Prenatal Diagnosis and Outcome of Congenital Corrected Transposition of the Great Arteries - A Multicenter Report of 69 Cases.

OBJECTIVE: Congenital corrected transposition of the great arteries (ccTGA) is a rare congenital cardiac anomaly which remains difficult to diagnose prenatally. We aim to investigate the natural history, associated anomalies and the outcome of patients in prenatally diagnosed ccTGA. METHOD: This was an international multicenter retrospective analysis of fetuses with a diagnosis of ccTGA from 2002 to 2017. We reviewed clinical and echocardiographic databases of seven centers. Anatomic survey and fetal echocardiography were performed according to international guidelines of ISUOG. RESULTS: We considered 69 fetuses with prenatally suspected ccTGA. There was an overall survival rate of 91 % among 54 patients with a confirmed diagnosis. Survival to live birth was 96 % (52/54) and survival on an intention-to-treat basis was 94 % (49/52). The mean gestational age at the time of diagnosis was 25.6 ±â€Š5.9 weeks of gestation. In 7 out of 54 fetuses (13 %), ccTGA was an isolated finding. Dextro/mesocardia was present in 15 cases (27.8 %). Intracardiac anomalies were present in 46/54 cases (85.2 %) with the most frequent anomaly being a ventricular septal defect present in 41 fetuses (75.9 %). Complete heart block was diagnosed in 10 cases (18.5 %). Extracardiac anomalies were observed in 9 out of 54 cases (16.7 %). Prenatal karyotyping of the fetus was available in 30/54 (55.6 %) cases with chromosomal anomalies in 4/30 (13.3 %). CONCLUSION: ccTGA is a rare cardiac anomaly often accompanied by a variable spectrum of further intracardiac abnormalities. Accurate diagnosis of ccTGA, which can be integrated into parental counselling, is feasible with a favorable short-term outcome for affected neonates.

Outcome and Associated Findings in Individuals with Pre- and Postnatal Diagnosis of Tetralogy of Fallot (TOF) and Prediction of Early Postnatal Intervention.

PURPOSE: The aim of our retrospective evaluation was to compare the outcome of patients with prenatal and postnatal diagnosis of Tetralogy of Fallot (TOF) and to analyze prenatal echocardiographic parameters predicting intervention within 30 days postnatal. MATERIALS AND METHODS: We evaluated 142 patients in our pediatric heart center and prenatal diagnosis center and prenatal practice Praenatal plus in Cologne between 01/08-06/16. RESULTS: Within the prenatal diagnosis group, 6/74 fetuses (8.1 %) had TOF with pulmonary atresia (TOF-PA), and 6 (8.1 %) had absent pulmonary valve syndrome (TOF-APVS). 14 (18.9 %) had an abnormal karyotype including 9/14 (64.3 %) with microdeletion 22q11.2. 25 (33.8 %) had extracardiac malformation. 4 (5.4 %) had agenesis of ductus arteriosus (DA), 22 (29.7 %) had right aortic arch (RAA) and 9 (12.2 %) had major aortopulmonary collateral arteries (MAPCAs). Within the postnatal diagnosis group, no patient had TOF-PA, 4/68 (5.9 %) had TOF-APVS. 12 (17.6 %) had extracardiac malformations, 9 (13.2 %) had an abnormal karyotype including 2/9 with microdeletion 22q11.2. 10 (14.7 %) had RAA, 9 (13.2 %) had MAPCAs. There were no cases with agenesis of DA. Increasing z-score values of the left/right pulmonary artery (LPA/RPA) prenatally were associated with a lower probability for early postnatal intervention (RPA: p = 0.017; LPA: p = 0.013). Within the prenatal diagnosis group, 12 of 41 (29.3 %) live-born patients with follow-up and intention to treat needed early intervention versus 7 (10.3 %) in the postnatal diagnosis group (p = 0.02). Within the postnatal diagnosis group, there were no deaths, while 2 (4.9 %) post-intervention deaths occurred in the prenatal diagnosis group. CONCLUSION: There are no significant differences concerning post-intervention survival in the prenatal diagnosis group versus the postnatal diagnosis group. Complex cases may be underrepresented in the postnatal diagnosis group. Smaller RPA/LPA values prenatally seem to be associated with early postnatal intervention.

Fetal Cardiac Axis in Fetuses with Conotruncal Anomalies.

Purpose The purpose of this study was to assess the cardiac axis in fetuses with conotruncal anomalies during four-chamber view scanning. Materials and Methods We retrospectively evaluated the cardiac axis of 150 fetuses with conotruncal anomalies within the second and third trimester between October 2008 and August 2014. The cardiac axis was obtained by the angle of two lines in a transverse view of the fetal thorax at the level of the four-chamber view. The first line divided the thorax into two equal halves starting from the spine posteriorly ending at the sternum. The second line was placed through the interventricular septum of the fetal heart. The angle was calculated using OsiriX software. Results 23 had double outlet right ventricle (DORV), 17 had truncus arteriosus communis (TAC), 36 had tetralogy of Fallot (TOF), and 74 had complete transposition of the great arteries (d-TGA). In fetuses with DORV ≤ 24 + 6 weeks of gestation (wks), the mean cardiac axis was 52.5° (p = 0.005), at ≥ 25 + 0 wks it was 51.1° (p = 0.0003). In fetuses with TAC ≤ 24 + 6 wks, the mean cardiac axis was 56.8° (p = 0.01), at ≥ 25 + 0 wks it was 50.0° (p = 0.05). In fetuses with TOF ≤ 24 + 6 wks, the mean cardiac axis was 67.5° (p < 0.0001), at ≥ 25 + 0 wks it was 63.8° (p < 0.0001). In fetuses with d-TGA ≤ 24 + 6 wks, the mean cardiac axis was 45.6°, at ≥ 25 + 0 wks it was 45.4° (not significant). Throughout gestation, the cardiac axis did not show a difference in the two separate examinations. Conclusion In fetuses with DORV, TAC and TOF, the cardiac axis is significantly different from the normal axis, but in fetuses with TGA there is no significant difference compared to the normal axis. Therefore, analysis of the heart axis could be useful for screening for conotruncal anomalies.

