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Objective: To identify fetal echocardiographic features, associations, and outcome of atrioventricular and ventriculoarterial discordance. Design: Retrospective analysis of videotape recordings of fetal echocardiograms and examination of departmental database and patient records to establish outcome. Setting: Tertiary centre for fetal cardiology. Patients: 34 consecutive fetuses with a prenatal diagnosis of double discordance seen between January 1993 and December 2003. Main outcome measures: Echocardiographic features including reversed differential insertion, identification of the right ventricle through the moderator band, and the origin and orientation of the great arteries. All associated cardiac abnormalities and outcome. Results: Reversed differential insertion was identified in 26 fetuses. The right ventricle could be identified by the moderator band in 30 fetuses and was left sided in all but one. A parallel or abnormal orientation of the great arteries was seen in all fetuses. Only five of 34 cases were isolated from a cardiac viewpoint. There was a ventricular septal defect in 21, Ebstein's anomaly in 8, pulmonary stenosis in 12, coarctation of the aorta in 6, tricuspid atresia in 2, congenital heart block in 2 fetuses prenatally, and dextrocardia in 6. Nineteen babies survived; one baby died in the neonatal period and three in infancy. Eleven pregnancies were interrupted. Conclusions: A left sided or posterior right ventricle, identified by reversed differential insertion or the moderator band, in association with abnormal orientation of the great arteries should alert a sonographer to the diagnosis of double discordance. Isolated cases are rare prenatally (14%) but the short term survival in our fetal series is good. Where pregnancy was continued, 19 of 23 babies (82%) remained alive. [ABSTRACT FROM AUTHOR]

Objective To determine whether heart failure is the mechanism underlying the association between increased fetal nuchal translucency and congenital heart defects. Methods Retrospective analysis of the types of congenital heart defect observed in fetuses with increased nuchal translucency and those with normal nuchal scans. Retrospective quantitative analysis of cardiac size and left ventricular ejection fraction in fetuses with ventricular septal defects or the hypoplastic left heart syndrome. Results Eighty-three fetuses with congenital heart defects had undergone nuchal screening of which 51 had increased nuchal translucency and 32 had normal nuchal translucency. A wide variety of different congenital cardiac lesions with different hemodynamic effects were observed in fetuses with increased nuchal translucency and those with normal nuchal scans. Defects primarily characterized by left heart obstruction, right heart obstruction and septal defects occurred in both groups. All measurements of cardiothoracic ratio and left ventricular ejection fraction fell within the normal range and there was no significant difference between fetuses with increased nuchal translucency and those with normal nuchal scans. Conclusions No specific type of congenital heart lesion is associated with increased nuchal translucency. The contention that heart failure explains the association between congenital heart defects and increased nuchal translucency is not supported by this study. [ABSTRACT FROM AUTHOR]

Congenital heart disease (CHD) is a common form of congenital malformation associated with significant morbidity and mortality. Antenatal diagnosis of life-threatening forms of CHD may help to improve survival and morbidity as well as allowing parental choice and preparation. The diagnosis of CHD during fetal life can be made with a very high level of diagnostic accuracy in tertiary centres dealing with high-risk pregnancies. However, most cases of CHD will occur in low-risk groups and will only be detected by screening at the time of obstetric ultrasound scans. The concept of antenatal screening for CHD was introduced in the UK over 20 years ago, and current national guidelines recommend that the heart should be examined at the time of the obstetric anomaly scan. However, there remains a large regional variation in prenatal detection rates of CHD. Widespread teaching and training is required to achieve a more uniform standard but this will be associated with significant resource implications. [ABSTRACT FROM AUTHOR]

BACKGROUND: Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome. METHODS AND RESULTS: We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000-2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1-21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age < 20 weeks, ventricular rate <= 50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of >= 1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4 ± 2.9 versus 24.9 ± 4.9 weeks; P=0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy. CONCLUSIONS: Risk factors associated with a poor outcome were gestation < 20 weeks, ventricular rate <= 50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen. [ABSTRACT FROM AUTHOR]

