Diagnostic accuracy of prenatal ultrasound in coarctation of aorta: systematic review and individual participant data meta-analysis.

OBJECTIVE: To determine the diagnostic accuracy of prenatal ultrasound in detecting coarctation of the aorta (CoA). METHODS: An individual participant data meta-analysis was performed to report on the strength of association and diagnostic accuracy of different ultrasound signs in detecting CoA prenatally. MEDLINE, EMBASE and CINAHL were searched for studies published between January 2000 and November 2021. Inclusion criteria were fetuses with suspected isolated CoA, defined as ventricular and/or great vessel disproportion with right dominance on ultrasound assessment. Individual participant-level data were obtained by two leading teams. PRISMA-IPD and PRISMA-DTA guidelines were used for extracting data, and the QUADAS-2 tool was used for assessing quality and applicability. The reference standard was CoA, defined as narrowing of the aortic arch, diagnosed after birth. The most commonly evaluated parameters on ultrasound, both in B-mode and on Doppler, constituted the index test. Summary estimates of sensitivity, specificity, diagnostic odds ratio (DOR) and likelihood ratios were computed using the hierarchical summary receiver-operating-characteristics model. RESULTS: The initial search yielded 72 studies, of which 25 met the inclusion criteria. Seventeen studies (640 fetuses) were included. On random-effects logistic regression analysis, tricuspid valve/mitral valve diameter ratio > 1.4 and > 1.6, aortic isthmus/arterial duct diameter ratio < 0.7, hypoplastic aortic arch (all P < 0.001), aortic isthmus diameter Z-score of < -2 in the sagittal (P = 0.003) and three-vessel-and-trachea (P < 0.001) views, pulmonary artery/ascending aorta diameter ratio > 1.4 (P = 0.048) and bidirectional flow at the foramen ovale (P = 0.012) were independently associated with CoA. Redundant foramen ovale was inversely associated with CoA (P = 0.037). Regarding diagnostic accuracy, tricuspid valve/mitral valve diameter ratio > 1.4 had a sensitivity of 72.6% (95% CI, 48.2-88.3%), specificity of 65.4% (95% CI, 46.9-80.2%) and DOR of 5.02 (95% CI, 1.82-13.9). The sensitivity and specificity values were, respectively, 75.0% (95% CI, 61.1-86.0%) and 39.7% (95% CI, 27.0-53.4%) for pulmonary artery/ascending aorta diameter ratio > 1.4, 47.8% (95% CI, 14.6-83.0%) and 87.6% (95% CI, 27.3-99.3%) for aortic isthmus diameter Z-score of < -2 in the sagittal view and 74.1% (95% CI, 58.0-85.6%) and 62.0% (95% CI, 41.6-78.9%) for aortic isthmus diameter Z-score of < -2 in the three-vessel-and-trachea view. Hypoplastic aortic arch had a sensitivity of 70.0% (95% CI, 42.0-88.6%), specificity of 91.3% (95% CI, 78.6-96.8%) and DOR of 24.9 (95% CI, 6.18-100). The diagnostic yield of prenatal ultrasound in detecting CoA did not change significantly when considering multiple categorical parameters. Five of the 11 evaluated continuous parameters were independently associated with CoA (all P < 0.001) but all had low-to-moderate diagnostic yield. CONCLUSIONS: Several prenatal ultrasound parameters are associated with an increased risk for postnatal CoA. However, diagnostic accuracy is only moderate, even when combinations of parameters are considered. © 2024 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

New method to predict need for Rashkind procedure in fetuses with dextro-transposition of the great arteries.

