Value of fetal echocardiographic examination in pregnancies complicated by preterm premature rupture of membranes.

OBJECTIVES: Cardiopulmonary and infectious complications are more common in preterm newborns after preterm premature rupture of membranes (pPROM). Fetal echocardiography may be helpful in predicting neonatal condition. Our aim was to assess the cardiovascular changes in fetuses from pregnancies complicated by pPROM and possible utility in predicting the intrauterine or neonatal infection, and neonatal heart failure (HF). METHODS: It was a prospective study enrolling 46 women with singleton pregnancies complicated by pPROM between 18+0 and 33+6 weeks of gestation and followed until delivery. 46 women with uncomplicated pregnancies served as a control group. Fetal echocardiographic examinations with the assessment of cardiac structure and function (including pulmonary circulation) were performed in all patients. RESULTS: Mean gestational age of pPROM patients was 26 weeks. Parameters suggesting impaired cardiac function in fetuses from pPROM were: higher right ventricle Tei index (0.48 vs. 0.42 p<0.001), lower blood flow velocity in Ao z-score (0.14 vs. 0.84 p=0.005), lower cardiovascular profile score (CVPS), higher rate of tricuspid regurgitation (18.2 % vs. 4.4 % p=0.04) and pericardial effusion (32.6 vs. 0 %). Intrauterine infection was diagnosed in 18 patients (39 %). 4 (8.7 %) newborns met the criteria of early onset sepsis (EOS). HF was diagnosed in 9 newborns. In fetal echocardiographic examination HF group had shorter mitral valve inflow time and higher left ventricle Tei index (0.58 vs. 0.49 p=0.007). CONCLUSIONS: Worse cardiac function was observed in fetuses from pPROM compared to fetuses from uncomplicated pregnancies.

Intrapartum cardiotocography in pregnancies with and without fetal CHD.

OBJECTIVES: Congenital heart defects (CHD) are the most common inherited abnormalities. Intrapartum cardiotocography (CTG) is still considered a "gold standard" during labor. However, there is a lack of evidence regarding the interpretation of intrapartum CTG in fetuses with CHD. Therefore, the study aimed to compare intrapartum CTG in normal fetuses and fetuses with CHD and describe the association between CTG and neonatal outcomes. METHODS: The present study is a retrospective analysis of the CTG of 395 fetuses. There were three study groups: Group 1: 185 pregnancies with a prenatal diagnosis of CHD, Group 2: 132 high-risk pregnancies without CHD, and Group 3: 78 low-risk pregnancies without CHD. RESULTS: Abnormal CTG was present statistically OR=3.4 (95%CI: 1.61-6.95) more often in Group 1. The rate of the emergency CS was higher in this group OR=3 (95%CI: 1.3-3.1). Fetuses with CHD and abnormal CTG were more often scored ≤7 Apgar, with no difference in acidemia. The multivariate regression model for Group 1 does not show clinical differences between Apgar scores or CTG assessment in neonatal acidemia prediction. CONCLUSIONS: CTG in fetuses with CHD should be interpreted individually according to the type of CHD and conduction abnormalities. Observed abnormalities in CTG are associated with the fetal heart defect itself. Preterm delivery and rapid cesarean delivery lead to a higher rate of neonatal complications. Health practitioners should consider this fact during decision-making regarding delivery in cases complicated with fetal cardiac problems.

Tetralogy of Fallot in the fetus - from diagnosis to delivery. 18-year experience of a tertiary Fetal Cardiology Center.

