Prenatal detection of cardiac abnormalities has increased significantly over the past few decades, such that fetal cardiology has developed into a sub-specialty of paediatric and congenital cardiology. As this speciality develops further and extends across Europe and more globally, it is important to standardize the requirements for training and subsequent practice, to optimize prenatal diagnosis and perinatal care. In addition to the knowledge and technical skills required to make a correct diagnosis, the counseling of families after diagnosis and the planning of appropriate perinatal management is equally important. The aim of these recommendations is to provide a framework for both basic and advanced training for paediatric cardiologists wishing to practice as fetal cardiologists, as well as highlighting requisites for a fetal cardiology service. All aspects regarding training in fetal cardiology and service provision are addressed including diagnosis, counseling and management.

The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.

Objectives: The aim of this study was to determine the timing of neonatal cardiac intervention in babies with antenatally diagnosed congenital heart disease and the impact on obstetric management. Methods: A retrospective review of all deliveries between January, 2008 and December, 2009 was conducted in a tertiary centre with foetal and paediatric cardiology, maternal–foetal medicine, and obstetric units. All live births with antenatally detected congenital heart disease were included. Data were collected from foetal, paediatric cardiology, and maternity databases and records. Induction, delivery mode, and timing of the first cardiac intervention in the neonate were studied. Results: 205 deliveries were included. Induction and elective Caesarean section rates were 51.2% (105/205) and 14.1% (29/205), respectively. The vaginal delivery rate was 56% (115/205). There was a non-significant trend towards a higher rate of vaginal delivery after spontaneous labour than after induction (75% versus 66%; p = 0.234). The rate of neonatal cardiac intervention during the initial stay was 59.5% (122/205); it was 18.5% (38/205) within 48 hours and 25.8% (53/205) within 72 hours. The median time to first intervention was 4 days (interquartile range 2–8). Babies with hypoplastic left heart syndrome (median 3, interquartile range 2–6), transposition of the great arteries (median 1, interquartile range 0–4.5), and arrhythmia (median 0.5, interquartile range 0–1) had a significantly earlier time to first intervention compared with those with other conditions (p = 0.001). Conclusion: Vaginal delivery can be achieved in women delivering babies with major congenital heart disease at a tertiary centre. Delivery in or near a tertiary centre is recommended for patients requiring early intervention, of which many can be identified in advance.

A case of a complete atrioventricular septal defect was detected in a 20-week gestation fetus, in whom the ventricular component of the defect was subsequently noted to close spontaneously with advancing gestational age. The antenatal and postnatal findings are discussed.

A case of left ventricular obstruction is reported in a fetus of 21 weeks gestation. Obstruction to the left ventricular inflow and outflow tracts was documented by prenatal echocardiography and, at autopsy, the cause was found to be a large blood cyst. It is likely that the associated hypoplasia of the left heart was a result of reduced blood flow beyond this obstructive lesion.

In a series of over 7,000 pregnancies referred for fetal echocardiography, atrioventricular septal defects were detected in 103 fetuses, (17% of 594 anomalies). A large proportion (42%) also had isomerism of the atrial appendages, abnormalities in arrangement and morphology of the thoracoabdominal organs, and a high incidence of associated cardiac malformations. When this was associated with abnormalities of rhythm, the fetus often showed signs of intrauterine congestive heart failure; hydrops, pleural effusion and ascites. Those with trisomic karyotypes (predominantly trisomy 21) were associated with usual atrial arrangement and concordant connections of the cardiac segments. Of the 103 fetuses, 53 were examined at autopsy. Extracardiac abnormalities were documented, and detailed morphologic examination and measurements of the heart were made. The ratios of inlet to outlet septal disproportion were not significantly different from those found in postnatal hearts. Again, as in postnatal life, those hearts with common atrioventricular orifice had a greater degree of ‘scooping’ of the ventricular septum compared to those with separate right and left atrioventricular valves. There were no significant differences in these findings in relation to the karyotype of the fetus. There was an increased incidence of ventricular dominance, and also of coarctation or hypoplasia of the aortic arch, in fetal hearts when compared to autopsied hearts obtained in postnatal life. In summary, the distinguishing morphological characteristics of atrioventricular septal defect are present from 16 weeks of gestation.

