Coarctation Of The Aorta Group Research Articles

The illness severity scores can predict the need for respiratory and inotropic support in newborns with critical congenital heart malformations

Background and objectives. The illness severity scoring systems have been developed and validated to assess mortality risks based on clinical conditions and aid critical patient decision-making. Dextro-transposition of the great arteries (D-TGA) and coarctation of the aorta (CoA) are critical congenital heart defects that require medical treatment from birth and surgical repair in the neonatal period. The study aimed to compare the values of three scoring systems in predicting the need for respiratory and inotropic support for newborns with D-TGA and CoA. Materials and methods. An observational study was conducted on inborn infants diagnosed with D-TGA and CoA admitted to the newborn intensive care unit (NICU) within 12 hours of birth at a referral unit for congenital heart disease between January 2018 and December 2020. Newborns with D-TGA and CoA who died within the first 24 hours of birth, outborn infants, and those with a late diagnosis were excluded. For each patient, the Clinical Risk Index of Babies (CRIB-II), Score for Neonatal Acute Physiology-II (SNAP-II), and Score for Neonatal Acute Physiology-Perinatal Extension-II (SNAPPE-II) were calculated. Results. A total of 69 newborns were included in the current study, 30 (43.48 %) of whom were infants with D-TGA (D-TGA Group) and 39 (56.52 %) with CoA (CoA group). Newborns who required respiratory support had significantly higher CRIBII scores (3.03 ± 0.63 vs. 1.34 ± 0.19; p = 0.001), SNAP-II scores (26.61 ± 13.96 vs. 12.43 ± 9.98; p = 0.001), and SNAPPE-II scores (33.04 ± 2.91 vs. 14.47 ± 12.35; p = 0.001) compared to those who did not require it. Significantly higher CRIB-II (3.22 ± 0.67 vs. 1.56 ± 0.23; p = 0.003), SNAP-II (22.91 ±14.57 vs. 14.28 ± 1.58; p = 0.003), and SNAPPE-II (28.82 ± 17.95 vs. 16.58 ± 13.85; p = 0.003) scores were also associated with the need for inotropic support. When comparing the predictive efficiency of illness severity scores for detecting the risk of needing respiratory and inotropic support, they are more effective at predicting the need for ventilation and less effective at predicting the need for inotropes. Conclusions. The illness severity scores can effectively predict the need for pre-surgery interventions. Their simplicity makes them suitable for routine use.

Fetal heart quantification technique improves the prenatal prediction of coarctation of the aorta: A retrospective analysis.

Coarctation of the aorta (CoA) ranks among the most prevalent congenital heart defects and poses a life-threatening risk if left undiagnosed. Herein, we utilized fetal heart quantification (HQ) technology to improve the prenatal prediction of CoA. A retrospective analysis was conducted on 64 fetal cases with suspected aortic arch constriction, identified through prenatal ultrasound findings between November 2020 and March 2022 at the Department of Ultrasound, Sir Run Run Shaw Hospital, Zhejiang University. According to the follow-up results, these cases were divided into two groups: 35 cases confirmed as CoA by postpartum surgery or induction, and 29 cases initially suspected of CoA prenatally but subsequently ruled out postnatally. Additionally, 88 cases of normal fetuses were randomly selected as the control group. Both conventional M-mode ultrasound techniques and Fetal HQ software were utilized for fetal analysis across all groups. Parameters related to the heart were measured, including fetal 4-CV length, width, Global Spherical Index (GSI), Mitral Annular Plane Systolic Excursion (MAPSE), areas and ratios of the left and right ventricles, as well as lengths and ratios of the left and right ventricles. Functional measurements of the left and right ventricles included ejection fraction (EF), fractional area change (FAC), global longitudinal strain (GLS), fractional shortening (FS), end-diastolic diameter (ED), and sphericity index (SI). Left ventricular (LV)-GLS, LV-FAC, LV-EF, and LV-EF Z-score could potentially differentiate between true CoA and false CoA or normal groups and serve as potential indicators for the clinical diagnosis of CoA. The receiver operating characteristic (ROC) curves indicated that LV-GLS and LV-EF Z-score have the greatest predictive power for CoA diagnosis. The segments 6-12 of FS in the confirmed CoA group were significantly lower than those in the false CoA and normal groups. Fetal HQ technology, by assessing changes in the size and shape of the heart, can provide relatively reliable parameter support for the prenatal diagnosis of fetal aortic coarctation.

Application of Computed Tomography Angiography in Preoperative Diagnosis of Coarctation of Aorta and Evaluation of Aortic Dilatation in Infants.