The authors estimated the prevalence of heart malformation during the first year of life, using five data sets with varying degrees of completeness from two English regional health authorities. These areas covered a total population of 6, 872, 000. Analysis was carried out using capture-recapture methods, including log-linear modeling, on data collected between June 1993 and August 1994. A large number of cases in the community were unrecorded by any of the current sources of information. In South East Thames, where an antenatal training screening program for detecting heart malformations had been implemented in the late 1980s, the estimated prevalence rate varied from 5.5 per 1, 000 births (95% confidence interval (Cl): 3.5, 10.8) to 9.0 per 1, 000 births (95% Cl: 6.4, 14.2), depending on the assumptions in the model and the number of sources used in the analysis. In the Wessex region, which did not have a formal training program, prevalence was lower and varied little, from 4.3 per 1, 000 (95% Cl: 3.4, 6.0) to 5.1 per 1, 000 (95% Cl: 4.0, 7.2), according to assumptions. These two estimates were reasonable rates in comparison with reports in the literature. This analysis was helpful in demonstrating that the training program designed to identify servere heart malformations during the antenatal period in one of these regions had no lasting impact on prevalence. Am J Epidemiol 1999; 150: 778–85. [ABSTRACT FROM PUBLISHER]

Objective: To report the timing of presentation and clinical profile of a cohort of fetuses with normal main cardiac connections but fetal echocardiographic signs suggestive of coarctation of the aorta. Design: Retrospective observational study. Setting: Tertiary fetal and paediatric cardiology centre. Patients: Between 1 January 1998 and 31 December 2002, 174 fetuses were studied, of whom 144 infants were born alive. Main outcome measures: Of the 144 liveborn infants, 43 had coarctation of the aorta, four had interruption of the aortic arch, and one was managed as having hypoplastic left heart syndrome. Hemianomalous pulmonary venous drainage was diagnosed in two infants. Three infants with coarctation presented late at 7-13 weeks of age, 6-12 weeks after closure of the arterial dud. Fetuses with cardiac asymmetry had a higher incidence of left superior vena cava than a control group. For fetuses with cardiac asymmetry, the incidence of left superior vena cava and ventricular septal defects was similar in infants who proved to have coarctation postnatally and in those who did not. The 30 day and one year surgical mortality of infants having repair of coarctation of the aorta was two of 41 (4.9%, 95% confidence interval (CI) 0.6 to 16.0). All cause mortality of liveborn infants with any abnormality of the aortic arch was five of 48 (10.4%, 95% CI 3.5 to 22.7) at 30 days and one year, which was heavily influenced by prematurity and extracardiac abnormalities. Conclusions: Precise diagnosis of coarctation of the aorta during fetal life remains difficult. Coarctation of the aorta may present several weeks after closure of the arterial duct and sequential echocardiography is recommended. [ABSTRACT FROM AUTHOR]

Background and Aims-Staged reconstructive surgery has radically altered the prognosis of hypoplastic left heart syndrome (HLHS). Antenatal diagnosis allows for appropriate counselling, and time to consider treatment options. We report outcome from a centre where most cases are antenatally diagnosed and delivered on site. Methods-Information was collated on 188 consecutive cases of HLHS between 1995 and 2000, including timing of diagnosis, outcome of pregnancy, and age and outcome at each stage of surgery. At Guy's Hospital, 174 cases were diagnosed antenatally, of whom 50 underwent surgery. Fourteen others (five diagnosed antenatally at other centres, and nine diagnosed postnatally) also underwent surgery. Results-Survival after stage I (the Norwood operation) was 52% (33/64). Postoperative survival after stage H (the hemi-Fontan operation, performed in 29), and stage III (the Fontan operation, performed in 10), was 100%. Two late deaths occurred 3 and 10 months after stage II, giving overall survival of 48% (31/64). At follow up, three children have neurological impairment, and one had poor right ventricular function necessitating cardiac transplantation. Conclusions-Antenatal diagnosis allows informed decisions about treatment options, and facilitates preoperative care. Mortality following stage I is high, irrespective of timing of diagnosis, but medium term outcome for survivors is good. [ABSTRACT FROM AUTHOR]

