Utility of Fetal Echocardiography with Acute Maternal Hyperoxygenation Testing in Assessment of Complex Congenital Heart Defects.

Fetal echocardiography is an excellent tool for accurately assessing the anatomy and physiology of most congenital heart defects (CHDs). Knowledge gathered from a thorough initial fetal echocardiogram and serial assessment assists with appropriate perinatal care planning, resulting in improved postnatal outcomes. However, fetal echocardiography alone provides limited information about the status of the pulmonary vasculature, which can be abnormal in certain complex CHDs with obstructed pulmonary venous flow (hypoplastic left heart syndrome with restrictive atrial septum) or excessive pulmonary artery flow (d-transposition of the great arteries, usually with a restrictive ductus arteriosus). Fetuses with these CHDs are at high risk of developing severe hemodynamic instability with the immediate transition from prenatal to postnatal circulatory physiology at the time of birth. Adjunctive use of acute maternal hyperoxygenation (MH) testing in such cases can help determine pulmonary vascular reactivity in prenatal life and better predict the likelihood of postnatal compromise and the need for emergent intervention. This comprehensive review discusses the findings of studies describing acute MH testing in a diverse spectrum of CHDs and congenital diagnoses with pulmonary hypoplasia. We review historical perspectives, safety profile, commonly used clinical protocols, limitations, and future directions of acute MH testing. We also provide practical tips on setting up MH testing in a fetal echocardiography laboratory.

Current State of Fetal Heart Disease Counseling and Training: Room for Improvement? : Endorsed by the Fetal Heart Society.

We sought to describe the fellowship experiences and current practice habits of pediatric cardiologists who counsel patients with fetal heart disease (FHD) and to identify fellowship experiences related to FHD counseling perceived as valuable by respondents as well as opportunities for improvement. A cross-sectional survey of attending pediatric cardiologists who care for patients with FHD was performed. The respondents' demographics, fellowship experiences related to FHD counseling, reflections on fellowship training, and current practice habits were collected. The Fetal Heart Society endorsed this survey. There were 164 survey responses. 56% of respondents did not have 4th-year subspecialty training in fetal cardiology. Observing and performing FHD counseling were the most commonly used methods of training, with the highest perceived effectiveness. The number of counseling sessions observed and performed correlated moderately with confidence in FHD counseling skills at fellowship graduation. Extracardiac pathology and neurodevelopment were the least frequently addressed topics in fellowship training and in current practice. Fewer than 50% of respondents received formal education and feedback in counseling techniques during fellowship training. A significant proportion of practicing pediatric cardiologists provide FHD counseling with only standard categorical training. This highlights the potential importance of expanding FHD counseling education into categorical fellowship curricula. We suggest increasing opportunities for fellows to perform FHD counseling and receive feedback as this is a valued and beneficial experience during training. A formalized curriculum including extracardiac pathology and neurodevelopment and the use of evidence-based workshops in counseling techniques may address identified gaps in fellowship education.

Prenatal Maternal Hyperoxygenation Testing and Implications for Critical Care Delivery Planning among Fetuses with Congenital Heart Disease: Early Experience.

BACKGROUND: Maternal hyperoxygenation (MH) during fetal ultrasound can characterize fetal pulmonary vasoreactivity (PVr) and its associations with postnatal physiology. OBJECTIVE: We explored MH testing to facilitate perinatal risk stratification for fetuses with congenital heart disease (CHD). METHODS: MH was performed in 12 fetuses: 2 with Ebstein anomaly, 2 with total anomalous pulmonary venous connection (TAPVC), 4 with hypoplastic left heart syndrome (HLHS) with (a) restrictive atrial septum (RAS) or (b) intact atrial septum (IAS) with decompressing vertical vein (VV), and 4 with D-loop transposition of the great arteries (TGA). PVr and physiologic and anatomic changes with MH and outcomes were recorded. RESULTS: Among Ebstein fetuses, pulmonary blood flow with MH mirrored postnatal findings. Among TAPVC fetuses, MH VV gradients correlated with postnatal gradients. One HLHS/IAS/VV fetus had no PVr and decreased pulmonary vein forward to reverse velocity time integral ratio with MH. Shortly after delivery, the infant experienced severe low cardiac output and required urgent atrial septoplasty. The remaining HLHS fetuses had PVr and underwent routine Stage 1 Norwood. Among TGA fetuses, septum primum position, foramen ovale flow, and the presence or absence of PVr with MH reflected postnatal findings. CONCLUSION: MH may help identify fetuses with CHD at risk for perinatal compromise. Additional study may yield insights into fetal PVr and elucidate predictors of perinatal outcomes.

Prevalence and pattern of executive dysfunction in school age children with congenital heart disease.

OBJECTIVE: Executive function, a set of cognitive skills important to social and academic outcomes, is a specific area of cognitive weakness in children with congenital heart disease (CHD). We evaluated the prevalence and profile of executive dysfunction in a heterogeneous sample of school aged children with CHD, examined whether children with executive dysfunction are receiving school services and support, and identified risk factors for executive dysfunction at school age. DESIGN: Ninety-one school aged patients completed questionnaires, including the Behavior Rating Inventory of Executive Function (BRIEF) and a medical history questionnaire. An age- and gender- matched control sample was drawn from a normative database. RESULTS: Children with CHD had a higher rate of parent reported executive dysfunction (OR = 4.37, P < .0001), especially for working memory (OR = 8.22, P < .0001) and flexibility (OR = 8.05, P < .0001). Those with executive dysfunction were not more likely to be receiving school services (P > .05). Gender, premature birth (≤37 weeks), and CHD with aortic obstruction were predictive of executive dysfunction, especially for behavior regulation skills. CONCLUSIONS: School aged children with CHD have an increased prevalence of executive dysfunction, especially problems with working memory and flexibility, and are underserved by the school system. The increased risk for executive dysfunction in those with CHD and prematurity or CHD with aortic obstruction suggests an etiology of delayed brain development in the fetal and neonatal periods, while male gender may increase susceptibility to brain injury. This study highlights the need for regular neurodevelopmental follow up in children with CHD, and a need to better understand mechanisms that contribute to adverse neurodevelopmental outcomes.