To evaluate the occurrence of aortic dilatation and its associated predictors with coarctation of the aorta (CoA) in infants using multi-slice computed tomography (MSCT). The clinical data of 47 infantile patients with CoA diagnosed by MSCT and 28 infantile patients with simple ventricular septal defect were analyzed retrospectively. Aortic diameters were measured at six different levels, and aortic sizes were compared by z score. The coarctation site-diaphragm ratio was used to describe the degree of narrowing. Relevant clinical data were collated and analyzed. The dilation rate and z score of the ascending aorta in the severe CoA group were significantly higher than those in the mild CoA group (11 [52.38%] vs. 21 [80.77%], P=0.038 and 2.00 ± 0.48 vs. 2.36 ± 0.43, P=0.010). Pearson's correlation analysis found that the z score of the ascending aorta was negatively correlated with the coarctation site-diaphragm ratio value (r=-0.410, P=0.004). A logistic retrospective analysis found that an increased degree of coarctation was an independent predictor of aortic dilatation (adjusted odds ratio 0.002; 95% confidence interval 0.00-0.819; P=0.043). The z score of the ascending aorta in the severe CoA group was significantly higher than that in the ventricular septal defect group (P<0.05). Most infants with CoA can also have significant dilatation of the ascending aorta, and the degree of this dilatation is related to the degree of coarctation. Assessment of aortic diameter and related malformations by MSCT can predict the risk of aortic dilatation in infants with CoA.

Evaluation of the aorta in infants with simple or complex coarctation of the aorta using CT angiography.

To assess aortic dilatation and determine its related factors in infants with coarctation of the aorta (CoA) by using computed tomography angiography (CTA). The clinical data of 55 infantile patients with CoA diagnosed by CTA were analyzed retrospectively. Aortic diameters were measured at six different levels and standardized as Z scores based on the square root of body surface area. The results of simple and complex CoA were compared. Univariate and multivariate logistic regression were used to analyze the effects of sex, age, hypertension, degree of coarctation, CoA type, bicuspid aortic valve (BAV), and other factors related to aortic dilatation. In total, 52 infant patients with CoA were analyzed, including 22 cases of simple CoA and 30 cases of complex CoA. The ascending aorta of the infants in the simple CoA group and the complex CoA group were dilated to different degrees, but the difference was not statistically significant (50.00% vs. 73.33%, P = 0.084, and 2.05 ± 0.40 vs. 2.22 ± 0.43 P = 0.143). The infants in the complex CoA group had more aortic arch hypoplasia than those in the simple CoA group (33.33% vs. 9.09%, P = 0.042). Compared to the ventricular septal defect (VSD) group, the Z score of the ascending aorta in the CoA group was significantly higher than that in the VSD group (P = 0.023 and P = 0.000). A logistic retrospective analysis found that an increased degree of coarctation (CDR value) was an independent predictor of ascending aortic dilatation (adjusted OR = 0.002; P = 0.034). Infants with simple or complex CoA are at risk of ascending aortic dilatation, and the factors of ascending aortic dilatation depend on the degree of coarctation. The risk of aortic dilatation in infants with CoA can be identified by CTA.

Computed tomography angiography assessment of the degree of simple coarctation of the aorta and its relationship with surgical outcome: Aretrospective analysis.

To investigate the correlation between the degree of aortic coarctation and surgical prognosis in infants with simple coarctation of the aorta (CoA) using computed tomography angiography (CTA). This study was a retrospective study. Twenty-seven infants with simple CoA who underwent surgical correction from January 2020 to June 2022 were enrolled. Aortic diameters were measured at five different levels and normalized to Z scores based on the square root of body surface area. The relevant data were collected and analyzed, and the predictors associated with surgical outcome were determined. Patients were divided into the mild CoA group and the severe CoA group according to the severity of coarctation. The mechanical ventilation duration and the length of ICU stay in the mild CoA group were significantly lower than those in the severe CoA group. Multiple linear regression analyses revealed that the degree of aortic coarctation was a significant risk factor for a prolonged postoperative ICU stay. In addition, gestational age and age at operation were risk factors for a prolonged postoperative ICU stay. Correlation analysis showed that the degree of aortic coarctation correlated with the Z scores of the ascending aorta and postcoarctation aorta. The degree of the CoA is an important predictor of surgical outcomes in infants with simple CoA and was significantly correlated with the ascending aorta and postcoarctation aorta Z scores. Therefore, preoperative CTA should be routinely performed to assess the degree of aortic coarctation and better identify risk factors.

