Mortality and Heart Transplantation After Hybrid Palliation of Hypoplastic Left Heart Syndrome: A Systematic Review and Meta-Analysis.

BACKGROUND: Newborns with hypoplastic left heart syndrome (HLHS) who are considered at increased risk for death following Norwood/Sano surgery often undergo hybrid palliation (HP) as initial surgery. We aimed to compile the HP experience in HLHS and its variants and assess the rates of, and risk factors for, death and heart transplantation. METHODS: CINAHL, CINAHL PLUS, PubMed/MEDLINE, and SCOPUS were systematically searched for HP outcome studies of death or heart transplantation in HLHS between 1998 and 2022. Pooled incidence was estimated, and potential risk factors were identified using random-effects meta-analysis and reconstructed time-to-event data from Kaplan-Meier curves. RESULTS: Thirty-three publications were included in our review. Overall, of 1,162 patients 417 died and 57 underwent heart transplantation, resulting in a combined outcome of 40.7%, (474/1,162). There was a trend toward decreasing mortality risk across the stages of palliation. Pooled mortality between HP and comprehensive stage 2 palliation was 25%, after stage 2 up to Fontan palliation was 16%, and 6% post-Fontan. The incidence of death or heart transplantation was higher in high-risk patients-43% died and 10% received heart transplantation. CONCLUSION: Our systematic review and meta-analysis found high rates of death or heart transplantation in HP of HLHS patients between HP and Fontan surgeries. All patients should be closely followed during the initial interstage period, which is associated with the highest hazard. Prospective studies on appropriate patient selection, indications, and / or alternatives, as well as refining HP strategies for managing newborns with HLHS are needed to improve outcomes.

Expectant management of patent ductus arteriosus for preterm infants: A meta-analysis of randomized controlled trials.

We conducted this meta-analysis to compare expectant management of patent ductus arteriosus (PDA) with active treatment for PDA closure in preterm infants. Data from 7 randomized controlled trials (RCTs) showed that all-cause mortality and other clinical adverse outcomes did not differ between expectant management of PDA and active treatment. Future large-scale and double-blinded RCTs with a consistent definition for hemodynamically significant PDA, and focusing on clearly delineated high-risk subgroups or later selective treatment are needed to further evaluate the role of expectant management.

Impaired myocardial deformation persists at 2 years in offspring of mothers with diabetes mellitus.

BACKGROUND: A diabetic intrauterine environment has been proposed as a potential etiological mechanism for in utero programming of cardiac disease, and is associated with impaired fetal cardiac function. We aimed to assess cardiac function in offspring of mothers with diabetes mellitus (ODM) and determine whether fetal cardiac abnormalities persist during follow-up. METHODS: Longitudinal observational study to evaluate and compare myocardial function in 40 ODM to age-matched control offspring (CO). Myocardial deformation was measured using speckle-tracking echocardiography (STE). RESULTS: Significant differences were detected in global longitudinal strain (-20.9 ± 3.1 vs. -23.6 ± 2.2%; p = 0.001), global circumferential strain (-24.4 ± 3.9 vs. -26.9 ± 2.7%; p = 0.017), average radial strain (29.0 ± 9.8 vs. 37.1 ± 7.2%; p = 0.003), average longitudinal systolic strain rate (-1.24 ± 0.25/s vs. -1.47 ± 0.30/s; p = 0.011) and average circumferential systolic strain rate (-1.56 ± 0.37/s vs. -1.84 ± 0.37/s; p = 0.013) in comparison to CO up to 2 years of follow-up. Minimal differences were observed within ODM over the 2-year period. CONCLUSION: Impaired cardiac function in ODM persists during 2 years follow-up. Functional cardiac assessment might therefore be useful to detect these unfavorable changes, independent of screening for congenital heart disease or hypertrophic cardiomyopathy in this population. IMPACT: We demonstrate persistence of subclinical myocardial deformation abnormalities in offspring of mothers with diabetes mellitus from fetal life to early childhood years. These results extend the cellular observations in basic and translational research of developmental programming into the clinical realm. Persistence of subclinical myocardial deformation abnormalities may shed light on the known incidence of early cardiovascular disease in offspring of mother with diabetes. Cardiac myocardial strain assessment can be useful to detect these abnormalities, independent of screening for congenital heart disease or hypertrophic cardiomyopathy in this population.

