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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. EA/TEF patients born between 1995 and 2018 at two centers were retrospectively reviewed. Clinical variables including sex, prenatal diagnosis, birth weight, prematurity, major congenital heart disease (MCHD), and pre-operative mechanical ventilation (POMV) were collected. Index admission composite morbidity was considered positive if: length-of-stay > 90th percentile (139 days), ventilation days > 90th percentile (24 days), and/or gastrostomy was used for long-term feeding. Multivariable regression determined predictors of index admission mortality and composite morbidity. A composite morbidity predictive algorithm was created. ROC curves evaluated model discrimination. Of 253 patients, 13 (5.1%) experienced index admission mortality. Of the patients not suffering mortality, 74 (31.6%) experienced composite morbidity. Only MCHD predicted mortality (p = 0.001); birth weight did not (p = 0.173). There was no difference between the Spitz classification and MCHD alone in predicting mortality risk (p = 0.198); both demonstrated very good discrimination. Prenatal diagnosis, POMV, prematurity, and male sex predicted composite morbidity risk (p < 0.001; p = 0.008; p = 0.009; p = 0.05). An algorithm incorporating these predictors demonstrated good discrimination (AUC = 0.784; 95% CI: 0.724, 0.844). The Spitz classification maintains contemporary relevance for mortality risk, though birth weight can be de-emphasized. A new morbidity risk algorithm is proposed for early postnatal counseling. Prognosis study. Level IV. • The Spitz classification remains relevant for early mortality risk prediction. • Birth weight < 1.5 kg does not independently predict index admission mortality. • Prenatal diagnosis is the strongest predictor of index admission morbidity risk. • A new morbidity risk algorithm is proposed for early postnatal prognostication. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objectives To describe the natural history of pulmonary hypertension (PH) and the risk of death and pulmonary morbidity associated with the persistence of PH through the neonatal hospitalization for these infants. Study design We performed a retrospective cohort study of infants with congenital diaphragmatic hernia (CDH) cared for at University of California San Francisco (2002-2012). Infants with other major anomalies or syndromes were excluded (n = 43). Clinical echocardiograms were performed weekly for up to 6 weeks or until PH resolved off respiratory support or until hospital discharge. Echocardiograms were re-read by a blinded reviewer and categorized by severity of elevation in estimated pulmonary arterial pressure. PH was defined as ≥2/3 systemic blood pressure. Severity was determined by a hierarchy of ductus arteriosus level shunt, interventricular septal position, and tricuspid regurgitant jet velocity. Results Of 140 infants with ≥1 echo, 98 resolved their PH prior to death/discharge. Mean time to resolution was 18 days (median 14 days, IQR 8, 21 days). Those with persistence of PH had a higher rate of extracorporeal membrane oxygenation (P < .001) and death (P < .001), and fewer ventilator-free days (P < .001). Persistence of PH at 14 days predicted mortality (area under the receiver operating characteristic curve 0.87) and adverse respiratory outcomes (area under the receiver operating characteristic curve 0.80-0.83). Conclusions The majority of infants with CDH resolve PH between 1 and 3 weeks of life. At 2 weeks of age, severity of PH by echocardiogram strongly predicts short-term pulmonary morbidity and death. Further evaluation of physiological alterations during that time may lead to novel therapies for severe CDH. [ABSTRACT FROM AUTHOR]

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 Overall neonatal survival in 167 fetuses was 44% (range, 0–75%) and was influenced by the underlying disease process. The anatomic distribution of fluid varied and was not significantly different between survivors and nonsurvivors. Univariate analysis indicated that resolution of hydrops and delivery at a later gestational age were predictive of survival (OR: 5.7 (95% CI: 2.5–13.2) and OR: 1.3 (95% CI: 1.1–1.4), respectively). Fetal intervention also improved survival in some diseases. Echocardiograms were reviewed to group fetuses with similar cardiac physiology and defined categories with high or low/normal cardiothoracic ratio (CTR). Among patients with a high CTR, the cardiovascular profile score was predictive of survival ( p = 0.009). Conclusion Survival in hydrops depends on the underlying disease, available fetal therapies to resolve hydrops, and the gestational age of delivery and not on the specific anatomic manifestations of hydrops. In hydropic fetuses with high CTRs, the cardiovascular profile score may be a useful prognostic indicator. [ABSTRACT FROM AUTHOR]