Abstract 14791: Evaluation of Cardiac Size, Shape and Function in Fetuses With Coarctation of the Aorta by Fetal Heart Quantitative Technique

Introduction: This study aimed to evaluate the size and shape of the four-chamber view (4CV) and right and left ventricles (RV and LV) as well as the function of the RV and LV in fetuses with coarctation of the aorta (CoA) using fetal heart quantitative (Fetal HQ) technique. Methods: A total of 67 CoA fetuses and 67 gestational age-matched controls were included. Fetal HQ technique was used to obtain the 4CV end-diastolic (ED) width, length, area, and global sphericity index (GSI), RV and LV 24-segment ED diameter (EDD), 24-segment sphericity index (SI), area, and the following functional parameters: (1) fractional area change (FAC); (2) global longitudinal strain (GLS); (3) 24-segment transverse fractional shortening (FS); (4) LV ejection fraction (LVEF), LV ED volume (LVEDV), LV stroke volume (LVSV) and LV output (LVO). Results: Compared with normal controls, the Z-score of 4CV ED width was increased ( P &lt; 0.05), while the GSI was decreased ( P &lt; 0.05) in fetuses with CoA. However, there were no significant differences in the Z-scores of 4CV ED length and area between two groups (both P &gt; 0.05) The RV 24-segment EDD Z-scores, RV area Z-score as well as the RV/LV 24-segment EDD were significantly higher in CoA fetuses than in controls (all P &lt; 0.05). In contrast, the LV 24-segment EDD Z-scores and area Z-score were significantly lower in fetuses with CoA ( P &lt; 0.05). The 24-segment SIs of the LV were significantly higher, while the 24-segment SIs of the RV were significantly lower in CoA group (all P &lt; 0.05). CoA fetuses presented lower GLS, FAC and 24-segment FSs for both RV and LV when compared with normal controls (all P &lt; 0.05). In addition, LVEF, LVEDV Z-score, LVSV Z-score and LVO Z-score were all decreased in CoA fetuses (all P &lt; 0.05). Conclusions: In fetuses with CoA, the 4CV was increased in width and spherical in shape. The LV area and width were decreased, while the transverse width and area of RV was increased. Abnormal SIs reflected a flatter LV and a more spherical RV. RV and LV global, longitudinal, and transverse contractility were all depressed. Fetal HQ can be used to quantitatively analyze cardiac size, shape and function in CoA fetuses, which may improve prenatal diagnosis of CoA.

Serum complement C1q level is associated with left ventricular hypertrophy induced by coarctation of the aorta: A retrospective observational study

BackgroundThe complement system plays an important role in the development of left ventricular hypertrophy. Complement C1q is an initial component of the classical complement pathway and is related to many inflammatory diseases. We aimed to determine whether there was an association between serum complement C1q and left ventricular hypertrophy induced by coarctation of the aorta (CoA).MethodsBased on whether CoA was combined with a large ventricular septal defect (VSD) or patent ductus arteriosus (PDA), the patients were divided into a simple CoA group (n = 15) and a complex CoA group (n = 13). Meanwhile, we selected simple large VSD (n = 14) patients and normal children (n = 28) as the control group. The serum complement C1q level was compared using immunity transmission turbidity among different groups.ResultsThe preoperative content of C1q in the simple CoA group was significantly lower than that in the complex CoA group and normal group (96.97 ± 20.66 vs. 130.73 ± 35.78, 96.97 ± 20.66 vs. 156.21 ± 29.14, P < 0.05). There was no significant difference in the preoperative content of C1q between the complex CoA group and the large VSD group (P > 0.05). There was a negative correlation between the preoperative complement C1q content and the interventricular septal thickness and left ventricular posterior wall thickness (r = − 0.035, r = − 0.288, P < 0.05). The percentage of postoperative decrease in C1q in children with simple CoA or complex CoA was positively correlated with the time of cardiopulmonary bypass and aortic cross clamp, respectively (r = 0.797, r = 0.622, r = 0.898, r = 0.920, P < 0.05). There was no significant difference in the content of preoperative triglycerides (TG), total cholesterol (TCHO), high-density lipoprotein cholesterol (HDL-C) or low-density lipoprotein cholesterol (LDL-C) among the different groups (P > 0.05). In the simple CoA group and complex CoA group, the preoperative complement C1q, TG, TCHO, HDL-C and LDL-C levels were significantly higher than those after the operation (P < 0.05). There was no significant correlation between preoperative complement C1q and TG, TCHO, HDL-C or LDL-C (P > 0.05).ConclusionsComplement C1q has an inhibitory effect on the formation of left ventricular hypertrophy, which may not be mediated by regulating lipid metabolism. During cardiac surgery, complement C1q may have a protective effect against myocardial injury.

Early Echocardiography Predicts Intervention Need in Antenatal Suspicion of Coarctation of the Aorta.