Vortex Formation Time is a Novel Measure for Early Detection of Diastolic Abnormalities in Adolescents with Hypertension.

Pediatric hypertension (HTN) has demonstrated an upward trend in recent years. Adolescent HTN has been linked to adult HTN, cardiovascular disease, and other health conditions. Thus, it is essential that HTN and its associated cardiac abnormalities be diagnosed and treated early to minimize lifelong adverse effects. In this study, we evaluated whether vortex formation time (VFT), a validated echocardiogram measure of left ventricular diastolic dysfunction, correlated with ambulatory blood pressure monitoring (ABPM) and HTN in adolescents. Echocardiogram data including systolic and diastolic function indices and ABPM data from 2015 to 2022 in adolescents age 13-21 years were analyzed retrospectively. We found that VFT was significantly lower in adolescents with HTN compared to those without HTN (3.69 ± 1.39 vs. 4.50 ± 1.73, p = 0.02). Standard echocardiographic indices of systolic and diastolic function were similar between the two groups, except indexed left atrial volume. Higher overall systolic blood pressure (SBP) (ß = - 0.01, CI - 0.02, - 2.2 × 10-3, p = 0.02), mean wake SBP (ß = - 0.01, CI - 0.02, - 9.4 × 10-4, p = 0.03), and mean sleep SBP (ß = - 0.01, CI - 0.02, - 1.2 × 10-3, p = 0.03) were significantly associated with lower VFT. This study demonstrates that VFT correlates to ABPM data and can be used a novel diagnostic measure in adolescents with HTN.

Accelerating integration of social needs into mainstream healthcare to achieve health equity in the COVID-19 era.

It is known that social inequities result in health disparities in outcomes, highlighted in the coronavirus disease 2019 (COVID-19) pandemic. This commentary discusses the actionable initiatives that have been implemented to address social inequities in healthcare in the United States. The publicly available social needs screening tools and International Classification of Disease Systems-10 Z codes for social determinants of health are introduced. In this context, policies, health system strategies and the larger role of implementation science in recognizing and alleviating the social needs are discussed.

Identification of research priorities in CHD: empowering patients and families through participation in the development of formal research agendas.

BACKGROUND: Conquering CHD, formerly known as the Pediatric Congenital Heart Association (PCHA), is the leading congenital heart disease (CHD) patient advocacy organisation in the United States of America, and places high priority on patient engagement in the research process. Participatory design is an approach to problem-solving that utilises the knowledge and opinions of groups of people to generate plans and new ideas. Utilising this mode of patient engagement, patients and families engaged with Conquering CHD assisted in developing a list of research priorities which was then distributed to the larger membership with instructions to rank the priorities in order of importance. Upon completion, these items were compared to the current scientific literature to assess correlation with current publications. This cross-sectional study and literature review aimed to assess the priorities of patients and families in CHD research and to determine the reflection of these areas in the current body of scientific literature. METHODS: This cross-sectional study utilised a survey asking participants to rank the importance of research items within categories including "Technology Advances," "Genetic and Cellular Research," "Broad Understanding of CHD," and "Psychosocial Outcomes" which was distributed through social media and email to 43,168 accounts across all platforms. Respondents were asked to place each item in a ranked order in each category, with the value "1" representing the most preferred for each participant. Anyone engaged with Conquering CHD was eligible to complete the study, including patients and families. Subsequently, a literature review of the largest medical databases including PubMed, Scopus, and ScienceDirect was undertaken to determine the number of articles published per each topic which was then assessed to determine if there is a correlation between patient-ranked priorities and the current body of literature. RESULTS: The study generated a total response of 527 participants. Regarding "Technology Advances," valve replacement was the preferred topic (mean rank 2.07, IQR 2). Stem cell research was the favoured topic in "Genetic and Cellular Research" (mean rank 2.53, IQR 2). Access to care was the priority in the "Broadening Understanding of CHD" (mean rank 1.24, IQR 1). Pertaining to "Psychosocial Outcomes", psychological/emotional effects was the highest ranked topic (mean rank 1.46, IQR 1). The literature review returned a total of 135,672 articles in the areas of interest. For "Valve Replacement", 8361 articles resulted reflecting a proportion of 0.097 of total articles. For "Stem Cell Research", 9921 articles resulted reflecting a proportion of 0.115 of total articles. For "Access to Care", 7845 articles resulted reflecting a proportion of 0.091 of total articles. For "Psychological/Emotional Effects", 6422 articles resulted reflecting a proportion of 0.074 of total articles. A Spearman's correlation demonstrated no correlation between the preferred domain of CHD research and the number of articles published for that domain (rs = 0.02, p = 0.94). CONCLUSIONS: This process demonstrates the effectiveness of participatory design, using a patient and family network to determine the research items of concern to those affected by CHD. The cross-sectional survey was effective in assessing patient and family priorities but was limited by access to reliable internet and delivery only in English. Though the study had a large response rate, it was limited to patients already engaged with Conquering CHD. For these reasons, it may not completely reflect the opinions of the total population affected by CHD. However, this offers valuable insight into patient-determined priorities and reveals that the current scientific literature does not correlate with these items. These data serve to inform individual and institutional research agendas to better reflect the needs and desires of this population.

