产前超声诊断单脐动脉与胎儿异常的相关性研究

目的探讨超声检测胎儿脐带血管数目预测胎儿先天异常的价值。方法2000年1月至2006年10月在中山大学附属第一医院采用超声检测胎儿脐血管数目，对单脐动脉胎儿行产前超声系统筛查及胎儿染色体检查；分析单脐动脉合并畸形类型，与染色体异常的关系及胎儿结局。结果产前超声诊断胎儿单脐动脉119例，包括左侧72例（60．5％），右侧47例（39．5％）。单纯性单脐动脉59例（49.6％）；合并其他结构畸形60例（50．4％），其中泌尿系统畸形25例（41．7％），心血管系统畸形17例（28.3％），中枢神经系统畸形15例（25.0％），下肢畸形9例（15．0％），前腹壁和消化道畸形各5例（各占8．3％），唇裂或唇腭裂畸形3例（5．0％），其他畸形3例（5．0％）。行胎儿染色体检查41例，发现染色体异常8例，包括18-三体综合征3例、13-三体综合征1例、21．三体综合征1例、染色体片段异常3例，均合并其他畸形。结论胎儿单脐动脉左侧发生率高于右侧，约50％合并其他畸形；合并畸形时，染色体异常发生率较高；大部分单纯性单脐动脉胎儿结局良好。     

Single umbilical artery--consequences of prenatal diagnosis]

The authors report about the prenatal diagnosis of 34 cases of fetuses with a single umbilical artery (SUA) observed at the Perinatal Center of Charité. Between January 1989 and June 1991 the SUA has been associated with some adverse perinatal events, such as low birth weight (35%), congenital malformations (35%), perinatal mortality (11%) and placental alterations (76%). The incidence was not higher in girls than in boys. We did not find any chromosomal anomalies in our cases. An accurate ultrasonographic examination of the SUA in the 16th to 20th week is very important. The prognosis of the newborn could be improved by efficient diagnosis and optimum management of pregnancy and of delivery at a perinatal center.     

Single umbilical artery and its siding in the second trimester of pregnancy: relation to chromosomal defects

OBJECTIVES: To determine the possible association between single umbilical artery (SUA) in the second trimester of pregnancy and the incidence of chromosomal abnormalities. To determine whether the presence of chromosomal defects in fetuses with SUA is related to the side of the missing artery. METHODS: Color flow imaging of the fetal pelvis was used to determine the number of umbilical arteries in 2147 fetuses immediately before amniocentesis for karyotyping in the second trimester of pregnancy. RESULTS: SUA was diagnosed in 102/2147 (4.8%) cases. The left umbilical artery was absent in 60/102 (58.8%) fetuses, compared with the 42/102 (41.2%) for the right artery. The rate of chromosome abnormalities was significantly higher among fetuses with SUA than among those with 2 umbilical arteries (19/102 or 18.6% versus 109/2045 or 5.3%; OR = 4.1, 95% CI 2.3-7.1, p < 0.0001). Among fetuses with SUA, there was no significant difference in the rate of chromosome abnormalities between those with absence of the left versus the right artery (11/60 or 18.3% versus 8/42 or 19.0%, p = 0.93). There was an SUA in 5/39 (12.8%) cases with trisomy 21, 8/16 (50%) with trisomy 18, 1/4 (25%) with trisomy 13 and 5/69 (7.2%) with other chromosomal defects. There were no chromosome abnormalities in fetuses where a single umbilical artery was an isolated sonographic finding. All fetuses with SUA and chromosomal defects had associated abnormalities detected by ultrasound. CONCLUSION: A single umbilical artery (SUA) in the second trimester of pregnancy has a high association with trisomy 18, 13, 21 and other chromosomal defects, but all chromosomally abnormal fetuses had associated malformations detected by ultrasound. The absence of the left artery is more frequent than the absence of the right artery. The association with chromosomal abnormalities seems to be equal on each side.     

Single umbilical artery: review of 12 cases

The umbilical cord normally consists of three vessels, two arteries and one vein. The absence of one umbilical artery or single umbilical artery (SUA) has been associated with some adverse perinatal events, such as low birth weight, prematurity, congenital malformations and perinatal mortality. The authors present 12 cases of SUA confirmed by histologic examination and the principal maternal and perinatal features of these cases are analyzed. In our study there were a prematurity rate of 58%, a low birth weight rate of 66% and a perinatal mortality rate of 580/1000. Among our patients, 42% of the infants with SUA had major congenital malformations. An accurate examination of the umbilical cord at delivery is suggested, mainly because SUA is an important index to detect associated congenital malformations.     

