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1.  [No title available] 
PMCID: PMC3946912  PMID: 24000004
2.  [No title available] 
PMCID: PMC3946929  PMID: 24096716
3.  Indomethacin Use for the Management of Patent Ductus Arteriosus in Preterms: A Web-Based Survey of Practice Attitudes Among Neonatal Fellowship Program Directors in the United States 
Pediatric cardiology  2007;28(3):193-200.
The objective of this study was to determine whether neonatal-perinatal fellowship programs (NFTPs) in the United States vary in indomethacin use for the management of patent ductus arteriosus (PDA) in ≤28 week gestational age infants at birth. A 53-item web-based survey was sent to 84 NFTP directors who received prenotification, followed 2 weeks later by a reminder letter. A total of 56 NFTP directors responded (67% maximum response rate). Wide variation exists in the maximum number of indomethacin courses used to close ductus, use of indomethacin for reopened PDA beyond 14 days, ductal closure definition, contraindications before consideration of indo-methacin, interventions for contraindications, and reported ductal closer rate after each indomethacin course. Indomethacin therapy for symptomatic PDA and short course of indomethacin are common practices. Indomethacin use for the management of PDA in premature infants varies among NFTP directors. Practice attitudes may explain variations in ductal closure and ligation rates. Because practice variations may have implications for long-term outcome of vulnerable premature infants, studies relevant to the management of PDA in premature infants are needed.
doi:10.1007/s00246-006-0093-1
PMCID: PMC4285413  PMID: 17457636
Indomethacin; Health care surveys; Premature infants
4.  Multiple Courses of Indomethacin and Neonatal Outcomes in Premature Infants 
Pediatric cardiology  2007;29(5):878-884.
The objective of this retrospective cohort study was to determine patent ductus arteriosus (PDA) closure rate with multiple short courses (three doses) of postnatal indomethacin and compare neonatal outcomes in infants who received two versus three courses of indomethacin for PDA closure. Infants <34 weeks’ gestational age born between January 2000 and December 2004 at the University of Maryland Medical Center and who received two or more short courses of indomethacin were included. Outcome measures were ductal closure rate and neonatal outcomes. Of 61 infants who were identified to have received two or more courses of indomethacin, 26 infants closed their ductus after the second course (response rate, 42%). Of the 35 infants who failed ductal closure after two courses, 11 infants had their ductus ligated and 23 received a third course of indomethacin. Of 23 who received a third course, 10 closed their ductus (response rate, 43%). There was no significant difference in the incidence of chronic lung disease, severe retinopathy of prematurity, necrotizing enterocolitis, renal function, or mortality between infants who received two and those who received three courses of indomethacin. Infants exposed to three courses of indo-methacin had a statistically nonsignificant increased incidence of periventricular leukomalacia (p = 0.08; adjusted odds ratio = 4.8; 95% CI, 0.8–30) and remained in the hospital for a longer duration (p = 0.02) compared to infants exposed to two courses of indomethacin. We conclude that multiple courses of indomethacin may be associated with a ductal closure. However, the requirement for a third course may be associated with an increased risk of periventricular leukomalacia.
doi:10.1007/s00246-007-9166-z
PMCID: PMC4285430  PMID: 18094917
Multiple courses of indomethacin; Patent ductus arteriosus; Periventricular leukomalacia
5.  First Case of Intracardiac Foregut Cyst Occurring in the Left Ventricular Outflow Tract 
Pediatric cardiology  2013;34(8):10.1007/s00246-012-0623-y.
An 11 day old female underwent resection of a mass in the subaortic region secondary to critical aortic stenosis. At 3 months of age, recurrent severe left ventricular outflow obstruction in the setting of heart failure prompted redo surgery and the resected mass revealed an intracardiac foregut cyst, a rare finding. To our knowledge this is the first case obstructing the left ventricular outflow tract (LVOT).
doi:10.1007/s00246-012-0623-y
PMCID: PMC3664127  PMID: 23341050
Congenital Heart Disease; Tumor; Left ventricle
6.  Active Surveillance of Sudden Cardiac Death in Young Athletes by Periodic Internet Searches 
Pediatric cardiology  2013;34(8):10.1007/s00246-013-0717-1.
Objective
We hypothesized that prospective, systematic Internet searches could identify occurrences of sudden cardiac death (SCD) in athletes, and would be useful to establish a system of active surveillance.
Methods
Weekly advanced Google searches of the Internet were conducted for cases of SCD in young athletes over a 12-month period (2007–8). Athletes aged 11 to 30 years who collapsed during a game, practice, or within an hour of exercise were included. Individuals with known histories of cardiac issues and events occurring outside the United States were excluded. Verification of SCD was by autopsy reports and death certificates from county coroner offices and vital record agencies.