Social Determinants of Disparities in Mortality Outcomes in Congenital Heart Disease: A Systematic Review and Meta-Analysis.

Background: Social determinants of health (SDoH) affect congenital heart disease (CHD) mortality across all forms and age groups. We sought to evaluate risk of mortality from specific SDoH stratified across CHD to guide interventions to alleviate this risk. Methods: We searched electronic databases between January 1980 and June 2019 and included studies that evaluated occurrence of CHD deaths and SDoH in English articles. Meta-analysis was performed if SDoH data were available in >3 studies. We included race/ethnicity, deprivation, insurance status, maternal age, maternal education, single/multiple pregnancy, hospital volume, and geographic location of patients as SDoH. Data were pooled using random-effects model and outcome was reported as odds ratio (OR) with 95% confidence interval (CI). Results: Of 17,716 citations reviewed, 65 met inclusion criteria. Sixty-three were observational retrospective studies and two prospective. Of 546,981 patients, 34,080 died. Black patients with non-critical CHD in the first year of life (Odds Ratio 1.62 [95% confidence interval 1.47-1.79], I2 = 7.1%), with critical CHD as neonates (OR 1.27 [CI 1.05-1.55], I2 = 0%) and in the first year (OR 1.68, [1.45-1.95], I2 = 0.3%) had increased mortality. Deprived patients, multiple pregnancies, patients born to mothers <18 years and with education <12 years, and patients on public insurance with critical CHD have greater likelihood of death after the neonatal period. Conclusion: This systematic review and meta-analysis found that Black patients with CHD are particularly vulnerable for mortality. Numerous SDoH that affect mortality were identified for specific time points in CHD course that may guide interventions, future research and policy. Systematic Review Registration: [https://www.crd.york.ac.uk/PROSPERO/display_record.php?ID=CRD42019139466&ID=CRD42019139466], identifier [CRD42019139466].

Influence of right ventricular pressure and volume overload on right and left ventricular diastolic function.

