The Prevalence of Congenital Anomalies of the Airway or Lung in Infants with Hypoplastic Left Heart Syndrome and Differences in Midterm Outcomes: A National Pediatric Cardiology Quality Improvement Collaborative Registry Analysis.

Infants with single ventricle physiology and congenital anomalies of the airway and/or lung are potentially poor candidates for staged palliation. The prevalence and midterm outcomes for patients with anomalies of the airway or lung with hypoplastic left heart syndrome are unknown. We performed an analysis of data in infants with hypoplastic left heart syndrome from the National Pediatric Cardiology Quality Improvement Collaborative registry. The prevalence of congenital anomalies of the airway or lung in the registry was determined. Clinical characteristics and midterm outcomes were compared between infants with hypoplastic left heart syndrome with and without anomalies of the airway or lung. Fifty-seven (2.3%) of 2467 infants with hypoplastic left heart syndrome enrolled in the registry had congenital airway or lung anomalies. Infants congenital anomalies of the airway or lung had significantly lower transplant-free survival at 1 year (49.5 vs 77.2%, p < 0.001). Infants with airway or lung anomalies had longer combined hospital length of stay for stage 1 and stage 2 palliation (102 vs 65.1 days, p < 0.001) and underwent more major procedures (2.04 vs 0.93 procedures, p < 0.001) than those without. There was no difference in the number of interstage readmissions (1.85 vs 1.89, p = 0.87) or need for non-oral feeding supplementation (71.4 vs 54.5%, p = 0.12) between groups. Infants enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry with hypoplastic left heart syndrome and anomalies of the airway or lung have increased morbidity and mortality at 1 year compared to those with hypoplastic left heart syndrome alone.

Hospitalisations of children and adults with Hypoplastic Left Heart Syndrome in Texas from 2009 to 2019.

INTRODUCTION: Hypoplastic Left Heart Syndrome accounts for a significant proportion of CHD morbidity and mortality, despite improvements in care and improved survival. This study evaluates number of, reasons for, and trends in discharges of patients with hypoplastic left heart syndrome over 11 years in Texas. METHODS: The Texas Inpatient Discharge Dataset Public Use File captures almost all discharges in Texas and was reviewed from 2009 to 2019. Discharges of patients ≥5 years of age and diagnosis codes for Hypoplastic Left Heart Syndrome were included. The admitting and principle diagnoses were categorised and all discharges were evaluated for procedures performed. Descriptive and univariate statistical analyses were performed. RESULTS: A total of 1024 discharges were identified with a 16.9% annual increase over the study period. Median length of stay was 4 [IQR: 2-8] and there were 17 (1.7%) in-hospital mortalities with no differences across age groups. Seven (17.1%) discharges of patients 25+ years were uninsured, higher than other age groups (p < 0.001). The most common admitting diagnosis was CHD and 224 (21.9%) of discharges included a procedure, including 23 heart transplants. Discharges occurred from 67 different hospitals with 4 (6.0%) representing 71.4% of all discharges. CONCLUSIONS: Discharges of Hypoplastic Left Heart Syndrome have increased rapidly, particularly in the older age groups and were spread over a large number of hospitals. Further work is needed to understand the interplay between Hypoplastic Left Heart Syndrome and other conditions and care experiences that occur within the general population, which will become more common as this population ages and grows.

Geographic Distribution of Congenital Heart Disease: A Single Surgical Center Experience.

OBJECTIVE: To determine presence of spatial clustering or dispersion of pre and postnatally detected hypoplastic left heart syndrome (HLHS) and d-transposition of the great arteries (TGA) cases. STUDY DESIGN: This retrospective study examined all patients with a prenatal or postnatal diagnosis of HLHS or TGA who had an initial visit or hospitalization at our tertiary care center over a 5-year period from 2012 to 2016 (n = 105). Using geographic information systems software, the nearest neighbor ratio (NNR) tool was used to determine whether statistically significant clustering or dispersion occurred. RESULTS: Geographic clustering was observed among prenatally diagnosed pooled cases of HLHS and TGA and all total cases (NNR = 0.73 and 0.66, respectively), but not postnatally detected cases (NNR = 1.08). Notably, there was significant dispersion of postnatally detected TGA cases (NNR = 1.22) There was no pattern for prenatally detected TGA or HLHS when analyzed individually. CONCLUSIONS: The spatial distribution of HLHS and TGA is not random; these conditions occur in geographic clusters. Clustering of all patients in the study population and dispersion of postnatal diagnosis of TGA represent opportunities for improved delivery of fetal cardiac care.