Results
A total of 71 events were initially identified. Verification of the cause of death by coroner reports was possible in 45 cases, of which 43 (96%) were confirmed to be SCDs. Sixty-nine individuals, 11 to 30 years of age (mean 17 ± 5), died suddenly from cardiovascular causes while participating in 15 different organized sports and a variety of non-organized physical activities. The most common cause of death was hypertrophic cardiomyopathy (30%), followed by coronary artery anomalies (9%), and myocarditis (9%). The incidence of athlete SCD, types of sports involved, and cardiac causes of death in our study were comparable to previous reports.
Conclusions
Readily available Internet searches have the potential to be a powerful tool for identifying occurrences of athlete SCD. An active surveillance system using Google searches followed by coroner report verification can provide important epidemiologic and clinical information.
doi:10.1007/s00246-013-0717-1
PMCID: PMC3838872  PMID: 23681420
epidemiology; surveillance; athlete; sudden death; cardiac
7.  Multi-Tiered Analysis of Brain Injury in Neonates with Congenital Heart Disease 
Pediatric cardiology  2013;34(8):1772-1784.
Early brain injury occurs in newborns with congenital heart disease (CHD) placing them at risk for impaired neurodevelopmental outcomes. Predictors for preoperative brain injury have not been well described in CHD newborns. This study aimed to analyze, retrospectively, brain magnetic resonance imaging (MRI) in a heterogeneous group of newborns who had CHD surgery during the first month of life using a detailed qualitative CHD MRI Injury Score, quantitative imaging assessments (regional apparent diffusion coefficient [ADC] values and brain volumes), and clinical characteristics. Seventy-three newborns that had CHD surgery at 8 ± 5 (mean ± standard deviation) days of life and preoperative brain MRI were included; 38 also had postoperative MRI. Thirty-four (34/73, 47%) had at least 1 type of preoperative brain injury, and 28/38 (74%) had postoperative brain injury. The 5-minute APGAR score was negatively associated with preoperative injury, but there was no difference between CHD types. Infants with intraparenchymal hemorrhage, deep gray matter injury, and/or watershed infarcts had the highest CHD MRI Injury Scores. ADC values and brain volumes were not different in infants with different CHD types, or in those with and without brain injury. In a mixed group of CHD newborns, brain injury was found preoperatively on MRI in almost 50%, and there were no significant baseline characteristic differences to predict this early brain injury, except 5-minute APGAR score. We conclude that all infants, regardless of CHD type, who require early surgery, should be evaluated with MRI as they are all at high risk for brain injury.
doi:10.1007/s00246-013-0712-6
PMCID: PMC3973037  PMID: 23652966
brain injury; brain magnetic resonance imaging; congenital heart disease; congenital heart surgery; neonate; infant
8.  Application of a Complication Screening Method to Congenital Heart Surgery Admissions A Preliminary Report 
Pediatric cardiology  2007;29(2):258-265.
There have been comprehensive screening methods developed to identify unwanted inpatient events. A comprehensive assessment of complication diagnoses during congenital heart surgery admissions has not been performed. We examined the frequency of complications identified by a complication screening method and their relationship to patient characteristics among congenital heart surgery admissions. Data were obtained from the Healthcare Cost and Utilization Project Kids' Inpatient Database 2000. Among congenital heart surgery admissions, age ≤20 years, we identified International Classification of Disease, 9th Revision, Clinical Modification (ICD-9-CM) codes indicating complication. Complication diagnoses were categorized as related to drug/biologic, procedures, devices, implants and grafts, and radiation. We used the Risk Adjustment for Congenital Heart Surgery risk categories (1–6) to examine the association between case complexity and complications. Multivariate analyses estimated the odds for a complication diagnosis by patient characteristics, including age, prematurity, chromosomal anomalies, noncardiac structural anomalies, and surgical risk category.
Among 12,717 cases, 4014 (32%) had at least 1 complication diagnosis code. Procedure-related complication diagnoses represented 75% of complication diagnoses; device, implant, or graft represented 21%; drug/biologic represented 4% and radiation represented 0%. Multivariate analyses demonstrated that higher surgical case complexity and older age had a greater risk for a complication diagnosis: Risk Category 2, odds ratio (OR) 1.8; 3 OR 2.9; 4 OR 2.9; 5 OR 5.0; 6 OR 4.1, relative to category 1, all p‹0.01; age ≥12 years, OR 1.3, p‹0.001; ‹1 year OR 1.1, p = 0.31. Premature cases had decreased odds OR 0.4, p‹0.001. This complication screening method indicates that unwanted patient events occur frequently during congenital heart surgery admissions. Children undergoing complex congenital heart surgery are at greatest risk for these unwanted events. Further study of these events is needed to determine their preventability and severity.
doi:10.1007/s00246-007-9110-2
PMCID: PMC4240226  PMID: 17912481
Outcomes; Surgery complications; CHD miscellaneous
9.  What is the Clinical Utility of Routine Cardiac Catheterization Before a Fontan Operation? 
Pediatric cardiology  2010;31(7):977-985.
