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Significance of lung anomalies in fetuses affected by tetralogy of Fallot with absent pulmonary valve syndrome

Published online by Cambridge University Press:  04 July 2017

Estelle Tenisch ,
Marie-Josée Raboisson ,
Françoise Rypens ,
Julie Déry ,
Andrée Grignon  and
Chantale Lapierre
Estelle Tenisch
Affiliation:
Radiodiagnostic and Interventional Radiology Department, CHU Vaudois, Lausanne, Suisse, Canada
Marie-Josée Raboisson
Affiliation:
Cardiology Department, CHU Sainte-Justine, Montréal, Canada
Françoise Rypens
Affiliation:
Medical Imaging Department, CHU Sainte-Justine, Montréal, Canada
Julie Déry
Affiliation:
Medical Imaging Department, CHU Sainte-Justine, Montréal, Canada
Andrée Grignon
Affiliation:
Medical Imaging Department, CHU Sainte-Justine, Montréal, Canada
Chantale Lapierre*
Affiliation:
Medical Imaging Department, CHU Sainte-Justine, Montréal, Canada
*
Correspondence to: Dr C. Lapierre, Medical Imaging Department, CHU Sainte-Justine, 3175 Cote-Sainte-Catherine, Montreal, QC, Canada, H3T 1C5. Tel: +514 345 4931, ext. 3499; Fax: +514 345 4816; E-mail: chantal_lapierre@ssss.gouv.qc.ca
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Abstract

Objectives

Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of tetralogy of Fallot with dilatation of large pulmonary arteries. Prognosis is related to the severity of the cardiac malformation and to bronchial tree compression by dilated pulmonary arteries. This study analyses the prenatal echographic lung appearance in fetuses with tetralogy of Fallot with absent pulmonary valve and discusses its significance.

Methods

We carried out a retrospective review of fetal and postnatal files of nine fetuses diagnosed with tetralogy of Fallot with absent pulmonary valve syndrome in our institution. Correlations of prenatal ultrasound and cardiac imaging findings were obtained with outcome.

Results

Abnormal heterogeneous fetal lung echogenicity was detected in eight cases out of nine, always associated with significant lobar arterial dilatation. This aspect was well correlated with postnatal imaging and outcome in the four neonatal cases. The only fetus with normal lung echogenicity also had lower degree of pulmonary artery dilatation in the series.

Conclusions

This study demonstrates that a heterogeneous ultrasound appearance of the fetal lungs can be detected in utero in the most severe cases. This aspect suggests an already significant compression of the fetal bronchial tree by the dilated arteries that may have prognostic implications.

Keywords

Prenatal diagnosisfetal ultrasoundtetralogy of Fallotabsence of pulmonary valvefetal lung anomaly
Type
Original Articles
Information
Cardiology in the Young , Volume 27 , Issue 9 , November 2017 , pp. 1740 - 1747
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Copyright
© Cambridge University Press 2017 

