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Prenatal identification of the pulmonary arterial supply in tetralogy of Fallot with pulmonary atresia

Published online by Cambridge University Press:  01 April 2009

Anna N. Seale ,
Siew Y. Ho ,
Elliot A. Shinebourne  and
Julene S. Carvalho
Anna N. Seale
Affiliation:
Brompton Fetal and Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom
Siew Y. Ho
Affiliation:
Brompton Fetal and Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom Imperial College, London, United Kingdom
Elliot A. Shinebourne
Affiliation:
Brompton Fetal and Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom
Julene S. Carvalho*
Affiliation:
Brompton Fetal and Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom Fetal Medicine Unit, St George’s Hospital, London, United Kingdom
*
Correspondence to: Dr Julene S Carvalho, Consultant Fetal and Paediatric Cardiologist, Royal Brompton Hospital, Sydney Street, London SW3 6NP. Tel: 0207 351 8704/8361; Fax: 0207 351 8758; E-mail: [email protected]
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Abstract

Objective

To define the patterns of flow of blood to the lungs in fetuses with tetralogy of Fallot and pulmonary atresia.

Background

In this condition, supply of blood to the lungs is provided via an arterial duct or systemic-to-pulmonary collateral arteries, or very rarely through other conduits such as coronary arterial fistulas or an aortopulmonary window. The intrapericardial pulmonary arteries vary in size, and may be absent. These variables influence the prognosis and management.

Methods

We carried out a retrospective review of cases from a tertiary service for fetal cardiology, identifying all cases of tetralogy of Fallot with pulmonary atresia diagnosed antenatally between January, 1997, and April, 2006. We established pre- and postnatal outcomes, and compared the prenatal diagnosis with postnatal or autopsy findings.

Results

Of 6587 fetuses scanned during this period, 11 were diagnosed as having tetralogy of Fallot with pulmonary atresia and no other cardiac defect. In 5, arterial flow to the lungs was via an arterial duct, and in the other 6, the main identified source of flow was systemic-to-pulmonary collateral arteries. Of the latter 6 pregnancies, 4 were terminated, along with 3 of the 5 with ductal supply. The presence of systemic-to-pulmonary collateral arteries was confirmed at postmortem examination in 3 instances, and in the two delivered neonates, in neither of whom was an infusion of prostaglandin commenced.

Conclusion

The patterns of pulmonary flow can be identified prenatally in the setting of tetralogy with pulmonary atresia. Supply through systemic-to-pulmonary collateral arteries impacts on counselling, introducing uncertainty regarding postnatal surgical management.

Keywords

Prenatal diagnosissystemic-to-pulmonary collateral vesselsechocardiography
Type
Original Article
Information
Cardiology in the Young , Volume 19 , Issue 2 , April 2009 , pp. 185 - 191
Copyright
Copyright © Cambridge University Press 2009

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References

1.Haworth, SG, Macartney, FJ. Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Br Heart J 1980; 44: 14–24.CrossRefGoogle ScholarPubMed
2.Baker, EJ. Tetralogy of Fallot with pulmonary atresia. In: Anderson RH, Baker EJ, Macartney FJ et al (eds). Paediatric Cardiology, 2nd ed. Churchill Livingstone, London, 2002, pp 1251–1280.Google Scholar
3.Hornberger, LK, Sanders, SP, Sahn, DJ, et al. In utero pulmonary artery and aortic growth and potential for progression of pulmonary outflow tract obstruction in tetralogy of Fallot. J Am Coll Cardiol 1995; 25: 739–745.CrossRefGoogle ScholarPubMed
4.Sharland, GK, Allan, LD. Normal fetal cardiac measurements derived by cross-sectional echocardiography. Ultrasound Obstet Gynecol 1992; 2: 175–181.CrossRefGoogle ScholarPubMed
5.Schneider, C, McCrindle, BW, Carvalho, JS, Hornberger, LK, McCarthy, KP, Daubeney, PE. Development of Z-scores for fetal cardiac dimensions from echocardiography. Ultrasound Obstet Gynecol 2005; 26: 599–605.CrossRefGoogle ScholarPubMed
6.Shapiro, I, Degani, S, Leibovitz, Z, Ohel, G, Tal, Y, Abinader, EG. Fetal cardiac measurements derived by transvaginal and transabdominal cross-sectional echocardiography from 14 weeks gestation to term. Ultrasound Obstet Gynecol 1998; 12: 404–418.CrossRefGoogle ScholarPubMed
7.Pepa, LP, Savis, A, Jones, A, Sharland, GK, Tulloh, RMR, Simpson, JM. An echocardiographic study of tetralogy of Fallot in the fetus and infant. Cardiol Young 2003; 13: 240–247.CrossRefGoogle Scholar
8.Volpe, P, Campobasso, G, Stanziano, A, et al. Novel application of 4D sonography with B-flow imaging and spatio-temporal image correlation (STIC) in the assessment of the anatomy of pulmonary arteries in fetuses with pulmonary atresia and ventricular septal defect. Ultrasound Obstet Gynecol 2006; 28: 40–46.CrossRefGoogle ScholarPubMed
9.Vesel, S, Rollings, S, Jones, A, Callaghan, N, Simpson, J, Sharland, G. Prenatally diagnosed pulmonary atresia with ventricular septal defect: echocardiography, genetics, associated abnormalities and outcome. Heart 2006; 92: 1501–1505.CrossRefGoogle ScholarPubMed
10.Miyashita, S, Chiba, Y. Prenatal demonstration of major aortopulmonary collateral arteries with tetralogy of Fallot and pulmonary atresia. Fetal Diagn Ther 2004; 19: 100–105.CrossRefGoogle ScholarPubMed
11.Dinarevic, S, Redington, A, Rigby, M, Shinebourne, EA. Outcome of pulmonary atresia and ventricular septal defect during infancy. Pediatr Cardiol 1995; 16: 276–282.CrossRefGoogle ScholarPubMed
12.Tulzer, G, Artz, W, Franklin, RC, Loughnan, PV, Mair, R, Gardiner, HM. Fetal pulmonary valvuloplasty for critical pulmonary stenosis or atresia with intact septum. Lancet 2002; 360: 1567–1568.CrossRefGoogle ScholarPubMed
13.Haworth, SG. Collateral arteries in pulmonary atresia with ventricular septal defect a precarious blood supply. Br Heart J 1980; 44: 5–13.CrossRefGoogle ScholarPubMed
14.Boshoff, D, Gewillig, M. A review of the options for treatment of major aortopulmonary collateral arteries in the setting of tetralogy of Fallot with pulmonary atresia. Cardiol Young 2006; 16: 212–220.CrossRefGoogle ScholarPubMed