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In spite of great advances in staged left ventricle recruitment strategy, some patients do not achieve biventricular circulation nor are candidates for reversal to single-ventricle palliation. We present a case of a successful reverse double switch operation in a patient with failure of left ventricle recruitment and pulmonary hypertension. This strategy provided a one-and-a-half repair with a sub-pulmonary hypoplastic left ventricle that improved the patient’s clinical status, becoming a novel alternative in this particular subset of patients.

The pandemic caused by the SARS-CoV-2 virus declared by the WHO in March 11th 2020, affects a small number of pediatric patients, who mostly present mild respiratory compromise and favorable evolution. However began to be observed in previously healthy children, an increase in cases defined as "Multisystemic Inflammatory Syndrome" (MIS-C) or "Kawasaki-like" post-COVID 19 (KLC) that evolve to shock and require hospitalization in the Pediatric Intensive Care Unit. MIS-C and KL-C are characterized by fever; signs of inflammation, gastrointestinal symptoms, and cardiovascular dysfunction, associated with sever forms of presentation with higher incidence of hypotension and/or shock. In the laboratory, markers of inflammation, hypercoagulability and myocardial damage are observed. Firstline drug treatment consists of intravenous immunoglobulin plus oral acetylsalicylic acid. A multidisciplinary approach is recommended for an accurate diagnosis and an early and effective treatment, in order to reduce morbidity and mortality.

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.

The pandemic caused by the SARS-CoV-2 virus declared by the WHO in March 11th 2020, affects a small number of pediatric patients, who mostly present mild respiratory compromise and favorable evolution. However began to be observed in previously healthy children, an increase in cases defined as "Multisystemic Inflammatory Syndrome" (MIS-C) or "Kawasaki-like" post-COVID 19 (KLC) that evolve to shock and require hospitalization in the Pediatric Intensive Care Unit. MIS-C and KL-C are characterized by fever; signs of inflammation, gastrointestinal symptoms, and cardiovascular dysfunction, associated with sever forms of presentation with higher incidence of hypotension and/or shock. In the laboratory, markers of inflammation, hypercoagulability and myocardial damage are observed. Firstline drug treatment consists of intravenous immunoglobulin plus oral acetylsalicylic acid. A multidisciplinary approach is recommended for an accurate diagnosis and an early and effective treatment, in order to reduce morbidity and mortality.La pandemia ocasionada por el nuevo coronavirus (SARS-CoV-2), declarada por la Organización Mundial de la Salud OMS) en marzo de 2020, afecta a un reducido número de pacientes pediátricos, quienes presentan, en su mayoría, compromiso respiratorio leve y evolución favorable. Sin embargo, en niños previamente sanos, comenzó a observarse un aumento de casos definidos como síndrome inflamatorio multisistémico (SIM-C) o similar a Kawasaki (Kawasaki-like) asociado a la enfermedad por el nuevo coronavirus (COVID-19) (KL-C) que evolucionan al shock y requieren internación en la unidad de cuidados intensivos. Los cuadros de SIM-C y los KL-C se caracterizan por fiebre, signos de inflamación, síntomas gastrointestinales y disfunción cardiovascular; las formas graves de presentación tienen mayor incidencia de hipotensión y/o shock. En el laboratorio se observan marcadores de inflamación, hipercoagulabilidad y daño miocárdico. El tratamiento farmacológico de primera línea consiste en la administración de inmunoglobulina por vía intravenosa más ácido acetilsalicílico por vía oral. Se recomienda un abordaje multidisciplinario para un diagnóstico certero y un tratamiento temprano y eficaz para disminuir la morbimortalidad.

Idiopathic dilatation of the right atrium is a rare condition. The real incidence of the disease is difficult to estimate since most patients are asymptomatic, although a wide spectrum of symptoms have been reported in children and adults. In the literature, prenatal diagnosis has been reported in few cases. We report a case of prenatally diagnosed right atrial dilation with a 3 years and 8 months follow up. The patient is on acetylsalicylic acid since birth and remained asymptomatic without any atrial thrombosis. We performed regular transthoracic echocardiograms during follow up, however was a cardiac magnetic resonance imaging the method for achieving diagnostic confirmation. The aim of this case report is to describe a rare, potentially serious disease and how prenatal diagnosis allows anticipated actions such as monitoring for cardiac arrhythmia detection and early starting of primary thrombosis prophylaxis.La dilatación idiopática de la aurícula derecha es una entidad poco frecuente. Estimar la verdadera incidencia es dificultoso dado que muchos pacientes son asintomáticos y los síntomas reportados, tanto en niños como en adultos, presentan un amplio espectro de manifestaciones clínicas. Pocos pacientes con diagnóstico prenatal fueron publicados. Se presenta un caso de diagnóstico prenatal de dilatación auricular derecha con un seguimiento de 3 años y 8 meses. El paciente se encuentra asintomático, sin evidencia de trombosis auricular, medicado con ácido acetilsalicílico desde su nacimiento. El seguimiento se realizó con ecocardiogramas regulares; sin embargo, la confirmación diagnóstica fue a través de la resonancia magnética nuclear cardíaca. La importancia del reporte radica en describir una enfermedad rara, potencialmente grave, y cómo el diagnóstico prenatal permite tomar conductas anticipadas, como el monitoreo cardíaco, para la detección de arritmias y el inicio temprano de profilaxis primaria antitrombótica.

