Objective:Interruption of aortic arch(IAA)is a rare congenital heart disease.This study aims to investigate echocardiographic features and pathological ultrastructural characteristics of fetal IAA and to further analyze its pathological evolution. Methods:A retrospective analysis was conducted on prenatal echocardiographic,post-surgical,or autopsy findings of fetuses prenatally diagnosed with IAA.Prenatal echocardiographic tracking was used to observe the internal diameters and Z-scores of different segments of the aortic arch and the changes in the narrowed section.These observations were combined with autopsy and pathological findings to explore the potential intrauterine evolution of IAA and its cytological basis. Results:The study included 34 fetuses with IAA,with 3,3,and 28 fetuses prenatally diagnosed with aortic arch dysplasia(AAD),coarctation of aorta(CoA),and IAA,respectively.The 3 AAD and 3 CoA fetuses chose termination of pregnancy 1 to 2 weeks after prenatal ultrasound diagnosis,and autopsy confirmed IAA.Among the 28 fetuses prenatally diagnosed with IAA,6 cases of CoA progressively worsened,eventually evolving into type A IAA as observed through echocardiographic follow-up.The remaining 22 cases were diagnosed as IAA on the first prenatal ultrasound.Postnatal surgery corrected 3 cases,while 27 cases opted for pregnancy termination,and 4 cases resulted in intrauterine death.Echocardiographic features of the fetal IAA included a significantly smaller left ventricle compared with the right or negligible difference on the four-chamber view,a significantly smaller aorta than the pulmonary artery on the three-vessel view,and a lack of connection between the aorta and the descending aorta on the three-vessel-trachea and aortic arch views.The aortic arch appears less curved and more rigid,losing the normal"V"shape between the aorta,ductus arteriosus,and descending aorta.Color Doppler ultrasound showed no continuous blood flow signal at the interruption site,with reversed blood flow visible in the ductus arteriosus.Transmission electron microscopy of 7 IAA fetuses revealed numerous disorganized smooth muscle cells between the elastic membranes near the aortic arch interruption site,significantly increased in number compared with the proximal ascending aorta.The elastic membranes were thicker and more twisted near the interruption site.The interruption area lacked normal endothelial cells and lumen,with only remnants of necrotic endothelial cells,disorganized short and thick elastic membranes,and randomly arranged smooth muscle cells. Conclusion:Prenatal echocardiography is the primary diagnostic tool for fetal IAA.Post-surgical follow-up and autopsy help identify complications and disease characteristics,enhancing diagnostic accuracy.Some fetal IAA may evolve from AAD or CoA,with potential pathogenesis related to ischemia,hypoxia,and migration of ductal constrictive components.

Objective:To investigate the ultrasonographic features and prognosis of fetal vascular anomalies.Methods:Thirty-one fetuses with vascular anomalies diagnosed from June 2013 to August 2018 in Changsha Hospital for Maternal and Child Health Care were retrospectively analyzed. The location, size, shape, internal echo and blood flow distribution of the lesions were observed by ultrasound carefully, and the prognosis was followed up and analyzed.Results:Among the 31 cases of fetal vascular anomalies, 10 cases were comfirmed by autopsy after induced labor, and 21 cases were confirmed by postpartum local observation or surgery and pathology. Among them, there were 4 cases of Klippel-Trenaunay syndrome (including 1 case of Parkes-Weber syndrome), 3 cases of hemangioma, 1 case of primary congenital lymphedema and 23 cases of lymphangioma. Thirty-one cases showed lesions coincident with ultrasound localization and diagnosis, including 9 cases of head and neck lesions, 15 cases of trunk lesions, 1 case of upper limb lesion and 6 cases of lower limb lesions. The blood flow spectra of arteriovenous fistulas were found in 3 cases of fetal lesions and no obvious blood flow signals were found in 28 cases of fetal lesions.Conclusions:Prenatal ultrasound examination and follow-up play an important role in the diagnosis and prognostic prediction of fetal vascular diseases. The prognosis of fetal vascular diseases is closely related to the location, size, effect on the surrounding tissue and shunt volume of the lesion.

Objective:To develop Z-scores for the aortic arch in normal fetuses as a reference for fetuses with suspected coarctation of aorta(CoA).Methods:The aortic arch inner diameters of 610 normal fetuses and 59 CoA fetuses from May 2010 to March 2015 in the Second Xiangya Hospital of Central South University were measured at the long axis of the aortic arch view. Gestational age(GA), femur length(FL) as the independent variable, the aortic arch inner diameters as the dependent variable, Z-scores were created relating the aortic arch inner diameters to the GA and FL. Z-score=[ln(measured diameter)-ln(predicted diameter)]/root MSE. Z-scores of the CoA fetuses were calculated with the above regression, and compared with the Z-scores of the control group.Results:A simple linear regression model was the best description of the data in each case and correlations between FL and the aortic arch inner diameters were excellent ( P<0.001). There was no significant difference in Z-scores calculated with FL or GA as independent variables ( P>0.05). Z-scores of the control group was between -2 and + 2, Z-scores of the CoA group was significantly lower and below -2( P<0.001). Conclusions:Z-scores of fetal aortic arch are sensitive indicators of fetal coarctation, and are of clinical importance for the diagnosis and follow-up study of CoA.