BACKGROUND: Ventricular interdependence may account for altered ventricular mechanics in congenital heart disease. The present study aimed to identify differences in load-dependent right ventricular (RV)-left ventricular (LV) interactions in porcine models of pulmonary stenosis (PS) and pulmonary insufficiency (PI) by invasive admittance-derived hemodynamics in conjunction with noninvasive cardiovascular magnetic resonance (CMR). METHODS: Seventeen pigs were used in the study (7 with PS, 7 with PI, and 3 controls). Progressive PS was created by tightening a Teflon tape around the pulmonary artery, and PI was created by excising 2 leaflets of the pulmonary valve. Admittance catheterization data were obtained for the RV and LV at 10 to 12 weeks after model creation, with the animal ventilated under temporary diaphragm paralysis. CMR was performed in all animals immediately prior to pressure-volume catheterization. RESULTS: In the PS group, RV contractility was increased, manifested by increased end-systolic elastance (mean difference, 1.29 mm Hg/mL; 95% confidence interval [CI], 0.57-2.00 mm Hg/mL). However, in the PI group, no significant changes were observed in RV systolic function despite significant changes in RV diastolic function. In the PS group, LV end-systolic volume was significantly lower compared with controls (mean difference, 25.1 mL; 95% CI, -40.5 to -90.7 mL), whereas in the PI group, the LV showed diastolic dysfunction, demonstrated by an elevated isovolumic relaxation constant and ventricular stiffness (mean difference, 0.03 mL-1; 95% CI, -0.02 to 0.09 mL-1). CONCLUSIONS: The LV exhibits systolic dysfunction and noncompliance with PI. PS is associated with preserved LV systolic function and evidence of some LV diastolic dysfunction. Interventricular interactions influence LV filling and likely account for differential effects of RV pressure and volume overload on LV function.

Continuous and intermittent administration of intravenous sildenafil in critically ill infants with pulmonary hypertension.

Intravenous (IV) sildenafil may be administered as a continuous infusion or intermittent bolus dosing in infants with pulmonary hypertension (PH). We aimed to compare these delivery methods. METHODS: We retrospectively evaluated subjects less than 12 months old treated with IV sildenafil for PH. Vital signs, oxygen requirement, vasoactive-inotropic score (VIS), and echocardiogram results before and after sildenafil initiation, and the need for discontinuation due to side effects, were noted. RESULTS: Forty-three subjects were identified (23 continuous, 20 intermittent). There were clinically significant differences in PH classifications between groups. The continuous group was significantly younger (p = 0.010) with higher baseline severity of illness suggested by higher inspired oxygen (FiO2 ) and VIS (p = 0.012). After sildenafil initiation, there were no significant differences in changes in blood pressure, oxygen saturation, FiO2 , or VIS between groups, and no difference in the number of subjects requiring discontinuation due to side effects (4 continuous, 1 intermittent, p = 0.351). Eight continuous group subjects (34.8%) and 3 intermittent group subjects (15.0%) died (p = 0.024), but echocardiographic improvement in PH degree was more common in the continuous group (77.8% vs. 33.3%, p = 0.007). CONCLUSION: In this small cohort of infants treated with continuous or intermittent IV sildenafil, in the setting of different baseline characteristics between groups, there were no significant differences in changes in vital signs, VIS, FiO2 , or need for discontinuation of therapy due to side effects. Higher continuous group mortality may be explained by greater baseline illness severity, but larger prospective, randomized studies are required to investigate these different delivery methods.

Social determinants of health and coronavirus disease 2019 in pregnancy.