Impact of Socioeconomic Status, Race and Ethnicity, and Geography on Prenatal Detection of Hypoplastic Left Heart Syndrome and Transposition of the Great Arteries.

BACKGROUND: Prenatal detection (PND) has benefits for infants with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but associations between sociodemographic and geographic factors with PND have not been sufficiently explored. This study evaluated whether socioeconomic quartile (SEQ), public insurance, race and ethnicity, rural residence, and distance of residence (distance and driving time from a cardiac surgical center) are associated with the PND or timing of PND, with a secondary aim to analyze differences between the United States and Canada. METHODS: In this retrospective cohort study, fetuses and infants <2 months of age with HLHS or TGA admitted between 2012 and 2016 to participating Fetal Heart Society Research Collaborative institutions in the United States and Canada were included. SEQ, rural residence, and distance of residence were derived using maternal census tract from the maternal address at first visit. Subjects were assigned a SEQ z score using the neighborhood summary score or Canadian Chan index and separated into quartiles. Insurance type and self-reported race and ethnicity were obtained from medical charts. We evaluated associations among SEQ, insurance type, race and ethnicity, rural residence, and distance of residence with PND of HLHS and TGA (aggregate and individually) using bivariate analysis with adjusted associations for confounding variables and cluster analysis for centers. RESULTS: Data on 1862 subjects (HLHS: n=1171, 92% PND; TGA: n=691, 58% PND) were submitted by 21 centers (19 in the United States). In the United States, lower SEQ was associated with lower PND in HLHS and TGA, with the strongest association in the lower SEQ of pregnancies with fetal TGA (quartile 1, 0.78 [95% CI, 0.64-0.85], quartile 2, 0.77 [95% CI, 0.64-0.93], quartile 3, 0.83 [95% CI, 0.69-1.00], quartile 4, reference). Hispanic ethnicity (relative risk, 0.85 [95% CI, 0.72-0.99]) and rural residence (relative risk, 0.78 [95% CI, 0.64-0.95]) were also associated with lower PND in TGA. Lower SEQ was associated with later PND overall; in the United States, rural residence and public insurance were also associated with later PND. CONCLUSIONS: We demonstrate that lower SEQ, Hispanic ethnicity, and rural residence are associated with decreased PND for TGA, with lower SEQ also being associated with decreased PND for HLHS. Future work to increase PND should be considered in these specific populations.

Post-operative arrhythmias in patients with hypoplastic left heart syndrome and anatomic variants: incidence, type, and course.

BACKGROUND: Arrhythmias are common in the post-operative course of patients with hypoplastic left heart syndrome. We sought to determine the types, incidence, risk factors, and impact of arrhythmias in patients with HLHS and anatomic variants. METHODS: We performed a retrospective chart review of 120 consecutive patients with HLHS and anatomical variants, who had single-ventricle palliation at our institution from January, 2006 to December, 2016. RESULTS: A total of thirty-one patients (26%) had 37 episodes of arrhythmias over a median follow-up period of 3.5 years. Of the 37 episodes, 12 (32.4%) were ectopic atrial tachycardia, 9 (24.3%) were paroxysmal supraventricular tachycardia, 4 (10.8%) were junctional ectopic tachycardia, 5 (13.6%) were sinus node dysfunction, 3 (8.1%) were heart block, 2 (5.4%) were atrial flutter, and 2 (5.4%) were ventricular tachycardia. Twenty-four (65%) of the arrhythmias occurred at post-stage 1 surgery. Most (64.8%) of the arrhythmias were resolved. Arrhythmias that occurred at post-stage 1 surgery were more likely to resolve compared to post-stages 2 or 3 (p = 0.006). No anatomical, surgical, or clinical variables were associated with arrhythmia except for age (OR per unit decrease in age at stage 1 palliation: 1.12 (95% CI 1.003, 1.250); p = 0.0439). Arrhythmias were not associated with length of hospital stay or mortality. CONCLUSION: Arrhythmias are common in patients with HLHS and anatomic variants, with EAT and PSVT being the most common types. Arrhythmias were associated with younger age at surgery, but did not affect mortality or length of hospital stay.

