TY - JOUR
T1 - Short-term outcome after the prenatal diagnosis of right aortic arch
AU - Bet, Bo B.
AU - Snoep, Maartje C.
AU - van Leeuwen, Elisabeth
AU - Linskens, Ingeborg H.
AU - Haak, Monique C.
AU - Rozendaal, Lieke
AU - Knobbe, Ingmar
AU - van Schuppen, Joost
AU - Hoekstra, Carlijn E. L.
AU - Koolbergen, David R.
AU - Clur, Sally-Ann
AU - Pajkrt, Eva
N1 - Funding Information: Bo B. Bet and Maartje C. Snoep collected the data. Bo B. Bet analyzed the data, interpreted the study results and wrote the manuscript. Elisabeth van Leeuwen, Ingeborg H. Linskens, Monique C. Haak, Lieke Rozendaal, Ingmar Knobbe, Joost van Schuppen, Carlijn E. L. Hoekstra, David R. Koolbergen, Sally-Ann Clur, and Eva Pajkrt are responsible for the clinical practice of the included cases, distributed over the three participating hospitals and their own department of expertise. JS and SC constructed Figure 3. Elisabeth van Leeuwen, Sally-Ann Clur, and Eva Pajkrt initiated and managed this study and manuscript. All authors contributed to the design of the study, interpreted and described the study findings and critically revised the manuscript. Publisher Copyright: © 2023 The Authors. Prenatal Diagnosis published by John Wiley & Sons Ltd.
PY - 2023/5
Y1 - 2023/5
N2 - Objectives: To determine the proportion of children that require surgery in the first year of life and thereafter in order to improve the counseling of parents with a fetus with a right aortic arch (RAA). Methods: Fetuses diagnosed with isolated RAA, defined as the absence of intra- or extracardiac anomalies, between 2007 and 2021 were extracted from the prospective registry PRECOR. Results: In total, 110 fetuses were included, 92 with a prenatal diagnosis of RAA and 18 with double aortic arch (DAA). The prevalence of 22q11 deletion syndrome was 5.5%. Six pregnancies were terminated and five cases were false-positive; therefore, the follow-up consisted of 99 neonates. Surgery was performed in 10 infants (10%) in the first year of life. In total, 25 (25%) children had surgery at a mean age of 17 months. Eight of these 25 (32%) had a DAA. Only one child, with a DAA, required surgery in the first week of life due to obstructive stridor. Conclusions: Children with a prenatally diagnosed RAA are at a low risk of acute respiratory postnatal problems. Delivery in a hospital with neonatal intensive care and pediatric cardiothoracic facilities seems only indicated in cases with suspected DAA. Expectant parents should be informed that presently 25% of the children need elective surgery and only incidentally due to acute respiratory distress.
AB - Objectives: To determine the proportion of children that require surgery in the first year of life and thereafter in order to improve the counseling of parents with a fetus with a right aortic arch (RAA). Methods: Fetuses diagnosed with isolated RAA, defined as the absence of intra- or extracardiac anomalies, between 2007 and 2021 were extracted from the prospective registry PRECOR. Results: In total, 110 fetuses were included, 92 with a prenatal diagnosis of RAA and 18 with double aortic arch (DAA). The prevalence of 22q11 deletion syndrome was 5.5%. Six pregnancies were terminated and five cases were false-positive; therefore, the follow-up consisted of 99 neonates. Surgery was performed in 10 infants (10%) in the first year of life. In total, 25 (25%) children had surgery at a mean age of 17 months. Eight of these 25 (32%) had a DAA. Only one child, with a DAA, required surgery in the first week of life due to obstructive stridor. Conclusions: Children with a prenatally diagnosed RAA are at a low risk of acute respiratory postnatal problems. Delivery in a hospital with neonatal intensive care and pediatric cardiothoracic facilities seems only indicated in cases with suspected DAA. Expectant parents should be informed that presently 25% of the children need elective surgery and only incidentally due to acute respiratory distress.
UR - http://www.scopus.com/inward/record.url?scp=85148498962&partnerID=8YFLogxK
U2 - https://doi.org/10.1002/pd.6326
DO - https://doi.org/10.1002/pd.6326
M3 - Article
C2 - 36738444
SN - 0197-3851
VL - 43
SP - 629
EP - 638
JO - Prenatal diagnosis
JF - Prenatal diagnosis
IS - 5
ER -