BACKGROUND: The social and physical environments in which people live affect the emergence, prevalence, and severity of both infectious and noninfectious diseases. There are limited data on how such social determinants of health, including neighborhood socioeconomic conditions, affect the risk of severe acute respiratory syndrome coronavirus 2 infection and severity of coronavirus disease 2019 during pregnancy. OBJECTIVE: Our objective was to determine how social determinants of health are associated with severe acute respiratory syndrome coronavirus 2 infection and the severity of coronavirus disease 2019 illness in hospitalized pregnant patients in New York during the global coronavirus disease 2019 pandemic. STUDY DESIGN: This cross-sectional study evaluated all pregnant patients who delivered and had polymerase chain reaction testing for severe acute respiratory syndrome coronavirus 2 between March 15, 2020, and June 15, 2020, at 7 hospitals within Northwell Health, the largest academic health system in New York. During the study period, universal severe acute respiratory syndrome coronavirus 2 testing protocols were implemented at all sites. Polymerase chain reaction testing was performed using nasopharyngeal swabs. Patients were excluded if the following variables were not available: polymerase chain reaction results, race, ethnicity, or zone improvement plan (ZIP) code of residence. Clinical data were obtained from the enterprise electronic health record system. For each patient, ZIP code was used as a proxy for neighborhood. Socioeconomic characteristics were determined by linking to ZIP code data from the United States Census Bureau's American Community Survey and the Internal Revenue Service's Statistics of Income Division. Specific variables of interest included mean persons per household, median household income, percent unemployment, and percent with less than high school education. Medical records were manually reviewed for all subjects with positive polymerase chain reaction test results to correctly identify symptomatic patients and then classify those subjects using the National Institutes of Health severity of illness categories. Classification was based on the highest severity of illness throughout gestation and not necessarily at the time of presentation for delivery. RESULTS: A total of 4873 patients were included in the study. The polymerase chain reaction test positivity rate was 11% (n=544). Among this group, 359 patients (66%) were asymptomatic or presymptomatic, 115 (21%) had mild or moderate coronavirus disease 2019, and 70 (13%) had severe or critical coronavirus disease 2019. On multiple logistic regression modeling, pregnant patients who had a positive test result for severe acute respiratory syndrome coronavirus 2 were more likely to be younger or of higher parity, belong to minoritized racial and ethnic groups, have public health insurance, have limited English proficiency, and reside in low-income neighborhoods with less educational attainment. On ordinal logit regression modeling, obesity, income and education were associated with coronavirus disease 2019 severity. CONCLUSION: Social and physical determinants of health play a role in determining the risk of infection. The severity of coronavirus disease 2019 illness was not associated with race or ethnicity but was associated with maternal obesity and neighborhood level characteristics such as educational attainment and household income.

Age-related changes in diastolic function in children: Echocardiographic association with vortex formation time.

BACKGROUND: This study aims to understand the age-related changes in vortex formation time (VFT) index in children, and thus, describe the ranges of VFT in different pediatric age groups with the ultimate goal of assessment of diastolic function. METHODS AND RESULTS: Transthoracic echocardiograms in healthy (n = 84) subjects from birth to 20 years were analyzed to compute VFT and diastolic performance. LV apical and short-axis views were used. Three separate measurements were performed, and the mean was used to derive VFT and other indices. Statistical comparisons were made amongst the groups, stratified by age. RESULTS: Vortex formation times in neonates (median 1.79, interquartile range 1.31-1.92) and infants (1.38, 1.07-1.72) were found to be significantly lower (P < .05) than the older age groups (1-5 years 2.47, 1.87-2.94, 5-10 years 2.18, 1.89-2.53, 10-20 years 2.34, 1.84-2.96). The changes in VFT correlate to the changes in diastolic function in children. CONCLUSION: Our results show that unlike adults, VFT changes along with the growth-related myocardial adaptations in children, and its range may be used to evaluate diastolic function. The present study is the first to test the significance of the trans-mitral VFT in children by comparing different age groups of healthy subjects.

Risk factors for death or heart transplantation in single-ventricle physiology (tricuspid atresia, pulmonary atresia, and heterotaxy): A systematic review and meta-analysis.

BACKGROUND: In this study we sought to evaluate risk factors (RFs) for death or heart transplantation (D-HT) in single-ventricle (SV) physiology due to tricuspid atresia (TA), pulmonary atresia‒intact ventricular septum (PA-IVS), and heterotaxy with SV (HX), clinical conditions for which outcome data are limited. METHODS: To conduct a systematic review, we included citations that evaluated occurrence of D-HT in SV physiology of TA, PA-IVS, and HX in English articles published between January 1998 and December 2017 based on inclusion and exclusion criteria, following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. The Cochrane Risk of Bias in Non-Randomized Studies-Interventions (ROBINS-I) tool for non-randomized studies was used to assess the risk of bias. Meta-analysis was performed if RF data were available in more than 3 studies. RESULTS: Of 11,629 citations reviewed, 30 met inclusion criteria. All 30 were observational, retrospective studies. In all, 1,770 patients were included, 481 died and 21 underwent HT (63 lost to follow-up); 723 patients reached Fontan completion. We found that systemic ventricular dysfunction (odds ratio [OR] 20.7, confidence interval [CI] 10.0-42.5, I2 = 0%) and atrioventricular valve regurgitation (AVR) were associated with risk of D-HT (OR 3.7, CI 1.9-6.9, I2 = 14%). RF associations with D-HT could not be derived for right ventricle‒dependent coronary circulation, pulmonary arteriovenous malformations, total anomalous pulmonary venous return, arrhythmias, and pulmonary atresia. CONCLUSIONS: This systematic review and meta-analysis has identified a high mortality rate in children born with non-HLHS SV heart disease and points to potential under-utilization of HT. Systemic ventricular dysfunction and AVR were identified as RFs for D-HT in this subset of patients SV with TA, PA-IVS, and HX.