Coarctation of the aorta (CoA) is challenging to diagnose in early postnatal life. We aimed to describe the resource utilization and predictors for the need of intervention in an antenatal suspicion of CoA. A retrospective study of infants with an antenatal suspicion of CoA born at ≥37 weeks was performed. Those not requiring intervention (normal) were compared with those who required cardiac surgery (CoA). Strain was measured using speckle-tracking echocardiography. A total of 51 newborns were included; 40 (78%) were considered normal and 11 (22%) underwent intervention. Echocardiography occurred within the first day of life for both groups. Right ventricular (RV) predominance was present in the CoA group, as demonstrated by the left ventricular (LV) end-systolic eccentricity index (1.60 [0.28] vs 2.16 [0.45]; P < 0.001) and by a larger RV end-diastolic area (EDA) in apical 4-chamber (A4C) relative to LV-EDA-with a ratio of 1.56 [0.23] vs 1.02 [0.2]; P < 0.001. An RV/LV EDA ratio in A4C ≥1.3 had a high probability for CoA (area under the curve= 0.97). Newborns with CoA had a lower RV deformation (peak systolic strain rate:-0.98 [0.17] vs-0.83 [0.2]; P= 0.02). Intraclass correlation coefficient for the EDA ratio revealed a good inter-rater agreement (0.76; 95% confidence interval: 0.55-0.87). Analysis with rater #2 revealed that the EDA ratio ≥1.3 predicted 100% of CoA. The majority of those with an antenatal suspicion of CoA did not require intervention but were high consumers of resources. Within the first day of life, the ventricular EDA ratio in A4C may help predicting those with true CoA requiring intervention.

Incremental Value of Myocardial Deformation in Predicting Postnatal Coarctation of the Aorta: Establishment of a Novel Diagnostic Model

Prenatal detection of coarctation of the aorta (CoA) still suffers from high false-positive and false-negative rates. The objective of this study was to develop a novel model to improve the diagnostic accuracy of fetal CoA. A retrospective study was conducted in 122 fetuses with suspected CoA who also had postnatal follow-up. Fetuses with confirmed diagnoses of CoA after birth were defined as the CoA group, and the non-CoA group comprised those with false positives. Conventional fetal echocardiographic measurements, including great arterial dimensions and flow characteristics, were obtained. Left ventricular (LV) functional parameters were determined using two-dimensional speckle-tracking echocardiography. A novel multiparametric diagnostic model, including gestational age at diagnosis, aortic isthmus (AOI) Z score, and LV longitudinal strain (LVLS), was developed in univariate and multivariate logistic regression analyses. The model was validated prospectively by a validation cohort of 48 fetuses. CoA was confirmed in 62 of 122 patients (50.8%) after birth. Fetuses with postnatal CoA were diagnosed significantly earlier than those with false-positives (median, 24.5weeks [interquartile range, 23.3-26.4weeks] vs 27.8weeks [interquartile range, 24.5-30.4weeks]; P<.001). The Z scores of aortic dimensions (aortic valve annulus, ascending aorta, transverse aortic arch, and AOI) were significantly smaller (P<.001 for all), whereas the Z scores of pulmonary dimensions (pulmonary valve annulus and main pulmonary artery) were significantly greater (P<.05 for all), in cases of confirmed CoA than false positives. Compared with the non-CoA group, the CoA group displayed lower LV ejection fraction (P=.005), LV fractional area change (P<.001), and LVLS (P<.001). A multivariate logistic regression model incorporating gestational age (odds ratio, 0.74; 95% CI, 0.60-0.88; P=.001), AOI Z score (odds ratio, 0.20; 95% CI, 0.08-0.41; P<.001), and LVLS (odds ratio, 1.79; 95% CI, 1.41-2.42; P<.001) was shown to diagnose CoA more accurately (Akaike information criterion=81.77, C statistic=0.945). The performance of this model was confirmed prospectively in the validation cohort. In fetuses with suspected CoA, speckle-tracking analysis of LVLS may have incremental value in predicting postnatal CoA. The diagnostic model presented here, including gestational age, AOI Z score and LVLS, may provide a good tool for stratification of risk in fetal CoA and contribute to patient-specific perinatal management.

Aortic Elasticity and Cardiac Function in Fetuses With Aortic Coarctation.

ObjectiveThe purpose of the study was to observe the elasticity of the ascending aorta (AAo) in normal fetuses and fetuses with coarctation of the aorta (CoA) by M-mode echocardiography.MethodsThis was a prospective clinical study performed on 16 fetuses with CoA and 48 gestational-age matched normal fetuses. The minimum internal diameter in the diastolic phase (Dmin) and the maximum internal diameter in the systolic phase (Dmax) of the AAo were measured by M-mode echocardiography. The aortic strain was calculated using the formula 100 × (Dmax−Dmin)/Dmin). Doppler echocardiography was performed to measure the cardiac function parameters. Correlations between aortic strain and cardiac function were assessed in fetuses with CoA.ResultsThe aortic strain of the ascending aorta in the fetuses with CoA was significantly lower than that in normal fetuses (18.12 ± 4.88% vs. 25.22 ± 4.92%, p < 0.01). The fetuses with CoA showed significantly higher combined cardiac output than the controls (471.89 ± 93.98 vs. 411.57 ± 46.35 ml/min/kg, p < 0.05). Compared with the normal group, the early diastolic velocities (E') and peak systolic velocities (S') of the left side were obviously decreased in the CoA group (p < 0.05), while the left early diastolic velocity ratio (E/E') was significantly increased in the fetuses with CoA (p < 0.01). For the fetuses with CoA, the aortic strain of the AAo was correlated with the left E/E' and S' (r = −0.522 and 0.504, respectively, P < 0.05).ConclusionsThe aortic strain of the ascending aorta was significantly decreased in fetuses with CoA in middle-late gestation. The impaired strain of the ascending aorta was correlated with the left ventricle function in the fetuses with CoA. These findings imply that the abnormalities of the intrinsic aortic wall of CoA might develop early in utero.