Myocardial Function Pre- and Post-Fetal Endoscopic Tracheal Occlusion (FETO) in Fetuses with Left-Sided Moderate to Severe Congenital Diaphragmatic Hernia.

Purpose To evaluate pre- and postprocedural myocardial function in fetuses with moderate to severe congenital diaphragmatic hernia (CDH) who underwent FETO to improve survival and to reduce morbidity and to compare these data with fetuses and CDH not undergoing FETO and normal controls. Materials and Methods 8 fetuses with isolated left-sided CDH were included and underwent FETO at our center between 2012 and 2013. Prior to and after the operation, myocardial function was assessed by measuring the mitral annular plane systolic excursion (MAPSE), the tricuspid annular plane systolic excursion (TAPSE), Tei index, isovolumetric contraction time (ICT), ejection time (ET), isovolumetric relaxation time (IRT) for the left ventricle in PW Doppler ultrasound as well as ICT, ET, IRT and Tei index in pulsed wave tissue Doppler imaging (PW-TDI) for the left and right ventricle. The E-, A-, E'- and A'-wave peak velocity and the systolic downward motion (S') were measured for both ventricles and the E/A, E/E' and E'/A' ratios were calculated. Results were compared to fetuses with CDH not undergoing FETO and to gestational age-matched healthy controls. RESULTS: FETO was performed at 32.5 (SD 2.4) weeks of gestation. There was no statistically significant change in myocardial function in fetuses treated by FETO except a slight prolongation of the ICT of the left ventricle in PW-TDI. The myocardial function of fetuses with CDH pre- and post-FETO and fetuses with CDH without FETO was comparable to that of healthy controls. Conclusion In our series FETO did not affect myocardial function in fetuses with left-sided CDH. Although ventricular preload increases, FETO seems to have no short-term impact on fetal myocardial performance.

Short-Time Impact of Laser Ablation of Placental Anastomoses on Myocardial Function in Monochorionic Twins with Twin-to-Twin Transfusion Syndrome.

Purpose To evaluate pre- and post-procedure myocardial function in monochorionic twins with TTTS who underwent laser ablation of placental anastomoses using pulsed wave tissue Doppler imaging (pw TDI). Materials and Methods 20 monochorionic twin gestations with TTTS were included and underwent laser ablation at our center between 2011 and 2014. Prior to and after the intervention, cardiac function was assessed by measuring the mitral annular plane systolic excursion (MAPSE), the tricuspid annular plane systolic excursion (TAPSE), Tei index, isovolumetric contraction time (ICT), ejection time (ET), isovolumetric relaxation time (IRT) for the left ventricle in pulsed wave Doppler (pw D) ultrasound as well as ICT, ET, IRT and Tei index in pw TDI for the left and right ventricle. E-, A-, E´- and A´-wave peak velocity and the systolic downward motion (S´) were measured for both ventricles and the E/A, E/E´ and E´/A´ ratios were calculated. In a mean of 1.3 (SD 0.6) days after laser ablation, this measurement protocol was repeated. Results Pre-intervention recipients had longer ICT, ET and IRT in pw D and pw TDI compared to donors not reaching statistical significance for most parameters. Statistically significant were prolonged ICT in pw D (p 0.01) and ET (p 0.01) in pw TDI in recipients. In donor fetuses preoperative myocardial function did not differ significantly from postoperative myocardial function except in increased left ventricular ejection time of the left ventricle in pw TDI (p 0.04) and an increased E´/A´ratio (p 0.01). After laser coagulation, myocardial function was slightly altered in recipients as ICT and IRT shortened and Tei indices decreased but only reaching statistical significance in shortened IRTs in pw TDI for both ventricles. Conclusion Laser ablation of placental anastomoses in TTTS might influence myocardial function in the postoperative period. Shortened IRT intervals may reflect an improvement of diastolic function in recipients.

Fetal and neonatal diagnosis of interrupted aortic arch: associations and outcomes.

OBJECTIVE: Interrupted aortic arch (IAA) is a rare but serious anomaly. Prenatal diagnosis is challenging and published data are limited. The aim of the study was to review the data of fetuses and neonates diagnosed with IAA during a 16-year period at Children's Hospital Giessen. METHODS: Retrospective ascertainment of 8 fetuses and 20 neonates with a confirmed diagnosis of IAA from 1994 to 2010 by reviewing the hospital database of the cardiovascular program of the prenatal and pediatric cardiology clinics at the University Hospital Giessen. RESULTS: Eighteen cases with IAA type B and 10 cases with IAA type A were found. After 2005, prenatal diagnosis was achieved in 8 cases and postnatal imaging confirmed IAA in all 8 neonates. Twenty-nine percent of individuals had a chromosomal anomaly, with microdeletion 22q11.2 being the most common abnormality (n = 6, 21%). In 46% (13/28) other complex cardiac anomalies were present. Mortality after surgery was 18%. Long-term morbidity and mortality was due to neurological impairment in the presence of microdeletion 22q11.2 and the need of surgical or catheter re-intervention. CONCLUSION: Despite the difficulties and challenges in diagnosis, the prenatal detection rate of IAA is increasing. Associated complex cardiac and chromosomal abnormalities influence the outcome of patients with IAA and are important issues of parental counseling.