OBJECTIVE: Prenatal congenital heart disease classification systems distinguish between critical dextro-transposition of the great arteries (d-TGA) with restriction of the foramen ovale (FO) (which requires a Rashkind procedure within the first 24 h following delivery) and d-TGA for which surgery is planned (after prostaglandin perfusion or Rashkind procedure later than 24 h after delivery). However, current prenatal diagnostic criteria for postnatal FO restriction in d-TGA are inadequate, resulting in a high false-negative rate. We aimed to identify echocardiographic features to predict the urgent need for Rashkind procedure. METHODS: We identified retrospectively 98 patients with singleton pregnancy diagnosed prenatally with fetal d-TGA at two European centers from 2006 to 2013. Two groups were compared: (1) those in whom the Rashkind procedure was performed within the first 24 h postnatally; and (2) those who did not undergo a Rashkind procedure before cardiac surgery. Exclusion criteria were: (1) no fetal echocardiography within 3 weeks prior to delivery (n = 18); (2) delivery before 37 weeks of gestation (n = 6); (3) improper or lack of measurement of pulmonary vein maximum flow velocity (n = 10); (4) lack of neonatal follow-up data (n = 9); (5) Rashkind procedure performed more than 24 h after delivery (n = 4). RESULTS: Fifty-one patients met the inclusion criteria: 29 who underwent the Rashkind procedure and 22 who did not. There were no differences between these two study groups in terms of maternal age, gestational age at time of fetal echocardiography, fetal biometric measurements, estimated fetal weight, rate of Cesarean delivery, newborn weight or Apgar score at 1 min. There were also no differences during prenatal life between the two groups in terms of fetal cardiac size (heart area/chest area ratio), rate of disproportion between left and right ventricle, FO diameter and maximum velocity of flow through the FO. However, the pulmonary vein maximum velocity was significantly higher in the group requiring a Rashkind procedure (47.62 ± 7.48 vs 32.21 ± 5.47 cm/s; P < 0.001). The cut-off value of 41 cm/s provided maximum specificity (100%) and positive predictive value (100%) at only a slight cost of sensitivity (82%) and NPV (86%). The prenatal appearance of the FO also differed between the groups, the FO valve being flat in 52% of those requiring a Rashkind procedure. CONCLUSIONS: In fetuses with d-TGA, prenatal sonographic findings of increased pulmonary venous blood flow and flattened FO valve were associated with the need for a Rashkind procedure within the first 24 h postnatally; these echocardiographic features could be used to predict prenatally a need for the procedure following delivery. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Fetal cardiology: changing the definition of critical heart disease in the newborn.

Infants born with congenital heart disease (CHD) may require emergent treatment in the newborn period. These infants are likely to benefit the most from a prenatal diagnosis, which allows for optimal perinatal planning. Several cardiac centers have created guidelines for the management of these high-risk patients with CHD. This paper will review and compare several prenatal CHD classification systems with a particular focus on the most critical forms of CHD in the fetus and newborn. A contemporary definition of critical CHD is one which requires urgent intervention in the first 24 h of life to prevent death. Such cardiac interventions may be not only life saving for the infant but also decrease subsequent morbidity. Critical CHD cases may require delivery at specialized centers that can provide perinatal, obstetric, cardiology and cardiothoracic surgery care. Fetuses diagnosed in mid-gestation require detailed fetal diagnostics and serial monitoring during the prenatal period, in order to assess for ongoing changes and identify progression to a more severe cardiac status. Critical CHD may progress in utero and there is still much to be learned about how to best predict those who will require urgent neonatal interventions. Despite improved therapeutic capabilities, newborns with critical CHD continue to have significant morbidity and mortality due to compromise that begins in the delivery room. Fetal echocardiography is the best way to predict the need for specialized care at birth to improve outcome. Once the diagnosis is made of critical CHD, delivery at the proper time and in appropriate institution with specific care protocols should be initiated. More work needs to be done to better delineate the risk factors for progression of critical CHD and to determine which newborns will require specialized care. The most frequently described forms of critical CHD requiring immediate intervention include hypoplastic left heart syndrome with intact or severely restricted atrial septum, obstructed total anomalous pulmonary venous return and transposition of the great arteries with restrictive atrial septum.

[The significance of cardiography in the diagnosis of cardiac ectopia of the fetus]. / Znaczenie echokardiograficznej diagnostyki ektopii serca plodu.

