Anita J Moon-Grady

UCSF University of California, San Francisco | UCSF · Department of Pediatrics

Introduction:: The management of anti-Ro/SSA positive pregnancies is challenging because fetal atrioventricular (AV) block is rare and fetal echo seldom detects reversible injury. Hypothesis: We hypothesized that ambulatory fetal heart rate/rhythm monitoring improves detection of fetal AV block when compared with echo. Methods: Anti-Ro antibody sub...

Objective This prospective study of pregnant patients, Surveillance To Prevent AV Block Likely to Occur Quickly (STOP BLOQ), addresses the impact of anti‐SSA/Ro titers and utility of ambulatory monitoring in the detection of fetal second‐degree atrioventricular block (AVB). Methods Women with anti‐SSA/Ro autoantibodies by commercial testing were s...

Introduction Fetal cardiac disease is strongly associated with maternal anti-SSA/Ro antibodies, but gaps in our knowledge include the influence of antibody specificity and titer, maternal diagnosis, overall non-cardiac adverse pregnancy outcomes (APOs), optimal surveillance protocols, and efficacy of rapid treatment. Methods The multi-center S ur...

BACKGROUND Echocardiographic measurements carry the promise of improving inter-rater agreement (IR) over subjective assessment. In this study we assess the effect of implementing a measurement-based protocol on the IR agreement and accuracy in reporting of right ventricular (RV) systolic pressure in children. We also evaluate the effect of this rep...

Background: Evidence based protocols for surveillance and treatment of fetal anti-Ro/ SSA antibody mediated AV block (AVB) are lacking. In September 2021 we initiated STOP BLOQ (Surveillance To Prevent AV Block Likely to Occur Quickly), a 3-step open label clinical trial including risk stratification by antibody titer, fetal heart rate monitoring (...

Objective This tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle. Methods This retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tert...

Background Impairments in fetal oxygen delivery have been implicated in brain dysmaturation seen in congenital heart disease (CHD), suggesting a role for in utero transplacental oxygen therapy. We applied a novel imaging tool to quantify fetal cerebral oxygenation by measuring T2* decay. We compared T2* in fetuses with CHD with controls with a focu...

Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge...

Objectives: Fetal cerebral vascular resistance is influenced by several factors in the setting of intact autoregulation to allow for normal cerebral blood flow and oxygenation. Maternal hyperoxia (MH) testing allows for acute alterations in fetal physiology and can be a tool to test cerebral vascular reactivity in late gestation fetuses. We utiliz...

( Am J Obstet Gynecol . 2019;220:494.e1–494.e7) Open maternal-fetal surgery (OMFS) for fetal myelomeningocele (fMMC) is associated with a significant reduction in morbidity related to spina bifida. OMFS for fMMC may be associated with adverse outcomes in subsequent pregnancies, but there are limited data available. There is a uterine rupture risk o...

Background/Purpose The Spitz classification for esophageal atresia with/without tracheoesophageal fistula (EA/TEF) predicts mortality. This study evaluates the contemporary relevance of the Spitz classification and investigates predictors of morbidity. Methods EA/TEF patients born between 1995 and 2018 at two centers were retrospectively reviewed....

Background Infants with critical congenital heart disease ( CCHD ) are more likely to be small for gestational age ( SGA ) or born to mothers with maternal placental syndrome. The objective of this study was to investigate the relationship between maternal placental syndrome, SGA , and gestational age ( GA ) on 1‐year mortality in infants with CCHD...

Pathogenic DNM1L mutations cause a mitochondrial disorder with a highly variable clinical phenotype characterized by developmental delay, hypotonia, seizures, microcephaly, poor feeding, ocular abnormalities, and dysarthria. We report the case of an 8‐month‐old female with autosomal dominant, de novo DNM1L c. 1228G>A (p. E410K) mutation and mitocho...

Background: Open maternal-fetal surgery for fetal myelomeningocele results in reduction in neonatal morbidity related to spina bifida but may be associated with fetal, neonatal, and maternal complications in subsequent pregnancies. Objective: The objective of this study was to ascertain obstetric risk in subsequent pregnancies after open materna...

Background Despite improvements in fetal survival for pregnancies that are affected by twin-twin transfusion syndrome since the introduction of laser photocoagulation, prematurity remains a major source of neonatal morbidity and death. Objective To investigate the indications and factors that influence the timing of delivery after laser treatment,...

Background: Fetal atrioventricular block (AVB) occurs in 2% to 4% of anti-Ro antibody–positive pregnancies and can develop in <24 h. Only rarely has standard fetal heart rate surveillance detected AVB in time for effective treatment. Objectives: Outcome of anti-Ro pregnancies was surveilled with twice-daily home fetal heart rate and rhythm monitori...