Patients with single-ventricle circulation presenting for Fontan completion routinely undergo cardiac catheterization despite ongoing debate concerning its additive value. Increasing interest in noninvasive preoperative evaluation alone led the authors to analyze the utility of routine pre-Fontan catheterization and to determine whether a subset of patients could avoid this invasive procedure. Patients younger than 5 years referred for pre-Fontan evaluation were retrospectively reviewed. Medical records and catheter angiograms were examined, and catheterizations were categorized as “additive” based on predetermined criteria. Associations between precatheterization variables, catheterization findings, and short-term postoperative outcomes were evaluated. Cardiac catheterization was clinically nonadditive for 89 of 175 patients undergoing pre-Fontan evaluation (51%). There were no robust precatheterization predictors of a nonadditive catheterization. Echocardiography did not fully demonstrate the relevant anatomy of 115 patients (66%), most frequently due to inadequate visualization of the pulmonary arteries, and 22 patients had additive catheterizations due to new diagnostic findings alone. Interventions at catheterization were frequent and deemed “important” for 64 patients (37%). Catheterization hemodynamic data were not associated with early postoperative outcomes. Minor catheterization complications occurred for 51 patients (29%) and major complications for 4 patients (2%). Although at least 50% of the patients presenting for Fontan completion may be able to avoid routine catheterization safely, an echocardiography-based imaging strategy alone is insufficient to allow proper identification of those who could be evaluated noninvasively. A more comprehensive imaging strategy not based solely on echocardiography should be considered.
doi:10.1007/s00246-010-9736-3
PMCID: PMC4237011  PMID: 20503042
Cardiac catheterization; CHD; Echocardiography; Fontan; Imaging; Univentricular heart
10.  Serial Cardiopulmonary Exercise Testing in Patients with Previous Fontan Surgery 
Pediatric cardiology  2010;31(2):175-180.
Patients with previous Fontan surgery have reduced peak oxygen consumption (VO2), and data regarding progression of exercise intolerance is limited. The purpose of this study was to assess the evolution of exercise tolerance in patients with previous Fontan surgery. We performed a retrospective cohort study of patients with previous Fontan surgery who underwent cardiopulmonary exercise testing between November 2002 and January 2009. Patients were required to have completed at least two tests, with adequate levels of effort, ≥6 months apart. We identified 78 patients (55% male) who had undergone a total of 215 cardiopulmonary exercise tests. Age at initial study was 19.7 ± 10.2 years; time interval since previous Fontan surgery 13.3 ± 5.7 years; and time interval between the initial and the most recent exercise test was 3.0 ± 1.4 years. Morphologic left ventricle was present in 58 (74.4%) patients. At baseline, peak VO2 averaged 24.7 ± 7.0 ml/kg/min (63.7% ± 15.8% predicted). At most recent study, peak VO2 averaged 23.2 ± 7.2 ml/kg/min, (60.4% ± 13.9% predicted). Percent predicted peak VO2 declined slowly, with a mean rate of decline of 1.25 ± 0.36 percentage points/y (p < 0.001). Most of the decline occurred in patients < 18 years of age (mean rate of decline 1.78 ± 0.46 percentage points/y; p = 0.0004). Thereafter, the rate of decline was less marked (mean rate of decline 0.54 ± 0.57 percentage points/y; p = not significant). Ventricular morphology, type of Fontan procedure, and cardiac medications were not predictive of progressive exercise intolerance. In conclusion, although the exercise function of previous Fontan patients tends to decline during late adolescence, it appears to stabilize during early adulthood.
doi:10.1007/s00246-009-9580-5
PMCID: PMC4235332  PMID: 19915891
Congenital heart disease; Exercise tests; Fontan procedure
11.  Truncus Arteriosus: Diagnostic Accuracy, Outcomes, and Impact of Prenatal Diagnosis 
Pediatric cardiology  2008;30(3):256-261.
Limited data exist on the impact of prenatal diagnosis and outcomes of fetal truncus arteriosus (TA). We sought to assess prenatal diagnostic accuracy and prenatal outcomes in fetuses with TA and compare postnatal outcomes in neonates with prenatally and postnatally diagnosed TA. Records were reviewed for patients diagnosed with TA in utero or at ≤60 days of life from 1992 to 2007. Forty-three (32%) of 136 TA patients had prenatal diagnosis. Five patients with TA were prenatally misdiagnosed, and 5 with other congenital heart diseases were misdiagnosed with TA prenatally. Of 28 fetuses diagnosed at <24 weeks gestation, 19 (68%) did not survive to birth because of spontaneous fetal death (n = 2) or because of elective termination (n = 17). Pregnancy termination was not more likely for fetuses with extracardiac anomalies. Of 19 live-born patients with correct prenatal diagnosis of TA, 2 (11%) died before surgery, and 4 (24%) died in the early postoperative period. All patients who died presurgically had been diagnosed prenatally. Overall, early postoperative mortality was 10%. Prenatal diagnosis of TA remains challenging and is associated with a high rate of elective termination. Fetal diagnosis was associated with younger age at repair but was not associated with improved neonatal survival.
