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Objectives: Generalized arterial calcification of infancy (GACI) is a rare autosomal recessive disorder characterized by subintimal fibrous proliferation and deposition of calcium salts in the internal elastic lamina, leading to extensive arterial calcification and stenosis of large and medium‐sized arteries. Prenatal diagnosis is usually made in the third trimester by detection of aortic and pulmonary calcification with associated nonimmune hydrops; earlier prenatal diagnosis is rare. This study was performed to examine the prenatal ultrasound and genetic features of fetuses with GACI. Methods: We retrospectively reviewed the ultrasound findings, their progression in utero, and the clinical features in three fetuses with GACI ascertained using ultrasound in the second trimester. GACI was subsequently confirmed through pathological examination and/or molecular genetic testing. Results: All three fetuses had hyperechogenic valves or annuli as the first detectable manifestation in the second trimester, followed by relatively rapid progression to arterial wall calcification. Three novel mutations of the ENPP1 gene associated with GACI were found in two of the cases (c.26dupG, c.1454A > G, and c.263C > G). Conclusions: GACI should be suspected when hyperechogenic cardiac valves, annuli, or arterial walls are noted after ruling out other causes of arterial calcification. Genetic testing is important for prenatal and future preimplantation genetic diagnosis. Key points: What's already known about this topic? Historically, generalized arterial calcification of infancy (GACI) has often been diagnosed retrospectively. Prenatal diagnosis is usually made in the third trimester by the detection of calcifications in the aortic, pulmonary, and other large arterial walls, with associated nonimmune hydrops in some cases. What does this study add? We present three cases of GACI diagnosed prenatally using ultrasound in the second trimester, in which hyperechogenic valves and/or annuli were the early manifestation. We also report three novel mutations of the ENPP1 gene associated with GACI. [ABSTRACT FROM AUTHOR]

Methods: The study included 59 patients with normal fetal heart structure, blood flow, and heart rhythm (fetal abnormality‐negative group) and 50 patients with abnormal fetal heart structure, blood flow, and/or heart rhythm (fetal abnormality‐positive group). Automated Cardiac Motion Quantification (aCMQ) was performed in both groups to obtain left and right ventricular endocardial global longitudinal strain (GLSendo), mid‐myocardial global longitudinal strain (GLSmid), and epicardial global longitudinal strain (GLSepi). Parameters between the two groups were compared and correlation analyses performed. A deformation analysis was performed by two trained observers, and reproducibility was assessed. Results: The fetal left ventricular and right ventricular global longitudinal strain (LV‐GLS and RV‐GLS, respectively) decreased in a gradient from the endocardium to the epicardium. LV‐GLS and RV‐GLS of all myocardial layers were lower in the fetal abnormality‐positive than ‐negative group (all P < 0.05). Correlation analysis showed that neither LV‐GLS nor RV‐GLS was significantly correlated with gestational age in the fetal abnormality‐negative group (all P > 0.05), whereas left ventricular GLSendo, GLSmid, and GLSepi were negatively correlated with gestational age in the fetal abnormality‐positive group (r = −.39 to −.44, all P < 0.05). Repeatability testing showed that the inter‐observer and intra‐observer intra‐class correlation coefficients for LV‐GLS and RV‐GLS in each myocardial layer were >.75 (all P < 0.001). Conclusions: As a new speckle tracking echocardiography tool, aCMQ has feasibility and repeatability in evaluating myocardial deformation of the fetal ventricle. This technique might provide helpful information on ventricular myocardial deformation in fetal hearts with abnormal structure or rhythm for clinical guidance in pregnancy. [ABSTRACT FROM AUTHOR]

Background: Placenta previa and accreta are serious obstetric conditions that are associated with a high risk of intraoperative massive hemorrhage.Purpose: To develop a scoring system for intraoperative massive hemorrhage combining MRI and clinical characteristics to predict the risk of massive hemorrhage in placenta previa and accreta STUDY TYPE: Retrospective cohort study.Subjects: In all, 374 patients consisting of 259 patients with placenta previa and accreta after previous cesarean section (CS) for the derivation cohort and 115 patients for the validation cohort.Field Strength/sequence: 1.5T single-shot fast spin-echo sequence. [Correction added on October 23, 2019, after first online publication: The field strength in the preceding sentence was corrected.] ASSESSMENT: Using the derivation cohort, clinical and MRI data were collected and multivariable logistic regressions analysis was conducted to develop a scoring system for prediction of intraoperative massive bleeding (blood loss volume > 2000 mL). Finally, the scoring system was validated on 115 patients.Statistical Tests: Student's t-test, Mann-Whitney U-test, X 2 statistics, multivariable analysis, and receiver operating characteristic (ROC) analysis.Results: Ten indicators, including clinically maternal age (1 point), preoperative hemoglobin level (1 point), gravidity number (1 point), number of CS (1 point), and MRI T2 dark intraplacental bands (4 points), cervical canal length (3 points), placenta thickness on the uterine scar area (4 points), empty vascular shadow of the uterus (1 point), low signal discontinuity in the muscular layer of the posterior wall of the bladder (6 points) and attachment position of the placenta (1 point) were imputed. From the ROC analysis, a total score of 7 points was identified as the optimal cutoff value, allowing good differentiation of intraoperative massive bleeding in the derivation cohort (AUC, 0.863; 95% confidence interval [CI]: 0.811-0.916) and in the validation cohort (AUC, 0.933; 95% CI: 0.885-0.980).Data Conclusion: The scoring system for intraoperative massive hemorrhage consists of MRI and clinical indicators, and using a cutoff value of 7 points for a high risk of massive bleeding, the developed scoring system could accurately assess the risk of intraoperative massive hemorrhage in patients with placenta previa and accreta. This scoring system can potentially reduce the incidence of intraoperative massive bleeding by identifying patients at high risk.Level Of Evidence: 3 Technical Efficacy: Stage 5 J. Magn. Reson. Imaging 2020;51:947-958. [ABSTRACT FROM AUTHOR]