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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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