Right ventricular (RV) function is one of the critical factors affecting the prognosis of fetuses with hypoplastic left heart syndrome (HLHS). Our study objectives included assessment of cardiac function and comprehensive measurement of cardiac microstructure. We retrospectively studied 42 fetuses diagnosed as HLHS by echocardiography. Myocardial deformation of the right ventricular wall was calculated automatically in offline software. Postmortem cardiac imaging for three control fetal hearts and four HLHS specimens was performed by a 9.4T DTI scanner. Myocardial deformation parameters of the RV (including strain, strain rate, and velocity) were significantly lower in HLHS fetuses (all p < 0.01). FA values increased (0.18 ± 0.01 vs. 0.21 ± 0.02; p < 0.01) in HLHS fetuses, but MD reduced (1.3 ± 0.15 vs. 0.88 ± 0.13; p < 0.001). The HLHS fetuses’ RV lateral base wall (−7.31 ± 51.91 vs. −6.85 ± 31.34; p = 0.25), middle wall (1.71 ± 50.92 vs. −9.38 ± 28.18; p < 0.001), and apical wall (−6.19 ± 46.61 vs. −11.16 ± 29.86, p < 0.001) had HA gradient ascent but HA gradient descent in the anteroseptal wall (p < 0.001) and inferoseptal wall (p < 0.001). RV basal lateral wall HA degrees were correlated with RVGLS (R2 = 0.97, p = 0.02). MD values were positively correlated with RVGLS (R2 = 0.93, p = 0.04). Our study found morphological and functional changes of the RV in HLHS fetuses, and cardiac function was related to the orientation patterns of myocardial fibers. It may provide insight into understanding the underlying mechanisms of impaired RV performance in HLHS.

Background Fetal ductal constriction (DC) is associated with excessive polyphenol-rich food (PRF) consumption during pregnancy. However, the effect of this hemodynamic change on fetal cardiac function still needs to be elucidated. Therefore, this study aimed to evaluate the cardiac function of fetuses with PRF-related DC and to describe serial observations of cardiac function changes. Methods We compared the traditional echocardiographic indices, including morphological, hemodynamic, and functional parameters, between study fetuses and controls. For global and segmental deformation analysis of the left and right ventricles, fetalHQ with the speckle-tracking technique was used to calculate sphericity index (SI), global longitudinal strain (GLS), fractional shortening (FS), fractional area change (FAC), etc. In addition, follow-up data were compared with the generalized linear model. Results A total of 60 DC fetuses and 60 gestational-matched controls were enrolled in our study, with 20 DC fetuses undertaking a follow-up echocardiogram after 2–3 weeks. Compared with controls, there was a distinct decrease in right ventricular GLS (RVGLS) (−13.39 ± 3.77 vs. −21.59 ± 2.51, p < 0.001), RVFAC (22.20 ± 9.56 vs. 36.01 ± 4.84, p < 0.001), left ventricular GLS (LVGLS) (−19.52 ± 3.24 vs. −23.81 ± 2.01 p < 0.001), and LVFAC (39.64 ± 7.32 vs. 44.89 ± 4.91, p = 0.004). For 24-segment FS analysis, DC fetuses showed lower FS in left ventricular (LV) segments 18–24, with no difference in LV segments 1–17. Right ventricular (RV) FS in segments 4–23 was also reduced in the DC group. The 24-segment SI analysis indicated significantly lower SI in DC than those in controls for LV segments 1–14 and RV segments 19–24. We found that the pulsatility index (PI) of ductus arteriosus (DA) was an independent variable for RVGLS (β = −0.29, p = 0.04). In 20 DC fetuses with follow-up echocardiograms, no obvious difference in myocardial deformation was found between the initial examination and follow-up data. Conclusion Left and right ventricular performances were both impaired in DC fetuses, along with a series of morphological and hemodynamic changes. Although the state of DA constriction improved on second examinations, cardiac function was not completely restored.

We investigated a custom congenital heart disease (CHD) geneset to assess the diagnostic value of whole‐exome sequencing (WES) in karyotype‐ and copy number variation (CNV)‐negative aborted fetuses with conotruncal defects (CTDs), and to explore the impact of postnatal phenotyping on genetic diagnosis.