OBJECTIVES We aimed to evaluate the effect of technical aspects of fetal aortic valvuloplasty (FAV) on procedural risks and pregnancy outcomes. BACKGROUND FAV is performed in cases of severe mid-gestation aortic stenosis with the goal of preventing hypoplastic left heart syndrome (HLHS). METHODS The International Fetal Cardiac Intervention Registry was queried for fetuses who underwent FAV from 2002 to 2018, excluding one high-volume center. RESULTS The 108 fetuses had an attempted cardiac puncture (mean gestational age [GA] 26.1 ± 3.3 weeks). 83.3% of attempted interventions were technically successful (increased forward flow/new aortic insufficiency). The interventional cannula was larger than 19 g in 70.4%. More than one cardiac puncture was performed in 25.0%. Intraprocedural complications occurred in 48.1%, including bradycardia (34.1%), pericardial (22.2%) or pleural effusion (2.7%) requiring drainage, and balloon rupture (5.6%). Death within 48 hr occurred in 16.7% of fetuses. Of the 81 patients born alive, 59 were discharged home, 34 of whom had biventricular circulation. More than one cardiac puncture was associated with higher complication rates (p

Among congenital malformations, heart defects are the most common type of anomaly, and these are associated with a high perinatal, longterm morbidity and mortality. The objective of this update was to review the rate of prenatal detection, screening characteristics throughout the pregnancy, in both the first and second trimesters, indications for advanced echocardiography, and to establish a management algorithm in case of prenatal diagnosis of a congenital heart disease. Potential invasive and non-invasive tests and obstetric follow-up will be discussed here. Finally, the main characteristics of fetal therapy in heart anomalies will be reviewed, both cardiac interventions and intrauterine treatment of arrhythmias.Dentro de las malformaciones congénitas, las cardiopatías son las anomalías más frecuentes y se asocian a una elevada morbimortalidad perinatal y a largo plazo. El objetivo de esta actualización es revisar la tasa de detección prenatal, las características del tamizaje a lo largo del embarazo, tanto en el primero como en el segundo trimestre, las indicaciones de ecocardiografía avanzada, y establecer un algoritmo de manejo ante el diagnóstico prenatal de una cardiopatía congénita. Se discutirán los estudios invasivos y no invasivos que pueden realizarse y el seguimiento obstétrico. Finalmente, se revisarán las características principales de la terapia fetal en anomalías cardíacas, tanto intervencionismo cardíaco como el tratamiento intrauterino de las arritmias.

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.

Background Invasive fetal cardiac intervention (FCI) has been reported in single-institution series, promoting technical and physiologic success. Objectives This study describes the creation of an international registry of cases presenting for FCI, intended to compile technical and outcome data from a multicenter cohort. Methods For this initial analysis, the entire database of the International Fetal Cardiac Intervention Registry (IFCIR) was queried for details of diagnoses, procedures, and outcomes. Maternal-fetal dyads from January 2001 through June 2014 were included. Results Eighteen institutions submitted data by data harvest. Of 370 cases entered, 245 underwent FCI: 100 aortic valvuloplasties from a previous single-center report (excluded from additional reporting here), an additional 86 aortic and 16 pulmonary valvuloplasties, 37 atrial septal cases, and 6 unclassified cases. FCI did not appear to affect overall survival to hospital discharge. Among live-born infants with a fetal diagnosis of aortic stenosis/evolving hypoplastic left heart syndrome, more than twice as many were discharged with biventricular circulation after successful FCI versus those meeting institutional criteria but without any or successful FCI (42.8% vs. 19.4%, respectively). When fetal deaths were counted as treatment failures, the percentages were similar: biventricular circulation at discharge was 31.3% versus 18.5% for those discharged with univentricular palliation. Survival to discharge for live-born fetuses with atrial restriction was similar to that of those undergoing technically successful versus unsuccessful FCI (63.6% vs. 46.7%, respectively), although criteria for diagnosis were nonuniform. Conclusions We describe the contents of the IFCIR and present post-natal data to suggest potential benefit to fetal therapy among pregnancies considered for possible intervention and support proposals for additional work.

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.