A propósito del artículo Síndrome del corazón izquierdo hipoplásico / A Propos of the Article Síndrome del corazón izquierdo hipoplásico [Hypoplastic Left Heart Syndrome]

Con gran interés leí el artículo: Síndrome del corazón izquierdo hipoplásico, de la autoría de Crespo Vallejo y otros investigadores ecuatorianos. Este síndrome (ICD10CM: Q23.4) representa entre el 7 y el 9 por ciento de las malformaciones congénitas cardiacas y constituye la causa más frecuente de insuficiencia cardiaca y de mortalidad neonatal por cardiopatías congénitas (CC) en la primera semana de vida.1 Los autores describen muy adecuadamente al síndrome de cavidades izquierdas hipoplásicas (SCIH)…como una combinación de varias malformaciones que, en su conjunto, son los responsables de todas las manifestaciones clínicas de la enfermedad y citando a Laranjo y otros, plantean que: …"Se conoce que todas estas afecciones tienen un componente genético; sin embargo, en el caso del SCIH no se ha identificado aún el tipo o localización del trastorno responsable de la aparición del...(AU)

Six-Year Neurodevelopmental Outcomes for Children With Single-Ventricle Physiology.

OBJECTIVES: To determine if neurodevelopmental deficits in children with single-ventricle physiology change with age and early developmental scores predict 6-year outcomes. METHODS: In the Single Ventricle Reconstruction Trial, Bayley Scales of Infant Development, Second Edition, were administered at 14 months of age, and parents completed the Behavior Assessment System for Children, Second Edition (BASC-2) annually from the ages of 2 to 6 years. Scores were classified as average, at risk, or impaired. We calculated sensitivities, specificities, and positive and negative predictive values of earlier tests on 6-year outcomes. RESULTS: Of 291 eligible participants, 244 (84%) completed the BASC-2 at 6 years; more Single Ventricle Reconstruction participants than expected on the basis of normative data scored at risk or impaired on the BASC-2 Adaptive Skills Index at that evaluation (28.7% vs 15.9%; P < .001). Children with Adaptive Skills Composite scores <2 SD below the mean at the age of 6 were more likely to have had delayed development at 14 months, particularly on the Psychomotor Development Index (sensitivity of 79%). However, the positive predictive value of the 14-month Mental Development Index and Psychomotor Development Index for 6-year BASC-2 Adaptive Scores was low (44% and 36%, respectively). Adaptive Skills Composite score impairments at the age of 6 were poorly predicted by using earlier BASC-2 assessments, with low sensitivities at the ages of 3 (37%), 4 (48%), and 5 years (55%). CONCLUSIONS: Many children with hypoplastic left heart syndrome who have low adaptive skills at the age of 6 years will not be identified by screening at earlier ages. With our findings, we highlight the importance of serial evaluations for children with critical congenital heart disease throughout development.

Higher Incidence of Protein-Losing Enteropathy in Patients with Single Systemic Right Ventricle.

Patients with single ventricle congenital heart disease are at risk of unpredictable protein-losing enteropathy (PLE) after surgical palliation. Based on prior reports of physiologic differences for patients with single morphologic right versus left ventricles, we hypothesized that those with right ventricular morphology would have a higher incidence of PLE. We performed a retrospective review of > 15 million pediatric hospitalizations from the Healthcare Cost and Utilization Project KID 2000-2012 databases for admissions 5-21 years old with ICD-9 codes for hypoplastic left heart syndrome (HLHS) and tricuspid atresia (TA) with and without PLE. Incidence of PLE was compared between those with HLHS and TA. In addition, outcomes and costs were compared between admissions with and without PLE and between HLHS and TA. Of 1623 HLHS admissions, 289 (17.8%) had PLE, and of 926 TA admissions, 58 (5.9%) had PLE (p < 0.001). Admissions with PLE were older compared to those without PLE (12 vs 10 years, p < 0.001) and PLE onset occurred at a younger age for HLHS than TA (11 vs 14 years, p < 0.001). There were no differences in hospital outcomes or costs. Review of this large administrative database suggests a higher incidence of PLE in patients with HLHS and a younger age of onset compared to those with TA. These data suggest that a single systemic right ventricle may be an independent risk factor for developing PLE.