Objective: To study myocardial deformation by speckle tracking echocardiography in fetuses with coarctation of the aorta (CoA) compared with gestational age-matched normal controls.Methods: This was a retrospective study of 12 fetuses with postnatally confirmed CoA and 12 gestational age-matched controls seen at a tertiary fetal cardiology unit between January 2013 and July 2014. Two-dimensional speckle tracking in standard grayscale four-chamber view of the fetal heart was performed to assess left and right myocardial deformation. Global longitudinal strain, strain rate and velocities, and regional longitudinal strain were analyzed and compared with controls.Results: Median gestational age at echocardiography was 25 + 4 weeks. Fetuses with CoA presented with a narrower, but not shorter, left ventricle when compared with controls (mitral valve diastolic diameter, 5.90 vs 8.50 mm; P = 0.002; left ventricular diastolic length, 16.50 vs 18.50 mm; P = 0.05). Global longitudinal systolic strain (P = 0.004), systolic strain rate (P = 0.01) and diastolic strain rate (P = 0.004) of the left ventricle were significantly lower in fetuses with CoA compared with controls. Similar findings were observed for longitudinal systolic (P = 0.03) and diastolic (P = 0.01) velocities of the left ventricle. Right ventricular parameters were not different between groups.Conclusions: Fetuses with CoA have lower left ventricular longitudinal systolic strain, systolic strain rate and diastolic strain rate when compared with gestational age-matched control fetuses. These differences in deformation might explain, at least in part, the cardiac asymmetry observed in fetuses with CoA. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd. [ABSTRACT FROM AUTHOR]

Objective: To assess the accuracy of prenatal diagnosis, the association with genetic and extracardiac anomalies, and outcome in fetuses with isolated pulmonary atresia with ventricular septal defect (PA-VSD). Design and setting: Retrospective study in a tertiary centre for fetal cardiology. Patients and outcome measures: Echocardiographic video recordings of 27 consecutive fetuses with PA-VSD were reviewed for: (1) intracardiac anatomy; (2) presence of confluence and size of the branch pulmonary arteries; (3) source of pulmonary blood supply; and (4) side of the aortic arch. Postmortem and postnatal data were added. Karyotyping was performed in 25 patients and, in 23 of these, fluorescent in situ hybridisation to identify 22q1 1.2 deletion. Results: PA-VSD was correctly diagnosed in 19 of 21 patients (90%) with postnatal or autopsy confirmation of diagnosis. Central pulmonary arteries were correctly identified in 79% (15/19), the source of pulmonary blood supply in 62% (13/21) and major aortopulmonary collateral arteries in 44% (4/9). Aneuploidy was detected in 4 of 25 patients (16%) and 22q1 1.2 deletion in 6 of 23 patients (26%). Five of 27 patients (19%) had extracardiac anomalies. Eleven pregnancies were interrupted. Eleven of 16 liveborn babies survived. Neonatal survival was 15 of 16 (94%, 95% confidence interval (CI) 70 to 100), one-year survival was 9 of 12 (75%, 95% Cl 43 to 95) and two-year survival was 5 of 9 (56%, 95% CI 21 to 86). Conclusion: PA-VSD can be diagnosed by fetal echocardiography with a high degree of accuracy. However, it can be difficult to determine the morphology of the central pulmonary arteries and to locate the source of pulmonary blood supply. In most liveborn infants, complete surgical repair can be achieved. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the incidence of fetal dextrocardia, associated cardiac and extracardiac malformations, and outcome. Design: Retrospective echocardiographic study. Setting: Two tertiary centres for fetal cardiology. Patients: 81 consecutive fetuses with a fetal dextrocardia presenting at Guy's Hospital, London, between 1983 and 2003 and at Hôpital Robert Debré, Paris, between 1988 and 2003. Fetal dextrocardia was defined as a condition in which the major axis of the heart points to the right. Results: The incidence was 0.22%. There were 38 fetuses (47%) with situs solitus (SS), 24 (30%) with situs ambiguus (SA), and 19 (23%) with situs inversus (SI). Structural cardiac malformations were found in 25 cases (66%) of SS, 23 cases (96%) of SA, and 12 cases (63%) of SI. Extracardiac malformations were identified in 12 cases (31%) of SS, in five cases (21%) of SA, and in two cases (10%) of SI. Of the 81 cases of fetal dextrocardia, there were 27 interrupted pregnancies (15 of 24 SA, 10 of 38 SS, and 2 of 19 SI), six intrauterine deaths (3 of 38 SS, 2 of 24 SA, and 1 of 19 SI), and five neonatal deaths (3 of 24 SA, 1 of 19 SI, and 1 of 38 SS). There were 43 survivors (24 of 38 SS, 15 of 19 SI, and 4 of 24 SA). Conclusion: The majority of fetuses with dextrocardia referred for fetal echocardiography have associated congenital heart disease. There is a broad spectrum of cardiac malformation and the incidence varies according to the atrial situs. Fetal echocardiography enables detection of complex congenital heart disease so that parents can be appropriately counselled. [ABSTRACT FROM AUTHOR]