Objective To investigate the clinical value of prenatal diagnosis of fetal double outlet ventricle . Methods T he data of double outlet ventricle from fetal echocardiography in Second Xiangya Hospital of Central South University and Changde Women and Children Health Hospital of Hunan Province from January 2000 to August 2018 were collected . T he statistical method was used to analyze characteristics of echocardiography ,related‐intracardiac and extracardiac abnormalities ,postnatal echocardiography ,surgery and autopsy findings . Results Ninety‐four fetuses were diagnosed with double outlet ventricle ,including 84 cases of double outlet right ventricle ( DORV ) and 10 cases of double outlet left ventricle ( DOLV ) . T he pregnancy was terminated in 45 cases . Autopsy was offered to all patients after termination of pregnancy ,42 cases were consistent with prenatal diagnosis ,1 case was tetralogy of fallot ,2 cases were transposition of great artery . Forty‐nine cases were decided to continue the pregnancy ,32 cases of them were confirmed by postpartum surgery ,17 cases were confirmed by postnatal echocardiography . Echocardiographic findings of fetal double outlet ventricle was characterized by the origin of the both great arteries arising predominantly or completely( ＞50% ) from the same ventricle . Conclusions Prenatal ultrasound diagnosis of double outlet ventricular has important clinical value ,facilitate appropriate prenatal counseling and postnatal management and it should be differentiated with transposition of the great arteries ,tetralogy of fallot and ventricular septal defect .

Objective To construct Z-score models for normal fetal heart size measurements derived from fetal echocardiography.Methods Fetal echocardiography were performed in 910 normal singleton fetuses from 14th to 40th gestational weeks.Fetal transverse heart diameter (HD),heart length (HL),heart circumference (HC) and heart area (HA) were derived from a standard four-chamber view during end diastole.Using fetal somatic sizes as independent variables and heart sizes as dependent variables,the regression analyses of the mean (M) and the standard deviation (SD) for each parameter were calculated separately.A group of fetal heart diseases were assessed using these models.Results Strong correlations were found between fetal heart sizes and somatic sizes.Linear-cubic regression equations were each fitted to the models of the means of the heart sizes,whereas linear-quadratic equations were fitted to the models of the SDs.HD (r =0.984-0.986) was a dependent variable that provided the highest correlation coefficient with all of the fetal sizes,followed by HL (r =0.981-0.984),HC (r =0.981-0.982) and HA (r =0.978-0.979).All fetuses with Ebstein' s anomaly and most with homozygous α thalassemia-1 demonstrated Z scores reflective of increased heart sizes.Conclusions The fetal heart sizes Z-scores models had been constructed.The calculation of Z-scores for heart sizes as a function of fetal somatic size is feasible and simple.They might be useful for quantitative assessment of some cardiac diseases and used as new predictive indicators for homozygous α-thalassemia-1 particularly.

OBJECTIVE: To evaluate the echocardiographic diagnosis for ventricular non-compaction cardiomyopathy (NCCM) in foetus and to analyze the pathologic features of NCCM.
 METHODS: A total of 9 patients with fetal NCCM were examined by prenatal echocardiography from 2004 to 2013, which was compared with postnatal echocardiography or autopsy to analyze the fetal characteristic of myocardial ultrastructure.
 RESULTS: The results of echocardiography displayed an excessive muscle trabecular meshwork and muscle trabecular crypt, and the ventricular myocardium and non-compaction/compaction ratio was ≥2.0. Among the 9 fetuses of NCCM, 6 fetuses were involved in left ventricle, 2 in both left and right ventricles and 1 in right ventricle. Two fetuses were confirmed by postnatal echocardiography, the remaining 7 patients were chosen to terminate their pregnancies, which were confirmed by autopsy later. Muscle biopsies revealed the abnormal myocardial mitochondria, sarcomeres and myocardial fibrosis.
 CONCLUSION It is feasible to accurately diagnose NCCM by prenatal echocardiography. Fetal NCCM most often involves the left ventricle, but it can involve the right ventricle or both, too. The myocardial ultrastructure of fetal NCCM possesses certain unique characteristics, such as the low maturation of the mitochondria, sarcomeres and myocardial fibers.
Cardiomyopathies ; diagnosis ; Echocardiography ; Fetus ; Heart Ventricles ; pathology ; Humans ; Myocardium ; pathology