Detection screening and seasonality evaluation of hypoplastic left heart syndrome in the polish national registry for fetal cardiac anomalies from the years 2004 to 2016.

OBJECTIVES: To evaluate the incidence of hypoplastic left heart syndrome (HLHS) and the efficiency of the screening program using data from the Polish National Registry for Fetal Cardiac Anomalies. To investigate whether HLHS incident rates in Poland are seasonally variable. METHODS: Data on 791 cases of HLHS from the Registry collected between 2004 and 2016 was analyzed. RESULTS: The median gestational age for the 734 cases of HLHS detected was 23 weeks. Comparing the age at time of HLHS detection between 2004 and 2016, a decrease from 26 to 20.8 weeks was observed. We noted a rapid increase in HLHS incidence during the initial years of the Registry data, the annual percentage change during that period was 22.0% and this trend lasted until 2010. In the following years, the Registry became representative of the general population which has an estimated incidence of HLHS of 20.93 cases per 100 000 live births. We observed no clear seasonal patterns of HLHS incidence in our population. CONCLUSION: The Registry reached a plateau state in terms of diagnosing new cases of HLHS. No evidence of seasonality has been noted. The average gestational age of patients identified as having HLHS decreased significantly during the study period.

Síndrome del corazón izquierdo hipoplásico / Hypoplastic Left Heart Syndrome

Introducción: El síndrome del corazón izquierdo hipoplásico es una rara anormalidad que se presenta en el periodo prenatal. Sus manifestaciones clínicas se relacionan con la presencia de hipoxemia a nivel sistémico. Objetivo: Socializar las manifestaciones clínicas y elementos diagnósticos del síndrome del corazón izquierdo hipoplásico. Caso clínico: Recién nacido masculino, a término, que presenta manifestaciones clínicas y ecosonográficas que permiten llegar al diagnóstico de un síndrome del corazón izquierdo hipoplásico. Conclusiones: A pesar de los avances de la ciencia y la técnica, el síndrome del corazón izquierdo hipoplásico presenta una elevada mortalidad. Una adecuada planificación de la gestación, el seguimiento preciso del embarazo, un correcto asesoramiento genético y la utilización de técnicas quirúrgicas para corregir las deformidades resultantes parecen ser las herramientas más eficaces para enfrentar esta mortal afectación(AU)

Introduction: Hypoplastic left heart syndrome is a rare abnormality that occurs in the prenatal period. Its clinical manifestations are related to the presence of hypoxemia at the systemic level. Objective: To describe the clinical manifestations and diagnostic elements of hypoplastic left heart syndrome. Clinical case: A male newborn, at term, who presented clinical and echographic manifestations that permitted the diagnosis of hypoplastic left heart syndrome. Conclusions: Despite advances in science and technology, hypoplastic left heart syndrome presents a high mortality. Proper planning for pregnancy, accurate pregnancy monitoring, proper genetic counseling, and the use of surgical techniques to correct the resulting deformities seem to be the most effective tools to deal with this deadly condition(AU)

Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia: A Swedish national cohort study.

BACKGROUND: Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia. METHODS: Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993-2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016. RESULTS: The 1993-2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome. CONCLUSION: The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.

Necrotizing Enterocolitis and Associated Mortality in Neonates With Congenital Heart Disease: A Multi-Institutional Study.