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References

1. Chevers, N. Recherches sur les maladies de l’artère pulmonaire. Arch Gen Med 1847; 15: 488–508.Google Scholar
2. Miller, RA, Lev, M, Paul, MH. Congenital absence of the pulmonary valve. The clinical syndrome of tetralogy of Fallot with pulmonary regurgitation. Circulation 1962; 26: 266–278.Google Scholar
3. Rao, PS, Lawrie, GM. Absent pulmonary valve syndrome. Surgical correction with pulmonary arterioplasty. Br Heart J 1983; 50: 586–589.Google Scholar
4. Emmanoulides, GC, Gutgesell, HP. Congenital absence of the pulmonary valve. In Emmanoulides GC, Riemenschneider TA, Allan LD, Gutgesell HP, (eds). Heart Disease in Infants, Children and Adolescents. Willimans and Wilkins, Baltimore, 1995: 1018–1026.Google Scholar
5. Jacobs, ML. Congenital heart surgery nomenclature and database project: tetralogy of Fallot. Ann Thorac Surg 2000; 69 (Suppl): S77–S82.CrossRefGoogle ScholarPubMed
6. Momma, K, Kondo, C, Matsuoka, R. Tetralogy of Fallot with pulmonary atresia associated with chromosome 22q11 deletion. J Am Coll Cardiol 1996; 27: 198–202.CrossRefGoogle ScholarPubMed
7. Razavi, RS, Sharland, GK, Simpson, JM. Prenatal diagnosis by echocardiogram and outcome of absent pulmonary valve syndrome. Am J Cardiol 2003; 91: 429–432.CrossRefGoogle ScholarPubMed
8. Zucker, N, Rozin, I, Levitas, A, Zalzstein, E. Clinical presentation, natural history, and outcome of patients with the absent pulmonary valve syndrome. Cardiol Young 2004; 14: 402–408.CrossRefGoogle ScholarPubMed
9. Becker, R, Schmitz, L, Guschmann, M, Wegner, RD, Stiemer, B, Entezami, M. Prenatal diagnosis of familial absent pulmonary valve syndrome: case report and review of the literature. Ultrasound Obstet Gynecol 2001; 17: 263–267.Google Scholar
10. Wertaschnigg, D, Jaeggi, M, Chitayat, D, et al. Prenatal diagnosis and outcome of absent pulmonary valve syndrome: contemporary single-center experience and review of the literature. Ultrasound Obstet Gynecol 2013; 41: 162–167.Google Scholar
11. Chelliah, A, Berger, JT, Blask, A, Donofrio, MT. Clinical utility of fetal magnetic resonance imaging in tetralogy of Fallot with absent pulmonary valve. Circulation 2013; 127: 757–759.CrossRefGoogle ScholarPubMed
12. Rabinovitch, M, Grady, S, David, I, et al. Compression of intrapulmonary bronchi by abnormally branching pulmonary arteries associated with absent pulmonary valves. Am J Cardiol 1982; 50: 804–813.Google Scholar
13. Milanesi, O, Talenti, E, Pellegrino, PA, Thiene, G. Abnormal pulmonary artery branching in tetralogy of Fallot with “absent” pulmonary valve. Int J Cardiol 1984; 6: 375–380.Google Scholar
14. Galindo, A, Gutiérrez-Larraya, F, Martinez, JM, et al. Prenatal diagnosis and outcome for fetuses with congenital absence of the pulmonary valve. Ultrasound Obstet Gynecol 2006; 28: 32–39.Google Scholar
15. Lee, SL, Cheung, YF, Leung, MP, Ng, YK, Tsoi, NS. Airway obstruction in children with congenital heart disease: assessment by flexible bronchoscopy. Pediatr Pulmonol 2002; 34: 304–311.Google Scholar
16. An, HS, Choi, EY, Kwon, BS, et al. Airway compression in children with congenital heart disease evaluated using computed tomography. Ann Thorac Surg 2013; 96: 2192–2197.CrossRefGoogle ScholarPubMed
17. Driscoll, DJ, Shaddy, RE, Feltes, TF. Moss and Adams’ Heart Disease in Infants, Children and Adolescents Including the Fetus and Young Adult , volume 2, 7th edn. Wolters Kluwer, Lippincott, Williams and Wilkins, Philadelphia, PA, 2008.Google Scholar
18. Yeager, SB, Van Der Velde, ME, Waters, BL, Sanders, SP. Prenatal role of the ductus arteriosus in absent pulmonary valve syndrome. Echocardiography 2002; 19: 489–493.Google Scholar
19. Volpe, P, Paladini, D, Marasini, M, et al. Characteristics, associations and outcome of absent pulmonary valve syndrome in the fetus. Ultrasound Obstet Gynecol 2004; 24: 623–628.Google Scholar
20. Fink, AM, Edis, B, Massie, J. The CT appearances of delayed amniotic fluid clearance from the lungs in an infant with absent pulmonary valve and congenital lobar emphysema. Pediatr Radiol 2005; 35: 891–894.Google Scholar
21. Beydon, N, Larroquet, M, Coulomb, A, et al. Comparison between US and MRI in the prenatal assessment of lung malformations. Pediatr Radiol 2013; 43: 685–696.Google Scholar
22. Olutoye, OO, Coleman, BG, Hubbard, AM, Adzick, NS. Prenatal diagnosis and management of congenital lobar emphysema. J Pediatr Surg 2000; 35: 792–795.Google Scholar
23. Pariente, G, Aviram, M, Landau, D, Hershkovitz, R. Prenatal diagnosis of congenital lobar emphysema: case report and review of the literature. J Ultrasound Med 2009; 28: 1081–1084.Google Scholar