Complex congenital heart defects are accurately detectable antenatally using fetal echocardiography. In continuing pregnancies with fetal cardiac abnormalities, regular follow-up with advancing gestation is essential. This report documents the changes that occurred in the fetal cardiac anatomy in a fetus detected during antenatal scanning to have an atrioventricular septal defect with isomerism of the left atrial appendages.

Twenty-six patients requiring palliation for the tetralogy of Fallot were treated by balloon dilation. The mean age at dilation was 8.5 months (range 0.5−20.6) and the mean weight 6.7 kg (range 2.6−10.7). Successful dilation was achieved in 25 patients. The mean ratio of the balloon to the diameter of the pulmonary outflow tract was 1.6 (range 1.2−2.0). The aortic oxygen saturation increased from mean±SD of 80±12.4% before to 91±4.3% after dilation (p<0.05). The pulmonary arterial oxygen saturation rose from 64±10.7% to 80±7.7% (p<0.05). Transient hypotension and arterial desaturation were noted in 19 patients during inflation of the balloon. One patient had a cardiac arrest prior to dilation and died afterwards. Seven patients had cyanotic spells related to the procedure. Attempts to cross the pulmonary valve resulted in infundibular perforation by the guide wire in one patient. Of the 24 survivors who initially had successful dilation procedures, three have needed a Blalock-Taussig shunt. Palliation without a shunt was achieved in the remaining 21 patients. In 12 patients, repeat angiography, 2.5−20 months (mean 9.1) after dilation, showed an increase in the diameter of the outflow tract from 7.1±2.1 mm before to 9.5±2.0 mm after dilation (p<0.05). Diameters of the pulmonary arteries increased from 6.3±2.2 mm on the right and 6.8±1.9 mm on the left before to 9.2±2.2 mm and 9.3±2.5 mm respectively after dilation (p<0.05 for both branches). Surgical correction has been achieved in 22 patients, 21 of these 2−22 months after balloon dilation and the other patient 20 months after construction of a shunt. At operation, the leaflets of the pulmonary valve were noted to be torn in five patients. In 12 patients, there was extensive scarring of the infundibulum. Balloon dilation of the right ventricular outflow tract achieves adequate palliation and may result in an increase in the diameter of the pulmonary valve and branches of the pulmonary trunk. It should be considered as an alternative to shunt procedures in children with tetralogy of Fallot.

One hundred-ninety-four fetuses with irregular heart rates were seen over a five-year period at a tertiary center for fetal cardiology. The median gestation at referral was 31 weeks, with a range from 19 to 41 weeks. Of these fetuses, 157 had extrasystoles of either atrial or ventricular origin. Blocked atrial ectopic beats had led to slow ventricular rates (80–110 beats per minute) in 37 fetuses. The structure of the heart was normal in all except two fetuses. Postnatal outcome was known for 165 of the fetuses. Of these, 157 (95%) had an uneventful antenatal and postnatal course. Tachyarrhythmias developed in eight fetuses (5%) in either the prenatal (n=4) or postnatal (n=4) period. Five of 37 fetuses with blocked atrial ectopic beats (13%) developed a tachyarrhythmia. No fetus developed hydrops, and all infants survived. All cases had required treatment with antiarrhythmic drugs. The occurrence of an irregular heart rhythm in the fetus, therefore, is not always benign. Fetuses with blocked atrial ectopic beats require particularly close monitoring.