Remodeling of the cardiovascular circulation in fetuses of mothers with diabetes: A fetal computational model analysis.

AIMS: Myocardial structural and functional abnormalities are known to occur in fetuses of mothers with diabetes mellitus (FMDM). The main aim of this investigation was to explore the cardiovascular circulatory patterns in FMDM using a validated lumped computational model of the cardiovascular system. METHODS: This was a multi-institutional study involving FMDM compared to fetuses of maternal controls (FC). Fetal echocardiographic Doppler data from left and right ventricular outflow tracts, aortic isthmus, middle cerebral and umbilical arteries were fitted into a validated fetal circulation computational model to estimate patient-specific placental and vascular properties. Non-parametric comparisons were made between resistances, compliances and flows in the brain and placenta in FMDM and FC. RESULTS: Data from 23 FMDM and 31 FC were fitted into the model. In FMDM, compared to FC, placental relative resistance was lower (0.59 ±â€¯0.50 versus 0.91 ±â€¯0.41; p < .05) with higher brain relative resistance (2.36 ±â€¯1.65 versus 1.60 ±â€¯0.85; p < .05). Middle cerebral artery flow was lower in FMDM than FC (0.12 ±â€¯0.14 vs. 0.27 ±â€¯0.21 ml/min; p 0.04) with a lower cerebral-placental flow ratio. Combined stroke volume was lower in FMDM (3.65 ±â€¯2.05 ml) than FC (4.97 ±â€¯2.45 ml) (p 0.04). CONCLUSIONS: Blood flow is redistributed in FMDM to the placenta, away from the brain. This alteration may play a role in the postnatal health of these fetuses.

Depressed left and right ventricular cardiac output in fetuses of diabetic mothers.

INTRODUCTION: We compared right and left ventricular cardiac output (RVCO and LVCO) in fetuses of diabetic mothers (FDM) to a large normal cohort. METHODS: We prospectively enrolled 264 normal fetuses and 30 FDM. Fetal CO parameters: semilunar valve velocity time integrals (AVVTI, PVVTI), ventricular outflow diameters (LVOTD, RVOTD), stroke volumes (AVSV, PVSV) were measured, and LVCO and RVCO calculated.  These were normalized using nonlinear regression to estimated fetal weight (EFW) to provide means and standard deviations. Among FDMs, mean Z-scores and 95% confidence limits (CL) were calculated, and compared to zero. RESULTS: LVCO, RVCO, and parameters they were calculated from, increased predictably and non-linearly with increasing EFW.  In FDM, LVCO was depressed (mean Z -1.679, 95% CL -2.404, -0.955, p<0.001), and AVVTI, LVOTD, AVSV significantly lower than normal.  Similarly, RVCO (mean Z = -1.119, CL -1.839, -0.400, p=0.003), RVOTD (mean -2.085, CL -3.077, -1.093, p<0.001), and PVSV (mean -1.184, CL -1.921, -0.446, p=0.003) were lower than normal, however, PVVTI was not different (mean Z 0.078, CL -0.552, +0.707, p=0.803). CONCLUSION: Normal biventricular stroke volumes and outputs follow a nonlinear regression with EFW. FDM have significantly lower right and left heart stroke volumes and outputs for weight than do normal fetuses.

Maternal Serum Aneuploidy Screen and Adverse Pregnancy Outcomes.