BACKGROUND: Tetralogy of Fallot (TOF) is a common congenital heart disease but very heterogeneous in terms of detailed cardiac anatomy, associated malformations, and genetic anomalies, especially when assessed prenatally. AIMS: We aimed to analyze the clinical spectrum of TOF in the prenatal period, including detailed cardiac morphology, coexisting anomalies, and their impact on short-term neonatal outcome. We also assessed changing trends in the prenatal diagnostic workup of TOF. METHODS: A retrospective cohort study including fetuses diagnosed with TOF between 2002 and 2019 was conducted in a tertiary Fetal Cardiology Center. Medical records and echocardiographic examinations were reviewed to collect demographic, sonographic, and genetic data. RESULTS: Among 326 TOF fetuses, 237 (73%) had pulmonary stenosis (TOF-PS), 72 (22%) pulmonary atresia (TOF-PA), and 17 (5%) absent pulmonary valve (TOF-APV). The yearly number of diagnoses increased during the study period, with decreasing fetal age at the time of diagnosis. Extracardiac malformations were found in 172 (53%) fetuses, cardiovascular malformations in 159 (49%), and genetic anomalies in 99 (39% of the tested group). Hypoplastic thymus, right aortic arch, and polyhydramnios were sonographic markers of microdeletion 22q11. Left-to-right ductal flow was predictive of postnatal ductal dependency. The perinatal outcome was dependent on the presence of associated anomalies and disease subtype, with TOF-APV having the worst prognosis. CONCLUSIONS: Extracardiac and genetic anomalies are common in fetuses with TOF, and, together with disease subtype and ductal flow assessment, they impact the perinatal management and outcomes. Genetic testing with array comparative genomic hybridization should be offered in all cases.

Paediatric and adult congenital cardiology education and training in Europe.

BACKGROUND: Limited data exist on training of European paediatric and adult congenital cardiologists. METHODS: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries. RESULTS: Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87-9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63-10.72 million), and one training centre per 4.29 million population (range 1.63-10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1-17), and duration of training was 3 years (range 2-5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R2 = 0.41). CONCLUSION: Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.

Liver herniation into the pericardium mimicking a pericardial tumor: unusual presentation of trisomy 13.

Aim of the study: Trisomy 13 is the third most common autosomal trisomy. The following case report shows an atypical case of trisomy 13, highlighting the usefulness of 3D volume storage and reconstruction, and the necessity of careful interpretation of the first trimester screening results. Case description: The results of the first trimester screening tests were interpreted as normal, and invasive tests were not recommended. At 21 weeks, a bright spot in the left ventricle was noted, and fetal echocardiography was performed at 33 weeks. The scan showed a massive pericardial effusion and a pericardial tumor located in front of the right ventricle. Conclusions: The final diagnosis, made postnatally, revealed an atypical right-sided diaphragmatic hernia. Part of the liver was displaced to the pericardial cavity, mimicking a pericardial tumor in a baby with trisomy 13. Following the diagnosis of the lethal disorder, the baby was discharged under a home-based palliative care program and died on the 49th day of life.

Fetal Cardiac Interventions-Are They Safe for the Mothers?

The aim of fetal cardiac interventions (FCI), as other prenatal therapeutic procedures, is to bring benefit to the fetus. However, the safety of the mother is of utmost importance. The objective of our study was to evaluate the impact of FCI on maternal condition, course of pregnancy, and delivery. 113 mothers underwent intrauterine treatment of their fetuses with critical heart defects. 128 percutaneous ultrasound-guided FCI were performed and analyzed. The patients were divided into four groups according to the type of FCI: balloon aortic valvuloplasty (fBAV), balloon pulmonary valvuloplasty (fBPV), interatrial stent placement (IAS), and balloon atrioseptoplasty (BAS). Various factors: maternal parameters, perioperative data, and pregnancy complications, were analyzed. There was only one major complication-procedure-related placental abruption (without need for blood products transfusion). There were no cases of: procedure-related preterm prelabor rupture of membranes (pPROM), chorioamnionitis, wound infection, and anesthesia associated complications. Tocolysis was only necessary only in two cases, and it was effective in both. None of the patients required intensive care unit admission. The procedure was effective in treating polyhydramnios associated with fetal heart failure in six out of nine cases. Deliveries occurred at term in 89%, 54% were vaginal. The results showed that FCI had a negligible impact on a further course of pregnancy and delivery.

Fetal Cardiac Interventions-Polish Experience from "Zero" to the Third World Largest Program.

This article presents the technical aspects of the Polish fetal cardiac interventions (FCI) program, including preparation of the team and modifications in the technique of the procedure that aim to increase its safety for the mother and the fetus. Over 9 years, 128 FCI in 113 fetuses have been performed: 94 balloon aortic valvuloplasties (fBAV), 14 balloon atrioseptoplasties (fBAS) with stent (BAS+), 5 balloon atrioseptoplasties without stent placement (BAS-), and 15 fetal pulmonary valvuloplasties (fBPS). The technical success rate ranged from 80% (BAS-) to 89% (fBAV), while the procedure-related death rate (defined as death within 72 hours following the procedure) ranged from 7% (fBAV and fBPV) to 20% (BAS). There were 98 live births after all FCI (3 pregnancies continue). Median gestational age at delivery was 39 weeks in our center and 38 weeks in other centers.