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 early ligation approach, with prompt PDA ligation if the ductus failed to close after indomethacin therapy (period 1: January 1999 to December 2004), to a selective ligation approach, with PDA ligation performed only if specific criteria were met (period 2: January 2005 to May 2009). All infants in both periods received prophylactic indomethacin. Multivariate analysis was used to compare the odds of a composite abnormal neurodevelopmental outcome (Bayley Mental Developmental Index or Cognitive Score <70, cerebral palsy, blindness, and/or deafness) associated with each treatment approach at age 18-36 months (n = 224). Results: During period 1, 23% of the infants in follow-up failed indomethacin treatment, and all underwent surgical ligation. During period 2, 30% of infants failed indomethacin, and 66% underwent ligation after meeting prespecified criteria. Infants treated with the selective ligation strategy demonstrated fewer abnormal outcomes than those treated with the early ligation approach (OR, 0.07; P = .046). Infants who underwent ligation before 10 days of age had an increased incidence of abnormal neurodevelopmental outcome. The significant difference in outcomes between the 2 PDA treatment strategies could be accounted for in part by the earlier age of ligation during period 1. Conclusion: A selective ligation approach for PDAs that fail to close with indomethacin therapy is not associated with worse neurodevelopmental outcomes at age 18-36 months. [Copyright &y& Elsevier]

Background: Congenital long-QT syndrome (LQTS) may present during fetal development and can be life-threatening. The molecular mechanism for the unusual early onset of LQTS during fetal development is unknown. Objective: We sought to elucidate the molecular basis for severe fetal LQTS presenting at 19 weeks'' gestation, the earliest known presentation of this disease. Methods: Fetal magnetocardiography was used to demonstrated torsades de pointes and a prolonged rate-corrected QT interval. In vitro electrophysiological studies were performed to determine functional consequences of a novel SCN5A mutation found in the fetus. Results: The fetus presented with episodes of ventricular ectopy progressing to incessant ventricular tachycardia and hydrops fetalis. Genetic analysis disclosed a novel, de novo heterozygous mutation (L409P) and a homozygous common variant (R558 in SCN5A). In vitro electrophysiological studies demonstrated that the mutation in combination with R558 caused significant depolarized shifts in the voltage dependence of inactivation and activation, faster recovery from inactivation, and a 7-fold higher level of persistent current. When the mutation was engineered in a fetal-expressed SCN5A splice isoform, channel dysfunction was markedly potentiated. Also, R558 alone in the fetal splice isoform evoked a large persistent current, and hence both alleles were dysfunctional. Conclusion: We report the earliest confirmed diagnosis of symptomatic LQTS and present evidence that mutant cardiac sodium channel dysfunction is potentiated by a developmentally regulated alternative splicing event in SCN5A. Our findings provide a plausible mechanism for the unusual severity and early onset of cardiac arrhythmia in fetal LQTS. [Copyright &y& Elsevier]

We hypothesized that infants with fetal growth restrictions have increased mortality and morbidity after congenital heart disease surgery. The study included patients in The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010-2016) who underwent cardiac surgery at a corrected gestational age of ≤44 weeks. Patients were classified as severely (birth weight Z-score −4 to −2), moderately (Z-score −2 to −1), and mildly growth restricted (Z-score −1.0 to −0.5) and compared with a reference population (Z-score 0-0.5). Multivariable logistic regression clustering on center was used to evaluate the association of birth weight Z-score with operative mortality and postoperative complications and its interaction with gestational age was assessed. In 25,244 patients, operative mortality was 8.6% and major complications occurred in 19.4%. Compared with the reference group, the adjusted odds ratio (AOR) of mortality was increased in infants with severe (AOR, 2.4; 95% confidence interval [CI], 2.0-3.0), moderate (AOR, 1.7; 95% CI, 1.4-2.0), and mild growth restriction (AOR, 1.4; 95% CI, 1.2-1.6). The AOR for major postoperative complications was increased for severe (AOR, 1.4; 95% CI, 1.2-1.7) and moderate growth restriction (AOR, 1.2; 95% CI, 1.1-1.4). There was significant interaction between birth weight Z-score and gestational age (P =.007). Even birth weight Z-scores slightly below average are independent risk factors for mortality and morbidity in infants who undergo cardiac surgery. The strongest association between poor fetal growth and operative mortality exists in early-term infants. These novel findings might account for some of the previously unexplained variation in cardiac surgical outcomes. Methods, main results, and implications of this study. This was a retrospective study using The Society of Thoracic Surgeons database to evaluate Z-score for birth weight as a predictor for mortality and postoperative complications in neonates who underwent congenital heart surgery. [Display omitted] [ABSTRACT FROM AUTHOR]