The impact of multiple pregnancies and malformations on perinatal mortality

AIM: To evaluate the impact of the rate of multiple pregnancies and congenital malformations on perinatal mortality. METHODS: The study is based on data from the perinatal audit in Vejle County Denmark. Fetal deaths with gestational age > or = 22 weeks and deaths in livebirths within the first 28 days after birth were included in the calculated perinatal mortality. Total number of births was 30,181 and 252 pregnancies and 268 fetuses/infants were evaluated. The study period was 1995-2000. There was no routine ultrasound screening for congenital malformations in the county, though midtrimester ultrasound was used to assess gestational age. RESULTS: Perinatal mortality was 8.9 per 1000 births with no significant change over time. Rate of multiple pregnancies was 1.94% ranging from 1.81% during the first 3 years to 2.06% for the last 3 years (not significant). Fetuses and infants from multiple pregnancies contributed 18% of all deaths. Perinatal mortality for single births was 7.6 per 1000 births and for multiple births 42.2/1000 (P<0.0001). The distribution of gestational age for single and multiple births was highly significant (P<0.0001) with 67% of multiple pregnancies with GA < 28 weeks compared to 26% of single pregnancies. Nineteen percent of all deaths were caused by congenital malformations and the majority of these were potentially detectable by ultrasound investigation. CONCLUSIONS: The increasing rate of multiple pregnancies makes it difficult to see improvements in perinatal mortality. Calculated from the perinatal mortality in single and multiple pregnancies in Vejle County assisted conceptions contribute with an an excess of 45 perinatal deaths per year in Denmark. The difference between countries in rate of multiple pregnancies and in prenatal ultrasound screening recommendations for malformations makes it difficult to compare perinatal mortality.     

Antenatal sonographic detection of single umbilical artery.

The absence of one umbilical artery of single umbilical artery (SUA) is one of the most common congenital malformations in man. This vascular anomaly of the umbilical cord is frequently associated with other congenital malformations as well as some adverse perinatal events such as intrauterine growth retardation (IUGR), premature delivery, and increased perinatal mortality. Five cases of SUA detected prenatally by ultrasound are reported here in detail, including the first reported case in a twin gestation. None of the 5 affected infants had associated anomalies, but 2 cases of intrauterine growth retardation (IUGR) and 1 stillborn infant were noted in this series. An umbilical vein/umbilical artery ratio less than 2 was invariably found in all cases, making this observation another useful sonographic characteristic to use in the antenatal detection of SUA. Since the umbilical cord can be easily seen prenatally by ultrasound, and SUA is recognized as an important index for detecting congenital malformations, examination of the umbilical cord for the absence of one umbilical artery is an extremely valuable tool in prenatal diagnosis. The prenatal detection of SUA demands an extensive search for associated anomalies and a close surveillance of fetal well-being, since these fetuses have a high risk of fetal death or IUGR. Sonologists and sonographers should be aware of the possibility of SUA, especially in those cases associated with congenital malformations or IUGR.     

Omphalocele and gastroschisis: prenatal diagnosis and peripartal management. A case analysis of the years 1989-1997 at the Department of Obstetrics and Gynecology, University of Homburg/Saar

OBJECTIVE: The article presents a retrospective analysis (1989-1997) of the prenatal diagnosis, the course and completion of pregnancy of 26 fetuses with omphalocele and 18 fetuses with gastroschisis. SUBJECTS: 44 pregnancies with anterior fetal wall defect diagnosed by prenatal ultrasound, clinical or patho-anatomic examination between 1989 and 1997 at the Department of Obstetrics and Gynecology, University of Homburg/Saar. RESULTS: In 40 of 44 pregnancies (91%) the fetal ventral abdominal wall defect could be detected antenatally with ultrasound. Associated malformations in fetuses with omphalocele were seen in 18 cases (69%), whereas only five fetuses with gastroschisis (28%) had an associated malformation. Nineteen of 26 fetuses (73%) with omphalocele had a normal karyotype. Seven of 26 fetuses (27%) with omphalocele had an abnormal karyotype. Eleven fetuses with omphalocele were live born, three of them with minor anomalies. Ten babies with omphalocele survived. No chromosomal anomalies were detected in fetuses with gastroschisis. There were four gastrointestinal malformations and one lethal associated malformation in fetuses with gastroschisis. There were 15 live born babies with gastroschisis, all of whom have survived. In 20 of 44 cases (45%) with ventral abdominal wall defect oligohydramnios could be detected by ultrasound. In 28 of 44 cases (64%) we found fetal growth retardation <10th percentile for gestational age. CONCLUSION: In case of a fetal ventral abdominal wall defect, the detection and appropriate classification of associated fetal anomalies is of great importance for the further course of pregnancy. Fetal karyotyping should be offered in case of a fetal abdominal wall defect. Early and close prenatal consultation of the neonatologist and the pediatric surgeon will favorably influence the perinatal outcome.     