Myocardial work indices and ventricular dyssynchrony in adults with aortic coarctation

Abstract Funding Acknowledgements Type of funding sources: None. Background Adults with coarctation of the aorta (CoA) may present residual aortic obstruction and develop arterial vasculopathy and subclinical left ventricular (LV) dysfunction. Myocardial work (MW) is a novel non-invasive index of myocardial efficiency calculated from echocardiographic LV pressure-strain loops, which yields incremental information over ejection fraction and global longitudinal strain. (GLS) Purpose Aim of the present study is to analyze LVMW in a cohort of adult patients with operated aortic CoA Methods CoA patients aged &amp;gt; 18 years who underwent transthoracic echocardiography between September 2020 and July 2021 at our tertiary centre were included. Exclusion criteria were significant recoarctation, impaired LVEF, significant valvular disease and suboptimal image quality. A group of healthy individuals with no cardiac abnormalities. GLS and peak strain dispersion(PSD) were measured. MW indices were calculated using the blood pressure measured in the right arm at the time of the exam. Results Sixty patients (26[22-33]years, 66%male) were included. Data on previous medical history, clinical status at last assessment and Coa-related echocardiographic findings are showed in table 1. No significant differences in traditional parameters of LV systolic and diastolic function were found between groups (EF 60[57-64] vs 57[53-61],p = 0.6), however Coa patients had higher LVmass (84[75-97] vs 68[56-75]g). GLS and MW indices in the study population and in the control group are reported in table 2. Coa group showed lower GLS values and higher PSD (p &amp;lt; 0.0001 for both). Global work index(GWI) and global constructive work(GCW) values were not significantly different between groups, whereas CoA group showed significant increase of global wasted work(GWW) and impaired global work efficiency(GWE,p = 0.003 and 0.0005 respectively). Spearman’s linear method illustrated that both GCW and GWI had a moderate positive relation with mean gradient across the descending aorta at continuous wave Doppler. PSD was positively related to GWW and inversely related to GWE(Figure 1). Using linear regression model with the log of GWE values as dependent variable,PSD,age(β:-0.002,p = 0.03) and LVmass(β:-0.06,p = 0.004)were related to GWE. However, only PSD retained a significant relation on multivariable analysis(β:-0.002,p &amp;gt; 0.0001). Conclusion MW indices assessment is feasible in Coa patients may provide a more comprehensive understanding of the overall myocardial mechanics and performance. In particular, MW demonstrated impaired LV efficiency, which was strongly related to increased mechanical dispersion in Coa patients. Abstract Figure. Abstract Figure.

Echocardiographic findings for improved prenatal diagnosis of aortic coarctation with ventricular septal defect.

Accurate prenatal diagnosis of coarctation of the aorta (CoA) associated with ventricular septal defect (VSD) remains challenging. The objective of the study was to identify which Doppler and/or two-dimensional sonographic findings are most useful for predicting fetal CoA/VSD. A retrospective cohort study identified 35 fetuses with suspected CoA/VSD. Prenatal imaging characteristics included the right ventricular/left ventricular, pulmonary artery (PA)/aorta ratio, aortic isthmus (AOI) Z score, diastolic velocity-time integral (VTID), and systolic velocity-time integral (VTIS) at the AOI. The area under the receiver operating characteristic curve (AUC), integrated discrimination improvement (IDI), and net reclassification improvement (NRI) were calculated. Significant differences in the PA/AO, VTID, VTID/VTIS, VTID/VTIS, VTID/(VTID + VTIS), and AOI Z score between the true CoA group and false positives were found. When associated with VSD, the VTID/VTIS and VTID/(VTID + VTIS) had the highest AUC (0.97, 95% confidence interval: 0.84-1.00), with 88.46% sensitivity and 100.00% specificity for predicting the true CoA. The AOI Z score had the highest sensitivity (92.31%). Adding the VTID/VTIS to the AOI Z score significantly improved the performance (IDI, 50%; NRI, 82%; P < 0.05), with an improvement in specificity (77.78% vs. 55.56%; non-Event P = 0.008) without sacrificing sensitivity (96.15% vs. 92.31%; Event P = 0.564). In fetuses with suspected CoA associated with VSD, the quantitativespectral Doppler metric aided accurate detection of the fetal CoA, with reduced false positives. The conventional AOI Z score plus spectral Doppler metric may improve the overall diagnostic accuracy of CoA/VSD.