Objective: We conducted a multicenter, randomized, controlled trial to determine whether higher doses of indomethacin would improve the rate of patent ductus arteriosus (PDA) closure. Study design: Infants (<28 weeks gestation) who received a conventional, prophylactic 3-dose course of indomethacin were eligible if they had continued evidence of persistent ductus patency on an echocardiogram obtained before the third prophylactic indomethacin dose. Infants (n = 105) were randomized to receive an extended 3-day course of either low-dose (0.1 mg/kg/d) or higher-dose (0.2 or 0.5 mg/kg/d) indomethacin. An echocardiogram was obtained 24 hours after the last dose of study drug. Results: Despite increasing serum indomethacin concentrations by 2.9-fold in the higher-dose group, we failed to detect a significant decrease in the rate of persistent PDA (low = 52%; higher = 45%, P = .50). The higher-dose group had a significantly higher occurrence of serum creatinine >2 mg/100 mL (low = 6%, higher = 19%, P < .05) and moderate/severe retinopathy of prematurity (ROP) (low = 15%, higher = 36%, P < .025). The incidence of moderate/severe ROP was directly related to the poststudy indomethacin concentrations (odds ratio = 1.75, confidence interval: 1.15-2.68, P < .01). Conclusion: Increasing indomethacin concentrations above the levels achieved with a conventional dosing regimen had little effect on the rate of PDA closure but was associated with higher rates of moderate/severe ROP and renal compromise. [Copyright &y& Elsevier]

Background: A coronary artery arising from the wrong aortic sinus, with or without a proximal course between the aorta and the pulmonary artery, may predispose to myocardial ischemia, myocardial infarction, or sudden death. Methods: We repaired this lesion in 5 symptomatic patients, all of whom had experienced exercise-related chest pain, dyspnea, or syncopal episodes. All patients underwent patch angioplasty of the proximal anomalous coronary artery with glutaraldehyde-treated autologous pericardium. When the coronary artery coursed between the aorta and the main pulmonary artery, the main pulmonary artery was translocated toward the left pulmonary artery to create additional space and prevent compression. Results: All patients recovered well and to date have demonstrated no evidence of recurrent myocardial ischemia. Conclusions: The combination of coronary artery angioplasty and translocation of the pulmonary artery seems to effectively address all the mechanisms that can generate ischemia. [Copyright &y& Elsevier]

Abstract: Proximal pulmonary artery stenosis is a common acquired lesion in infants treated for congenital heart disease. We hypothesized that a large number of stenoses develop at the site of ductal insertion in patients with right ventricular outflow tract obstruction (RVOTO) and that these patients are at risk for developing hypoplasia of the ipsilateral pulmonary artery. The surgical and cardiac catheterization databases at our institution during the years 1988–2000 were searched for all patients under 1 year of age carrying a diagnosis of pulmonary atresia with intact ventricular septum (PA), tetralogy of Fallot (TOF) or pulmonary stenosis (PS), yielding 700 patients (62 PA, 373 TOF, 265 PS). The cardiac catheterization database was also searched for all patients with any diagnosis under 1 year of age found at catheterization to have proximal pulmonary artery stenosis. Proximal pulmonary artery stenosis associated with the ductal insertion site was diagnosed at catheterization in 33 infants (18 with PA, 5 with TOF, 6 with PS, 4 other diagnoses). This represents 29% of patients with PA, 1% with TOF and 2% with PS. Among patients with RVOTO and ductal insertion site-associated stenosis, there was a high prevalence (59%) of associated distal pulmonary arterial hypoplasia, defined as diameter of the stenosed vessel at first distal branch ≤80% the diameter of the contralateral vessel. Symptomatology failed to identify this lesion; therefore, a high index of suspicion is necessary if proximal pulmonary artery stenosis is to be detected early in these patients. [Copyright &y& Elsevier]