Pregnancy outcome in isolated single umbilical artery

Our objective was to determine whether the rate of small for gestational age (SGA) infants and adverse perinatal outcome are increased in pregnancies diagnosed with an isolated single umbilical artery (SUA). We compared 297 pregnancies with a SUA diagnosed on routine obstetrical ultrasound with 297 pregnancies with a three-vessel cord control. Pregnancies complicated by major fetal anomalies were excluded. The rate of SGA, fetal death, and neonatal outcomes were compared between the two groups. Data analysis were performed using the T-test and chi-square test. The sample size had 80% power to detect a 50% difference between groups assuming a SGA rate of 20% in the SUA group and 10% in the control, alpha = 0.05. Among the SUA group, in 21 neonates (7.1%) the presence of a SUA could not be confirmed by postnatal examination, and 21 (7.1%) had major congenital anomalies, leaving 255 for final analysis. In the control group, 8 of the 297 (2.7%) had major congenital anomalies, leaving 289 for final analysis. The incidence of SGA neonates was 35 of 255 (13.7%) in the isolated SUA group compared with 38 of 289 (13.1%) in the control group ( P = 0.93). The composite perinatal outcomes (fetal death and/or SGA) were also similar between the groups (16.1% versus 14.5%; P = 0.72). We concluded that pregnancies with isolated SUA have a similar rate of SGA to those with 3VC.When a SUA is identified antenatally, a targeted ultrasound is warranted to rule out associated anomalies. Serial antepartum ultrasound for fetal growth is not necessary in managing pregnancies complicated by isolated SUA.     

Detection of fetal structural abnormalities by an 11-14-week ultrasound dating scan in an unselected Swedish population

BACKGROUND: To determine the detection rate of fetal structural abnormalities by a routine 11-14-week ultrasound scan for dating in an unselected pregnant population. METHODS: A prospective observational cohort study of 2,708 unselected pregnant women attending an abdominal ultrasound examination at 11-14 weeks gestation. The number of major fetal structural abnormalities diagnosed after birth was obtained from a computerized database at the same unit. RESULTS: Out of 2,708 pregnant women, 89 (3.3%) were found to have a missed abortion at the time of the ultrasound scan and 33 (1.2%) were diagnosed as twins. Thirteen major structural abnormalities were detected, three cases of anencephaly (one case also had a spina bifida), one case with hydranencephaly, one fetus with Dandy-Walker syndrome, two cases with gastroschisis, one case with a bilateral hydronephrosis, one case with a generalized hydrops, one fetus with multiple malformations, and three cystic hygromas. An additional 19 major structural defects were detected at birth. Four cases of neural tube defects and nine fetuses with congenital heart defects were diagnosed. The antenatal ultrasound detection rate was 40.6% (13/32). Nine patients had a nuchal translucency greater than 3.0 mm (excluding cystic hygromas); two of them had chromosomal abnormalities (trisomy 21 and trisomy 18). CONCLUSIONS: Fetal structural abnormalities were detected in 41% (95%CI = 24-59) of the cases in an unselected pregnant population at a routine 11-14-week ultrasound scan for dating purpose. Two out of nine fetuses with a nuchal translucency greater than 3.0 mm had a chromosomal abnormality.     

Prenatally diagnosed fetal lung lesions with associated conotruncal heart defects: is there a genetic association?

