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Published on: April 7, 2023
Accurate prenatal diagnosis of coarctation of the aorta by 3-step echocardiographic diagnostic protocol
Hong Meng1, Zhi-Ling Luo2, Yan Shen2
1Department of Echocardiography, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 167 Beilishi Rd, Xicheng District, Beijing, 100037, P. R. China. drfwmh@126.com.
Insights
A new 3-step diagnostic protocol accurately detects fetal coarctation of the aorta (CoA), a common congenital heart defect. This algorithm improves prenatal diagnosis, reducing false positives and negatives for better consultation and management.
Area of Science:
- Fetal cardiology
- Diagnostic imaging
- Congenital heart disease
Background:
- Coarctation of the aorta (CoA) is the most frequent undiagnosed congenital heart defect during prenatal screening.
- High rates of false positives and negatives in prenatal screening complicate consultation and postnatal care.
- Accurate prenatal diagnosis of CoA is crucial for effective management.
Purpose of the Study:
- To evaluate the effectiveness of various fetal echocardiographic measurements in predicting prenatal CoA.
- To develop a diagnostic algorithm for improved prenatal detection of CoA.
- To enhance the accuracy of prenatal diagnosis for coarctation of the aorta.
Main Methods:
- A cohort of 154 fetuses with suspected CoA was analyzed, divided into confirmed CoA (n=47) and false positive (n=107) groups.
- Measurements included aortic arch dimensions, the transverse aortic arch-descending aorta (TAO-DAO) angle, and Z-scores.
- A 3-step diagnostic protocol was developed, incorporating TAO-DAO angle, transverse arch diameter, isthmus Z-score, and other indices.
Main Results:
- Confirmed CoA cases exhibited significantly thinner transverse arches, lower isthmus Z-scores, smaller TAO-DAO angles, and a larger distance between the left subclavian artery (LSA) and left common carotid artery (LCCA).
- The 3-step diagnostic protocol achieved high predictive capability with an Area Under the Curve (AUC) of 0.975.
- The protocol demonstrated excellent diagnostic accuracy, with 97.8% sensitivity and 97.2% specificity, validated by a kappa value of 0.936.
Conclusions:
- The developed 3-step diagnostic protocol effectively integrates key echocardiographic measurements for fetal CoA detection.
- This algorithm offers a highly accurate method for identifying coarctation of the aorta in fetuses.
- The protocol aids in improving prenatal diagnosis and subsequent management of congenital heart defects.
Background:
Coarctation of the aorta (CoA) is the most common undiagnosed congenital heart defect during prenatal screening. High false positive and false negative rates seriously affect prenatal consultation and postnatal management. The objective of the study was to assess the utility of various measurements to predict prenatal CoA and to derive a diagnostic algorithm.
Methods:
One hundred and fifty-four fetuses with suspected CoA who presented at Fuwai Hospital between December 2017 and August 2021 were enrolled and divided into confirmed CoA cases (n = 47) and false positive cases (n = 107), according to their postnatal outcomes. The transverse aortic arch, isthmus, and descending aorta were measured in the long-axis view of the aortic arch. The angle between the transverse aortic arch (TAO) and the descending aortic arch (DAO) was defined as the TAO-DAO angle and measured in the long axis or sagittal view. Based on the database in GE Voluson E10 and the formula (Z = [Formula: see text]), the standard score (Z-score) of the dimensions of the aorta were calculated in relation to the gestational age. The main echocardiographic indices were combined to design a 3-step diagnostic protocol. The TAO-DAO angle was used as the first step in the diagnostic model. The diameter of the transverse arch and the Z-score of the isthmus were the second step. The third-step indices included a Z-score of the transverse arch, diameter of the isthmus, distance from the left subclavian artery (LSA) to left common carotid artery (LCCA), the ratio of isthmus diameter and LSA diameter and ratio of the distances (the distance between the LSA and LCCA to the distance between the right innominate artery and LCCA). The receiver operating characteristic (ROC) curve determined the predictive capability of each diagnostic parameter, and the kappa test determined the diagnostic accuracy of the proposed model.
Results:
The cases with confirmed CoA had thinner transverse arches (1.92 ± 0.32 mm vs. 3.06 ± 0.67 mm, P = 0.0001), lower Z-scores of the isthmus (-8.97 ± 1.45 vs. -5.65 ± 1.60, P = 0.0001), smaller TAO-DAO angles (105.54 ± 11.51° vs. 125.29 ± 8.97°, P = 0.0001) and larger distance between the LSA and LCCA (4.45 ± 1.75 mm vs. 2.74 ± 1.07 mm, P = 0.0001) than the false positive cases. The area under the curve (AUC) was 0.947 (95% CI 0.91-0.98) for the TAO-DAO angle ≤ 115.75°, 0.942 (95% CI 0.91-0.98) for the transverse arch diameter ≤ 2.31 mm, 0.937 (95% CI 0.90-0.98) for the Z-score of the isthmus ≤ -7.5, and 0.975 (95% CI 0.95-1.00) for the 3-step diagnostic protocol with 97.8% sensitivity and 97.2% specificity. The kappa test showed that the model's diagnostic accuracy was consistent with postnatal outcomes (kappa value 0.936, P = 0.0001).
Conclusions:
The 3-step diagnostic protocol included the three most useful measurements and the additional indices with appropriate cut-off values. The algorithm is useful for the detection of aortic coarctation in fetuses with a high degree of accuracy.
Trial Registration:
Retrospectively registered.