Umbilical artery catheterization is often performed in critically ill neonates. Infection and thromboembolic events are relatively frequent complications, but aneurysm formation is rare. We describe the case of a premature baby who developed a rapidly expanding aneurysm of the descending thoracic aorta, secondary to a highly placed infected umbilical catheter. The rapid progression under medical therapy prompted us to replace the thoracic aorta with an 8-mm polytetrafluoroethylene graft, using extracorporeal circulation and core cooling. The baby had an excellent recovery and was discharged home after a prolonged antibiotic course. He is being followed with repeated imaging studies for a small abdominal aneurysm. We describe a novel approach to this rare and complicated problem and provide a review of the literature on the subject. [Copyright &y& Elsevier]

We describe clinical and echocardiographic features of tetralogy of Fallot with absent pulmonary valve complex (TOF/APVC) and hypothesized that outcome might be related to pulmonary artery enlargement or severity of illness. We examined the clinical records of all 23 patients evaluated at our institution before death or surgical correction of TOF/APVC between 1990 and 2000. Echocardiograms for 16 patients (including 5 fetuses) were also reviewed, and measurements of the semilunar valves and pulmonary arteries were obtained and compared with patient’s aortic annulus size and with established normal subjects. Actuarial survival was 15 of 23 patients (68%) at 4 years. Four fetuses were hydropic and none survived; 7 patients were ventilator dependent at operation and only 3 survived. No difference was noted in pulmonary artery diameters in survivors versus nonsurvivors. Pulmonary valve annulus size was larger in nonsurvivors (103 ± 25% vs 71 ± 24% of normal, p = 0.03); however, when fetal examinations were excluded, this difference did not persist. Thus, only hydrops and ventilator dependence at diagnosis predicted mortality. There was no correlation between postnatal measurements of pulmonary arteries and outcome. Larger pulmonary annulus size in hydropic fetuses and poor survival among patients diagnosed in utero suggests that the pathophysiology in TOF/APVC is not due entirely to the aneurysmal dilation of the pulmonary arteries but may be related to right-sided cardiac dysfunction. [Copyright &y& Elsevier]

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 maternal-fetal surgery for fetal myelomeningocele closure.Study Design: An international multicenter prospective observational registry created to track and report maternal, obstetric, fetal/neonatal, and subsequent pregnancy outcomes following open maternal-fetal surgery for fetal myelomeningocele was evaluated for subsequent pregnancy outcome variables. Institutional Review Board approval was obtained for the registry.Results: From 693 cases of open maternal-fetal surgery for fetal myelomeningocele closure entered into the registry, 77 subsequent pregnancies in 60 women were identified. The overall live birth rate was 96.2%, with 52 pregnancies delivering beyond 20 weeks gestational age and median gestational age at delivery of 37 (36.3-37.1) weeks. The uterine rupture rate was 9.6% (n = 5), resulting in 2 fetal deaths. Maternal transfusion was required in 4 patients (7.7%).Conclusion: The risk of uterine rupture or dehiscence in subsequent pregnancies with associated fetal morbidity after open maternal-fetal surgery is significant, but is similar to that reported for subsequent pregnancies after classical cesarean deliveries. Future pregnancy considerations should be included in initial counseling for women contemplating open maternal-fetal surgery. [ABSTRACT FROM AUTHOR]

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 repair of tetralogy of Fallot. Methods This is a retrospective single site study of patients younger than age 2 years with tetralogy of Fallot undergoing complete repair between 2007 and 2015. Medical record data and discharge echocardiograms were reviewed to ascertain component and composite technical performance scores. Primary outcomes included postoperative length of stay, major complications, and total hospital costs. Multivariable logistic and linear regression identified determinants of each outcome. Results Patient population (n = 115) had a median postoperative length of stay of 8 days (interquartile range, 6-10 days), and a median total cost of $71,147. Major complications occurred in 33 patients (29%) with 1 death. Technical performance scores assigned were optimum in 28 patients (25%), adequate in 59 patients (52%), and inadequate in 26 patients (23%). Neither technical performance score components nor composite scores were associated with increased postoperative length of stay. Optimum or adequate repairs versus inadequate had equal risk of a complication ( P = .79), and equivalent mean total cost ($100,000 vs $187,000; P = .25). Longer cardiopulmonary bypass time per 1-minute increase ( P < .01) was associated with longer postoperative length of stay and reintervention ( P = .02). The need to return to bypass also increased total cost ( P < .01). Conclusions Current tetralogy of Fallot technical performance scores were not associated with selected outcomes in our postoperative population. Although returning to bypass and bypass length are not included as components in the current score, these are important factors influencing complications and resource use in our population. Revisions anticipated from a prospective trial should consider including these variables. [ABSTRACT FROM AUTHOR]