OBJECTIVE: To find out whether maternal serum screening for fetal chromosomal aneuploidy predicts adverse pregnancy outcomes. METHODS: A two-year retrospective case-control study was conducted at a tertiary hospital. Pregnant women with a high-risk serum screen but with chromosomally normal fetuses (n = 189) were compared to those with low-risk screen (controls, n = 157) for adverse pregnancy outcomes. RESULTS: Women with high-risk double marker or combined screen were found to have higher prevalence of LBW [OR 2.56; 95 % CI (1.01-6.53), p < 0.05] and PT [OR 2.93; 95 % CI (1.11-7.65), p < 0.05], while women with high-risk triple screen had higher prevalence of PIH [OR 3.72; 95 % CI (1.23-11.18); p < 0.05], Oligo [OR 4.50; 95 % CI (1.30-15.64); p < 0.05], delivery by C-section [OR 2.51; 95 % CI (1.41-4.47); p < 0.005] as compared to low-risk women. PAPP-A was found to be a significant predictor of birth weight (R (2) = 12.2 %, ß ± SE = 0.224 ± 0.069; p < 0.005) and gestational age (R (2) = 4.9 %, ß ± SE = 0.613 ± 0.296; p < 0.05). Beta hCG in first and hCG in second trimester predicted oligohydramnios (R (2) = 9.2 %, ß ± SE = -0.077 ± 0.025; p < 0.005). The areas under the ROC curves of PAPP-A for LBW and PT were 0.70(p < 0.01) and 0.684 (p < 0.05), respectively. CONCLUSION: A "high-risk" maternal serum screen with abnormal PAPP-A and/or beta hCG/HCG is associated with adverse pregnancy outcomes and may help identifying women requiring additional fetal surveillance.

Lack of Association of ST-T Wave Abnormalities to Congenital Heart Disease in Neonates.

BACKGROUND: ST-T wave (STTW) abnormalities have been described in 20-40% of normal newborns. We sought to describe the associations of these Electrocardiogram (ECG) abnormalities to perinatal course and congenital heart disease (CHD). METHODS: A retrospective chart review was performed on all neonatal ECGs between January 2008 and March 2013 identified from electronic medical records. Electronic medical records were reviewed for perinatal course and maternal medical conditions. Neonates <37 weeks gestation, >3 days age, requiring hemodynamic support in the first 3 days, with oxygen saturation <90% on room air, or with arrhythmias and significant abnormalities of axis and voltage were excluded from the analysis. ST segment elevation or depression of >2 mm in at least one lead and flat or inverted T waves in at least one lead except aVR were considered abnormal. Statistical relationships were explored between STTW abnormalities, perinatal variables and CHD. RESULTS: ECGs were performed on 1043 neonates, of which 664 were included. STTW abnormalities were found in 236 (35.5%) neonates. T wave abnormalities were identified in 191 (28.7%), ST segment abnormalities in 77 (11.6%) and both on 32 (4.8%) neonates. No relationship was found between the ECG abnormalities and perinatal variables, except maternal cefazolin administration during labor. Noncritical CHD was diagnosed by echocardiography in 59/84; STTW abnormalities were seen in 17/59 (29%) patients with and 9/25 (34%) without noncritical CHD, P = .6. CONCLUSION: STTW abnormalities on ECG are commonly found in 35.5% of normal neonates and do not predict noncritical CHD.

Outcomes and risk factors for listing for heart transplantation after the Norwood procedure: An analysis of the Single Ventricle Reconstruction Trial.