Background Racial/ethnic and socioeconomic disparities exist in outcomes for children with congenital heart disease. We sought to determine the influence of race/ethnicity and mediating socioeconomic factors on 1‐year outcomes for live‐born infants with hypoplastic left heart syndrome and dextro‐Transposition of the great arteries. Methods and Res...

Background Infants with critical congenital heart disease (CCHD) are more likely to be small for gestational age (GA). It is unclear how this affects mortality. The authors investigated the effect of birth weight Z score on 1‐year mortality separately in preterm (GA <37 weeks), early‐term (GA 37–38 weeks), and full‐term (GA 39–42 weeks) infants wit...

Genetic, epigenetic, intrauterine, and extrauterine factors impact the embryogenesis, development, and function of the human cardiovascular system. Although many of these interactions are incompletely understood, a number of maternal, placental, and fetal conditions that disrupt normal fetal cardiac development and/or function have been identified....

Fetal echocardiography facilitates the prenatal diagnosis of infants with congenital heart disease (CHD) and through sequential examinations, allows assessment of fetal hemodynamics and cardiovascular status from the time of diagnosis to delivery. Fetal cardiologists have created diagnostic protocols aimed at risk stratifying severity and potential...

Background and objectives: It is unknown how gestational age (GA) impacts neonatal morbidities in infants with critical congenital heart disease (CCHD). We aim to quantify GA-specific mortality and neonatal morbidity in infants with CCHD. Methods: Cohort study using a database linking birth certificate, infant hospital discharge, readmission, an...

Introduction: Umbilical artery (UA) Doppler ultrasound is used to assess uteroplacental insufficiency. Absent or reversed end diastolic flow (AREDF) in the UA is associated with increased perinatal mortality in fetuses with intrauterine growth restriction. We describe the incidence of UA Doppler abnormalities during open fetal surgery. Methods:...

Objective: To evaluate the incidence, etiology, and 1-year mortality of nonimmune hydrops fetalis (NIHF) and to identify risk factors for mortality in a contemporary population-based dataset. Study design: The California Office of Statewide Health Planning and Development maintains a database linking maternal and infant hospital discharge, readm...

Objective: Although an important quality metric, current technical performance scores may not be generalizable and may omit operative factors that influence outcomes. We examined factors not included in current technical performance scores that may contribute to increased postoperative length of stay, major complications, and cost after primary re...

Background: In severe right heart obstruction (RHO), redistribution of cardiac output to the left ventricle (LV) is well tolerated by the fetal circulation. Although the same should be true of severely regurgitant tricuspid valve disease (rTVD) with reduced or no output from the right ventricle, affected fetuses more frequently develop hydrops or...

Ectopia cordis (EC) is a rare congenital anomaly often associated with congenital heart disease (CHD). There is a lack of contemporary information on EC diagnosed prenatally. We sought to combine the experiences of two regional referral centers in order to evaluate current outcomes for EC. Clinical, echocardiographic features and perinatal outcomes...

Prenatal diagnosis of critical congenital heart disease (CHD) is associated with decreased morbidity. It is also associated with lower birth weights and earlier gestational age at delivery. The University of California Fetal Consortium (UCfC) comprises five tertiary medical centers, and was created to define treatment practices. We utilized this co...

The objective ot this study was to investigate whether lymphatic markers measured in women during the second trimester are associated with critical congenital heart defects (CCHDs) in offspring. This is a retrospective cohort study of pregnant women who participated in the California Prenatal Screening Program. CCHD data in the offspring was captur...

Coarctation of the aorta (CoA) is the most common ductal-dependent lesion missed on neonatal exam screening. Prenatal diagnosis of CoA improves outcomes through early initiation of prostaglandin. Fetal echocardiographic parameters including 2D and Doppler findings have been studied as predictive measures for fetal diagnosis of CoA, but diagnosis ra...

Objective: Fetuses exposed to anti-SSA (Sjögren's) antibodies are at risk of developing irreversible complete atrioventricular block (CAVB), resulting in death or permanent cardiac pacing. Anti-inflammatory treatment during the transition period from normal heart rhythm (fetal heart rhythm (FHR)) to CAVB (emergent CAVB) can restore sinus rhythm, b...

Mutation of highly conserved residues in transcription factors may affect protein-protein or protein-DNA interactions, leading to gene network dysregulation and human disease. Human mutations in GATA4, a cardiogenic transcription factor, cause cardiac septal defects and cardiomyopathy. Here, iPS-derived cardiomyocytes from subjects with a heterozyg...

Linked Comment: Ultrasound Obstet Gynecol 2016; 48: 426–433

Health registries are databases that are managed by an organized network of centers that contribute clinical data for research purposes. Within these registries, analysts, researchers, and physicians work together to generate new knowledge from pooled data that may improve treatments, outcomes, and systems of care. By combining cases from multiple...