Fetal Cardiac Intervention for Pulmonary Atresia with Intact Ventricular Septum: International Fetal Cardiac Intervention Registry.

INTRODUCTION: Invasive fetal cardiac intervention (FCI) for pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (PS) has been performed with small single-institution series reporting technical and physiological success. We present the first multicenter experience. OBJECTIVES: Describe fetal and maternal characteristics of those being evaluated for FCI, including pregnancy/neonatal outcome data using the International Fetal Cardiac Intervention Registry (IFCIR). METHODS: We queried the IFCIR for PAIVS/PS cases evaluated from January 2001 to April 2018 and reviewed maternal/fetal characteristics, procedural details, pregnancy and neonatal outcomes. Data were analyzed using standard descriptive statistics. RESULTS: Of the 84 maternal/fetal dyads in the registry, 58 underwent pulmonary valvuloplasty at a median gestational age of 26.1 (21.9-31.0) weeks. Characteristics of fetuses undergoing FCI varied in terms of tricuspid valve (TV) size, TV regurgitation, and pulmonary valve patency. There were fetal complications in 55% of cases, including 7 deaths and 2 delayed fetal losses. Among those who underwent successful FCI, the absolute measurement of the TV increased by 0.32 (±0.17) mm/week from intervention to birth. Among 60 liveborn with known outcome, there was a higher percentage having a biventricular circulation following successful FCI (87 vs. 43%). CONCLUSIONS: Our data suggest a possible benefit to fetal therapy for PAIVS/PS, though rates of technically unsuccessful procedures and procedure-related complications, including fetal loss were substantial. FCI criteria are extremely variable, making direct comparison to nonintervention patients challenging and potentially biased. More uniform FCI criteria for fetuses with PAIVS/PS are needed to avoid unnecessary procedures, expose only fetuses most likely to sustain a benefit, and to enable comparisons to be made with nonintervention patients.

Complete closure of the ductus arteriosus in the foetus with transposition of the great arteries.

Prenatal restriction of the ductus arteriosus can manifest as persistent pulmonary hypertension in the newborn, especially dangerous with the transposition of the great arteries. Its aetiology has long been related to maternal intake of non-steroidal anti-inflammatory drugs; however, some other substances, including polyphenols, may have similar properties. We describe a case of complete prenatal closure of the ductus arteriosus in the foetus with transposition of the great arteries. The newborn presented with pulmonary hypertension unresponsive to pharmacotherapy and died of multi-organ failure.

Spontaneous resolution of prenatally diagnosed isolated pleural effusion: An unusual early sign of a newborn disease.

We present a case report of a fetus with a diagnosed pleural effusion in the first trimester on nuchal translucency scan. The effusion resolved spontaneously by 17 weeks of pregnancy. Toxoplasmosis, rubella, cytomegalovirus, herpes simplex (TORCH) - negative. Array comparative genomic hybridization (aCGH) - normal. Serial Doppler scans normal - no prenatal signs of anemia. Maternal antibodies against red cell antigens - negative. Delivery at term by cesarean section because of macrosomia. Neonate suffered from prolonged jaundice. At 3 weeks of life diagnosed with hereditary spherocytosis. Literature review shows that this may be the first connection between this disease and prenatal life.

Fetal echocardiography gives a clue for the maternal diagnosis of tuberous sclerosis complex.

The detection of multiple cardiac tumors during fetal echocardiography allowed us to make the diagnosis of tuberous sclerosis complex in the mother and establish the reason of her first epileptic seizures.

Echocardiographic analysis of left ventricular function in term and preterm neonates at week 40 of postconceptional life.