doi:10.1007/s00246-008-9328-7
PMCID: PMC4227613  PMID: 19015910
Fetus; Aortic valve; Pulmonary valve; Tetralogy of Fallot; Pulmonary atresia; Conotruncal anomaly
12.  Evaluating the Diagnostic Gap: Statewide Incidence of Undiagnosed Critical Congenital Heart Disease Before Newborn Screening With Pulse Oximetry 
Pediatric cardiology  2013;34(7):1680-1686.
Screening for critical congenital heart disease (CCHD) using pulse oximetry has been endorsed by the American Academy of Pediatrics and the American Heart Association. We sought to determine the incidence of undetected CCHD in Tennessee and the diagnostic gap of CCHD in Middle Tennessee prior to screening implementation. The Tennessee Initiative for Perinatal Quality Care (TIPQC) Undetected CCHD Registry is a quality improvement initiative established to identify neonates discharged from the nursery with undetected CCHD. The TIPQC database was queried and a simultaneous review of all neonates with CCHD in the Middle Tennessee region was performed to define the incidence and identify the pre-screen diagnostic gap of undetected CCHD at the time of hospital discharge. In 2011, of 79,462 live births in Tennessee, 12 newborns had undiagnosed CCHD (incidence 15 per 100,000; 95 % CI 9–26 per 100,000). Nine of 12 (75 %) had coarctation of the aorta (CoA). There were no deaths due to undiagnosed CCHD. In the Middle Tennessee region, 6 of 45 neonates with CCHD were missed, for a diagnostic gap of 13 % (95 % CI 6–26 %). Prior to implementation of CCHD screening using pulse oximetry, 12 Tennessee neonates with CCHD were missed by prenatal ultrasound and newborn examination. CoA was the most common lesion missed and is also the CCHD most likely to be missed despite addition of screening using pulse oximetry. Continued evaluation of the diagnostic gap with particular attention to missed diagnoses of CoA should accompany institution of CCHD screening programs.
doi:10.1007/s00246-013-0697-1
PMCID: PMC3783532  PMID: 23595939
Coarctation of the aorta; Congenital heart disease; Newborn screening; Pulse oximetry
14.  Fifteen-year Single Center Experience with the “Giessen Hybrid” Approach for Hypoplastic Left Heart and Variants: Current Strategies and Outcomes 
Pediatric Cardiology  2014;36:365-373.
Presented is a retrospective outcome study of a 15-year single institutional experience with a contemporary cohort of patients with hypoplastic left heart syndrome and complex that underwent a “Giessen Hybrid” stage I as initial palliation. Hybrid approach consisting of surgical bilateral pulmonary artery banding and percutaneous duct stenting with or without atrial septum manipulation was developed from a rescue approach to a first-line procedure. Comprehensive Aristotle score defined pre-operative condition. Fifteen-year follow-up mortality is reported as occurring within the staged univentricular palliation or before and after biventricular repair. Hybrid stage I was performed in 154 patients; 107 should be treated by single ventricle palliation, 33 by biventricular repair (BVR), 7 received heart transplantation, and 7 were treated by comfort care, respectively. Overall 34 children died. The Aristotle score (mean value 18.2 ± 3) classified for univentricular circulations in newborns did not have statistical impact on the outcome. Two patients died during stage I (1.2 %), and the interstage I mortality was 6.7 %, and stage II mortality 9 %, respectively. Stage III was up to now performed in 57 patients without mortality. At 1 year, the overall unadjusted survival of HLHS and variants was 84 % and following BVR 89 %, respectively. The Fifteen-year survival rate for HLHS and variants was 77 %, with no significant impact of birth weight of less than 2.5 kg. In conclusion, Hybrid stage I fulfilled the criteria of life-saving approach. In our institution, Hybrid procedure replaced Norwood-staged palliation with a considerable mid- and long-term survival rate. Considering interstage mortality close surveillance is mandatory.
doi:10.1007/s00246-014-1015-2
PMCID: PMC4303711  PMID: 25179460
Hypoplastic left heart syndrome; Hypoplastic left heart complex; Hybrid approach
15.  Development of a Charge Adjustment Model for Cardiac Catheterization 
Pediatric Cardiology  2014;36:264-273.
