Hostname: page-component-848d4c4894-x24gv Total loading time: 0 Render date: 2024-04-30T15:51:59.121Z Has data issue: false hasContentIssue false
  • English
  • Français

An echocardiographic study of tetralogy of Fallot in the fetus and infant

Published online by Cambridge University Press:  24 May 2005

Litha P. Pepas ,
Alexandra Savis ,
Annette Jones ,
Gurleen K. Sharland ,
Robert M.R. Tulloh  and
John M. Simpson
Litha P. Pepas
Affiliation:
Department of Congenital Heart Disease, Guy's Hospital, London, UK
Alexandra Savis
Affiliation:
Department of Congenital Heart Disease, Guy's Hospital, London, UK
Annette Jones
Affiliation:
Department of Congenital Heart Disease, Guy's Hospital, London, UK
Gurleen K. Sharland
Affiliation:
Department of Congenital Heart Disease, Guy's Hospital, London, UK
Robert M.R. Tulloh
Affiliation:
Department of Congenital Heart Disease, Guy's Hospital, London, UK
John M. Simpson
Affiliation:
Department of Congenital Heart Disease, Guy's Hospital, London, UK
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective: To document the echocardiographic features of tetralogy of Fallot during fetal and postnatal life. Correlation of echocardiographic findings with the requirement for early intervention prior to definitive repair. Design: Retrospective observational study. Setting: A tertiary fetal cardiology unit. Patients: Fetuses with a diagnosis of tetralogy of Fallot identified from a prospective database between 1 January 1999 and 31 October 2002. Main measures of outcome: Growth of aorta and pulmonary trunk during fetal and postnatal life. Doppler assessment of the great arteries both prenatally and postnatally. Clinical outcome to definitive repair. Results: We identified 25 fetuses with tetralogy of Fallot, 23 having a pulmonary valvar diameter below the normal range at some point during gestation. The ratio of the diameter of the aortic to the pulmonary valve was abnormal in all cases. The pulmonary arterial Doppler velocity was within the normal range in six fetuses at presentation, and elevated in the remainder. In two fetuses, the right ventricular outflow tract was patent during fetal life, but had become atretic at birth. Both of these fetuses had reversal of flow in the arterial duct at presentation during fetal life. In 2 fetuses in whom we showed poor growth of the pulmonary trunk in late gestation, it was necessary to intervene early. The Doppler velocity across the pulmonary valve during fetal life did not differentiate between babies who required early intervention and those who were repaired electively. There was a marked increase in pulmonary arterial Doppler velocity following birth, which became more elevated with age. Of 18 liveborn infants, 17 have survived, with 2 having balloon dilation of the right ventricular outflow tract, and 3 insertion of a Blalock-Taussig shunt prior to definitive repair. Conclusions: In tetralogy of Fallot, features of pulmonary valvar hypoplasia and obstruction are evident during fetal life. Progression of obstruction in the right ventricular outflow tract was observed during fetal life as well as postnatally. Reversal of flow in the arterial duct, and failure of growth of the pulmonary trunk, predicted the need for early surgery to maintain pulmonary blood flow. Parents should be counselled about the possibility of emergency intervention being required after birth. Affected fetuses should be delivered at units with experience of managing the cyanosed newborn.

Keywords

Subpulmonary obstructionoverriding aortacongenital heart diseaseechocardiography
Type
Original Article
Information
Cardiology in the Young , Volume 13 , Issue 3 , June 2003 , pp. 240 - 247
Copyright
© 2003 Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Fyler DC. Tetralogy of Fallot. In: Fyler DC (ed.). Nadas' Pediatric Cardiology. Hanley and Belful, Philadelphia, 1992, pp 471–491.
Gatzoulis MA, Soukias N, Ho SY, Josen M, Anderson RH. Echocardiographic and morphological correlations in tetralogy of Fallot. Eur Heart J 1999; 20: 221–231.Google Scholar
Hornberger LK, Saunders SP, Sahn DJ, et al. In utero pulmonary artery and aortic growth and potential for progression outflow tract obstruction in tetralogy of Fallot. J Am Coll Cardiol 1995; 25: 739–745.Google Scholar
Lee W, Smith RS, Comstock CH, Kirk JS, Riggs T, Weinhouse E. Tetralogy of Fallot: Prenatal diagnosis and postnatal survival. Obstst Gynecol 1995; 86: 583–588.Google Scholar
Azancot A, Eydoux P, Vuillard E, Cusin V, Baumann C, Blot P. Spectre clinique de la tetralogie de Fallot antenatale. Arch Mal Coeur Vaiss 2000; 93: 587–593.Google Scholar
Sharland GK, Allan LD. Normal fetal cardiac measurements derived by cross-sectional echocardiography. Ultrasound Obstet Gynecol 1992; 2: 175–181.Google Scholar
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.Google Scholar
Simpson JM. Echocardiographic evaluation of cardiac function in the human fetus. MD Thesis. University of London, 2000.
Snider AR, Enderlein MA, Teitel DF, Juster RP. Two-dimensional echocardiographic determination of aortic and pulmonary artery sizes from infancy to adulthood in normal subjects. Am J Cardiol 1984; 53: 218–224.Google Scholar
Tagrett RC, Heldt GP, McIlroy MB. Doppler velocity in the pulmonary artery of infants, children and adults. Cardiovascular Research 1986; 20: 816–821.Google Scholar
Ettedgui JA, Sharland GK, Chita SK, Cook A, Fagg N, Allan LD. Am J Cardiol 1990; 66: 233–234.
Altman D. Construction of age related reference centiles using absolute residuals. Stat in Medicine 1993; 12: 917–921.Google Scholar
Allan LD, Sharland GK. Prognosis in fetal tetralogy of Fallot. Pediatric Cardiol 1992; 13: 1–4.Google Scholar
Chessa M, Butera G, Bonhoeffer PP, et al. Relation of genotype 22q11 deletion to phenotype of pulmonary vessels in tetralogy of Fallot and pulmonary atresia-ventricular septal defect. Heart 1998; 79: 186–190.Google Scholar