Right Ventricular Global Longitudinal Strain in Fetuses with Hypoplastic Left Heart Syndrome Does Not Differ Between Those With and Without Genetic Conditions.

The presence of a genetic condition is a risk factor for increased mortality in hypoplastic left heart syndrome (HLHS). Speckle tracking strain analysis in interstage echocardiograms have shown promise in identifying patients with HLHS at increased risk of mortality. We hypothesized that fetuses with a genetic condition and HLHS have impaired right ventricular global longitudinal strain compared with fetuses with HLHS and no evident genetic condition.

Left ventricular strain and left atrial strain are impaired during hemodialysis in children.

We aimed to investigate intradialytic changes in ventricular and atrial function using speckle tracking echocardiography (STE) in pediatric hemodialysis (HD). Children with HD vintage > 3 months were enrolled, and echocardiography was performed prior to, during, and after HD. STE was analyzed using GE EchoPAC.

Fetal endoscopic tracheal occlusion and pulmonary hypertension in moderate congenital diaphragmatic hernia.

Objective: To study the role of fetal endoscopic tracheal occlusion (FETO) on resolution of pulmonary hypertension (PH) in fetuses with isolated moderate left-sided diaphragmatic hernia (CDH).

Methods: This retrospective study included fetuses with CDH evaluated between February 2004 and July 2017. Using the tracheal occlusion to accelerate lung growth (TOTAL) trial definition, we classified fetuses into moderate left CDH if O/E-LHR (observed/expected-lung head ratio) was 25-34.

Chronic Maternal Hyperoxygenation and Effect on Cerebral and Placental Vasoregulation and Neurodevelopment in Fetuses with Left Heart Hypoplasia.

Introduction: In a pilot study of chronic maternal hyperoxygenation (CMH) in left heart hypoplasia (LHH), we sought to determine effect estimates of CMH on head size, vascular resistance indices, and neurodevelopment compared to controls.

Material And Methods: Nine gravidae meeting the inclusion criteria (fetal LHH, ≥25.9 weeks' gestation, and ≥10% increase in percent aortic flow after acute hyperoxygenation) were prospectively enrolled.

Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia - association with severity and impact on postnatal outcomes.

Objectives: Fetuses with congenital diaphragmatic hernia (CDH) demonstrate varying degrees of left heart hypoplasia. Our study assesses the relationship between fetal left-sided cardiac structural dimensions, lung size, percentage liver herniation, lung-to-head ratio, postnatal left-sided cardiac structural dimensions, and postnatal outcomes.

Methods: We performed a retrospective cohort study of fetuses with left-sided CDH who had prenatal echocardiographic, ultrasound, and magnetic resonance imaging examinations at our institution between January 2007 and March 2015.

Left ventricular rotational mechanics in early infancy: Normal reference ranges and reproducibility of peak values and time to peak values.

Introduction: Left ventricular cardiac twist and torsion values have been described in premature and term neonates, but not in early infancy. Early and late peak untwist rates and time to peak (TTP) values have not been described in infants.

Methods: 53 term infants were enrolled prospectively.

A genome-wide association study of congenital cardiovascular left-sided lesions shows association with a locus on chromosome 20.

Congenital heart defects involving left-sided lesions (LSLs) are relatively common birth defects with substantial morbidity and mortality. Previous studies have suggested a high heritability with a complex genetic architecture, such that only a few LSL loci have been identified. We performed a genome-wide case-control association study to address the role of common variants using a discovery cohort of 778 cases and 2756 controls.

Estimated combined cardiac output and laser therapy for twin-twin transfusion syndrome.

Introduction: Twin-twin transfusion syndrome (TTTS) is the major cause for fetal demise in monochorionic diamniotic pregnancies. Estimated combined cardiac output (CCO) has not been studied in this setting. The primary aims of this study were to compare CCO in twin donor and recipient fetuses and assess differences in CCO before and after laser coagulation therapy.

Echocardiographic Parameters and Outcomes in Primary Fetal Cardiomyopathy.

Objectives: Primary fetal cardiomyopathy is a rare entity, with a poor prognosis. We sought to describe its echocardiographic characteristics and outcomes.

Methods: We performed a 12-year retrospective review of fetuses with primary cardiomyopathy.

Late outcomes in children with Shone's complex: a single-centre, 20-year experience.

Objective: Shone's syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. There are a few studies evaluating the long-term outcomes in this population. We sought to determine the long-term outcomes in our paediatric population with Shone's syndrome and the factors associated with left heart growth.

Fetal and Neonatal Diastolic Myocardial Strain Rate: Normal Reference Ranges and Reproducibility in a Prospective, Longitudinal Cohort of Pregnancies.

Background: Normative fetal diastolic strain rate values have not been comprehensively reported. The aims of this study were to report normative data for diastolic strain rate parameters across gestation and upon delivery, determine the effect of advancing gestational age on these measures, and compare interobserver variability at multiple gestational age time points.

Methods: Sixty gravid women were enrolled before 20 weeks' gestation.