A methodology that would allow for comparison of charges across institutions has not been developed for catheterization in congenital heart disease. A single institution catheterization database with prospectively collected case characteristics was linked to hospital charges related and limited to an episode of care in the catheterization laboratory for fiscal years 2008–2010. Catheterization charge categories (CCC) were developed to group types of catheterization procedures using a combination of empiric data and expert consensus. A multivariable model with outcome charges was created using CCC and additional patient and procedural characteristics. In 3 fiscal years, 3,839 cases were available for analysis. Forty catheterization procedure types were categorized into 7 CCC yielding a grouper variable with an R2 explanatory value of 72.6 %. In the final CCC, the largest proportion of cases was in CCC 2 (34 %), which included diagnostic cases without intervention. Biopsy cases were isolated in CCC 1 (12 %), and percutaneous pulmonary valve placement alone made up CCC 7 (2 %). The final model included CCC, number of interventions, and cardiac diagnosis (R2 = 74.2 %). Additionally, current financial metrics such as APR-DRG severity of illness and case mix index demonstrated a lack of correlation with CCC. We have developed a catheterization procedure type financial grouper that accounts for the diverse case population encountered in catheterization for congenital heart disease. CCC and our multivariable model could be used to understand financial characteristics of a population at a single point in time, longitudinally, and to compare populations.
doi:10.1007/s00246-014-0994-3
PMCID: PMC4303716  PMID: 25113520
Resource utilization; RVU; Congenital heart disease; Catheterization; Outcomes
16.  Cardiorespiratory Fitness in Children: A Simple Screening Test for Population Studies 
Pediatric Cardiology  2014;36:27-32.
Cardiorespiratory fitness is one measure of body functions, and its assessment should play an important role in the activities associated with the promotion of physical activity as an important component of a healthy lifestyle. This study aimed to develop a reference system of the mean post-exercise heart rate (HRmean post-ex) after a 3-min step test for use in screening the cardiorespiratory fitness of 6- to 12-year-old children. The study included 14,501 children ages 6–12 years from primary schools in Gdansk. The participants were subjected to the 3-min Kasch Pulse Recovery Test (KPR Test). The reference range for the classification of cardiorespiratory fitness was developed on the basis of the age-specific percentile distribution of HRmean post-ex in 6- to 9- and 10- to 12-year-old children. This study showed that the 3-min KPR Test is easy to perform and well tolerated by school-age children. As such, it can constitute a useful tool for health promoters and educators. The presented age- and gender-specific reference range of HRmean post-ex enables the assessment and monitoring of submaximal exercise-induced changes in the cardiovascular system and, consequently, the physical fitness of a given individual.
doi:10.1007/s00246-014-0960-0
PMCID: PMC4284398  PMID: 25070386
Cardiorespiratory fitness; Heart rate; Reference range; Step test; Screening
17.  Common Variations in BMP4 Confer Genetic Susceptibility to Sporadic Congenital Heart Disease in a Han Chinese Population 
Pediatric Cardiology  2014;35(8):1442-1447.
Congenital heart disease (CHD) is the most common birth defect in humans. The genetic causes of sporadic CHD remain largely unknown. Bone morphogenetic protein 4 (BMP4), a member of the transforming growth factor-β (TGF-β) family, is required for normal heart development. Loss of BMP4 gene expression in mice is associated with septal defects, defective endocardial cushion remodeling, and abnormal semilunar valve formation. This study evaluated the contribution of single nucleotide polymorphisms (SNPs) in BMP4 to CHD susceptibility in a case–control study of 575 patients with CHD and 844 non-CHD control subjects in a Chinese population. The BMP4 SNP rs762642 was associated with CHD in an additive model (odds ratio [OR]add 1.22; 95 % confidence interval [CI] 1.04–1.43; Padd = 0.02). Stratified analysis by CHD subtypes showed a significant association only between rs762642 and atrial septal defect (ORadd 1.33; 95 % CI 1.04–1.72; Padd = 0.03) in the additive model. This study was the first to indicate that a common variant of BMP4 may contribute to susceptibility to sporadic CHD in a Chinese population.
doi:10.1007/s00246-014-0951-1
PMCID: PMC4236636  PMID: 25022354
Congenital heart disease; Single nucleotide polymorphism; Bone morphogenetic protein 4
18.  Effects of Race, Ethnicity, and Gender on Surgical Mortality in Hypoplastic Left Heart Syndrome 
Pediatric cardiology  2013;34(8):1829-1836.
