Prenatal sonography of extracorporeal ductus venosus in association with large fetal gastroschisis

Liver herniation commonly associated with omphalocele occurs in only approximately 2.3% to 16% of fetuses with gastroschisis. Liver herniation in such cases is associated with considerably decreased survival rates (43% vs 97% with or without liver herniation, respectively). Rarely, abnormally positioned fetal hepatic vasculature has been reported mainly in association with congenital diaphragmatic hernia. In these rare cases, intrathoracic depiction of hepatic venous vasculature has assisted in confirming intrathoracic displacement of the fetal liver. We present a case of a large gastroschisis with complete herniation of the fetal liver in which prenatal sonography depicted an extracorporeal ductus venosus.     

Trends in fetal echocardiography and implications for clinical practice: 1985 to 2003.

OBJECTIVE: The purpose of this study was to determine whether patterns of referral for fetal echocardiography (FE) and the subsequent yield for structural congenital heart disease (CHD) have changed between 1985 and 2003. METHODS: All FE performed between 1985 and 2003 at Yale-New Haven Hospital was reviewed. The primary indication for study and the presence of structural CHD were recorded, and data were analyzed for trends. Linear regression with Pearson coefficient calculation and Mantel-Haenszel chi(2) analysis were performed (P < .05 significant). RESULTS: Between 1985 and 2003, 10,806 patients had FE at Yale-New Haven Hospital, and 774 cases of structural CHD were detected. The annual number of studies and rate of detected structural CHD remained constant through the study period. There was a significant increase in the proportion of studies for diabetes, maternal structural CHD, suspicious 4-chamber heart, and family history of cardiac disease. There was a significant decrease in the proportion of studies for a previous child with structural CHD, cardiac teratogen exposure, other fetal anomalies, aneuploidy, fetal arrhythmia, and nonimmune hydrops. The percentage of structural CHD detected by indication remained constant through the study period. Subgroup analysis of diabetes revealed an increase in class B diabetes, while classes C and D remained stable. CONCLUSIONS: This is one of the largest series of FE and suggests that the pattern of indications has changed since 1985. Specifically, referral for diabetes (mostly class B) has increased without a change in yield of structural CHD by indication for sonography. The changing referral patterns reflect a change in obstetric demographics and has implications for obstetric care.     

Detection rate of early fetal echocardiography and in utero development of congenital heart defects.

OBJECTIVE: The purpose of this study was to evaluate the detection rate of early fetal echocardiography and the in utero development of congenital heart defects (CHD). METHODS: Cases were selected from all singleton pregnancies between 1997 and 2003 in which detailed fetal 2-dimensional and color-coded Doppler echocardiography was performed in our prenatal unit between 11 weeks' and 13 weeks 6 days' gestation; 2165 cases with complete outcome parameters were analyzed. RESULTS: During this study period, CHD were diagnosed in 46 fetuses. Between 11 and 13 weeks' gestation, 29 CHD were diagnosed (11 weeks, 9 cases; 12 weeks, 8 cases; and 13 weeks, 12 cases); 9 CHD were found in the second trimester and 2 in the third trimester. The in utero detection rate of fetal echocardiography was 86.96% (n = 40). Six additional CHD (13.04%) were detected postnatally. The spectrum of detected CHD changed with advancing gestational age and was different from the postnatal detected heart defects. CONCLUSIONS: Early fetal echocardiography is feasible and allows the detection of most CHD. Congenital heart defects vary in appearance at different stages of pregnancy and may evolve in utero with advancing gestational age. Therefore, early fetal echocardiography should always be followed by echocardiography at mid gestation.     

Prenatal detection of congenital heart disease in southern Nevada: the need for universal fetal cardiac evaluation.

OBJECTIVE: Congenital cardiac malformations are common developmental anomalies. In the United States, congenital heart disease is the number one cause of infant mortality from congenital malformations. Prenatal diagnosis of congenital heart defects aids treatment coordination. Our aim was to evaluate prenatal detection of serious congenital heart malformations in Clark County, Nevada. METHODS: We electronically searched our research department-maintained computer database for patients with serious congenital heart disease born in Clark County between May 2003 and April 2006. We excluded patients that did not have at least 1 local prenatal ultrasound examination. All pre-natal ultrasound studies were performed in obstetric offices, radiology imaging centers, or maternal-fetal medicine specialty practices. Fetal echocardiography was performed in maternal-fetal medicine specialists' offices under the supervision of a fetal cardiologist. Pediatric cardiologists performed all postnatal echocardiographic examinations. RESULTS: During the study period, we diagnosed serious congenital heart malformations in 161 patients among a total estimated 77,000 births (2/1000). Of the 161 patients, 58 (36%) had a prenatal diagnosis, and 103 (64%) had an exclusively postnatal diagnosis. CONCLUSIONS: Standard prenatal ultrasound fails to show congenital heart disease in most fetuses.     