Longitudinal Changes and Interobserver Variability of Systolic Myocardial Deformation Values in a Prospective Cohort of Healthy Fetuses across Gestation and after Delivery.

Background: Normative data for fetal myocardial deformation values have not been comprehensively described in a longitudinal cohort. The effect of gestational age on these values and on interobserver variability require further investigation.

Methods: Sixty gravid women were prospectively enrolled before 20 weeks' gestation.

5, 4, 3, 2, 1: embryologic variants of pentalogy of Cantrell.

Background: The purpose of this study was to evaluate our experience with pentalogy of Cantrell and the various embryologic variants.

Materials And Methods: Patient charts and diagnostic imaging studies of all fetuses evaluated at Texas Children's Fetal Center for pentalogy of Cantrell between April 2004 and June 2014 were reviewed retrospectively. Data collected from patient charts included demographic information, clinical presentation, fetal and postnatal imaging findings, operative treatment, pathologic evaluation, and outcomes.

Giant omphaloceles: surgical management and perinatal outcomes.

Background: The purpose of this study was to describe the current management and outcomes of infants with omphalocele.

Methods: The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003-February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies.

Fetal cardiac tamponade in a case of right-side congenital diaphragmatic hernia.

Background: Intrafetal fluid collection is a rare ultrasound finding in fetuses with right-side congenital diaphragmatic hernia.

Case: Our patient had a fetus with a large right-side congenital diaphragmatic hernia with a significant amount of the fetal liver herniated into the chest. At 31 weeks of gestation, the fetus had significant ascites and high-pressure intrathoracic fluid accumulation, hydrops fetalis, deviation of the mediastinum, and tamponade-like physiology that compromised cardiac function.

EXIT-to-resection for fetuses with large lung masses and persistent mediastinal compression near birth.

Purpose: To identify prenatal diagnostic features that will help select fetuses with lung masses (LM) who may benefit from ex-utero intrapartum treatment (EXIT procedure) as the preferred mode of delivery.

Methods: The CCAM-volume ratio (CVR), fetal treatment, and outcomes of all fetuses with LM evaluated between 2001 and 2011 were reviewed retrospectively. Fetuses with hydrops or CVR>1.

Defining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors.

Purpose: The aim of this study was to identify the most accurate prenatal predictors of outcomes and need for fetal surgery for fetuses with high-risk lung masses and vascular tumors.

Methods: The records of all fetuses with high-risk lung mass (congenital cystic adenomatoid malformation-volume ratio > 1.6 or findings of hydrops) and vascular tumor evaluated between July 2001 and March 2011 were reviewed retrospectively.

Fetal lung interstitial tumor: a cause of late gestation fetal hydrops.

Most fetal lung masses present by mid gestation, grow during the canalicular phase of lung development (18-26 weeks of gestation), and plateau in growth or shrink after 26 weeks of gestation. We describe the unique case of a fetal lung mass presenting at 37 weeks of gestation with hydrops and fetal heart failure. The late growth of this lesion and resultant hydrops prompted resection as part of the ex utero intrapartum treatment.

Prenatal diagnosis and outcome of fetal lung masses.

Aim: The purpose of this study is to evaluate the accuracy of prenatal diagnostic features, particularly congenital cystic adenomatoid malformation volume ratio (CVR), in predicting outcomes for fetuses with lung masses.

Methods: The records and imaging features of all fetuses referred to the Texas Children's Fetal Center with a fetal lung mass between July 2001 and May 2010 were reviewed retrospectively. Data collected included gestational age (GA) at diagnosis, fetal magnetic resonance imaging findings, CVR, mass size, nature of fetal treatment, surgical findings, pathology, and outcome.

Patent ductus arteriosus ligation in neonates: preoperative predictors of poor postoperative outcomes.

Purpose: The purpose of this study was to identify preoperative predictors of adverse outcomes in infants undergoing surgical ligation of patent ductus arteriosus (PDA).

Methods: Charts of all neonates who underwent PDA ligation at Texas Children's Hospital (Houston, TX) between 2001 and 2006 were retrospectively reviewed with specific attention to preoperative clinical characteristics, echocardiographic details, operative morbidity, and postoperative outcomes. Infants with other cardiac anomalies or right-to-left or bidirectional PDA shunt were excluded.

Myocardial deformation abnormalities in pediatric hypertrophic cardiomyopathy: are all etiologies identical?

Aims: Hypertrophic cardiomyopathy (HCM) is a disease with a heterogeneous clinical and morphological presentation. It can be secondary to mutations in genes encoding for sarcomeric and non-sarcomeric proteins. The pattern of ventricular hypertrophy can vary from isolated basal septal to concentric hypertrophy.

Fetal cardiac malposition: incidence and outcome of associated cardiac and extracardiac malformations.

Cardiac malposition is a rare but important finding when detected on fetal ultrasound. The purpose of this study was to evaluate the incidence of fetal cardiac malposition, associated abnormalities, and clinical outcome in a tertiary-care medical center. Records of fetuses (1993 to 2006) with dextroposition, dextrocardia, mesocardia, ectopia cordis, or heterotaxy were reviewed.