OBJECTIVE: There are scarce data about the prevalence and mortality of necrotizing enterocolitis in neonates with congenital heart disease. The purpose of this study is to provide a multi-institutional description and comparison of the overall prevalence and mortality of necrotizing enterocolitis in neonates with congenital heart disease. DESIGN: Retrospective multi-institutional study. SETTING: The Pediatric Health Information System database. PATIENTS: Neonates with congenital heart disease between 2004 and 2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The primary study measure is the prevalence of necrotizing enterocolitis. Secondary measures include in-hospital mortality, hospital charges, ICU length of stay, hospital length of stay, and 30-day readmission. The prevalence of necrotizing enterocolitis was 3.7% (1,448/38,770) and varied significantly among different congenital heart disease diagnoses. The lowest prevalence of necrotizing enterocolitis was in transposition of the great arteries (n = 104, 2.1%). Compared with transposition of the great arteries, necrotizing enterocolitis occurred more frequently in neonates with hypoplastic left heart syndrome (odds ratio, 2.7; 95% CI, 2.1-3.3), truncus arteriosus (odds ratio, 2.6; 95% CI, 1.9-3.5), common ventricle (odds ratio, 2.1; 95% CI, 1.5-2.8), and aortic arch obstruction (odds ratio, 1.4; 95% CI, 1.1-1.7). Prematurity is a significant risk factor for necrotizing enterocolitis and for mortality in neonates with necrotizing enterocolitis, conferring varying risk by cardiac diagnosis. Unadjusted mortality associated with necrotizing enterocolitis was 24.4% (vs 11.8% in neonates without necrotizing enterocolitis; p < 0.001), and necrotizing enterocolitis increased the adjusted mortality in neonates with transposition of the great arteries (odds ratio, 2.5; 95% CI, 1.5-4.4), aortic arch obstruction (odds ratio, 1.8; 95% CI, 1.3-2.6), and tetralogy of Fallot (odds ratio, 1.6; 95% CI, 1.1-2.4). Necrotizing enterocolitis was associated with increased hospital charges (p < 0.0001), ICU length of stay (p = 0.001), and length of stay (p = 0.001). CONCLUSIONS: The prevalence of necrotizing enterocolitis among neonates with congenital heart disease is 3.7% and is associated with increased in-hospital mortality, length of stay, and hospital charges. The prevalence and associated mortality of necrotizing enterocolitis in congenital heart disease vary among different heart defects.

Trends in long-stay admissions to a UK paediatric intensive care unit.

OBJECTIVE: Prolonged admission to a paediatric intensive care unit (PICU) consumes significant healthcare resource. An increase in the number of long-stay admissions and bed utilisation has been reported elsewhere in the world but not in the UK. If an increasing trend of long-stay admissions is evident, this may have significant implications for provision of paediatric intensive care in the future. DESIGN/SETTING/PATIENTS: We retrospectively analysed prospectively collected data from Birmingham Children's Hospital, UK, over a 20-year period from 1998 to 2017. PICU admissions, bed-days, length of stay and mortality trends were analysed and reported over four different epochs (1998-2002, 2003-2007, 2008-2012 and 2013-2017) for long-stay admissions (PICU length of stay ≥28 days) and others. Differences in patient demographics, diagnostic categorisation and hospital utilisation were also analysed. RESULTS: In total, 24 203 admissions accounted for 131 553 bed-days over the 20-year period. 705 (2.9%) long-stay admissions accounted for 42 312 (32%) bed-days. Proportion of long-stay admissions and corresponding bed-days increased from 1.6% and 20.5% in 1998-2002 to 4.5% and 42.6%, respectively, in 2013-2017 (p<0.001). Long-stay patients had a significantly higher number of hospital admissions (median: 4 vs 2, p<0.001) per patient and overall hospital length of stay (median: 98 vs 15, p<0.001) bed-days compared with other patients. Long-stay admissions were associated with significantly higher crude mortality (23% vs 6%, p<0.001) compared with other admissions. CONCLUSIONS: A significant increase in the proportion of prolonged PICU admissions with disproportionately high resource utilisation and mortality is evident over two decades.

Outcomes of hypoplastic left heart syndrome and fetal aortic valvuloplasty in a country with suboptimal postnatal management.

BACKGROUND: Fetal aortic stenosis (AoS) may progress to hypoplastic left heart syndrome (HLHS) in utero. There are currently no data, prenatal or postnatal, describing survival of fetuses or neonates with AoS or HLHS in a country with suboptimal postnatal management. STUDY DESIGN: Prospective cohort study performed in Mexico, including cases diagnosed with AoS and HLHS within a 6-year period. AoS patients fulfilling previously published criteria for evolving HLHS (eHLHS) were offered fetal aortic valvuloplasty. Outcome variables were perinatal mortality, postnatal management, type of postnatal circulation, and overall survival. RESULTS: Fifty-four patients were included: 16 AoS and 38 HLHS. Eighteen patients had associated anomalies and/or an abnormal karyotype. Seventy-four percent of HLHS received comfort measures, with only three cases reporting an attempt at surgical palliation, and one survivor of the first stage. Fetal aortic valvuloplasty was performed successfully in nine cases of eHLHS. Overall postnatal survival was 44% in AoS with fetal aortic valvuloplasty, and one case (ongoing) in the HLHS group. CONCLUSIONS: HLHS in Mexico carries more than a 95% risk of postnatal death, with little or no experience at surgical palliation in most centers. Fetal aortic valvuloplasty in AoS may prevent progression to HLHS and in this small cohort was associated with ≈50% survival.