Early fetal echocardiography: heart biometry and visualization of cardiac structures between 10 and 15 weeks' gestation.

OBJECTIVE: The purpose of this prospective cross-sectional study was to compile normative data about biometry of the fetal heart and great vessels between 10 and 15 weeks in 123 normal singleton pregnancies. Additionally, we investigated the different methods and the optimal examination time of early fetal echocardiography. METHODS: The interrogated parameters included total heart diameter; heart area and circumference; right and left ventricular diameter; diameter, circumference, and area of the thorax; and diameter of the aorta and pulmonary trunk. Visualization of the 4-chamber view, 3-vessel view, origin and crossover of the great arteries, aortic arch, ductus arteriosus, superior and inferior venae cavae, and pulmonary veins was analyzed, and the success rates by transvaginal sonography (TVS) and transabdominal sonography (TAS) were calculated. RESULTS: Complete evaluation of the fetal heart was impossible at 10 weeks; the total success rate increased from 45% at 11 weeks to 90% between 12 and 14 weeks and 100% at 15 weeks. Between 10 and 13 weeks, TVS was superior to TAS. At 14 weeks, both methods were similar to each other, and at 15 weeks, TAS allowed adequate visualization of all structures. Linear regression analysis showed a significant correlation between the interrogated parameters and gestational age, crown-rump length, and biparietal diameter (P < .05). The ratio of right and left ventricular diameters and the ratio of pulmonary trunk and aortic diameters were constant. CONCLUSIONS: Early fetal heart evaluation by TVS or TAS or both is reasonable and feasible. Our normative data could be helpful for understanding the normal development of the fetal heart and great arteries and for detection of cardiac defects in early pregnancy.     

声触诊组织量化技术评估经颈静脉肝内门体分流术联合胃冠状静脉栓塞术疗效

目的 探讨应用声触诊组织量化(VTQ)技术测量脾脏剪切波速度(SSWV)无创评价经颈静脉肝内门体分流术(TIPS)联合胃冠状静脉栓塞术(GCVE)疗效的价值。方法 将19例接受TIPS联合GCVE治疗的肝硬化门静脉高压患者分为A(13例,接受1次治疗)、B(6例,首次治疗后因支架闭塞再次接受治疗)两组。应用VTQ技术分别与治疗前1天、治疗后6天测量SSWV,以脉冲波多普勒测量门静脉主干血流速度(PVV)、脾静脉主干血流速度(SVV)。于TIPS联合GCVE治疗过程中,以直接测压法测量支架植入前及植入后的门静脉压(PPG),并进行统计分析。结果 TIPS联合GCVE治疗后,A、B两组PPG均较治疗前明显减低(P均<0.05);SSWV均较治疗前明显减低,PVV、SVV均较治疗前明显升高(P均<0.05)。A、B两组治疗前及治疗后SSWV均与PPG呈正相关(P均<0.05),PVV、SVV均与PPG无相关性(P均>0.05)。结论 应用VTQ技术所测SSWV与PPG存在相关性,可作为TIPS联合GCVE治疗前后监测门静脉压力的无创定量评估指标。     

Prenatal diagnosis of absent pulmonary valve syndrome in association with 22q11 deletion.

OBJECTIVE: To describe the prenatal sonographic appearances in cases of absent pulmonary valve syndrome and the importance of investigating the presence of 22q11 deletion. METHODS: We describe 2 cases, which were referred because of a suspicion of a cardiac malformation. In both cases, a large anechoic mass emerging from the right ventricle was visualized and identified as an aneurysmal dilatation of the pulmonary trunk with hypertrophy of the right ventricle. The diagnosis of tetralogy of Fallot with absent pulmonary valve syndrome and a secondary diverticular dilatation of the pulmonary artery was made. A review of the literature revealed another 18 cases of prenatal diagnosis of absent pulmonary valve syndrome with or without knowledge of chromosomal abnormalities. RESULTS: Pathologic examinations confirmed the diagnosis of absent pulmonary valve syndrome in both cases. Final results of fetal karyotyping revealed a 22q11 deletion in the first case. CONCLUSIONS: An abnormal 4-chamber view with an aneurysmal dilatation of the pulmonary trunk should suggest the diagnosis of this rare congenital anomaly. Perinatal death occurs in more than 60% of cases and is usually associated with hydrops fetalis, the presence of other malformations, or both. Even in the absence of extracardiac malformations, investigation for 22q11 deletion in cases of conotruncal cardiac abnormalities is recommended.     

The role of the sagittal view of the ductal arch in identification of fetuses with conotruncal anomalies using 4-dimensional ultrasonography.