Information is limited regarding the effect of race, ethnicity, and gender on the outcomes of the three palliative procedures for hypoplastic left heart syndrome (HLHS). This study examined the effects of race, ethnicity, gender, type of admission, and surgical volume on in-hospital mortality associated with palliative procedures for HLHS between 1998 and 2007 using data from the University HealthSystem Consortium. According to the data, 1,949 patients underwent stage 1 palliation (S1P) with a mortality rate of 29 %, 1,279 patients underwent stage 2 palliations (S2P) with a mortality rate of 5.4 %, and 1,084 patients underwent stage 3 palliation (S3P) with a mortality rate of 4.1 %. The risk factors for increased mortality with S1P were black and “other” race, smaller surgical volume, and early surgical era. The only risk factors for increased mortality with S2P were black race (11 % mortality; odds ratio [OR], 3.19; 95 % confidence interval [CI] 1.69–6.02) and Hispanic ethnicity (11 % mortality; OR 3.30; 95 % CI 1.64–6.64). For S2P, no racial differences were seen in the top five surgical volume institutions, but racial differences were seen in the non-top-five surgical volume institutions. Mortality with S1P was significantly higher for patients discharged after birth (37 vs 24 %; p = 0.004), and blacks were more likely to be discharged after birth (12 vs 5 % for all other races; p < 0.001). No racial differences with S3P were observed. The risk factors for increased mortality at S1P were black and “other” race, smaller surgical volume, and early surgical era. The risk factors for increased in-hospital mortality with S2P were black race and His-panic ethnicity.
doi:10.1007/s00246-013-0723-3
PMCID: PMC4023351  PMID: 23722968
Congenital heart disease; Congenital heart surgery; Hypoplastic left heart syndrome; Racial disparities
19.  Contractility–Afterload Mismatch in Patients With Protein-Losing Enteropathy After the Fontan Operation 
Pediatric Cardiology  2014;35(7):1225-1231.
This study aimed to clarify the relationship between onset of protein-losing enteropathy (PLE) and Fontan circulation, with special reference to the development of contractility–afterload mismatch. The PLE group comprised 9 patients who experienced PLE after undergoing the Fontan operation, and the control group consisted of 32 patients had did not experienced PLE more than 10 years after the Fontan operation. The study compared the pre- and postoperative values of arterial elastance (Ea), end-systolic elastance (Ees), and contractility–afterload mismatch (Ea/Ees). Furthermore, the variations in the values were examined during the preoperative, postoperative, and midterm postoperative periods in seven PLE patients who underwent cardiac catheterization at the onset of PLE and during the pre- and postintervention periods in three PLE patients who underwent surgical intervention to improve the Fontan circulation after the onset of PLE. Comparison of the values obtained before and after Fontan operations showed that the Ea values increased significantly in the PLE group. However, the pre- and postoperative Ees values did not differ in the two groups. During the postoperative period, Ea/Ees increased significantly, and the Ea and Ea/Ees values increased continuously until the onset of PLE in the PLE group. In the patients who underwent surgical intervention to improve the Fontan circulation after the onset of PLE, the Ea/Ees decreased significantly, and the serum albumin levels improved after the intervention. Contractility–afterload mismatch, mainly caused by the increase in the afterload of the systemic ventricle, may have an important role in the development of PLE after the Fontan operation.
doi:10.1007/s00246-014-0920-8
PMCID: PMC4164859  PMID: 24827079
Fontan; Protein-losing enteropathy; Contractility–afterload mismatch
20.  Development and Validation of a Fetal Cardiovascular Disease Severity Scale 
Pediatric Cardiology  2014;35(7):1174-1180.
Prenatal heart disease spans the spectrum of severity from very mild to severe life-threatening conditions. An accepted scale for grading fetal cardiovascular disease severity would aid in anomaly standardization, counseling, and future research. The Fetal Cardiovascular Disease Severity Scale with seven severity grades ranging from mild (grade 1) to severe (grade 7) disease was developed. Severity grade relates to the cardiovascular condition diagnosed by fetal echocardiography, with factors including postnatal intervention, number of interventions anticipated, likelihood of two-ventricle repair versus single-ventricle palliation, and overall prognosis. A survey describing 25 cardiac anomalies was offered to fetal cardiologists at six institutions for validation of scale reliability among practitioners. The study participants graded defects using this scale. A smaller group graded anomalies again more than 2 weeks after the initial survey. The intraclass correlation coefficient (ICC) was used to assess agreement of the respondents. The survey participants were 14 experienced fetal cardiologists: 9 from the Children’s Hospital of Philadelphia (CHOP) and 5 from five additional institutions in the United States. The initial survey ICC was high [0.93; 95 % confidence interval (CI) 0.88–0.96]. The subanalysis showed a higher ICC for the participants outside CHOP (0.95; 95 % CI 0.91–0.98 vs. 0.92; 95 % CI 0.86–0.96, respectively). The ICCs were high for all the fetal cardiologists participating in the repeat evaluation, ranging from 0.92 to 0.99 (95 % CI 0.65–1.00). The Fetal Cardiovascular Disease Severity Scale demonstrated good inter- and intrarater reliability among experienced fetal cardiologists and is a valid tool for standardization of prenatal cardiac diagnostic assessment across institutions. The scale has applications for parental counseling and research in fetal cardiovascular disease.
doi:10.1007/s00246-014-0911-9
PMCID: PMC4164841  PMID: 24801674
Severity; Congenital heart disease; Scale; Fetal echocardiography; Cardiac anomaly
21.  Comparison of Maximum Vasoactive Inotropic Score and Low Cardiac Output Syndrome As Markers of Early Postoperative Outcomes After Neonatal Cardiac Surgery 
Pediatric cardiology  2012;33(4):633-638.