Objectives To explore the relationship between cardiac function and pulmonary flow characteristics in tricuspid valve malformation (TVM) fetuses by conventional and speckle tracking echocardiography. Methods Eighty-eight TVM fetuses were retrospectively included and compared with 80 normal controls. TVM fetuses in each trimester were divided into two subgroups according to pulmonary flow characteristics: those with normal pulmonary flow (TVM-N) and those with reduced or absent pulmonary flow (TVM-R/A). Cardiac measurements, Celermajer index, and Simpson-Andrews-Sharland (SAS) score were obtained. Speckle tracking echocardiography was used to compute ventricular deformation parameters. Results TVM fetuses demonstrated significantly lower global longitudinal strain (GLS) and strain rate for both ventricles than controls (all P < .05). When compared with TVM-N, left ventricular (LV) ejection fraction, LV fractional area change, right ventricular (RV) fractional area change, LVGLS, and RVGLS were significantly reduced (all P < .05), while the RV diameter Z-score, RV/LV diameter, SAS score, and Celermajer index were obviously increased in TVM-R/A in both trimesters (all P < .05). Both LVGLS and RVGLS correlated with cardiothoracic circumference ratio, RV diameter Z-score, RV/LV diameter, Celermajer index, and SAS score (all P < .01). There was a slow decline for LVGLS and RVGLS in TVM-N fetuses throughout the gestation. Conversely, these two parameters worsened rapidly in TVM-R/A group. Conclusions TVM fetuses present biventricular dysfunction by deformation analysis in the second and third trimester of pregnancy. TVM fetuses with reduced or absent pulmonary flow exhibit significantly greater impairment and more rapid deterioration of cardiac function, which may contribute to poor outcomes.

Background: 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. Methods: 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 pa-rameters were determined using two-dimensional speckle-tracking echocardiography. A novel multiparamet-ric 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 vali-dated prospectively by a validation cohort of 48 fetuses. Results: CoA was confirmed in 62 of 122 patients (50.8%) after birth. Fetuses with postnatal CoA were diag-nosed significantly earlier than those with false-positives (median, 24.5 weeks [interquartile range, 23.3-26.4 weeks] vs 27.8 weeks [interquartile range, 24.5-30.4 weeks]; 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 in-formation criterion = 81.77, C statistic = 0.945). The performance of this model was confirmed prospectively in the validation cohort. Conclusions: 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. (J Am Soc Echocardiogr 2022;35:1298-310.)

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 < 0.05), while the GSI was decreased ( P < 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 > 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 < 0.05). In contrast, the LV 24-segment EDD Z-scores and area Z-score were significantly lower in fetuses with CoA ( P < 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 < 0.05). CoA fetuses presented lower GLS, FAC and 24-segment FSs for both RV and LV when compared with normal controls (all P < 0.05). In addition, LVEF, LVEDV Z-score, LVSV Z-score and LVO Z-score were all decreased in CoA fetuses (all P < 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.

Right ventricular (RV) function has important prognostic value in a variety of cardiovascular diseases. Due to complex anatomy and mode of contractility, conventional two-dimensional echocardiography does not provide sufficient and accurate RV function assessment. Currently, three-dimensional echocardiography (3DE) allows for an excellent and reproducible assessment of RV function owing to overcoming these limitations of traditional echocardiography. This review focused on 3DE and discussed the following points: (i) acquisition of RV dataset for 3DE images, (ii) reliability, feasibility, and reproducibility of RV volumes and function measured by 3DE with different modalities, (iii) the clinical application of 3DE for RV function quantification.

Background This study aimed to investigate the serial changes in left ventricular (LV) myocardial deformation in patients with sepsis using three-dimensional (3D) and two-dimensional (2D) speckle tracking echocardiography (STE). Methods In this single-center, prospective, and observational study, we included 59 patients diagnosed with sepsis or septic shock in the intensive care unit and 40 healthy controls. Left ventricular ejection fraction (LVEF), left ventricular global longitudinal strain (GLS), and global circumferential strain (GCS) assessed by 3D STE and 2D STE were obtained on the first, third, fifth, seventh to the tenth day after sepsis or septic shock. Results In patients with sepsis or septic shock, 3D and 2D LVEF were not different at each time point. GLS and GCS obtained by 3D STE and 2D STE decreased on the first day compared with the healthy group (all P < 0.01). Compared with the values on the first day, GLS and GCS further decreased on the third day, while 3D and 2D LVEF did not differ. 3D and 2D STE strains were lowest on the third day and gradually improved on the seventh to the tenth day compared with values on the third day. When compared with values on the first day, 3D and 2D GLS gradually improved on the seventh to the tenth day, whereas 3D and 2D GCS on the seventh to the tenth day was not different. Although 3D and 2D STE strains were significantly increased on the seventh to the tenth day, they were not fully recovered to normality. Conclusion Although patients with sepsis or septic shock demonstrated gradual improvements in 3D and 2D STE parameters during the ten-day period, LV myocardial strain was not fully recovered to normality by the seventh to the tenth days. 3D and 2D strain imaging, used as a helpful tool for monitoring the evolution of myocardial deformation, can provide clinicians with a useful additional imaging parameter.