Congenital lung malformation can easily be diagnosed by prenatal ultrasound. Associated extrapulmonary malformations such as heart defects and chromosomal aberrations are rare.OBJECTIVE: The objective of this study was to describe the natural history, outcome and other associated malformations in fetuses with lung lesions and an associated heart defect. METHODS: Retrospective analysis of 4 cases of prenatally diagnosed fetal CCAMs and hybrid lesions with an associated heart defect and review of 8 cases in the literature. RESULTS: At a single referral center 1.9% of the fetuses with Congenital cystic adenomatoid malformation (CCAM) were diagnosed with an associated heart defect. Seven of the total 12 cases (58%) reviewed had a conotruncal heart abnormality. Chromosomal abnormalities were found in 5 (42%) of the cases. CONCLUSION: This retrospective review shows that karyotyping in fetal lung lesions with an associated heart defect or isolated large lung lesions is indicated. It also suggests that there is a subpopulation of fetuses with CCAMs who have conotruncal heart defects. This finding may suggest a common genetic background.     

Should determination of the karyotype be systematic for all malformations detected by obstetrical ultrasound?

OBJECTIVE: The aim of this study was to determine whether karyotyping should be performed for every fetal malformation detected in low risk populations. METHODS: A karyotype was obtained from 428 fetuses examined over a 10-year period after fetal malformation was diagnosed using obstetrical ultrasound. These fetuses were separated into two groups, one with isolated malformations and the other with multiple malformations. The association between each type of malformation and the result of karyotype was evaluated. RESULTS: Forty-eight chromosomal abnormalities were encountered in 428 fetuses (11.2%). The karyotype was abnormal in 32/343 (9.3%) fetuses with isolated malformations and 16/85 (18.8%) fetuses with multiple malformations (p=0.022). The probability of an abnormal karyotype among the group of isolated malformation depended on the anatomical system involved (p<0.001). Our study demonstrated several isolated malformations without chromosomal abnormality (hydronephrosis with high obstruction, unilateral multicystic dysplastic kidney, gastroschisis, intestinal dilatation, meconium peritonitis, cystic adenomatoid malformation, pulmonary sequestration, tumor, vertebral anomaly). CONCLUSION: Each fetus with multiple malformations needs a chromosomal analysis. Within the group of isolated malformations, our study emphasizes that medical maternal history and the type of malformation need to be taken into account before performing a fetal karyotype.     

Evaluation of prenatal diagnosis by a registry of congenital anomalies.

Prenatal diagnosis performed by fetal karyotype and ultrasound scan is now a routine part of antenatal care in many countries. How many fetal anomalies are actually detected by these procedures? We have used our registry of congenital malformations to answer this question. In our region, prenatal diagnosis was performed in 23.1 per cent of fetuses with a chromosomal aberration and in 20.1 per cent of fetuses with non-chromosomal anomalies. Only 6.9 per cent of the pregnancies with fetuses with non-chromosomal anomalies were terminated. The sensitivity of prenatal diagnosis by ultrasonographic examination was much lower for isolated malformations (fetuses with only one anomaly) than for multiple malformed children, 15.3 and 48.3 per cent respectively, chromosomal anomalies excluded.     

Ultrasound diagnosis of fetal abnormalities and cytogenetic evaluation.

Over a 6 1/2 year period, in 288 pregnancies a variety of fetal malformations were detected by ultrasound. Two hundred and ten fetuses (73 per cent) were karyotyped. Gestational age at detection ranged from 11 to 38 weeks. The incidence of an abnormal karyotype in the total series was 14 per cent and 14.7 per cent in the 210 pregnancies in which a karyotype was performed. Single structural anomalies were found in 149 cytogenetically investigated fetuses, of which 25 had a chromosomal abnormality (17 per cent). Multiple structural malformations were present in 61 fetuses, of which 16 had an abnormal karyotype (26 per cent). Trisomy 18 was the most frequent finding. The most constant ultrasound finding in cases of an abnormal karyotype was polyhydramnios and severe IUGR in combination with structural defects. There is a need for extensive detailed ultrasound examination in high-risk pregnancies.     

Demographic and perinatal outcome data of fetuses with SUA/PRUV

Aim: Identify structural anomalies and adverse pregnancy outcomes accompanying single umbilical artery (SUA) and persistent right umbilical vein (PRUV) and to investigate whether SUA and PRUV are associated with chromosomal abnormalities and if these defects warrant invasive antenatal diagnosis.

Methods: We retrospectively analyzed pregnancies with an antenatal diagnosis of SUA/PRUV from the International Peace Maternity and Child Health Hospital (IPMCHH) database. Data of structural malformations, fetal karyotyping, and pregnancy outcomes were analyzed.