The buffer-perfused Langendorff heart is significantly vasodilated compared with the in vivo heart. In this study, we employed ultrasound to determine if this vasodilation translated into changes in left ventricular wall thickness (LVWT), and if this effect persisted when these hearts were switched to the "working" mode. To investigate the effects of perfusion pressure, vascular tone, and oxygen availability on cardiac dimensions, we perfused hearts (from male Wistar rats) in the Langendorff mode at 80, 60, and 40 cm H2O pressure, and infused further groups of hearts with either the vasoconstrictor endothelin-1 (ET-1) or the blood substitute FC-43. Buffer perfusion induced a doubling in diastolic LVWT compared with the same hearts in vivo (5.4 ± 0.2 mm vs. 2.6 ± 0.2 mm, p < 0.05) that was not reversed by switching hearts to "working" mode. Perfusion pressures of 60 and 40 cm H2O resulted in an increase in diastolic LVWT. ET-1 infusion caused a dose-dependent decrease in diastolic LVWT (6.6 ± 0.4 to 4.8 ± 0.4 mm at a concentration of 10–9 mol/L, p < 0.05), with a concurrent decrease in coronary flow. FC-43 decreased diastolic LVWT from 6.7 ± 0.5 to 3.8 ± 0.7 mm (p < 0.05), with coronary flow falling from 16.1 ± 0.4 to 8.1 ± 0.4 mL/min (p < 0.05). We conclude that the increased diastolic LVWT observed in buffer-perfused hearts is due to vasodilation induced by the low oxygen-carrying capacity of buffer compared with blood in vivo, and that the inotropic effect of ET-1 in the Langendorff heart may be the result of a reversal of this wall thickening. The implications of these findings are discussed. [ABSTRACT FROM AUTHOR]

Objective: To review the diagnosis, treatment, and outcome of fetal atrial flutter compared with supraventricular tachycardia. Design: Retrospective review of published reports: 11 papers about fetal tachyarrhythmia published between 1991 and 2002 were selected for review. Main outcome measures: All selected studies were analysed for the type of arrhythmia, degree of atrioventricular block in atrial flutter, occurrence of hydrops fetalis, gestational age at diagnosis, first and second line drug treatment, associated cardiac and extracardiac malformations, and mortality of the fetuses. Results: Atrial flutter accounted for 26.2% of all cases of fetal tachyarrhythmias, and supraventricular tachycardia for 73.2%. Hydrops fetalis was reported in 38.6% and 40.5% of fetuses with atrial flutter and supraventricular tachycardia, respectively (NS). Hydropic fetuses with atrial flutter had higher ventricular rates (median 240 beats/min, range 240-300) than non-hydropic fetuses (220 beats/min, range 200-310) (p = 0.02), whereas the atrial rates were not significantly different (median 450 beats/min, range 370-500). Digoxin treatment resulted in a higher conversion rate in non-hydropic fetuses with fetal tachyarrhythmias than in hydropic fetuses (p < 0.001 ). The overall mortality of atrial flutter was similar to that of supraventricular tachycardia, at 8.0% v 8.9% (p = 0.7). Conclusions: The prevalence of hydrops fetalis did not differ in fetal atrial flutter and supraventricular tachycardia with 1:1 conduction. There was no difference between the response rate to digoxin in fetus with atrial flutter or supraventricular tachycardia. Mortality was similar in the two types of tachyarrhythmia. [ABSTRACT FROM AUTHOR]

There are few data on the outcome of truncus arteriosus when this diagnosis is made during fetal life. Such prognostic information is important to assist parental counseling during pregnancy. This study aimed to analyze, retrospectively, the echocardiographic features and outcome of fetuses with truncus arteriosus. A database of those presenting to a tertiary center for fetal cardiology between 1990 and 1999 was reviewed. Cases in which truncus arteriosus was identified as a firm or differential diagnosis were selected. Outcome data were derived from clinical records, and fetal echocardiograms were reviewed retrospectively. At presentation, truncus arteriosus was firmly diagnosed in 16 patients and was a differential diagnosis in 12. Fourteen of 16 (87%) of the firm diagnoses were correct. There were 17 confirmed cases of truncus arteriosus. Pregnancy was terminated in 4 patients (24%) and there were 13 live births. One child was not actively treated, 4 (31%) died preoperatively, and 8 (61%) underwent surgery. Thirty-day surgical mortality was 2 of 8 (25%). There was 1 late death after cardiac catheterization, and overall survival on an intention-to-treat basis was 5 of 12 (42%). Five of 6 patients with a prenatal truncal valve Doppler velocity above the normal aortic range were found to have postnatal truncal valve stenosis. Two fetuses with stenotic valves died preoperatively with sudden cardiovascular collapse. Counseling of parents for fetuses with truncus arteriosus should include the relatively high nonsurgical mortality as well as surgical results. Elevated prenatal truncal valve Doppler velocity predicts postnatal truncal valve stenosis. Fetuses with truncal valve stenosis may be at risk of early sudden death. [ABSTRACT FROM AUTHOR]