Background: A novel, fully automated right ventricular (RV) software for three-dimensional quantification of RV volumes and function was developed. The direct comparison of the software performance with cardiac magnetic resonance (CMR) was limited. Therefore, the aim of this study was to test the feasibility, accuracy, and reproducibility of a fully automated RV quantification software against CMR imaging as a reference.Methods: A total of 170 patients who underwent both CMR and three-dimensional echocardiography were enrolled. RV end-diastolic volume (RVEDV), RV end-systolic volume (RVESV), and RV ejection fraction (RVEF) were obtained using fully automated three-dimensional RV quantification software and compared with a CMR reference. For inter-technical agreement, Spearman correlation and Bland-Altman analysis were used.Results: The fully automated RV quantification software was feasible in 149 patients. RVEDV and RVESV were underestimated, and RVEF was overestimated compared with CMR values. RV measurements obtained from the manual editing method correlated better with CMR values than that without manual editing (RVEDV, 0.924 vs. 0.794: RVESV, 0.955 vs. 0.854; RVEF, 0.941 vs. 0.781 respectively, all p < 0.0001) with less bias and narrower limit of agreement (LOA). The bias and LOA for RV volumes and EF using the automated software without and with manual editing were greater in patients with severely impaired RV function or low frame rate than those with normal and mild impaired RV function, or high frame rate. The fully automated RV three-dimensional measurements were highly reproducible.Conclusion: The novel fully automated RV software shows good feasibility and reproducibility, and the measurements had a high correlation with CMR values. These findings support the routine application of the novel 3D automated RV software in clinical practice.

Background: Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart defects (CHDs). The patterns of fetal myocardial deformations in TOF have not been well-studied. This study aimed to assess biventricular myocardial deformations in fetuses with TOF compared with normal fetuses.Methods: A retrospective cohort study of fetuses with TOF and gestational age (GA)-matched controls was conducted at a single tertiary referral center from 2014 to 2020. All enrolled fetuses underwent detailed echocardiography, and four-chamber video-clips were recorded and analyzed offline for deformation assessment by using two-dimensional speckle tracking echocardiography (2D-STE). Comparisons for baseline characteristics, cardiac morphological measurements (ventricular, atrial, and great arterial diameters or ratios, global sphericity index), systolic function parameters [ejection fraction (EF), fractional area change (FAC)], and strain parameters [global longitudinal strain (GLS), global longitudinal strain rate in systole and diastole (GLSRs, GLSRd)] were performed between fetuses with TOF and GA-matched controls.Results: Fifty-two fetuses with TOF and 52 GA-matched controls were enrolled in this study. Fetuses with TOF exhibited similar left ventricular (LV) EF (58.51 ± 5.11% vs. 57.59 ± 5.38%, P = 0.16) and right ventricular (RV) FAC (43.64 ± 2.89% vs. 44.27 ± 3.04%, P = 0.25), compared to normal fetuses. While, in deformational analysis, TOF fetuses demonstrated significantly lower LV and RV GLS values (−22.57 ± 2.91% vs. −27.39 ± 4.38%, P < 0.001 for LV GLS; −24.27 ± 3.18% vs. −28.71 ± 4.48%, P < 0.001 for RV GLS). Both LV GLS (r = −0.518, P < 0.001) and RV GLS (r = −0.534, P < 0.001) were found negatively correlated with the aortic valve-to-pulmonary valve diameter ratio (AV:PV ratio). Z-scores of PV annulus and main pulmonary artery (MPA) also had positive correlation with LV and RV GLS, respectively.Conclusions: Decreased biventricular myocardial deformations can appear even in fetuses with TOF with normal systolic ventricular function. Both LV and RV GLS values are correlated with the severity of right ventricular outflow tract obstruction. It indicates 2D-STE may be a more sensitive tool to assess fetal cardiac function than the conventional echocardiographic methods.