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, readmissions, and birth and death certificate date from 1 year before to 1 year after birth. We searched the database (2005-2012) for infants with NIHF (identified by the International Classification of Diseases, 9th Revision, Clinical Modification code). Hazard models were used to identify risk factors for mortality in infants with NIHF; results are presented as hazard ratios (HRs, 95% CI).Results: The incidence of NIHF was 2.5 out of 10 000 among live born infants. Neonatal mortality was 35.1% (364 out of 1037) and overall mortality was 43.2% (448 out of 1037) at 1 year of age. Gestational age (GA) was predictive of mortality with a HR of 2.4 (95% CI 1.9-3.2) for preterm compared with term infants. The GA-adjusted HR for mortality was 1.3 (95% CI 1.1-1.6) for polyhydramnios and 1.5 (95% CI 1.2-2.0) for large for gestational age infants compared with appropriate for GA infants. Aneuploid infants with critical congenital heart disease had an adjusted HR of 2.3 (95% CI 1.5-3.6) compared with euploid infants without a structural birth defect.Conclusions: In this large, population-based study, prematurity, polyhydramnios, and large for gestational age were predictors of increased mortality. Mortality is highly variable among euploid and aneuploid infants with and without structural birth defects and critical congenital heart disease. [ABSTRACT FROM AUTHOR]

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 twin-twin transfusion syndrome, to assess for predictors of disease behavior, and to compare pregnancy outcomes after intervention at stage I vs expectant management.Study Design: Ten North American Fetal Therapy Network centers submitted well-documented cases of stage I twin-twin transfusion syndrome for analysis. Cases were retrospectively divided into 3 management strategies: those managed expectantly, those who underwent amnioreduction at stage I, and those who underwent laser therapy at stage I. Outcomes were categorized as no survivors, 1 survivor, 2 survivors, or at least 1 survivor to live birth, and good (twin live birth ≥30.0 weeks), mixed (single fetal demise or delivery between 26.0-29.9 weeks), and poor (double fetal demise or delivery <26.0 weeks) pregnancy outcomes. Outcomes were analyzed by initial management strategy.Results: A total of 124 cases of stage I twin-twin transfusion syndrome were studied. In all, 49 (40%) cases were managed expectantly while 30 (24%) underwent amnioreduction and 45 (36%) underwent laser therapy at stage I. The overall fetal mortality rate was 20.2% (50 of 248 fetuses). Of those managed expectantly, 11 patients regressed (22%), 4 remained stage I (8%), 29 advanced in stage (60%), and 5 experienced spontaneous previable preterm birth (10%) during observation. The mean number of days from diagnosis of stage I to a change in status (progression, regression, loss, or delivery) was 11.1 (SD 14.3) days. Intervention by amniocentesis or laser therapy was associated with a lower risk of fetal loss (P = .01) than expectant management. The unadjusted odds of poor outcome were 0.33 (95% confidence interval, 0.09-01.20), for amnioreduction and 0.26 (95% confidence interval, 0.09-0.77) for laser therapy vs expectant management. Adjusting for nulliparity, recipient maximum vertical pocket, gestational age at diagnosis, and placenta location had negligible effect. Both amnioreduction and laser therapy at stage I decreased the likelihood of no survivors (odds ratio, 0.11; 95% confidence interval, 0.02-0.68 and odds ratio, 0.07; 95% confidence interval, 0.01-0.37, respectively). Only laser therapy, however, was protective against poor outcome in our data (odds ratio, 0.29; 95% confidence interval, 0.07-1.30 for amnioreduction vs odds ratio, 0.12, 95% confidence interval, 0.03-0.44 for laser), although the estimate for amnioreduction suggests a protective effect.Conclusion: Stage I twin-twin transfusion syndrome was associated with substantial fetal mortality. Spontaneous resolution was observed, although the majority of expectantly managed cases progressed. Progression was associated with a worse prognosis. Both amnioreduction and laser therapy decreased the chance of no survivors, and laser was particularly protective against poor outcome independent of multiple factors. Further studies are justified to corroborate these findings and to further define risk stratification and surveillance strategies for stage I disease. [ABSTRACT FROM AUTHOR]

