Impact of Neonatal Intervention on Left Ventricular Performance in Ebstein's Anomaly and Tricuspid Valve Dysplasia.

Background: Severe neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are associated with high perinatal morbidity and mortality. The authors recently demonstrated left ventricular (LV) dysfunction and dyssynchrony to be prevalent in affected newborns and to contribute to poor outcomes. The aim of this study was to investigate the impact of patent ductus arteriosus (PDA) closure, spontaneous or surgical ligation, or right ventricular exclusion (Starnes procedure) on LV performance in neonatal EA and TVD.

Left Ventricular Dysfunction in Neonatal Ebstein's Anomaly and Tricuspid Valve Dysplasia.

Background: The mechanisms and prognostic importance of left ventricular (LV) dysfunction in neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are not well understood. The authors recently demonstrated reduced cardiac output and dyssynchrony to be common in fetal EA/TVD and therefore hypothesized that LV dysfunction may be associated with worse outcomes in neonatal EA/TVD.

Methods: A multicenter retrospective case-control study was conducted among neonatal patients with EA/TVD (n = 32) and a healthy control cohort (n = 17) encountered from 2004 to 2019.

Extracardiac Doppler indices predict perinatal mortality in fetuses with Ebstein anomaly and tricuspid valve dysplasia.

Objectives: Ebstein anomaly and tricuspid valve dysplasia (EA/TVD) carry high perinatal mortality. Past studies have focused on cardiac predictors of mortality; we sought to describe the fetal echo (FE) extracardiac Dopplers in this cohort and determine their association with perinatal mortality.

Method: Fetuses with EA/TVD at 23 centers from 2005-2011 were included for retrospective study.

Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study.

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. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia.

Challenges and lessons learned from the Pediatric Heart Network Normal Echocardiogram Database study.

Background: The Pediatric Heart Network Normal Echocardiogram Database Study had unanticipated challenges. We sought to describe these challenges and lessons learned to improve the design of future studies.

Methods: Challenges were divided into three categories: enrolment, echocardiographic imaging, and protocol violations.

Quantitative Assessment of Left Ventricular Dysfunction in Fetal Ebstein's Anomaly and Tricuspid Valve Dysplasia.

Background: Fetal Ebstein's anomaly and tricuspid valve dysplasia (EA/TVD) are associated with high perinatal mortality relative to pulmonary atresia with intact ventricular septum (PAIVS), despite both requiring redistribution of the cardiac output (CO) to the left ventricle (LV). LV dysfunction is suspected to contribute to adverse outcomes in EA/TVD.

Objective: We sought to examine global and segmental LV function in fetal EA/TVD with comparison to normal controls and PAIVS.

Challenges With Left Ventricular Functional Parameters: The Pediatric Heart Network Normal Echocardiogram Database.

Background: The reliability of left ventricular (LV) systolic functional indices calculated from blinded echocardiographic measurements of LV size has not been tested in a large cohort of healthy children. The objective of this study was to estimate interobserver variability in standard measurements of LV size and systolic function in children with normal cardiac anatomy and qualitatively normal function.

Methods: The Pediatric Heart Network Normal Echocardiogram Database collected normal echocardiograms from healthy children ≤18 years old distributed equally by age, gender, and race.

Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.

Background: Ebstein anomaly and tricuspid valve dysplasia 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 multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era.

Echocardiographic diagnosis of right ventricular inflow compression associated with pectus excavatum during spinal fusion in prone position.

Introduction: Pectus excavatum is commonly viewed as a benign condition. Associated alterations in hemodynamics are rare. We present an unusual case of right ventricular inflow obstruction and hemodynamic compromise as a consequence of pectus excavatum encountered during surgical intervention.

Severe hypotension associated with the prone position in a child with scoliosis and pectus excavatum undergoing posterior spinal fusion.

Study Design: Case report.

Objective: We report a case of severe hypotension associated with prone positioning in a child with scoliosis and pectus excavatum.

Summary Of Background Data: Pectus excavatum is commonly associated with scoliosis and can impact cardiac function.

Accuracy of the fetal echocardiogram in double-outlet right ventricle.

Objective: Double-outlet right ventricle (DORV) is a complex congenital heart defect with heterogeneous anatomy. Patients require a variety of surgical interventions, and the long-term prognosis is variable. Therefore, accurate prenatal diagnosis is important in providing parents with appropriate counseling.

Balloon dilation of severe aortic stenosis in the fetus: potential for prevention of hypoplastic left heart syndrome: candidate selection, technique, and results of successful intervention.

Background: Preventing the progression of fetal aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS) requires identification of fetuses with salvageable left hearts who would progress to HLHS if left untreated, a successful in utero valvotomy, and demonstration that a successful valvotomy promotes left heart growth in utero. Fetuses meeting the first criterion are undefined, and previous reports of fetal AS dilation have not evaluated the impact of intervention on in utero growth of left heart structures.

Methods And Results: We offered fetal AS dilation to 24 mothers whose fetuses had AS.

Creation of an atrial septal defect in utero for fetuses with hypoplastic left heart syndrome and intact or highly restrictive atrial septum.

Background: Infants born with hypoplastic left heart syndrome and an intact or highly restrictive atrial septum face a neonatal mortality of at least 48% despite early postnatal left atrial decompression and palliative surgery. Prenatal left atrial decompression has been suggested as a means of improving these outcomes. This study reports the feasibility of fetal catheterization to create an interatrial communication and describes technical considerations.

Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: outcome after neonatal transcatheter atrial septostomy.

Background: Hypoplastic left heart syndrome (HLHS) with intact or very restrictive atrial septum is a highly lethal combination. We review our 13-year institutional experience treating this high-risk subgroup of patients with emergent catheter therapy.

Methods And Results: Infants with HLHS requiring catheter septostomy within the first 2 days of life were compared with a matched control group with adequate interatrial communication.

Association between cardiac tumors and tuberous sclerosis in the fetus and neonate.

A retrospective review was performed in 94 patients with > or =1 cardiac tumors seen on prenatal or neonatal echocardiography at 5 major referral centers. Tuberous sclerosis was present in 68 patients diagnosed with a cardiac tumor in utero or during the neonatal period, including 61 of 64 with multiple tumors.

Prenatal role of the ductus arteriosus in absent pulmonary valve syndrome.

Absent pulmonary valve with ventricular septal defect is associated with ductal agenesis and markedly dilated main and branch pulmonary arteries. The less common variant with intact ventricular septum generally exhibits a patent ductus and smaller branch pulmonary arteries, and may be associated with tricuspid atresia. We identified 7 patients with the prenatal diagnosis of absent pulmonary valve, 5 with ventricular septal defect (Group 1) and 2 with an intact ventricular septum (Group 2).

Transesophageal Echocardiography During Interventional Catheterization in Congenital Heart Disease.

The rapidly expanding role of transesophageal echocardiography (TEE) in the management and treatment of congenital heart disease includes guidance of interventional cardiac catheterization procedures and monitoring of results. TEE is particularly helpful for assessing device position during device closure of atrial septal defects (ASD), ventricular septal defects, and perivalvar defects, especially when multiple or complex defects are present. It is also useful during balloon dilation of certain congenital and postoperative obstructions.