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.

Introduction Aorto‐left ventricular tunnel (ALVT) accounts for <0.1% of congenital heart defects. Evidence on the prognosis from a fetal perspective is limited. With this retrospective international case series, we provide information on the outcome of fetuses with ALVT. Methods All members of the Association for European Pediatric and Congenital Cardiology's (AEPC) fetal working group and fetal medicine units worldwide were invited for participation. We observed antenatal parameters, neonatal outcome and postnatal follow‐up. Additionally, a systematic search of the literature was performed. Results Twenty fetuses with ALVT were identified in 10 participating centers (2001–2019). Fetal echocardiographic characteristics of ALVT included an increased cardiac–thorax ratio (95%), left ventricular end‐diastolic diameter (90%) and a dysplastic aortic valve (90%). Extracardiac malformations were rare (5%). Eight fetuses died at a median gestational age (GA) of 21 + 6 weeks (range, 19–24): all showed signs of hydrops prior to 24 weeks or at autopsy. All others (60%, 12/2) were live–born (median GA 38 + 4, range 37–40), underwent surgery and were alive at last follow up (median 3.2 years, range 0.1–17). The literature reported 22 ALVT fetuses with similar outcome. Conclusions In the absence of fetal hydrops, ALVT carries a good prognosis. Fetuses who survive to 24 weeks without hydrops are likely to have a good outcome.

Myocardial ischemia caused by microvascular dysfunction is an important pathophysiologic component of hypertrophic cardiomyopathy (HCM), promoting myocardial fibrosis, adverse left ventricular remodeling, and impacting on clinical course and outcome in HCM patients. The aim of study was to assess the prevalence and clinical significance of myocardial ischemia in children with HCM using 99mTc-MIBI single-photon emission computed tomography (SPECT). Ninety-one children with HCM, median age 13.6 years, underwent SPECT evaluation from 2006 to 2017. Imaging was performed at rest and after maximal exercise. Myocardial perfusion defects were identified in 70 children (76.9%; group I), median age 13.8 years. Fixed perfusion defects were evident in 22 of them, while reversible at rest in 48. In 21 children (23.1%; group II), median age 11 years, myocardial perfusion defects were not detected. Patient demographics, echocardiography, resting electrocardiogram (ECG), 24-h Holter ECG, myocardial fibrosis in cardiovascular magnetic resonance imaging, and cardiovascular events were analyzed and compared between the groups. During follow-up at a median of 8.3 years in children with myocardial ischemia, clinical endpoints occurred more often (47 vs. 5; p = 0.02) and more patients reached a clinical endpoint (28 [40%] vs. 3 [14.3%]; p = 0.036). In children with myocardial ischemia, myocardial fibrosis was observed with greater frequency. Myocardial perfusion defects may reflect an ischemic process which (1) affects the clinical manifestations and (2) is an important predictor of adverse clinical events and risk of death in children with HCM. Myocardial ischemia in HCM patients frequently correlates with myocardial fibrosis.

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 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.

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.

Functional analysis of the fetal cardiovascular system is crucial for the assessment of fetal condition. Evaluation of the right ventricle with standard 2D echocardiography is challenging due to its complex geometry and irregular muscle fibers arrangement. Software package TOMTEC 4D RV-Function is an analysis tool which allows assessment of right ventricular function based on volumetric measurements and myocardial deformation. The aim of this study was to determine the feasibility of this method in fetal echocardiography. The retrospective study was conducted in the high-flow Referral Center for Fetal Cardiology. We recorded 4D echocardiographic sequences of 46 fetuses with normal hearts. Following parameters were calculated: end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV) and ejection fraction (EF), right ventricle longitudinal free-wall (RVLS free-wall) and septal strain (RVLS septum). Tei index was calculated as a standard measure or RV function for comparison. 4D assessment was feasible in 38 out of 46 fetuses (83%). RV volumetric parameters—EDV, ESV and SV—increased exponentially with gestational age. Functional parameters—RV Tei index, EF and strains—were independent of gestational age. Mean EF was 45.2% (± 6%), RV free-wall strain was − 21.2% and RV septal strain was − 21.5%. There was a statistically significant correlation between septal and free-wall strains (r = 0.51, p = 0.001) as well as between EF and RV free-wall strain (r = − 0.41, p = 0.011). 4D RV assessment is feasible in most fetuses. Its clinical application should be further investigated in larger prospective studies.

ObjectivesLiterature on cerebroplacental hemodynamics in fetuses with transposition of the great arteries (TGA) is scarce and provides conflicting results regarding possible brain‐sparing effect. Therefore, the aims of our study were to examine the Doppler parameters of the middle cerebral artery (MCA) and the umbilical artery (UA) in a large cohort of fetuses with TGA, and to assess their possible utility in predicting urgent balloon atrial septostomy (BAS) in neonates.MethodsA retrospective observational study including fetuses diagnosed with TGA between 2008 and 2022 and age‐matched normal cohort was conducted in a single tertiary Fetal Cardiology Center. Medical records and echocardiographic examinations were reviewed to collect demographic, sonographic and follow‐up data. Selected Doppler parameters were compared between TGA and normal fetuses, as well as between TGA with and without associated ventricular septal defect (VSD) to assess the impact of this congenital heart defect on cerebroplacental circulation. Additionally, Doppler indices in patients with restrictive foramen ovale (FO) were analyzed to search for potential predictors of urgent BAS. Statistical analyses were performed with Statistica 13 software using descriptive statistics, T‐Student test or U‐Mann‐Whitney test for comparisons and ROC curves to assess predictive value.Results541 examinations of 159 fetuses with TGA (performed between 19 and 40 weeks of pregnancy) and 1300 examinations of age‐matched normal fetuses were included in the study. MCA PI and UA PI followed expected trends throughout pregnancy, with slightly higher values in TGA fetuses, however within the limits for normal population. Cerebroplacental ratio (CPR) values were similar in normal and TGA fetuses. Presence of small VSD did not have clinically important impact on Doppler parameters. Peak systolic velocity (PSV) in the MCA gradually increased after 35 weeks of pregnancy, especially in fetuses who did not develop restriction of the FO after birth. MCA PSV values below 1.16 multiples of median (MoM) measured at 38 weeks of pregnancy or later predicted the need for urgent BAS with 81.4% sensitivity and 52.4% specificity.ConclusionsMCA PI, UA PI, and CPR values in fetuses with TGA usually fall within normal limits throughout pregnancy. Presence of small coexisting VSD does not affect the Doppler parameters significantly. MCA PSV increases in TGA fetuses after 35 weeks of pregnancy, and its value measured in the last prenatal study (optimally after 37 weeks) may serve as an additional predictive factor for urgent BAS.This article is protected by copyright. All rights reserved.

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.