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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.

Fetal aortic valvuloplasty (FAV) aims at modifying the progression of aortic stenosis evolving to hypoplastic left heart syndrome (Donofrio et al. Circulation 129(21):2183–2242, 2014). In the last decades, the observation of mid-term outcome of the patients that have been intervened prenatally has allowed further understanding of the disease and the effects of FAV, as well as of postnatal treatment of these patients. FAV is also being studied with animal models, but still with limited results. Given the small number of cases, multicenter studies are ongoing. We present a review of the most recently reported data and central aspects of our experience in the procedure.

ObjectivesTo develop a prediction model based on echocardiographic findings to estimate the probability of the need for neonatal cardiac invasive therapy, including cardiac surgery or catheter-based therapy, in foetuses with CHD.MethodsRetrospective cohort study: a prediction model was developed based on echocardiographic findings on the examination of the four-chamber, the three-vessel, and the three-vessel and tracheal views. We assessed performance using the area under the curve of the receiver operating characteristic.ResultsAmong 291 patients with prenatal diagnosis of CHD and complete follow-up, 175 (60.1%) required neonatal cardiac invasive therapy. The variables “functionally single ventricle”, “great artery reverse flow”, and “congenital heart block” had a discrimination value of 100% and were excluded from the model. In univariate and multivariate analysis, “non-visualisation of a great vessel”, “asymmetry of the great vessels”, “visualisation of one atrioventricular valve”, and “ventricular asymmetry” were significantly associated with the need for neonatal cardiac invasive therapy. The area under the receiver operating characteristic curve was 0.9324 (95% CI 0.92–0.97).ConclusionsA prediction model based on echocardiographic findings in foetuses with CHD, even without a definite diagnosis, allows an accurate estimation of the probability of requiring neonatal cardiac invasive therapy. This can modify patient care, especially in regions where a Foetal Medicine Specialist or a Paediatric Cardiologist is not available and referral may be extremely difficult due to social and economic barriers.

Fetal aortic valvuloplasty is intended to alter the natural history of aortic stenosis evolving to hypoplastic left heart syndrome. The most recently reported data and advances on this procedure were reviewed.The highlights of the latest experience are the advances in further understanding of the prenatal and postnatal natural history of this disease, and the way in which fetal aortic valvuloplasty impacts on it, the identification of new predictors of biventricular outcome, and the report of postnatal survival of intervened patients. Recently reported short-term and middle-term results are encouraging. Experimental research on procedural aspects is ongoing, with no definite results. Multicenter studies are also ongoing.In recent years, there have been advances in the understanding of the prenatal and postnatal process of aortic stenosis evolving to hypoplastic left heart syndrome and the effects of fetal aortic valvuloplasty, as well as the need of adequate postnatal therapeutic strategies for these patients. Procedural aspects are being studied with animal models, but still need far more experience before human application. Long-term results are still to be discovered, and multicenter studies may provide a new perspective. Continuing research is mandatory so that ultimately fetal heart intervention finds its place among the therapeutic resources for congenital heart disease.

ObjectivesFoetal aortic valvuloplasty has been proposed as a strategy to improve left heart growth and function in foetuses with severe aortic stenosis at risk of progression to hypoplastic left heart syndrome. We report our experience with this intervention.Methods and resultsBetween 2005 and 2010, five foetuses with aortic stenosis and at risk of progression to hypoplastic left heart syndrome underwent ultrasound-guided percutaneous foetal aortic valvuloplasty. There were no associated maternal complications or foetal demise. In one case, the pregnancy was terminated a couple of weeks after the intervention, one foetus evolved to hypoplastic left heart syndrome, and three did not.ConclusionsFoetal aortic valvuloplasty seems to be a safe and feasible procedure. It has been reported that it has the potential to prevent progression to hypoplastic left heart syndrome in selected foetuses with severe aortic stenosis. Further investigation regarding physiological and clinical aspects of this disease both prenatally and postnatally will probably allow to improve therapeutic strategies and clinical outcome.