Our aims were to examine the diagnostic accuracy of prenatal diagnosis of concordant atrioventricular and discordant ventriculo-arterial connections, the accuracy of predictions made concerning the postnatal surgical approach, and a description of the different subtypes related to outcome. All fetuses were evaluated at a tertiary centre for fetal cardiology between January, 1994, and December, 2003. In this period, we identified congenitally malformed hearts in 1,835 fetuses, of whom 56 (3%) met the criterions of inclusion. Of the total, 30 (54%) had an intact ventricular septum, 9 (16%) had an associated ventricular septal defect, 7 (13%) had a ventricular septal defect and pulmonary stenosis, 1 (2%) had pulmonary stenosis and an intact ventricular septum, 8 (14%) had a ventricular septal defect and aortic coarctation, and 1 (2%) had coarctation of the aorta with an intact ventricular septum. All the discordant ventriculo-arterial connections were correctly identified. For associated ventricular septal defects, the diagnostic sensitivity was 96%, with specificity of 88%, positive predictive value of 85%, and negative predictive value of 97%. For aortic coarctation, the sensitivity was 100%, specificity 96%, positive predictive value 82%, and negative predictive value 100%. The prediction of the surgical approach was accurate in 41 of 48 cases (85%). For those fetuses with pulmonary stenosis and ventricular septal defect, the ratio of the diameters of the pulmonary trunk was shown to be helpful in predicting the possibility of an arterial switch as opposed to the Rastelli type of repair. Of the 49 liveborn infants, 46 were alive at 30 days (94%, with 95% confidence intervals from 83 to 99%), and 43 at one year (88%, 95% confidence intervals from 75 to 95%). Deaths were mainly related to the anatomy of the coronary arteries, and associated cardiac lesions.

Objectives: To establish the prevalence of coronary arterial abnormalities in mid-trimester fetuses with pulmonary atresia with intact ventricular septum, and whether their presence correlates with right ventricular morphology. Background: The presence of coronary arterial fistulas significantly alters the surgical options and prognosis for patients with pulmonary atresia with intact ventricular septum. The lesion can reliably be diagnosed using fetal echocardiography, and further definition of the prognosis is important for counselling parents. Methods: We examined the hearts of 39 pathological specimens diagnosed during fetal life, 3 of whom died postnatally. Coronary arterial abnormalities were defined as non-connection of the left or right coronary arteries to the aorta, ostial stenosis, marked tortuosity, dilation, thickening or abnormal myocardial branching. Mild tortuosity, or myocardial bridging, were considered normal. We measured the dimensions of the tricuspid valve along with the inlet and outlet portions of the ventricles. Ebstein's malformation, tricuspid valvar dysplasia, and the presence or absence of the infundibulum, were especially noted. We examined also 12 normal hearts as controls. Results: Coronary arterial abnormalities were found in 14/39 (36%). The dimensions of the right ventricle and tricuspid valves, and the gestational ages of the fetuses, were compared for these 14 with the 25 having no abnormalities using independent t-tests. The gestational ages were similar, 21.9 vs 21.1 weeks. The mean dimensions of the tricuspid valve, median z-scores, and right ventricle were smaller, 2.9 vs 7.2 mm; p < 0.002; −4.46 vs 0.23; p < 0.03; and 6.9 vs 13.7 mm; p < 0.002, for those with coronary arterial abnormalities. Ebstein's malformation, or dysplasia of the tricuspid valve, was present in 4 of 14 with, vs 15 of 25 without, coronary arterial abnormalities. A patent infundibulum was noted in 34 of 39 specimens. Conclusions: Mid-trimester fetuses with pulmonary atresia with intact ventricular septum already exhibit coronary arterial abnormalities, with a prevalence of 36%. The presence of a patent infundibulum confirms that atresia of the pulmonary valve is an acquired process. Coronary arterial abnormalities are seen in 50% of those with hypoplastic right ventricles, but less frequently in the presence of well developed ventricles. This is important information for those involved in counselling parents.