BACKGROUND Both gestational and chronological age of the neonate may influence and impair the function of the delicate and immature myocardium. However, the transition from fetal to neonatal circulation in preterm neonates is poorly understood. AIMS This study aimed to compare left ventricular (LV) systolic and diastolic function between premature neonates at expected term and term neonates during the postnatal cardiovascular transitional period. METHODS Using echocardiography, we assessed systolic and diastolic function of the LV in 89 preterm neonates at week 40 of postconceptional age and 29 term neonates after closure of the patent ductus arteriosus (PDA) and on the 28th day of life. Based on M­mode images, we measured myocardial thickness and fractional shortening (FS%). Using pulsed­wave Doppler echocardiography, we estimated cardiac output, myocardial performance index (MPI), and LV diastolic function (E and A waves, E/A ratio). Systolic and diastolic function was also assessed by tissue Doppler imaging. RESULTS Compared with term neonates on the 28th day of life, preterm neonates had reduced myocardial thickness (P ≤0.04), FS% (P = 0.002), and cardiac output (P = 0.01). However, preterm neonates had a lower MPI than term neonates after PDA closure (P <0.001) and on the 28th day of life (P = 0.02). The E/A ratio and S' wave values were similar in preterm and term neonates (P >0.05). CONCLUSIONS Preterm neonates at 40 weeks of postconceptional age have preserved systolic and diastolic function of the LV.

Foetal goitrous hypothyroidism - easy to recognise, difficult to treat. Is combined intra-amniotic and intravenous L-thyroxine therapy an option?

INTRODUCTION: Foetal hypothyroidism negatively impacts somatic and neurological child development and can be the cause of serious obstetric and perinatal complications. We present a rare case of a large foetal dyshormonogenetic goitre, causing foetal neck hyperexten-sion, oesophageal compression, and cardiac high-output failure. MATERIAL AND METHODS: A foetal goitre complicated by cardiomegaly and polyhydramnios was diagnosed at 23 weeks of gestation (WG) on a routine ultrasonographic (US) assessment in a healthy nullipara. Foetal blood sampling was performed and a severe foetal hypothyroid-ism was diagnosed. Treatment was undertaken with an intra-amniotic followed by combined intra-amniotic and intravenous injections of L-thyroxine (L-T4). A total of 11 doses of L-T4 were administered between 24-37 WG to the foetus. RESULTS: A complete regression of foetal goitre, cardiomegaly, and polyhydramnios was observed. At 38 WG the patient delivered vagi-nally a male infant with mild hypothyroidism and no signs of goitre or cardiomegaly on postnatal US. Neurological development of the one year old baby is normal. CONCLUSIONS: The effective diminishing of serum TSH concentration and goitre size was reached after combined intra-amniotic and in-travenous L-T4 injections were given. L-T4 requirement in the foetus is equal to or above 15 µg/kg daily and should be given in weekly intervals due to its rapid metabolism by the foetus and by placental type 3 deiodinase. Intra-amniotic L-T4 administration may be inef-fective when a large goitre indisposes amniotic fluid swallowing by the foetus, so then the combined L-T4 injections into the umbilical vein and intra-amniotically in experienced hands seems to be a reasonable and effective option.

Early vesico-amniotic shunting - does it change the prognosis in fetal lower urinary tract obstruction diagnosed in the first trimester?

OBJECTIVES: The aim of the study was to assess the outcome of vesico-amniotic shunting performed before 16 weeks of pregnancy in fetuses with severe megacystis diagnosed in the first trimester of pregnancy. MATERIAL AND METHODS: Between January 2008 and October 2012 severe megacystis with the bladder length > 15 mm was diagnosed in 17 fetuses. The procedure of early vesico-amniotic shunting (VAS) was offered to 8 patients with presumably isolated LUTO. The procedure of VAS was performed in 6 fetuses. Before the intervention one or two procedures of vesicocentesis and urine analysis were performed. RESULTS: In all treated cases shunts provided urinary tract decompression. All babies were born prematurely, 2 of them died due to premaurity, 3 of them survived and have normal renal function at the age of 5-6 years. In 4/5 children accompanying malformations were later diagnosed, in 1 born prematurely neonate necropsy was not performed. CONCLUSIONS: Our results suggest that early vesico-amniotic shunting in fetal LUTO is feasible and may potentially prevent not only pulmonary hypoplasia but also renal insufficiency. However, the rationale of the procedure needs further investigation due to a high risk of long-term morbidity and co-existing malformations in children Before offering the therapy detailed counseling of the parents about the possible pros and cons of the therapy is necessary.