Abstract 9008: Echocardiographic Markers in Early Postnatal Life to Predict Intervention in Coarctation of Aorta

Background: Coarctation of the aorta (CoA) is challenging to diagnose in the early neonatal life due to the patent ductus arteriosus (PDA). We aimed to describe the resource utilization, early post-natal cardiac profile and predictors for need of cardiac surgery. Methods: Retrospective study of infants with an antenatal suspicion of CoA, without major associated cardiac anomalies, born at ≥37 weeks during 2014 to 2020. The analyzed echocardiography (ECHO) was performed within 48 hours after birth. Those not requiring post-natal intervention (normal group) were compared to those that required post-natal cardiac surgery (CoA). Strain was measured using speckle-tracking ECHO (STE). Results: A total of 51 newborns were included, of which 40 (78%) were normal and 11 (22%) underwent intervention. In the normal group, median hospitalization was 4 [2-6] days, 6 (15%) were exposed to prostaglandin E (PGE) infusion, 38 (95%) were on intravenous fluids, 9 (23%) required gavage and age at full feeds was 3 days (IQR: 2-5). Ductus was patent in all newborns and aortic arch measurements were significantly smaller in the CoA (ascending [p=0.01], proximal, distal, isthmus [ p &lt;0.001]). Right ventricular (RV) predominance was higher in the CoA group, as demonstrated by the left ventricular (LV) end-systolic eccentricity index (EI) (1.60 [0.28] vs 2.16 [0.45]; p &lt;0.001) and by a larger end-diastolic area (EDA) of the RV in apical 4 chamber (A4C) relative to the LV-EDA - with a ratio of 1.02 [0.2] vs 1.56 [0.23]; p &lt;0.001. Isthmus and EI were predictive for intervention (OR 26, 95%CI (-42 to -9); p =0.002, OR 4.5, 95%CI (1.5-7.5); p =0.003). A RV to LV EDA ratio in A4C greater than 1.3 demonstrated a high probability for intervention (AUC = 0.97). CoA newborns had a lower RV deformation by STE (peak systolic strain rate (SR); -0.98 [0.17] vs -0.83 [0.2]; p =0.02, early diastolic SR; 1.05 [0.2] vs 0.85 [0.26]; p =0.01). Conclusions: Most infants with antenatal suspicion of CoA did not require intervention but were high consumers of resources. Despite PDA patency, isthmus and RV predominance on first post-natal ECHO were predictors for surgery. The novel markers of strain by STE and ventricular EDA ratio in A4C may help predicting those with true CoA requiring intervention.

Analysis of 3-Dimensional Arch Anatomy, Vascular Flow, and Postnatal Outcome in Cases of Suspected Coarctation of the Aorta Using Fetal Cardiac Magnetic Resonance Imaging.

Identifying fetuses at risk of severe neonatal coarctation of the aorta (CoA) can be lifesaving but is notoriously challenging in clinical practice with a high rate of false positives. Novel fetal 3-dimensional and phase-contrast magnetic resonance imaging (MRI) offers an unprecedented means of assessing the human fetal cardiovascular system before birth. We performed detailed MRI assessment of fetal vascular morphology and flows in a cohort of fetuses with suspected CoA, correlated with the need for postnatal intervention. Women carrying a fetus with suspected CoA on echocardiography were referred for MRI assessment between 26 and 36 weeks of gestation, including high-resolution motion-corrected 3-dimensional volumes of the fetal heart and phase-contrast flow sequences gated with metric optimized gating. The relationship between aortic geometry and vascular flows was then analyzed and compared with postnatal outcome. Seventy-two patients (51 with suspected fetal CoA and 21 healthy controls) underwent fetal MRI with motion-corrected 3-dimensional vascular reconstructions. Vascular flow measurements from phase-contrast sequences were available in 53 patients. In the CoA group, 25 of 51 (49%) required surgical repair of coarctation after birth; the remaining 26 of 51 (51%) were discharged without neonatal intervention. Reduced blood flow in the fetal ascending aorta and at the aortic isthmus was associated with increasing angulation (P=0.005) and proximal displacement (P=0.006) of the isthmus and was seen in both true positive and false positive cases. A multivariate logistic regression model including aortic flow and isthmal displacement explained 78% of the variation in outcome and correctly predicted the need for intervention in 93% of cases. Reduced blood flow though the left heart is associated with important configurational changes at the aortic isthmus in fetal life, predisposing to CoA when the arterial duct closes after birth. Novel fetal MRI techniques may have a role in both understanding and accurately predicting severe neonatal CoA.