Objectives: To describe the echocardiographic features, underlying causes, and outcome of fetuses with dilated cardiomyopathy. Design: A retrospective observational study between 1983 and 2003 at a tertiary centre for fetal cardiology. Patients: Affected fetuses were identified using a computerised database. We included fetuses with dilation and reduced systolic function of either the right ventricle, left ventricle, or both. We excluded fetuses with abnormal cardiac connections, arrhythmias, or stenosis of the aortic or pulmonary valves. In all, we identified 50 fetuses, born to 46 mothers. Of the fetuses, 24 had biventricular cardiomyopathy, 17 had isolated right ventricular cardiomyopathy, and 9 had isolated left ventricular cardiomyopathy. Two-thirds of the fetuses (32) were hydropic at some point during gestation. Main outcomes: A cause of cardiomyopathy was identified in 37 cases (74 per cent). This was genetic or metabolic in 11 fetuses; infective in 11; fetal anaemia, without proven parvovirus infection, in 5; of cardiac origin in 5; and an association with renal disease in 5. In 10 cases (20 per cent), the pregnancy was terminated. Based on an intention to treat, the survival to delivery was 25 of 40 (62.5 per cent, 95 per cent confidence intervals from 46 to 77 per cent), at 28 days was 17 of 40 (42.5 per cent, 95 per cent confidence intervals from 27 to 59 per cent), and at 1 year was 15 of 40 (37.5 per cent, 95 per cent confidence intervals from 23 to 54 per cent). The overall survival of non-hydropic fetuses was 9 of 18 (50 per cent), compared to 6 of 32 (18 per cent) hydropic fetuses. Conclusions: Genetic, metabolic, infective, and cardiac diseases may present with dilated cardiomyopathy during fetal life. There is a high rate of spontaneous intra-uterine and early neonatal death. The prognosis is particularly poor for hydropic fetuses.

Objective: To document the echocardiographic features of tetralogy of Fallot during fetal and postnatal life. Correlation of echocardiographic findings with the requirement for early intervention prior to definitive repair. Design: Retrospective observational study. Setting: A tertiary fetal cardiology unit. Patients: Fetuses with a diagnosis of tetralogy of Fallot identified from a prospective database between 1 January 1999 and 31 October 2002. Main measures of outcome: Growth of aorta and pulmonary trunk during fetal and postnatal life. Doppler assessment of the great arteries both prenatally and postnatally. Clinical outcome to definitive repair. Results: We identified 25 fetuses with tetralogy of Fallot, 23 having a pulmonary valvar diameter below the normal range at some point during gestation. The ratio of the diameter of the aortic to the pulmonary valve was abnormal in all cases. The pulmonary arterial Doppler velocity was within the normal range in six fetuses at presentation, and elevated in the remainder. In two fetuses, the right ventricular outflow tract was patent during fetal life, but had become atretic at birth. Both of these fetuses had reversal of flow in the arterial duct at presentation during fetal life. In 2 fetuses in whom we showed poor growth of the pulmonary trunk in late gestation, it was necessary to intervene early. The Doppler velocity across the pulmonary valve during fetal life did not differentiate between babies who required early intervention and those who were repaired electively. There was a marked increase in pulmonary arterial Doppler velocity following birth, which became more elevated with age. Of 18 liveborn infants, 17 have survived, with 2 having balloon dilation of the right ventricular outflow tract, and 3 insertion of a Blalock-Taussig shunt prior to definitive repair. Conclusions: In tetralogy of Fallot, features of pulmonary valvar hypoplasia and obstruction are evident during fetal life. Progression of obstruction in the right ventricular outflow tract was observed during fetal life as well as postnatally. Reversal of flow in the arterial duct, and failure of growth of the pulmonary trunk, predicted the need for early surgery to maintain pulmonary blood flow. Parents should be counselled about the possibility of emergency intervention being required after birth. Affected fetuses should be delivered at units with experience of managing the cyanosed newborn.