Fetal cardiac findings and hemodynamic changes associated with severe lower urinary tract obstruction in utero.

OBJECTIVES: To describe fetal echocardiographic findings associated with lower urinary tract obstruction (LUTO) and to compare anatomic and hemodynamic measurements between fetuses with LUTO and gestational age (GA)-matched controls, with an emphasis on quantitative indices of diastolic function and cardiac output. METHODS: This was a retrospective cohort study of fetuses diagnosed with severe LUTO with giant bladder, which underwent at least one fetal echocardiogram at our center between January 2005 and June 2018. Fetuses with major congenital heart disease were excluded. Control fetuses did not have any structural or functional abnormalities and were GA-matched to the LUTO fetuses based on the time of the first fetal echocardiogram. Cardiac anatomy and hemodynamic measurements were compared between fetuses with LUTO and controls. In infants with LUTO, serial fetal and postnatal echocardiographic data were assessed, when available, and clinical outcomes were reviewed. RESULTS: Twenty-six fetuses with LUTO and at least one fetal echocardiogram available were identified, one of which was excluded due to hypoplastic left heart syndrome, leaving 25 LUTO fetuses in the final cohort. The mean GA at the first fetal echocardiogram was 25.4 ± 5.1 weeks in the LUTO group and 25.3 ± 5.0 weeks in the control group. Common findings in fetuses with LUTO included cardiomegaly (40%), pericardial effusion (44%), right ventricular (RV) hypertrophy (64%) and left ventricular (LV) hypertrophy (48%). Compared with GA-matched controls, LUTO fetuses had lower ascending aorta Z-score (-0.10 ± 0.94 vs -0.93 ± 1.03; P = 0.02) and aortic isthmus Z-score (-0.14 ± 0.86 vs -1.62 ± 1.11; P < 0.001), shorter mitral valve inflow time indexed to cardiac cycle length (0.46 ± 0.04 vs 0.41 ± 0.06; P = 0.002), and worse (increased) LV myocardial performance index (0.39 ± 0.03 vs 0.44 ± 0.04; P < 0.001). In addition, the ratio of RV to LV cardiac index was higher in LUTO fetuses compared with controls (1.62 ± 0.13 vs 1.33 ± 0.11; P < 0.001). Of the 25 LUTO pregnancies, two were lost to follow-up, three underwent elective termination of pregnancy and three ended in intrauterine fetal demise. Four (16%) patients had mildly hypoplastic left-heart structures, comprising two with aortic arch hypoplasia and two with mitral and aortic stenosis. CONCLUSION: In addition to presenting with cardiomegaly, pericardial effusion and ventricular hypertrophy, fetuses with LUTO demonstrate LV diastolic dysfunction and appear to redistribute cardiac output as compared to control fetuses, which may contribute to the development of left-heart hypoplasia. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Prenatally diagnosed foramen ovale restriction in fetuses with hypoplastic left heart syndrome may be a predictor of longer hospitalization, but not of a need for an urgent rashkind procedure.

OBJECTIVES: This prospective study aimed to assess the effectiveness of foramen ovale examination in classifying prenatal hypoplastic left heart syndrome (HLHS) in accordance with the new classification groupings for congenital heart defects. MATERIAL AND METHODS: The analysis included 145 fetuses with HLHS, diagnosed and monitored between 2008 and 2015 in Prenatal Cardiology Department at Polish Mother's Memorial Hospital Research Institute in Lodz. The main criteria for classifying our study population into three sub-groups was was the presence of a foramen ovale restriction, which we diagnosed by evaluating the diameter and blood flow through the foramen ovale. Of the total group, 73.8% (n = 107) were classified as severe planned, 24.1% (n = 35) as severe urgent, and 2.1% (n = 3) as the severest group. RESULTS: Comparing the severe planned and the severe urgent HLHS groups showed: gestational age of delivery 38 vs 38 weeks respectively (p = 0.45); cesarean delivery 62% vs 79.2% (p = 0.15); neonatal birth weight 3110 g vs 2985 g (p = 0.2); Apgar score 9 vs 9 points; survival rate 65.8% vs 61.9% (p = 0.8); and hospitalization 38 vs 46.5 days (p = 0.059). Prenatal qualification for the group of severe urgent HLHS was characterized by 100% sensitivity, 80.6% specificity and a low posi- tive predictive value of 9.5%. CONCLUSIONS: 1. Prenatal qualification into the group of severe urgent CHD based on the features of foramen ovale was characterized by high sensitivity, a satisfying specificity and a low positive predictive value. 2. Prenatally diagnosed foramen ovale restriction may be a predictor of longer hospitalization, but not of a need for an urgent Rashkind procedure. 3. New classifications of CHDs allowed clinicians to determine prognoses and to plan optimal multi-specialized care which resulted in similar outcomes between the severe planned and severe urgent HLHS groups.

