Key ultrasound predictors in the prenatal assessment of congenital pulmonary airway malformation: a single-center experience

BackgroundThis study assesses the sensitivity and specificity of congenital pulmonary airway malformation (CPAM) Volume Ratio (CVR) in predicting the need for fetal therapy (FT) and explores the role of additional ultrasound indicators, including the Observed/Expected Lung to Head Ratio (O/E LHR) an...

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Main Authors: Isabella Fabietti, Alice Novak, Laura Valfrè, Chiara Vassallo, Domenico Umberto De Rose, Milena Viggiano, Andrea Conforti, Chiara Iacusso, Marco Bonito, Pietro Bagolan, Leonardo Caforio
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-05-01
Series:Frontiers in Pediatrics
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Online Access:https://www.frontiersin.org/articles/10.3389/fped.2025.1555539/full
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author Isabella Fabietti
Alice Novak
Alice Novak
Laura Valfrè
Chiara Vassallo
Domenico Umberto De Rose
Milena Viggiano
Andrea Conforti
Chiara Iacusso
Marco Bonito
Pietro Bagolan
Pietro Bagolan
Leonardo Caforio
author_facet Isabella Fabietti
Alice Novak
Alice Novak
Laura Valfrè
Chiara Vassallo
Domenico Umberto De Rose
Milena Viggiano
Andrea Conforti
Chiara Iacusso
Marco Bonito
Pietro Bagolan
Pietro Bagolan
Leonardo Caforio
author_sort Isabella Fabietti
collection DOAJ
description BackgroundThis study assesses the sensitivity and specificity of congenital pulmonary airway malformation (CPAM) Volume Ratio (CVR) in predicting the need for fetal therapy (FT) and explores the role of additional ultrasound indicators, including the Observed/Expected Lung to Head Ratio (O/E LHR) and Mediastinal Angle Shift (MSA), in improving FT prediction.MethodsWe retrospectively studied all CPAM cases referred to our Center from 2018 to 2022. FT was provided at any CVR value in cases of hydrops, rapid lesion growth, or polyhydramnios. The worst CVR, O/E LHR, and MSA values between 20 and 28 weeks of gestation were analyzed.ResultsAmong 62 CPAM cases, 56.4% right-sided and 43.5% left-sided. Hydrops occurred in 5 cases, all right-sided. FT was required in 14 cases (11 receiving steroids and 3 thoraco-amniotic shunt). CVR was significantly higher in the FT group compared to the non-FT group (p < 0.0001), with an optimal cut-off of 1.25 (Sn 100%; Sp 89.6%) for predicting FT. The O/E LHR was significantly lower in the FT group (mean 44.8 vs. 58.3; p = 0.0046, AUC 0.75), with a Sn of 84% and Sp of 62%. MSA was significantly higher in the FT compared to the non-FT group (p < 0.0001), with a threshold of 13.3° providing high Sn (92.8%) and Sp (89.3%) for predicting FT.ConclusionsCVR is the most reliable predictor of the need for FT, even at lower thresholds. MSA can effectively complement CVR in predicting FT, and using multiple parameters may improve parental counseling and identify cases needing closer monitoring.
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spelling doaj-art-667fb3a25145471386b3ff14bbbfc33c2025-08-20T02:31:44ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602025-05-011310.3389/fped.2025.15555391555539Key ultrasound predictors in the prenatal assessment of congenital pulmonary airway malformation: a single-center experienceIsabella Fabietti0Alice Novak1Alice Novak2Laura Valfrè3Chiara Vassallo4Domenico Umberto De Rose5Milena Viggiano6Andrea Conforti7Chiara Iacusso8Marco Bonito9Pietro Bagolan10Pietro Bagolan11Leonardo Caforio12Clinical Area of Fetal, Neonatal and Cardiological Sciences and Research Area of Perinatal Medicine – “Bambino Gesù” Children's Hospital IRCCS, Rome, ItalyClinical Area of Fetal, Neonatal and Cardiological Sciences and Research Area of Perinatal Medicine – “Bambino Gesù” Children's Hospital IRCCS, Rome, ItalyObstetrics and Gynecology Unit, Maternal and Child Department, San Pietro Fatebenefratelli Hospital, Rome, ItalyClinical Area of Fetal, Neonatal and Cardiological Sciences and Research Area of Perinatal Medicine – “Bambino Gesù” Children's Hospital IRCCS, Rome, ItalyClinical Area of Fetal, Neonatal and Cardiological Sciences and Research Area of Perinatal Medicine – “Bambino Gesù” Children's Hospital IRCCS, Rome, ItalyClinical