Research on relevant factors and the value on prenatal ultrasound diagnosis for coarctation of the aorta

Objective To improve the accuracy of prenatal diagnosis for coarctation of the aorta(CoA) by comparatively analyzing the relevant factors and their application value. Methods Cases of disproportion of ventricles and great vessels were selected between January 2011 and July 2018. Only liveborn fetuses with complete postnatal follow-up were included in the study. One hundred and twelve cases were retrieved and analyzed. According to the postnatal ultrasound and CT results, the patients were divided into gruop A and goup B. Different fetal echocardiography parameters and features were selected to evaluate the diagnostic value. Logistic regression analysis was used to select the best predictors of CoA and optimal cut-offs for these parameters were identified by ROC analysis. Results One hundred and thirty eight fetuses were suspected to be CoA, and 112 of them were born. They included 59 cases (52.9%) with CoA(Group A) and 53 cases (47.3%) without CoA(Group B). There were statistically significant differences between the two groups in gestational age, Z-score of diameter of left ventricle, ascending aorta (AAO) and aortic arch isthmus, main pulmonary artery (MPA)/AAO diameters ratio, and arterial duct/isthmus diameters ratio. The parameters most predictive of postnatal CoA selected by logistic regression and the cut-off values identified by ROC analysis were: gestational age at first diagnosis ≤34.5 weeks, Z-score of diameter of left ventricle 1.6. AUC yielded by these parameters in combination was 0.94(95%CI: 0.89-0.99). The study group were divided into two subgroups by gestational age at first diagnosis.The parameters and cut-off values in subgroups were selected by the same analysis as above: Group Ⅰ(≤34.5 weeks), Z-score of diameter of right ventricle >2.1, MPA/AAO diameters ratio>1.6; Group Ⅱ(>34.5 weeks), MPA/AAO diameters ratio>1.7, arterial duct/isthmus diameters ratio>2.3. Meanwhile, some ultrasound features(including aortic arch hypoplasia, retrograde blood flow of aortic isthmus) should also be considered to improve diagnostic accuracy. Conclusions Combined with the use of different ultrasound parameters and features at different gestational weeks can improve the accuracy of the prenatal diagnosis of CoA in the right heart dominant fetus. Key words: Echocardiography; Fetal; Congenital heart disease; Aorta coarctation

Fetal Echocardiographic Measures to Improve the Prenatal Diagnosis of Coarctation of the Aorta.

The objective of this study is to identify fetal echocardiographic measures that predict postnatal coarctation of the aorta (CoA). A retrospective review of patients from 2013 to 2017 identified 13 cases of prenatal diagnosis of CoA confirmed postnatally and 14 cases of prenatal diagnosis of CoA with normal arches postnatally. There were 30 controls. Measurements were made and indices applied on all available longitudinal fetal echocardiograms for each patient. Linear mixed effects models were used to examine the between-group differences in the trajectories of the measurements. Significant differences were seen in the true CoA group for the following: smaller distal transverse arch diameter to distance between the left common carotid and left subclavian arteries (DT/LCA-LSCA) index (p = 0.04), smaller distal transverse arch diameter (p = 0.005), and longer brachiocephalic to left common carotid artery (LCA) (p = 0.004) and LCA-left subclavian artery (LSCA) distances (p < 0.0001). Additionally, the LCA/DT index trend appears to differentiate false positives from true coarctations (p < 0.03). The fetal echocardiographic DT/LCA-LSCA index, brachiocephalic-LCA distance and LCA-LSCA distance are significant predictors of postnatal coarctation. The LCA/DT index trend over time may differentiate which of those patients with prenatal concern for coarctation are more likely to develop coarctation postnatally. The use of fetal echocardiographic measures may improve prenatal detection and predication of postnatal coarctation.

Abstract 16980: Defining the Arrhythmia Burden in Adults With Repaired Coarctation of the Aorta

Introduction: Ventricular and atrial arrhythmias have been shown to correlate with the risk of sudden cardiac death (SCD) in adult congenital heart disease (ACHD). Patients with repaired coarctation of the aorta (CoA) remain one of the highest risk ACHD populations for late SCD. However, the etiology of SCD is poorly understood in this population. Hypothesis: We hypothesize that adults with repaired CoA have an increased prevalence of atrial and ventricular arrhythmia. Methods: We identified 165 adults with repaired CoA (39±11years old, 45% women) and retrospectively analyzed all cardiac rhythm testing (2008-2018). The prevalence of atrial fibrillation (AF), atrial flutter (AFL), supraventricular tachycardia (SVT) and non-sustained ventricular tachycardia (NSVT) were analyzed and compared to prevalence in the general (non-repaired CoA) population. Results: Ventricular and atrial arrhythmia were found to be more common in adults with repaired CoA. The odds of NSVT occurring were 2.4 (95% CI: 1.5-3.8). Atrial arrhythmia risk varied by type with the odds of atrial arrhythmia in the CoA group ranging from 4.4 to 129 times the risk of the general population (See Figure). Conclusions: In this relatively young cohort of adults with repaired CoA, the incidence of ventricular and atrial arrhythmia was significantly higher than the general population. This data indicates that further study of arrhythmogenic substrate for SCD in repaired CoA is important.