Changing Epidemiology of Hypoplastic Left Heart Syndrome: Results of a National Swedish Cohort Study.

Background Norwood surgery provides a palliative surgical option for hypoplastic left heart syndrome and has been available in Sweden since 1993. The practice of prenatal ultrasound screening was gradually implemented in the same era, resulting in an increased prenatal detection rate. Our primary aims were to study changes in the incidence of live births, prenatal detection rate, and the termination of pregnancies over time. The secondary aims were to study the proportion of live-borns undergoing surgery and to identify factors that influenced whether surgery was or was not performed. Methods and Results Neonates with hypoplastic left heart syndrome with aortic atresia born 1990-2010 were identified through national databases, surgical files, and medical records. The fetal incidence was estimated from the period when prenatal screening was rudimentary. The study period was divided into the presurgical, early surgical, and late surgical periods. The incidence was calculated as the overall yearly incidence for each time period and sex separately. Factors influencing whether surgery was performed were analyzed using Cox-logistic regression. The incidence at live birth decreased from 15.4 to 8.4 per 100 000. The prenatal detection rate increased from 27% to 63%, and terminations increased from 19% to 56%. The odds of having surgery was higher in the late period and higher in the group with prenatal diagnosis. Conclusions We observed a decrease in incidence of live-borns with hypoplastic left heart syndrome aortic atresia. There was in increase in prenatal detection rate and an increase in termination of pregnancy. The proportion of live-borns who underwent surgery increased between time periods.

The effect of right ventricular function on survival and morbidity following stage 2 palliation: An analysis of the single ventricle reconstruction trial public data set.

OBJECTIVE: Limited information is known on how right ventricular function affects outcomes after stage 2 palliation. We evaluated the impact of different right ventricular indices prior to stage 2 palliation on morbidity and mortality. DESIGN: Retrospective study design. SETTING: Pediatric Heart Network Single Ventricle Reconstruction Trial Public Data Set. PATIENT: Any variant of stage 1 palliation and all anatomic hypoplastic left heart syndrome variants in the trial were evaluated. Echocardiograms prior to stage 2 palliation were analyzed and compared between those who failed and those who survived. INTERVENTION: None. OUTCOME MEASURES: Mortality was defined as death, listed for transplant, or transplanted after stage 2 palliation. Morbidity was evaluated as hospital length of stay and duration of intubation. RESULTS: A total of 283 patients met criteria for analysis. Of those, only 18 patients failed stage 2. Right ventricular fractional area change was less in those who failed (30% vs 34%, P = .039) and right ventricular indexed end-diastolic volume and end-systolic volume were larger in those who failed (142.74 mL/ BSA1.3 vs 111.29 mL/BSA1.3 , P = .023, 88.45 mL/ BSA1.3 vs 62.75 mL/ BSA1.3 , P = .025, respectively). Larger right ventricular indexed end-diastolic and systolic volumes were associated with failure (OR 1.17 [1.01-1.35] P = .021, OR 1.25 [1.03-1.52] P = .021, respectively). Every 10% increase in RV ejection fraction had a 63% decrease in length of stay and a 68% decrease in duration of intubation (P = .014, and P = .039, respectively). CONCLUSION: Patients with decreased right ventricular fractional area change and larger right ventricular indexed end-diastolic and systolic volumes were more likely to fail stage 2 palliation. Those with preserved right ventricular function had a shorter hospital length of stay and duration of intubation. Echocardiographic measurements of right ventricular indices during the interstage period can be utilized to determine the prognosis following stage 2 palliation.