Retrospective analysis of echocardiographic examinations of 7 fetuses with ectopia cordis was presented. The majority of abnormalities were found in low risk pregnancies. There was only one case of isolated ectopia cordis with normal heart anatomy and no extracardiac malformations. There was no survival. Theoretically the prenatal diagnosis of isolated ectopia cordis may help to choose the proper term, place and method of delivery and also to plan the optimal care of newborn. Parents should be counseled about the prognosis in such cases, at the tertiary center, after detailed fetal echocardiography.

[Analysis of perinatal death at the Institute of the Health Center of the Polish mother in 1995, 1996 and 1997. The reason for making changes in the accountability for perinatal death]. / Analiza zgonów w okresie perinatalnym w Instytucie Centrum Zdrowia Matki Polki w latach 1995, 1996 I 1997. Przyczynek do koniecznosci wprowadzenia zmian w sprawozdawczosci na temat umieralnosci okoloporodowej.

Perinatal death's causes of fetuses and newborns from single and twin pregnancies delivered at the PMMHI from 1995-1997 were discussed. Data from the Pathology Department were analysed and compared to information regarding prenatal US + ECHO diagnoses coming from the Department for Diagnoses of Congenital Malformations at the PMMHI. The most frequent cause of death of fetuses and newborns from single pregnancies were congenital malformations (42%). In twins there prevailed such typical for multiple pregnancies' death causes as TTTS (27%), intrauterine demise of one of the twins (17%). Premature labor occupies the second most frequent cause of death both in single and multiple pregnancies. Most of perinatal deaths may be predicted prenatally by means of ultrasound and fetal echocardiography.

Isolated, persistent functional tricuspid valve regurgitation in a fetus with normal heart anatomy (and no extracardiac malformation). Case report.

Functional fetal tricuspid valve regurgitation was diagnosed by echocardiography at 27 weeks of gestation as an isolated anomaly with a maximum velocity of the regurgitation jet of 3 m/s. This was also observed at 30, 32 and 35 weeks of gestation and at term. Just after delivery pneumonia was diagnosed in the newborn baby based on clinical symptoms, chest X-ray and laboratory findings. To date persistent fetal tricuspid valve regurgitation has not been described in the literature on normal fetal heart anatomy. Various etiologies and possible pathomechanisms are discussed.

Can we suspect fetal down syndrome by heart evaluation during the second half of pregnancy?

UNLABELLED: Thirty fetuses with Down syndrome, who had detailed fetal echocardiography and sonography at the tertiary center with videotape recordings, were retrospective analyzed by one observer with a specially prepared flow sheet. The mean gestational age of the fetuses at the time of the study was 31 +/- 5.6 (minimum 21, maximum 39) weeks. The 'main' fetal abnormalities were congenital heart defect (CHD): in 13 cases (43.3%) an abnormal 4-chamber view was recorded, including 6 cases (20%) of isolated CHD and 7 (23.3%) of coexisting CHD + extracardiac malformation. Of the 13 cases of CHD, there were 12 cases of atrio-ventricular canal and 1 case of ventricular septal defect. Normal heart anatomy was recorded in 17 cases (56.7%), including 2 with tricuspid value regurgitation. From the videotape recordings also some 'minor' abnormalities were noticed in a few cases such as: femur length shortening; sandal gap; pericardial effusion; macroglossia; echogenic bowel; absent diastolic flow in the umbilical artery, and others. The prevalence of CHD in the study group was similar to the prevalence of CHD in the comparison group of 20 newborns with Down syndrome, born during the same period of time at the same institution, who had not had prenatal scanning at all (chi2, p > 0.05). CONCLUSIONS: (1) the main 'major' abnormality which might be detected in a fetus with Down syndrome after 20 weeks of pregnancy is CHD, which was presented in 43.3% of this series; (2) the presence of any extracardiac malformation should prompt the sonographer for detailed heart evaluation, and (3) fetal echocardiography may increase the accuracy of 'genetic sonogram' in Down syndrome.