Retrospective cohort study of prenatally and postnatally diagnosed coarctation of the aorta (CoA): prenatal diagnosis improve neonatal outcome in severe CoA

Introduction: Prenatal diagnosis of congenital heart disease (CHD) leads to improved outcome but not mortality rate. This may not be the case for coarctation of the aorta (CoA). The objective of this study is to estimate the effect of a prenatal diagnosis of CoA by comparing neonates with CoA by the time of diagnosis.Materials and methods: The study included 38 neonates with CoA diagnosed prenatally and 102 neonates diagnosed postnatally. The postnatal group was divided into two subgroups: (1) severe CoA: symptoms of CoA within the first 7 days (n = 43) and (2) mild CoA: symptoms within the 8–28th day (n = 34). The neonates diagnosed more than 28 days after delivery were excluded from the study (n = 25). Severe CoA was defined as CHD diagnosed postnatally with clinical symptoms that presented in the first week after birth. Mild CoA was defined as CHD that presented clinical symptoms later than 7 days of life.Results: Prostaglandins were initiated at lower doses (p < .001) in the prenatal group. Severe postnatal CoA was associated with more frequent Neonatal Intensive Care Unit (NICU) visits than mild postnatal CoA (p = .005). The length of hospitalization of neonates with severe postnatal CoA was 10 days longer than compared to the prenatal group, but the difference was not statistically significant. The highest mortality rate was in the severe postnatal CoA group (18.6%) which was significantly higher than the mortality rate in the prenatal group (p = .005).Conclusion: 1. Prenatal identification of fetuses at increased risk of developing CoA may reduce mortality and improve outcome only in neonates with severe CoA (symptoms of CoA within the first 7 days after birth); 2. Prenatal diagnosis of severe CoA was associated with lower prostaglandin doses and lower mortality rate.

Non-invasive measurement of cardiac output in children with repaired coarctation of the aorta using electrical cardiometry compared to transthoracic Doppler echocardiography

Objective: To evaluate the equivalence of the ICON® electrical cardiometry (EC) haemodynamic monitor to measure cardiac output (CO) relative to transthoracic Doppler echocardiography (TTE) in paediatric patients with repaired coarctation of the aorta (CoA). Approach: A group of n = 28 CoA patients and n = 27 matched controls were enrolled. EC and TTE were performed synchronously on each participant and CO measurements compared using linear regression and Bland–Altman analysis. The CoA group was further subdivided into two groups, with n = 10 and without n = 18 increased left ventricular outflow tract velocity (iLVOTv) for comparison. Main results: CO measurements from EC and TTE in controls showed a strong correlation (R = 0.80, p < 0.001) and an acceptable percentage error (PE) of 28.1%. However, combining CoA and control groups revealed a moderate correlation (R = 0.57, p < 0.001) and a poor PE (44.2%). We suspected that the CO in a subset of CoA participants with iLVOTv was overestimated by TTE. Excluding the iLVOTv CoA participants improved the correlation (R = 0.77, p < 0.001) and resulted in an acceptable PE of 31.2%. Significance: CO measurements in paediatric CoA patients in the absence of iLVOTv are clinically equivalent between EC and TTE. The presence of iLVOTv may impact the accuracy of CO measurement by TTE, but not EC.

The outcome of percutaneous stent implantation in congenital heart disease: experience of a single institute.

PurposeThe efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups.MethodsStents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the aorta (CoA) group, 5 lesions in 5 patients; (3) Fontan group, 13 lesions in 12 patients; (4) ductal stent group, 3 lesions in 3 patients; and (5) other CHD group, 4 lesions in 4 patients. Mean follow-up duration was 2.1 years (0.1–4 years). Medical records were reviewed retrospectively.ResultsThe minimum lumen diameter (MLD) in PAS and CoA increased from 5.0±1.9 mm and 8.4±1.6 mm to 10.1±3.6 mm and 12.3±2.5 mm, respectively (P<0.01). In the PAS group, pressure gradient decreased from 25.7±15.6 mmHg to 10.4±10.1 mmHg, and right ventricular to aortic pressure ratio from 0.56±0.21 to 0.46±0.19. In the CoA group, the pressure gradient decreased from 50±33 mmHg to 17±8 mmHg. In the ductal stent group, the MLD of the ductus increased from 2.3 mm to 4.3 mm and arterial oxygen saturation from 40%–70% to 90%. No deaths were associated with stent implantation. Stent migration occurred in 3 patients, but repositioning was successful in all. Stent redilation was performed successfully in 26 cases after 29±12 months.ConclusionPercutaneous stent implantation was safe and effective, with acceptable short and mid-term outcomes in Korean CHD patients.