Objective Congenital diaphragmatic hernia (CDH) results in morbidity and death from lung hypoplasia and persistent pulmonary hypertension (PH). We sought to define the relationship between fetal ultrasound markers of severity in CDH and the time to resolution of neonatal PH. Study Design We conducted a retrospective study of fetuses with an antenatal ultrasound scan and left-sided CDH cared for at the University of California San Francisco (2002-2012). Fetal liver position was classified on ultrasound scan as abdominal (entire liver within the abdomen) or thoracic (any portion of the liver within the thorax). Fetal stomach position was classified from least to most aberrant: abdominal, anterior left chest, mid-posterior left chest, or retrocardiac (right chest). Lung-to-head ratio (LHR) was determined from available scans at 20-29 weeks of gestational age (GA). Routine neonatal echocardiograms were performed weekly for up to 6 weeks or until PH resolved or until discharge. PH was assessed by echocardiogram with the use of a hierarchy of ductus arteriosus level shunt, interventricular septal position, and tricuspid regurgitant jet velocity. Days to PH-free survival was defined as the age at which pulmonary artery pressure was estimated to be <2/3 systemic blood pressure. Cox proportional hazards models adjusted for GA at birth, era of birth, fetal surgery, and GA at ultrasound scan (LHR model only), with censoring at 100 days. Results Of 118 patients, the following fetal markers were available: LHR (n = 53), liver position (n = 112), and stomach position (n = 80). Fewer infants experienced resolved PH if they had LHR <1 ( P = .006), thoracic liver position ( P = .001), or more aberrant stomach position ( P < .001). There was also a decreased rate of resolution of PH in infants with LHR <1 (hazard ratio, 0.30; P = .007), thoracic liver position (hazard ratio, 0.38; P < .001), and more aberrant stomach position (hazard ratios, 0.28 [ P = .002]; 0.1 [ P < .001]; and 0.07 [ P < .001]). Conclusion Fetal ultrasound markers of CDH severity are predictive not only of death but also of significant morbidity. LHR <1, thoracic liver, and aberrant stomach position are associated with delayed time to resolution of PH in infants with CDH and may be used to identify fetuses at high risk of persistent PH. [ABSTRACT FROM AUTHOR]

Objective: We sought to identify differences in echocardiographic profiles of monochorionic (MC)/diamniotic (DA) pregnancies with early or mild twin-twin transfusion syndrome (TTTS), compared to MC/DA twins affected only by discordant growth or discordant fluid. Study Design: This was a retrospective evaluation of sonograms and echocardiograms of twin pregnancies referred for suspected TTTS. Results: A total of 112 MC/DA pairs were studied. In all, 41 did not have/develop TTTS, and 61 had stage I/II TTTS. Ten developed TTTS after initially not meeting criteria. TTTS recipients had a higher rate of venous Doppler or tricuspid inflow abnormalities than purported recipients in non-TTTS pregnancies (86% vs 37%, P < .001). TTTS recipients had shorter tricuspid inflow duration/R-R intervals than non-TTTS fetuses (32 ± 6% vs 37 ± 4%, P < .001). Logistic regression and recursive partitioning identified shorter tricuspid inflow duration, longer isovolumic relaxation, and ductus venosus abnormality associated with TTTS. Conclusion: Diastolic pathology, specifically shorter tricuspid inflow duration, may be considered a hallmark of TTTS distinguishing these pregnancies from other MC/DA twin complications. [ABSTRACT FROM AUTHOR]