BACKGROUND: Infants with hypoplastic left heart syndrome after palliation have the worst survival among heart transplant recipients. Heart transplantation is often reserved for use in patients with sub-optimal results after palliative surgery. This study characterized outcomes after listing in infants with a single ventricle who had undergone the Norwood procedure and identified predictors of the decision to list for heart transplantation. METHODS: The public-use database from the multicenter, prospective randomized Single Ventricle Reconstruction trial was used to identify patients who were listed for heart transplantation. Outcomes on the waiting list and after transplantation were determined. Risk factors were compared between those who were listed and those who survived without listing. RESULTS: Among 555 patients, 33 patients (5.9%) were listed and 18 underwent heart transplantation. Mortality was 39% while waiting for a heart and was 33% after heart transplantation. Overall, 1-year survival after listing (including death after transplantation) was 48%. Factors associated with listing were a lower right ventricular fractional area change at birth, non-hypoplastic left heart syndrome diagnosis, and a more complicated post-Norwood course, defined as a higher need for extracorporeal membrane oxygenation, longer intensive care unit stay, more complications, and a higher number of discharge medications. CONCLUSIONS: Worse right ventricular function, non-hypoplastic left heart syndrome diagnosis, and complex intensive care unit stay were significant risk factors for listing for heart transplantation after the Norwood procedure. Heart transplantation as a rescue procedure after the Norwood procedure in the first year of life carries a significant risk of mortality.

Computed Tomography in Congenital Heart Disease: Clinical Applications and Technical Considerations.

Computed tomography (CT) is increasingly applied as an aid to clinical decision making in congenital heart disease (CHD) with regard to surgery, interventional catheterizations, and follow-up. It is particularly useful for the assessment of vascular and airway abnormalities. Updated knowledge of CT imaging techniques is mandatory not only for the optimization of image acquisition protocols, but also for the postprocessing reconstruction and interpretation of CT findings. The purpose of this article was to review the clinical indications, techniques, and dose reduction methods for CT imaging in children and adults with CHD. Relevant discussion and illustrations of common forms of CHD evaluated by CT are presented.

Mortality-related resource utilization in the inpatient care of hypoplastic left heart syndrome.

BACKGROUND: Quantifying resource utilization in the inpatient care of congenital heart diease is clinically relevant. Our purpose is to measure the investment of inpatient care resources to achieve survival in hypoplastic left heart syndrome (HLHS), and to determine how much of that investment occurs in hospitalizations that have a fatal outcome, the mortality-related resource utilization fraction (MRRUF). METHODS: A collaborative administrative database, the Pediatric Health Information System (PHIS) containing data for 43 children's hospitals, was queried by primary diagnosis for HLHS admissions of patients ≤21 years old during 2004-2013. Institution, patient age, inpatient deaths, billed charges (BC) and length of stay (LOS) were recorded. RESULTS: In all, 11,122 HLHS admissions were identified which account for total LOS of 277,027 inpatient-days and $3,928,794,660 in BC. There were 1145 inpatient deaths (10.3%). LOS was greater among inpatient deaths than among patients discharged alive (median 17 vs. 12, p < 0.0001). BC were greater among inpatient deaths than among patients discharged alive (median 4.09 × 10(5) vs. 1.63 × 10(5), p < 0.0001). 16% of all LOS and 21% of all BC were accrued by patients who did not survive their hospitalization. These proportions showed no significant change year-by-year. The highest volume institutions had lower mortality rates, but there was no relation between institutional volume and the MRRUF. CONCLUSIONS: These data should alert providers and consumers that current practices often result in major resource expenditure for inpatient care of HLHS that does not result in survival to hospital dismissal. They highlight the need for data-driven critical review of standard practices to identify patterns of care associated with success, and to modify approaches objectively.

Experiences from Garbha-Swasthya helpline.

Garbha-Swasthya helpline is a telephone helpline run by a tertiary care private hospital to address issues related to pregnancy and its complications. A review of the helpline data from September 2010 to December 2012 was conducted to study caller characteristics, number of calls and related information, and the reasons to call. Out of the total 696 voice calls, 421 were new callers and 275 were repeat callers and they comprised mainly pregnant women (73.3%), their relatives (24.8%), and medical professionals (1.9%). Most calls were related to first pregnancy and were mainly from Pune and other cities of Maharashtra. All calls by the medical professionals were regarding drug safety in pregnancy. The commonest category of questions (27.4%) was about exposures (drugs, alcohol, eclipse, radiation, etc.) and preexisting medical illness followed by pregnancy complications (18.5%), common medical issues such as nausea/vomiting during pregnancy, and delivery and postpartum period (14% each). Although the total number of calls received has steadily increased, efforts to create public and professional awareness to increase the usage of this helpline are warranted.