Low cardiac output syndrome (LCOS) and maximum vasoactive inotropic score (VIS) have been used as surrogate markers for early postoperative outcomes in pediatric cardiac surgery. The objective of this study was to determine the associations between LCOS and maximum VIS with clinical outcomes in neonatal cardiac surgery. This was a secondary retrospective analysis of a prospective randomized trial, and the setting was a pediatric cardiac intensive care unit in a tertiary care children's hospital. Neonates (n = 76) undergoing corrective or palliative cardiac operations requiring cardiopulmonary bypass were prospectively enrolled. LCOS was defined by a standardized clinical criteria. VIS values were calculated by a standard formula during the first 36 postoperative hours, and the maximum score was recorded. Postoperative outcomes included hospital mortality, duration of mechanical ventilation, intensive care unit (ICU) and hospital lengths of stay (LOS), as well as total hospital charges. At surgery, the median age was 7 days and weight was 3.2 kg. LCOS occurred in 32 of 76 (42%) subjects. Median maximum VIS was 15 (range 5–33). LCOS was not associated with duration of mechanical ventilation, ICU LOS, hospital LOS, and hospital charges. Greater VIS was moderately associated with a longer duration of mechanical ventilation (p = 0.001, r = 0.36), longer ICU LOS (p = 0.02, r = 0.27), and greater total hospital costs (p = 0.05, r = 0.22) but not hospital LOS (p = 0.52). LCOS was not associated with early postoperative outcomes. Maximum VIS has only modest correlation with duration of mechanical ventilation, ICU LOS, and total hospital charges.
doi:10.1007/s00246-012-0193-z
PMCID: PMC3989285  PMID: 22349666
Vasoactive inotropic score; Low cardiac output syndrome; Neonates; Cardiac surgery; Postoperative outcomes; Cardiopulmonary bypass
22.  Neurodevelopmental Outcomes for Children With Hypoplastic Left Heart Syndrome at the Age of 5 Years 
Pediatric cardiology  2013;34(7):1597-1604.
This study aimed to determine the neurodevelopmental (ND) outcome for children with hypoplastic left heart syndrome (HLHS) at early school age. English-speaking patients who underwent the Norwood procedure between 2000 and 2005 were eligible at 4–6 years of age for ND testing. Of the 72 eligible patients, 44 (61 %) agreed to participate, and 37 completed ND testing before the close of the study. Three subjects were excluded from analyses due to late stroke. The ND testing included intelligence, visual motor integration, memory and motor and language skills. Parents and teachers completed measures of behavior and attention problems. Subjects’ scores and parent/teacher ratings were converted to z-scores and compared with test norms. Higher scores on child measures represent better outcomes, whereas higher scores on parent and teacher rating scales indicate more problems. The average ND performance of the tested cohort fell within one standard deviation of the test norms for all measures. However, the subjects performed significantly lower than the test norms on measures of visual-motor integration, fine motor skills, memory, and word structure (z = −0.42 to −0.54; p < 0.005). On the parent and teacher completed measures, the subjects scored higher than the test norms on attention problems (z = 0.40–0.62; p < 0.005). Although the overall ND performance of the cohort was normal, the subjects showed relative weakness in visual motor and attention skills. Ongoing developmental monitoring of these children is recommended to guide interventions that may improve individual outcomes and to assess the impact of changes in clinical management strategies on functional outcomes.
doi:10.1007/s00246-013-0679-3
PMCID: PMC3982227  PMID: 23503929
Congenital heart disease/defects; Developmental outcomes; Hypoplastic left heart syndrome
23.  Cranial Irradiation as an Additional Risk Factor for Anthracycline Cardiotoxicity in Childhood Cancer Survivors: An Analysis from the Cardiac Risk Factors in Childhood Cancer Survivors Study 
Pediatric cardiology  2012;34(4):826-834.
Background
Anthracycline-treated childhood cancer survivors suffer cardiac damage that results in decreased left ventricular (LV) mass, leading to increased LV wall stress, which underlies their greater risk of cardiomyopathy. Many of these survivors are also at risk of growth hormone (GH) abnormalities from cranial irradiation exposure though it is unknown if such exposure is associated with cardiotoxicity.
Methods
Echocardiograms and insulin-like growth factor-1 (IGF-1), a marker of GH, were measured in 130 anthracycline-treated childhood cancer survivors, 59 of whom had been exposed to cranial irradiation, a mean 10 years from cancer diagnosis. Echocardiographic parameters and IGF-1 were standardized relative to age or body-surface area using data from sibling controls and expressed as the percent difference from normal.
