais 2000; 93: 653–656.Google Scholar
4.Van Praagh, R, Jung, WK. The arterial switch operation in transposition of the great arteries: anatomic indications and contraindications. Thorac Cardiovasc Surg 1991; 39 (suppl 2): 138–150.CrossRefGoogle Scholar
5.Soongswang, J, Adatia, I, Newman, C, Smallhorn, JF, Williams, WG, Freedom, RM. Mortality in potential arterial switch candidates with transposition of the great arteries. J Am Coll Cardiol 1998; 32: 753–757.CrossRefGoogle ScholarPubMed
6.Kumar, RK, Newburger, JW, Gauvreau, K, Kamenir, SA, Hornberger, LK. Comparison of outcome when hypoplastic left heart syndrome and transposition of the great arteries are diagnosed prenatally versus when diagnosis of these two conditions is made only postnatally. Am J Cardiol 1999; 83: 1649–1653.CrossRefGoogle ScholarPubMed
7.Bonnet, D, Coltri, A, Butera, G, et al. . Detection of transposition of the great arteries in fetuses reduces neonatal mortality and morbidity. Circulation 1999; 99: 916–918.CrossRefGoogle Scholar
8.Chantepie, A, Schleich, JM, Gournay, V, Blaysat, G, Maragnes, P. Mortalité préopératoire de la transposition isolée des gros vaisseaux. Arch Pediatr 2000; 7: 34–39.CrossRefGoogle Scholar
9.Maeno, YV, Kamenir, SA, Sinclair, B, van der Velde, ME, Smallhorn, JF, Hornbeger, LK. Prenatal features of ductus arteriosus constriction and restrictive foramen ovale in d-transposition of the great arteries. Circulation 1999; 99: 1209–1214.CrossRefGoogle ScholarPubMed
10.Sharland, GK, Allan, LD. Normal fetal cardiac measurements derived by cross-sectional echocardiography. Ultrasound Obstet Gynecol 1992; 2: 175–181.CrossRefGoogle ScholarPubMed
11.Mielke, G, Benda, N. Blood flow velocity waveforms of the fetal pulmonary artery and the ductus arteriosus: reference ranges from 13 weeks to term. Ultrasound in Obstet Gynecol 2000; 15: 213–218.CrossRefGoogle ScholarPubMed
12.Bull, C. Current and potential impact of fetal diagnosis on prevalence and spectrum of serious congenital heart disease at term in the UK. Lancet 1999; 354: 1242–1247.CrossRefGoogle ScholarPubMed
13.Hyett, J, Perdu, M, Sharland, G, Snijders, R, Nicolaides, K. Using fetal nuchal translucency to screen for major congenital cardiac defects at 10-14 weeks of gestation: population based cohort study. BMJ 1999; 318: 81–85.CrossRefGoogle ScholarPubMed
14.Makrydimas, G, Sotiriadis, A, Huggon, IC, et al. . Nuchal translucency and fetal cardiac defects: a pooled analysis of major fetal echocardiography centers. Am J Obstet Gynecol 2005; 192: 89–95.CrossRefGoogle ScholarPubMed
15.Gill, H, Splitt, M, Sharland, G, Simpson, J. Patterns of recurrence of congenital heart disease. An analysis of 6,640 consecutive pregnancies evaluated by detailed fetal echocardiography. J Am Coll Cardiol 2003; 42: 923–929.CrossRefGoogle Scholar
16.Burn, J, Brennan, P, Little, J, et al. . Recurrence risks in offsprings of adults with major heart defects: results from first cohort of British collaborative study. Lancet 1998; 351: 311–316.CrossRefGoogle ScholarPubMed
17.Sharland, GK, Chan, Kit-Yee, Allan, LD. Coarctation of the aorta: difficulties in prenatal diagnosis. Br Heart J 1994; 71: 70–75.CrossRefGoogle ScholarPubMed
18.Hornberger, LK, Sahn, DJ, Kleinman, CS, Copel, J, Silverman, NH. Antenatal diagnosis of coarctation of the aorta: a multicentre experience. J Am Coll Cardiol 1994; 23: 417–423.CrossRefGoogle Scholar
19.Pepas, L, Savis, A, Sharland, GK, Tulloh, RMR, Simpson, JM. An echocardiographic study of tetralogy of Fallot in fetal life and infancy. Cardiol Young 2003; 13: 240–247.CrossRefGoogle Scholar