Area of Fetal, Neonatal and Cardiological Sciences and Research Area of Perinatal Medicine – “Bambino Gesù” Children's Hospital IRCCS, Rome, ItalyClinical Area of Fetal, Neonatal and Cardiological Sciences and Research Area of Perinatal Medicine – “Bambino Gesù” Children's Hospital IRCCS, Rome, ItalyClinical Area of Fetal, Neonatal and Cardiological Sciences and Research Area of Perinatal Medicine – “Bambino Gesù” Children's Hospital IRCCS, Rome, ItalyClinical Area of Fetal, Neonatal and Cardiological Sciences and Research Area of Perinatal Medicine – “Bambino Gesù” Children's Hospital IRCCS, Rome, ItalyObstetrics and Gynecology Unit, Maternal and Child Department, San Pietro Fatebenefratelli Hospital, Rome, ItalyClinical Area of Fetal, Neonatal and Cardiological Sciences and Research Area of Perinatal Medicine – “Bambino Gesù” Children's Hospital IRCCS, Rome, ItalyDepartment of Systems Medicine, University of Rome “Tor Vergata”, Rome, ItalyClinical Area of Fetal, Neonatal and Cardiological Sciences and Research Area of Perinatal Medicine – “Bambino Gesù” Children's Hospital IRCCS, Rome, ItalyBackgroundThis study assesses the sensitivity and specificity of congenital pulmonary airway malformation (CPAM) Volume Ratio (CVR) in predicting the need for fetal therapy (FT) and explores the role of additional ultrasound indicators, including the Observed/Expected Lung to Head Ratio (O/E LHR) and Mediastinal Angle Shift (MSA), in improving FT prediction.MethodsWe retrospectively studied all CPAM cases referred to our Center from 2018 to 2022. FT was provided at any CVR value in cases of hydrops, rapid lesion growth, or polyhydramnios. The worst CVR, O/E LHR, and MSA values between 20 and 28 weeks of gestation were analyzed.ResultsAmong 62 CPAM cases, 56.4% right-sided and 43.5% left-sided. Hydrops occurred in 5 cases, all right-sided. FT was required in 14 cases (11 receiving steroids and 3 thoraco-amniotic shunt). CVR was significantly higher in the FT group compared to the non-FT group (p < 0.0001), with an optimal cut-off of 1.25 (Sn 100%; Sp 89.6%) for predicting FT. The O/E LHR was significantly lower in the FT group (mean 44.8 vs. 58.3; p = 0.0046, AUC 0.75), with a Sn of 84% and Sp of 62%. MSA was significantly higher in the FT compared to the non-FT group (p < 0.0001), with a threshold of 13.3° providing high Sn (92.8%) and Sp (89.3%) for predicting FT.ConclusionsCVR is the most reliable predictor of the need for FT, even at lower thresholds. MSA can effectively complement CVR in predicting FT, and using multiple parameters may improve parental counseling and identify cases needing closer monitoring.https://www.frontiersin.org/articles/10.3389/fped.2025.1555539/fullcongenital pulmonary airway malformationCPAMCVRO/E LHRMSAfetal surgery
spellingShingle Isabella Fabietti
Alice Novak
Alice Novak
Laura Valfrè
Chiara Vassallo
Domenico Umberto De Rose
Milena Viggiano
Andrea Conforti
Chiara Iacusso
Marco Bonito
Pietro Bagolan
Pietro Bagolan
Leonardo Caforio
Key ultrasound predictors in the prenatal assessment of congenital pulmonary airway malformation: a single-center experience
Frontiers in Pediatrics
congenital pulmonary airway malformation
CPAM
CVR
O/E LHR
MSA
fetal surgery
title Key ultrasound predictors in the prenatal assessment of congenital pulmonary airway malformation: a single-center experience
title_full Key ultrasound predictors in the prenatal assessment of congenital pulmonary airway malformation: a single-center experience
title_fullStr Key ultrasound predictors in the prenatal assessment of congenital pulmonary airway malformation: a single-center experience
title_full_unstemmed Key ultrasound predictors in the prenatal assessment of congenital pulmonary airway malformation: a single-center experience
title_short Key ultrasound predictors in the prenatal assessment of congenital pulmonary airway malformation: a single-center experience
title_sort key ultrasound predictors in the prenatal assessment of congenital pulmonary airway malformation a single center experience
topic congenital pulmonary airway malformation
CPAM
CVR
O/E LHR
MSA
fetal surgery
url https://www.frontiersin.org/articles/10.3389/fped.2025.1555539/full
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