Results: Results revealed that 13.9 and 7% of SUA and PRUV cases, respectively, had malformations. Furthermore, 60% of the malformations accompanying SUA were isolated congenital heart disease (CHD), and 50% of the malformations accompanying PRUV were multiple malformations including CHD. All primarily diagnosed isolated PRUV (iPRUV) cases and 92.6% of primarily diagnosed iSUA cases had normal fetal echocardiography. Cases of iSUA and iPRUV with indications of invasive antenatal diagnosis showed no chromosomal abnormalities. The emergency cesarean section rate did not differ between the study population and the IPMCHH general population (p?=?.184).

Conclusion: Patients of SUA and PRUV diagnosed in secondary hospitals should be referred to tertiary medical center for further ultrasonography. SUA and PRUV are not indicators for invasive antenatal diagnosis and selective cesarean section.     

Brain damage to the survivor within 30 min of co-twin demise in monochorionic twins

Single fetal death in a twin pregnancy in the late second or early third trimester is associated with significant morbidity and mortality rate in the surviving co-twin, especially in monochorionic twin pregnancies. The common causes are twin-to-twin transfusion syndrome, chromosomal abnormalities, and congenital anomalies of the fetus or anomalies of the umbilical cord-placenta. Here we report a case of monochorionic twin pregnancy in which one fetus had a single umbilical artery (SUA) while the co-twin had two umbilical arteries. The twin with SUA died in utero at the 30th week of gestation and the other fetus was delivered by cesarean section immediately due to fetal distress diagnosed by cardiotocography. Disseminated intravascular coagulation and multicystic encephalomalacia have been observed in the surviving neonate. This case and review of the literature suggest that neurologic complication rates are also increased in monochorionic twin pregnancies with single fetal demise despite the immediate delivery as in our case.     

The effect of associated structural malformations in the prediction of chromosomal abnormality risk of fetuses with echogenic bowel

Objective: Our aim is to determine the frequency of chromosomal abnormalities and also to identify the role of structural malformations on the chromosomal abnormality risk among fetuses with echogenic bowel.

Methods: Over a 6-year period fetuses with echogenic bowel (FEB) were retrospectively evaluated. The pregnancies with intra-amniotic bleeding history, congenital infection, cystic fibrosis and intrauterine growth retardation were excluded from the study. Types and frequency of sonographically detected fetal malformations were identified. Chromosomal abnormality incidences according to association with soft markers and major fetal abnormalities were compared.

Results: Of the 281 fetuses with echogenic bowel, 105 (37.37%) were isolated, 78 (27.76%) were associated with soft markers and 98 (34.87%) were associated with major abnormalities. There were 30 (10.7%) fetuses with abnormal karyotypes. The chromosomal abnormality rate of the groups of isolated FEB, FEB?+?soft markers and FEB?+?major abnormalities were 6.7%, 7.7% and 17.4%, respectively.

Conclusions: Chromosomal abnormality risk in fetuses with echogenic bowel should be evaluated according to additional sonographic findings. Association of structural malformations increases the chromosomal abnormality risk, although this risk is not significant with the presence of soft markers alone.     

Perinatal outcome after ultrasound prenatal diagnosis of persistent right umbilical vein

Objective

Our aim was to describe ultrasound findings and perinatal outcome after prenatal diagnosis of persistent right umbilical vein (PRUV).

Study design

We performed a retrospective analysis of fetuses with an ultrasound-based prenatal diagnosis of PRUV on record at 2 tertiary centers in Madrid, Spain. We describe clinical, maternal, fetal and perinatal variables for all cases.

Results

A total of 20,426 fetuses were delivered between the study centers. We detected 22 cases (0.1%) of PRUV. The male-to-female ratio was 1:1. All cases were intrahepatic type and diagnosed during the second and third trimesters (median, 21 weeks; IQR, 20–29 weeks). Doppler ultrasound revealed normal flow in the ductus venosus in all cases. Nine fetuses (40.9%) had additional ultrasound anomalies but no chromosomal abnormalities. Cardiovascular malformations were the most frequently associated congenital anomalies (4/9), followed by neurological malformations (2/9). In 5 of the cases with no concomitant anomalies, the weight of the newborn was below the tenth percentile for gestational age. Gestational development was normal for the remaining newborns. Delivery was unremarkable, and post-natal evolution was favorable.

Conclusion

Our results point to a potential association between PRUV and other fetal malformations and a very low rate of chromosomal abnormalities. Prenatal diagnosis of PRUV should be followed by detailed anatomical evaluation and echocardiography in order to rule out other structural malformations. The indication for a fetal karyotype study must be made on an individual basis considering PRUV type and other ultrasound findings.     