Background: Stage I twin-twin transfusion syndrome presents a management dilemma. Intervention may lead to procedure-related complications while expectant management risks deterioration. Insufficient data exist to inform decision-making. Objective: The aim of this retrospective observational study was to describe the natural history of stage I t...

Advances in fetal echocardiography have improved prenatal diagnosis of congenital heart disease (CHD) and allowed better delivery and perinatal management. Some newborns with CHD require urgent intervention after delivery. In these cases, delivery close to a pediatric cardiac center may be considered, and the presence of a specialized cardiac team...

Objective: Seven public and private California cardiac centers have joined to form the first statewide consortium created to improve the quality and value of congenital cardiac care. The overall goals of the consortium are: 1) to understand variation in modifiable perioperative processes of care among participating hospitals, and 2) to determine wh...

Objective: To determine the current maternal and fetal selection criteria and operative approaches used at centers performing fetal myelomeningocele surgery. Methods: The 17 principal investigators participating in the Fetal Myelomeningocele Consortium were asked to participate in an anonymous online survey regarding the current practice of mate...

Congenital diaphragmatic hernia (CDH) and congenital pulmonary airway malformation (CPAM) are diseases in which chest-occupying lesions can result in severe pulmonary hypoplasia. However, significant postnatal mortality due to pulmonary hypertension (PH) is more often seen in patients with CDH. We analyzed prenatal echocardiographic parameters of p...

Background: A growing body of evidence indicates that right ventricular dysfunction in patients with palliated hypoplastic left heart syndrome (HLHS) originates in fetal life. In this study, the systolic-to-diastolic time index (SDI) was used to study the presence of ventricular dysfunction in single right ventricles in fetuses with HLHS or evolvi...

Invasive fetal cardiac intervention (FCI) has been reported in single-institution series, promoting technical and physiologic success. 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. For this initial analysis, the entire database of...

-Ebstein anomaly and tricuspid valve dysplasia (EA/TVD) are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multi-center study to assess outcomes and factors associated with mortality after fetal diagnosis in...

Because they share a common placenta, monochorionic gestations are subject to unique pregnancy complications that can threaten the life and health of both fetuses and therefore impose a disproportionate disease burden on overall perinatal morbidity and mortality. Early detection of these unique disease processes may allow for prompt referral to a r...

Owing to vascular connections within a single placenta, monochorionic gestations present distinctive prenatal management challenges. Complications that can arise as a result of unbalanced hemodynamic exchange (twin–twin transfusion syndrome and twin anemia polycythemia sequence) and unequal placental sharing (selective fetal growth restriction) sho...

Congenital diaphragmatic hernia (CDH) is associated with small left ventricle (LV), mitral and aortic valve (MV, AV) in utero. We hypothesized that CDH severity would correlate with degree of left heart hypoplasia and decreased LV output. We also hypothesized that factors leading to abnormal fetal hemodynamics such as compression and reduced LV pre...

Purpose: Fetal hydrops arises from multiple disease processes and can portend a grim prognosis. We reviewed our experience with hydropic fetuses to understand relevant antenatal anatomic and physiologic predictors of survival. Methods: We reviewed fetal ultrasounds and echocardiograms of hydropic fetuses evaluated from 1996 to 2013. Results: O...

Background B-type natriuretic peptide (BNP) has not been evaluated in newborns with congenital diaphragmatic hernia (CDH). We hypothesized that BNP and severity of pulmonary hypertension (PH) would predict clinical outcome in these infants. Methods We measured BNP levels and assessed severity of PH by echocardiography at one day and one week of li...

Objectives To better understand the natural history and spectrum of fetal aortic stenosis (AS), we aimed to 1) determine the prenatal diagnosis rate of neonates with critical AS and a biventricular (BV) outcome; and 2) describe the findings at fetal echocardiography in prenatally diagnosed patients.MethodsA multi-center, retrospective study was per...

Maturational and structural differences and external constraint on the fetal myocardium give rise to observed differences in cardiac function between fetal and postnatal life. Traditional M-Mode, gray-scale 2-dimensional, and Doppler blood flow techniques give indirect insight into myocardial function and its interactions with the vasculature. Over...

Objectives Twin reversed arterial perfusion (TRAP) sequence affects 1% of monochorionic (MC) pregnancies and is caused by abnormal vascular connections between a pump twin and an acardiac mass. The effects of abnormal vascular connections on cerebral vasculature in the pump twin are unknown. We hypothesize that abnormal cerebral vascular impedance,...

Objectives: Optimal perinatal management of total anomalous pulmonary venous return (TAPVR) involves timely identification followed by surgical correction. Antenatal diagnosis, however, has long been a challenge. We aimed to identify consistent prenatal sonographic features in this condition in a large cohort in whom the diagnosis was made antenat...