OBJECTIVE: Conotruncal anomalies represent one fifth of all congenital heart defects (CHDs) detected in the fetus. However, the spatial relationship of the great vessels is incorrectly defined in about 20% of these cases. The sagittal view of the ductal arch is considered a standard ultrasonographic view in fetal echocardiography and can be easily visualized using 4-dimensional (4D) ultrasonography. This study was designed to determine the role of this ultrasonographic plane for the prenatal diagnosis of conotruncal anomalies. METHODS: We reviewed 4D volume data sets, acquired with the spatiotemporal image correlation technique, from fetuses with and without confirmed conotruncal anomalies. The visualization rate of the sagittal view of the ductal arch was compared among groups using standardized multiplanar views. RESULTS: This study included 183 volume data sets from fetuses in the following groups: (1) normal echocardiographic findings (n = 130); (2) conotruncal anomalies (n = 18); and (3) other CHDs (n = 35). Volumes of poor image quality were excluded from analysis (8.2% [15/183]). The visualization rate of the sagittal view of the ductal arch was significantly lower in fetuses with conotruncal anomalies (5.6% [1/18]) than that in fetuses without abnormalities (93.1% [108/116]) and that in fetuses with other CHDs (79.4% [27/34]; P < .01). Absence of visualization of the sagittal view of the ductal arch was associated with a likelihood ratio of 9.44 (95% confidence interval, 5.8-15.5) to have conotruncal anomalies. CONCLUSIONS: The sagittal view of the ductal arch may play an important role in the screening and prenatal diagnosis of conotruncal anomalies in 4D ultrasonography.     

Validation of volume and mass assessments for human fetal heart imaging by 4-dimensional spatiotemporal image correlation echocardiography: in vitro balloon model experiments.

OBJECTIVE: This study was designed to validate a slow-sweep real-time 4-dimensional (4D) spatiotemporal image correlation method for producing quantitatively accurate dynamic fetal heart images using an in vitro pulsatile balloon model and apparatus. METHODS: To model fetal heart chambers, asymmetric double-walled finger stalls (tips of surgical latex gloves) were used and attached to a laboratory-designed circuit that allowed calibrated changes in the inner balloon volume as well as an intermediate gel mass interposed between the 2 layers. The water-submerged model was attached to a small-volume pulsatile pump to produce phasic changes in volume within the inner balloon at a fixed rate. A sonography system with 4D spatiotemporal image correlation (STIC) capabilities was used for 3-dimensional (3D) and 4D data acquisition. Volume data were analyzed by customized radial summation techniques with 4D data analysis software and compared with known volumes and masses. RESULTS: Fifty-six individual volumes ranging from 2.5 to 10 mL were analyzed. Volume and mass measurements with 4D STIC were highly correlated (R2 > 0.90). The mean percentage error was better (<6%) for volumes exceeding 4 mL and was as low as 0.3% for 6-mL estimations. Measurements in the diastolic phase were the most accurate, followed by mass estimations equivalent to chamber walls. There was a wider range of percentage error in the lowest volumes tested (2.5 mL), which might have arisen from difficulties in spatial resolution or distortions from within the model apparatus itself. Resolution limitations of 4D technology in combination with extremely small volume targets may explain higher error rates at these small volumes. CONCLUSIONS: Four-dimensional STIC is an acceptably accurate method for volume and mass estimations in the ranges comparable with mid- and late-gestation fetal hearts. It is particularly accurate for diastolic estimations, for chamber wall mass measurements, and at volumes of greater than 2.5 mL. This study validates use of 4D STIC technology to overcome the limitations of nongated 3D technology for phasic and quantitative assessments in fetal echocardiography.     

Applications of 2-dimensional matrix array for 3- and 4-dimensional examination of the fetus: a pictorial essay.

OBJECTIVES: Two-dimensional (2D) matrix array is a new technology for the performance of 3-dimensional and 4-dimensional (4D) ultrasonography. In this study, we report the use of a 2D matrix array transducer for examination of fetal structures including the fetal heart. METHODS: Thirty-four fetuses without abnormalities and 19 fetuses with congenital anomalies were examined with a 2D matrix array transducer (x3-1, IE-33; Philips Medical Systems, Bothell, WA). Median gestational age was 25 6/7 weeks (range, 13 0/7-40 1/7 weeks). RESULTS: (1) A 360 degrees rotation and examination of selected structures was possible in the second trimester. (2) Structures were examined by maintaining the transducer in a fixed position and rotating the volume using the system trackball. (3) Dorsal and ventral parts of the hands and feet were visualized in a single volume data set, in real time, without moving the transducer. (4) Real-time en face visualization of atrioventricular valves was possible from the ventricular or atrial chambers. (5) Four-dimensional images of bones were obtained by decreasing gain settings only, with no need for cropping. (6) Four-dimensional reconstruction of vascular structures was possible with color Doppler imaging. Two limitations were identified: (1) lower resolution than mechanical volumetric transducers, and (2) narrow volume display. CONCLUSIONS: Real-time direct 4D imaging with 360 degrees rotation for examination of fetal anatomic structures is feasible. This technology allows examination of fetal structures from multiple perspectives, in real time, without the need to move the transducer in the maternal abdomen. Further technological developments may overcome the limitations identified in this study.