Fetal obstructive uropathies. Importance of chromosomal abnormalities and associated anomalies to perinatal outcome.

The ultrasound records of 30 fetuses suspected of having an obstructive uropathy were reviewed retrospectively. A prenatal karyotype was obtained with amniocentesis on each patient. After delivery, neonatal urologic records, renal ultrasound reports and autopsy information were reviewed and compared to the ultrasound records and fetal karyotype results. Chromosomal defects were found in 23% of fetuses with a suspected obstructive fetal uropathy. In five patients the chromosomal abnormality was lethal and caused 45% of the perinatal deaths in this series. If a fetus with an obstructive uropathy was female, there was a significant likelihood of an extrarenal anomaly or a complex genitourinary tract malformation. Seventeen percent of patients with an obstructive uropathy had a coexistent extrarenal defect. A prenatal karyotype should be obtained if a fetal obstructive uropathy is suspected antenatally since lethal chromosomal defects are an important cause of perinatal death. A female karyotype may indicate a fetus at higher risk of extrarenal anomalies or complex genitourinary malformations.     

Antenatal ultrasound diagnosis of fetal malformations: possibilities, limitations and dilemmas

The antenatal ultrasound diagnosis of fetal abnormalities in 150 fetuses over the period 1975-early 1983 is reported. The perinatal outcome of the whole series has been assessed. The most frequently encountered malformations involved the central nervous system (34.9%), the nephro-urological system (17.2%), and the gastro-intestinal tract, abdominal wall and diaphragm (17.1%). Neonatal findings in incompletely explained polyhydramnions, fetal hydrops, and severe intrauterine growth retardation are also commented upon. Numerical chromosomal abnormalities were present in at least 13.3% of the malformed fetuses. The accuracy of the antenatal ultrasound diagnosis in a defined subgroup with congenital malformations resulting in perinatal death was analysed: in 76.9% one (or more) malformations were correctly diagnosed or relevant information was obtained. Comment is made on the obstetrical management and its inherent dilemmas, with appropriate references to the literature. Antenatal ultrasound, together with complementary techniques, can reach an acceptable level of accuracy, but associated anomalies may be missed. Groups for which there are different prognoses can be assessed, and serve as a guideline in perinatal management. Ethical attitudes, psychological aspects, and the possible promises of fetal treatment are briefly discussed.     

Outcome for fetuses with prenatally detected congenital heart disease and cardiac arrhythmias in Taiwan.

BACKGROUND/PURPOSE: Outcome for fetuses with prenatally detected congenital heart disease (CHD) and/or cardiac arrhythmias is important for prenatal counseling and perinatal management; however, there exists little literature regarding the outcome for CHD diagnosed in utero in Taiwan. Therefore, we attempted to investigate the outcome for fetuses with CHD and/or cardiac arrhythmias diagnosed prenatally at a tertiary care medical center in Taiwan. METHODS: Between January 1995 and December 2000, 339 patients referred to the National Taiwan University Hospital for fetal echocardiography were included in this study. Medical records were reviewed retrospectively to determine the salient clinical characteristics for all fetuses. RESULTS: CHD was found in 103 fetuses. Gestational age at diagnosis ranged from 17 to 40 weeks; in 37 cases (35.9%) the diagnosis was made before 24 weeks. Mean gestational age at diagnosis was 27.8 weeks. Of the 103 cases, 15 fetuses (14.6%) had major extra cardiac malformations and 15 fetuses (14.6%) had chromosomal abnormalities (five had both) and 30 pregnancies (29.1%) were terminated. Of the remaining 73 pregnancies, three (4.1%) of the fetuses died in utero and 28 (38.4%) postnatally, with 42 (57.5%) surviving. The mortality rates were both 60% in cases with extracardiac or chromosomal anomalies. Arrhythmias were identified in 25, and two pregnancies involving hydrops fetalis were terminated. Of the remaining 23 continued pregnancies, two (8.7%) with long QT syndrome expired postnatally. CONCLUSION: Outcome for fetuses with prenatally detected CHD remains poor, with the prognosis negatively influenced by the presence of complex heart defects as well as extracardiac and chromosomal anomalies. However, prognosis is good for fetuses with cardiac arrhythmia, except with long QT syndrome or hydrops fetalis.