Results
After adjusting for other risk factors, survivors exposed to cranial irradiation had an additional 12% decrease in LV mass compared to unexposed survivors (P<.01), and an additional 3.6% decrease in LV dimension (P=.03). Survivors exposed to cranial irradiation also had a 30.8% decrease in IGF-1 relative to normal, which was greater than the 10.5% decrease in unexposed survivors (P<.01).
Conclusions
In anthracycline-treated childhood cancer survivors, a mean 10 years from diagnosis, those with cranial irradiation exposure had significantly greater decreases in LV mass and dimension. Because cranial irradiation was also associated with decreased IGF-1, it is possible that GH deficiencies mediated this effect suggesting that GH replacement therapy may help prevent the development of cardiotoxicity.
doi:10.1007/s00246-012-0539-6
PMCID: PMC3594453  PMID: 23080542
Cardiomyopathy; Anthracyclines; Cranial Irradiation; Cancer; Survivors
24.  Acute Pulmonary Vasodilator Testing With Inhaled Treprostinil in Children With Pulmonary Arterial Hypertension 
Pediatric cardiology  2012;34(4):1006-1012.
Acute pulmonary vasodilator testing (AVT) is essential to determining the initial therapy for children with pulmonary arterial hypertension (PAH). This study aimed to report the initial experience with inhaled treprostinil used for AVT in children with PAH and to evaluate the hemodynamic change after inhaled treprostinil compared with inhaled nitric oxide. This prospective cohort study was designed for 13 children who underwent AVT with inhaled treprostinil or oxygen plus inhaled nitric oxide (iNO) during catheterization. Inhaled treprostinil was delivered during cardiac catheterization by adapting the Optineb ultrasonic nebulizer via either a flow-inflating bag or the manual mode of the anesthesia system. The median age of the patients was 10 years (range 4–17 years). The etiologies of PAH included idiopathic PAH and associated PAH. All the patients tolerated inhaled treprostinil without marked clinical worsening and received six or nine breaths (36 or 54 µg) of treprostinil. The median of the total treprostinil doses was 1.53 µg/kg (range 0.71–2.89 µg/kg). Inhaled treprostinil was administrated via an endotracheal tube (n = 8), anesthesia mask (n = 3), or laryngeal mask airway (n = 2). Inhaled nitric oxide (iNO) and inhaled treprostinil significantly decreased the mean pulmonary artery pressure and the pulmonary vascular resistance index compared with baseline. Three adverse events were reported after inhaled treprostinil, including cough and mild to moderate hypotension with higher doses. All adverse events resolved without any intervention. This study report is the first to describe the use of inhaled treprostinil for AVT in children with PAH. In this small pediatric cohort, inhaled treprostinil was effectively delivered and well tolerated and may be useful for AVT.
doi:10.1007/s00246-012-0597-9
PMCID: PMC3608847  PMID: 23184020
Acute pulmonary vasodilator testing; Children; Iloprost; Inhaled treprostinil; Nitric oxide; Pulmonary arterial hypertension
25.  Sudden Unexplained Cardiac Arrest in Apparently Healthy Children: A Single Center Experience 
Pediatric cardiology  2012;34(3):639-645.
Objective
To determine the causes of sudden cardiac arrest (SCA) in apparently healthy children in a single center in the era of primary prevention (screening questionnaire, SQ) and secondary prevention (automated external defibrillator, AED and automated implantable cardioverter defibrillator, AICD).
Study Design
Any child (0–18 year’s age) without prior known disease except for attention deficit disorder who underwent out-of-the hospital cardiopulmonary resuscitation was included in the study as SCA. Using retrospective chart review we evaluated the efficacy of the SQ, electrocardiogram (EKG), chest roentgenogram (CXR) and an echocardiogram.
Results
We found 44 out of 6,656 children admitted to intensive care with SCA; an AED was used in 39%, AICD placed in 18% and survival to hospital discharge was 50%. The etiology for SCA was identified in 57% of the cases, mostly in those above one year of age and among these the majority of had a cardiac etiology (50%) while 7% had rupture of an arteriovenous malformation. Stimulant medication use was seen in 11% of the SCA. In the best case scenario of hypothesized primary prevention, a prior SQ, CXR, EKG and echocardiogram may have detected 18%, 9%, 23% and 16% of at-risk cases respectively and 32% may have been detected with EKG and SQ together. Based on a historical control cohort, a positive EKG was significantly higher in children with SCA (p = 0.014).
Conclusion
An EKG along with a screening SQ may be more effective in identifying children who are potentially at-risk for SCA than a SQ alone.
doi:10.1007/s00246-012-0516-0
PMCID: PMC3566361  PMID: 23052663
Stimulants; attention deficit hyperactivity disorder; pseudoephedrine; arteriovenous malformation; sudden cardiac death

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