Objectives To construct reference range of fetal optic tract mean diameter and to report the measured optic tract mean diameter in fetuses with agenesis of the septum pellucidum (SP).Methods Three-dimensional volumes of the optic chiasm were acquired in 254 normal fetuses during routine sonographic examination at 21 ～ 40 weeks' gestation and the diameters of posterior left and right optic tracts were measured offline.A polynomial regression approach was used to calculate reference charts for the fetal optic tract mean diameter.In addition,16 three-dimensional volumes were acquired in fetuses with SP agenesis for offline measurement of optic tract diameter.The complete follow-up data were obtained in 7 of these 16 cases.Results In normal fetuses,the optic tract diameter increased linearly throughout gestation.Normal charts and equations were constructed.Among 7 fetuses with SP agenesis and complete follow-up,one had postnatal normal vision,two had hypoplastic optic tract,and four underwent termination of pregnancy.Three cases of normal childbirth were tested after the baby was born as a bundle of apparent dysplasia,including 1 case of neonatal characterized by lack of vision at the one year old and 2 cases of impaired vision.Four cases of termination of pregnancy included 1 case with tracking pathological confirmation of optic nerve hypoplasia,and the other 3 cases without tracking the pathological results.Conclusions We present new reference charts for fetal optic tract mean diameter.In fetuses with agenesis of the SP,sonography of the optic tract might be a useful tool to assess its development and may help prenatal counseling.

Objective To investigate the differences in myocardial deformation between donor and recipient in twin-twin transfusion syndrome(TTTS) using vector velocity imaging( VVI). Methods Digital dynamic two-dimensional four chamber views were interrogated off-line using VVI software. Global longitudinal strain(S) , systolic strain rate(SRs) and diastolic strain rate(SRd) were measured in the left ventricles(LV) and right ventricles (RV) of 30 fetal pairs with TTTS and compared to 30 gestational age-matched normal controls. Results Donor LV-SRs was higher, while donor RV-SRd was significantly lower than controls. Recipient S, SRs and SRd were significantly lower for both LV and RV in comparison to controls. Conclusions In TTTS,donor LV systolic function is hyperdynamic while the RV diastolic function is low. Recipient RV and LV are both globally depressed with systolic and diastolic dysfunction.

OBJECTIVE: To investigate the value of 4-dimensional reconstruction by inversion mode of spatio-temporal image correlation (STIC) in prenatal diagnosis of congenital heart diseases. METHODS: Cardiac volume images of 30 normal fetuses and 22 fetuses with congenital heart diseases were obtained by STIC gray-scale 2-dimensional imaging technique. Under the guidance of cross-sectional display, the images were collected by inversion mode. RESULTS: A total of 53 and 38 qualified volume images were obtained in the 30 normal fetuses and 22 fetuses with congenital heart diseases respectively. All volume images were inverted successfully and showed the spacial position of the heart and blood vessel directly. CONCLUSION Four-dimensional reconstruction by inversion mode of STIC is feasible. This new method can provide more morphologic information, and may be used for prenatal diagnosis of congenital heart diseases.
Adult ; Echocardiography, Four-Dimensional ; methods ; Female ; Heart Defects, Congenital ; diagnostic imaging ; Humans ; Image Processing, Computer-Assisted ; Pregnancy ; Ultrasonography, Prenatal ; methods

Objective To determine the correlation of cardiovascular MRI with cardiac biomarkers and electrocardiography(ECG)in acute myocardial infarction(MI).Methods Nineteen patients with first acute MI were selected to undergo MRI on a 1.5 T system within 3-7 days after the onset of symptoms.A first-pass perfusion scan was performed with the administration of Gd-DTPA at a speed after cine MRI for global left ventricle(LV)funotions.Delay-enhanced MRI was performed by using an ECG-gated inversionrecovery fast-gradient echo-pulse sequence 5 to 10 minutes later with second bolus injection at a s peed.Infarct mass(IM),percentage size of infarction(PSI)and LV functions were compared with peak troponin T (peak TnT)and peak ereatine kinase-MB fraction (peak CK-MB).The 12-lead ECG was analysed for STelevation on admission.Pearson and Spearman correlation test and independent-Sample t test wel-e used for statistics.Results The IM (median 6.3 g) was correlated with peak TnT(median 0.8μg/L,r=0.487,P=0.0340)and left ventricle end-systolic volume index(LVESVI)(median 23.4 ml/m2,r=0.480,P=0.038).IM showed a negative correlation with left ventricle ejection fraction(LVEF)(54.1±15.4)/(r=-0.563.P:0.012).The PSI(median 6.0/)was correlated with peak TnT(r=0.583,P=0.009),peak CK.Mn(median 43.0 U/L,r=0.470,P=0.042)and LVSV[(57.6 ±15.0)ml,r=-0.482,P=0.036],peak TnT was also correlated with LVSV(r=-0.524,P=0.021).There were more involved segments(IS)(t=2.972,P=0.009),higher peak TnT(t=2.245,P=0.041)and peak CK-MB(t=2.508,P=0.024)in ST-elevation MI(STEMI)than in non ST-elevation MI(NSTEMI).Conclusions IM directly influences LV functions in acute MI.Peak TnT was a better biomarker reflecting PSI and LV functions.There were more involved segments in STEMI than in NSTEMI.