Twin-twin transfusion syndrome is a complex disease process affecting monochorionic twin pregnancies that has implications for the cardiovascular system in both recipient and donor cotwins. Systolic, diastolic, and structural cardiac changes can occur; these have been the subject of intense study over the past two decades, and the use of echocardio...

Prenatal heart disease spans the spectrum of severity from very mild to severe life-threatening conditions. An accepted scale for grading fetal cardiovascular disease severity would aid in anomaly standardization, counseling, and future research. The Fetal Cardiovascular Disease Severity Scale with seven severity grades ranging from mild (grade 1)...

The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to...

Prenatal diagnosis of critical congenital heart disease (CHD) is associated with decreased morbidity. It is also associated with lower birth weights and earlier gestational age at delivery. The University of California Fetal Consortium (UCfC) comprises five tertiary medical centers, and was created to define treatment practices. We utilized this co...

Advances in both imaging technology and understanding of fetal cardiac disease have contributed to a dramatic increase in the sensitivity and specificity of fetal echocardiography over the last few decades. Fetal echocardiography now plays an important role in the prenatal evaluation of fetuses with known or suspected aneuploidy. In this article, s...

Introduction: Tricuspid valve (TV) size at birth correlates with intervention strategy in patients with severe pulmonary stenosis (SPS) or pulmonary atresia/intact ventricular septum (PA/IVS). Prenatal features that might predict postnatal TV size have not been well studied. We hypothesized that prenatal echocardiographic measurements predict the...

Background: Small left heart structures are observed in fetuses with left-sided congenital diaphragmatic hernia (CDH). Fetoscopic tracheal occlusion (FETO) in mid-gestation promotes lung growth in fetuses with CDH, however cardiac effects of FETO are poorly described. We studied the effects of FETO on cardiac structure size at birth, hypothesizing...

Purpose: Congenital diaphragmatic hernia (CDH) and congenital pulmonary airway malformation (CPAM) are diseases in which a space-occupying lesion in the chest can be associated with pulmonary hypoplasia. However, significant postnatal morbidity and mortality due to pulmonary hypertension (PH) are more often seen with CDH than CPAM. We examined pren...

-Fetal arrhythmias characteristic of long QT syndrome (LQTS) include torsades de pointes (TdP) and/or 2° atrioventricular block (AVB), but sinus bradycardia, defined as fetal heart rate <3% for gestational age, is most common. We hypothesized that prenatal rhythm phenotype might predict LQTS genotype and facilitate improved risk stratification and...

Background Congenital diaphragmatic hernia (CDH) represents a spectrum of lung hypoplasia and consequent pulmonary hypertension is an important cause of postnatal morbidity and mortality. We studied biomarkers at the maternal-fetal interface to understand factors associated with the persistence of pulmonary hypertension. Methods Maternal and cord...

Background: The aim of this study was to compare the reproducibility, agreement, and sensitivity of pulsed-wave Doppler tissue imaging (DTI) versus spectral Doppler assessment of right ventricular (RV) myocardial performance index (MPI) in midgestation fetuses in both a normal and a disease state. Methods: RV MPI was calculated using pulsed-wave...

Background: The aim of this study was to determine the feasibility of performing complete early fetal echocardiography (FE) at <17 weeks of gestation with comparison with standard FE in the midtrimester (17-23 weeks). Methods: Fetal echocardiograms obtained in pregnancies studied at <17 weeks at the University of California, San Francisco, over...

Objective: We sought to evaluate the completeness of echocardiographic diagnosis of fetal tetralogy of Fallot (fTOF) at 12-17 weeks gestation, and compare assessment and clinical outcomes to diagnoses made at >17 weeks gestation. Methods: We identified all fTOF diagnoses made in our experience from 2003 to 2008. Referral indication, anatomic det...

Objective: To examine whether a change in the approach to managing persistent patent ductus arteriosus (PDA) from early ligation to selective ligation is associated with an increased risk of abnormal neurodevelopmental outcomes. Study design: In 2005, we changed our PDA treatment protocol for infants born at ≤27 6/7 weeks' gestation from an earl...

To compare the course of hypoplastic left heart syndrome (HLHS) patients diagnosed prenatally with any degree of atrial restriction with those without evidence of atrial restriction. Retrospective, cohort. Prenatally diagnosed HLHS patients from August 1999 to January 2009 were categorized as nonrestrictive versus restrictive, defined by left atria...

Fetal supraventricular tachycardia (SVT) and atrial flutter (AF) can be associated with significant morbidity and mortality. Digoxin is often used as first-line therapy but can be ineffective and is poorly transferred to the fetus in the presence of fetal hydrops. As an alternative to digoxin monotherapy, we have been using sotalol at presentation...