Abstract Objective To assess the effect of echogenic foci in the fetal heart on the risk for Down’s syndrome. Design Prospective evaluation of fetal echocardiograms at a fetal cardiology referral center and systematic postnatal follow-up. A relative risk was calculated from the prevalence of echogenic foci in fetuses subsequently demonstrated to have trisomy 21 divided by that in normal fetuses. For a subgroup of 548 fetuses with echogenic foci but otherwise normal detailed anomaly scans, the expected number of trisomy 21 fetuses calculated from maternal age risks was compared with the observed number to derive a relative risk for isolated echogenic foci. Results Echogenic foci occurred in 905 of 6904 fetuses scanned, but after excluding those referred specifically because of an echogenic focus and those with heart defects, the incidence was 9.5%. Overall, echogenic foci were more frequent in fetuses with trisomy 21 than those without by a factor of 2.93. For the 548 fetuses with echogenic foci but otherwise normal detailed anomaly scans, the actual number of trisomy 21 fetuses exceeded that expected on the basis of maternal age risks by a factor of 5.54. Combination with data from several previous studies suggests a consensus relative risk of about 3.0. Conclusions Echogenic foci are associated with increased risk of trisomy 21 even when present as an isolated finding. Their significance in an individual should be interpreted in the light of prior risk assessment based on maternal age and results of any first-trimester screening tests. We suggest that the prior risk is increased by a factor of 3.0. [ABSTRACT FROM AUTHOR]

Prenatal alleviation of severe fetal aortic valve obstructions by percutaneous ultrasound-guided balloon valvuloplasty has been performed to improve the fate of affected fetuses. The purpose of this study was to analyze the current world experience of these procedures in human fetuses. Data from 12 human fetuses were available for analysis. The mean gestational age at intervention was 29.2 weeks (range 27 to 33). The mean time period between initial presentation and intervention was 3.3 weeks (range 3 days to 9 weeks). Technically successful balloon valvuloplasties were achieved in 7 fetuses, none of whom had an atretic valve. Only 1 of these fetuses remains alive today. Of the 5 remaining technical failures, 1 patient with severe aortic stenosis underwent successful postnatal intervention and remains alive. Six patients who survived prenatal intervention died from cardiac dysfunction or at surgery in the first days or weeks after delivery. Four fetuses died early within 24 hours after the procedure, 1 from a bleeding complication, 2 from persistent bradycardias, and 1 at valvotomy after emergency delivery. Thus, the early clinical experience of percutaneous ultrasound-guided fetal balloon valvuloplasty in human fetuses with severe aortic valve obstruction has been poor due to selection of severe cases, technical problems during the procedure, and high postnatal operative mortality in fetuses who survived gestation. Improved patient selection and technical modifications in interventional methods may hold promise to improve outcome in future cases. [ABSTRACT FROM AUTHOR]

ABSTRACT A double aortic arch is a congenital anomaly associated with the formation of a vascular ring, which can cause varying degrees of airway compression. This can be subclinical or clinical, manifesting as acute stridor, severe respiratory compromise or symptoms of chronic airway compression. An accurate diagnosis of double aortic arch can be made prenatally by fetal echocardiography. In this report, we describe two infants with normal karyotypes in whom a prenatal diagnosis of double aortic arch was made at our fetal cardiology center, between July 2012 and August 2013. The diagnosis was confirmed postnatally by two-dimensional echocardiography and cardiac magnetic resonance imaging ( MRI). The presence of a right aortic arch should prompt more careful evaluation to exclude a double aortic arch. Postnatal assessment should comprise echocardiography and cross-sectional imaging in the form of cardiac MRI/computed tomography. Bronchoscopy may be considered to exclude subclinical airway compression and reduce potential long-term respiratory sequelae. Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd. [ABSTRACT FROM AUTHOR]

An abstract of the article "Use of customised birthweight centiles in fetuses with congenital heart disease," by L. Story, D. Pasupathy, S. Sankaran, P. Desai, G. Sharland and P. Kyle is presented.