Objective: To examine whether a more conservative approach to treating patent ductus arteriosus (PDA) is associated with an increase or decrease in morbidity compared with an approach involving early PDA ligation. Study design: In January 2005, we changed our approach to infants born at age ≤27 weeks gestation who failed indomethacin treatment. We changed from an early surgical approach, in which feedings were stopped and all PDAs were ligated (period 1: January 1999 to December 2004; n = 216) to a more conservative approach in which feedings continued and PDAs were ligated only if cardiopulmonary compromise developed (period 2: January 2005 to August 2009; n = 180). All infants in both periods received prophylactic indomethacin therapy. Results: The 2 periods had similar rates of perinatal/neonatal risk factors and indomethacin failure (24%), as well as ventilator management and feeding advance protocols. The conservative approach (period 2) was associated with decreased rates of duct ligation (72% vs 100%; P <.05). Even though infants subjected to this approach were exposed to larger PDA shunts for longer durations, the rates of bronchopulmonry dysplasia, sepsis, retinopathy of prematurity, neurologic injury, and death were similar to those in period 1. The overall rate of necrotizing enterocolitis was significantly lower in period 2 compared with period 1. Conclusions: These findings support the need for new controlled, randomized trials to reexamine the benefits and risks of different approaches to PDA treatment. [ABSTRACT FROM AUTHOR]

Objectives: To define current frequency of prenatal detection of congenital heart disease (CHD), factors affecting prenatal detection, and its influence on postnatal course. Study design: We prospectively identified all fetuses and infants ≤6 months of age with major CHD at 3 referral centers in Northern California over 1 year; we obtained prenatal and demographic data, reviewed prenatal ultrasound (US) and postnatal records, and used logistic regression to analyze maternal, fetal, and prenatal-care provider risk factors for prenatal diagnosis. Results: Ninety-eight of 309 infants with major CHD had prenatal diagnosis (36% accounting for 27 pregnancy terminations); 185 infant-families participated in the postnatal survey, and although 99% had prenatal US, only 28% were prenatally diagnosed. Anomalous pulmonary venous return (0%), transposition of the great arteries (19%), and left obstructive lesions (23%) had the lowest prenatal detection. Heterotaxy (82%), single ventricle (64%), and HLHS (61%) had the highest. Prenatal diagnosis was higher at university versus community practices (P = .001). Sociodemographics were not associated with prenatal diagnosis. Infants diagnosed prenatally were less frequently ventilated (P < .01) or treated with prostaglandin (P < .05). Conclusions: Prenatal detection of major CHD significantly alters postnatal course but remains low despite nearly universal US. CHD type and US practice type are important determinants of prenatal detection. [Copyright &y& Elsevier]

Abstract: We report an infant who presented with cyanosis after birth and was unresponsive to all resuscitative efforts. Emergent echocardiogram showed d-transposition of the great arteries (d-TGA) with an intact ventricular septum. Attempts to perform a bedside balloon atrial septostomy failed due to inability to traverse the atrial septum. Autopsy revealed d-TGA with intact atrial and ventricular septa and patent ductus arteriosus. Though no survivors have been reported for this rare lethal combination of lesions thus far in the literature, we speculate that recent advances in fetal treatment may soon be applicable to infants with this condition. [Copyright &y& Elsevier]

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. [ABSTRACT FROM AUTHOR]

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 monitoring (FHRM) and surveillance echocardiography.Methods: Anti-Ro pregnant women were recruited from 16 international centers in a prospective observational study. Between 18 and 26 weeks' gestation, mothers checked FHRM twice daily with a commercially available Doppler monitor and underwent weekly or biweekly surveillance fetal echocardiograms. If FHRM was abnormal, a diagnostic echocardiogram was performed. Cardiac cycle length and atrioventricular interval were measured, and cardiac function was assessed on all echocardiograms. After 26 weeks, home FHRM and echocardiograms were discontinued, and mothers were monitored during routine obstetrical visits. Postnatal electrocardiograms were performed.Results: Most mothers (273 of 315, 87%) completed the monitoring protocol, generating 1,752 fetal echocardiograms. Abnormal FHRM was detected in 21 mothers (6.7%) who sought medical attention >12 h (n = 7), 3 to 12 h (n = 9), or <3 h (n = 5) after abnormal FHRM. Eighteen fetuses had benign rhythms, and 3 had second- or third-degree AVB. Treatment of second-degree AVB <12 h after abnormal FHRM restored sinus rhythm. Four fetuses had first-degree AVB diagnosed by echocardiography; none progressed to second-degree AVB. No AVB was missed by home FHRM or developed after FHRM.Conclusions: Home FHRM confirms the rapid progression of normal rhythm to AVB and can define a window of time for successful therapy. (Prospective Maternal Surveillance of SSA [Sjögren Syndrome A] Positive Pregnancies Using a Hand-held Fetal Heart Rate Monitor; NCT02920346). [ABSTRACT FROM AUTHOR]