James Cnota

Boston Children's Hospital, Boston, Massachusetts, United States

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Publications (41)118.76 Total impact

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    ABSTRACT: Data regarding the value of B-type natriuretic peptide (BNP) measurements in infants with a single-ventricle (SV) physiology are lacking. This analysis aimed to describe the BNP level changes in infants with an SV physiology before and after superior cavopulmonary connection (SCPC) surgery. Levels of BNP were measured by a core laboratory before SCPC (at 5.0 ± 1.6 months) and at the age of 14 months during a multicenter trial of angiotensin-converting enzyme inhibition therapy for infants with SV. Multivariable longitudinal analysis was used to model the associations between BNP levels and three sets of grouped variables (echocardiography, catheterization, growth). Multivariable analysis was performed to assess associations with patient characteristics at both visits. Associations between BNP levels and neurodevelopmental variables were investigated at the 14 month visit because neurodevelopmental assessment was performed only at this visit. The BNP level was significantly higher before SCPC (n = 173) than at the age of 14 months (n = 134). The respective median levels were 80.8 pg/ml (interquartile range [IQR], 35-187 pg/ml) and 34.5 pg/ml (IQR, 17-67 pg/ml) (p < 0.01). A BNP level higher than 100 pg/ml was present in 72 subjects (42 %) before SCPC and in 21 subjects (16 %) at the age of 14 months. In the 117 patients who had BNP measurements at both visits, the median BNP level decreased 32 pg/ml (IQR, 1-79 pg/ml) (p < 0.01). In the longitudinal multivariable analysis, higher BNP levels were associated with a higher end-systolic volume z-score (p = 0.01), a greater degree of atrioventricular (AV) valve regurgitation (p < 0.01), a lower weight z-score (p < 0.01), and a lower length z-score (p = 0.02). In multivariable analyses, a higher BNP level at the age of 14 months was associated with arrhythmia after SCPC surgery (p < 0.01), a prior Norwood procedure (p < 0.01), a longer hospital stay after SCPC surgery (p = 0.04), and a lower Bayley psychomotor developmental index (p = 0.02). The levels of BNP decreases in infants with SV from the pre-SCPC visit to the age of 14 months. A higher BNP level is associated with increased ventricular dilation in systole, increased AV valve regurgitation, impaired growth, and poorer neurodevelopmental outcomes. Therefore, BNP level may be a useful seromarker for identifying infants with SV at risk for worse outcomes.
    Pediatric Cardiology 02/2014; · 1.20 Impact Factor
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    ABSTRACT: To assess the relationship between commonly reported fetal cardiomyopathy scoring systems in early stage twin-twin transfusion syndrome (TTTS). We retrospectively reviewed 100 cases of Quintero stage I and II TTTS referred to our center for evaluation from 2008 to 2010. The cases were divided into groups of 25, representing each of four grades of TTTS cardiomyopathy as assessed by Cincinnati Stage: No cardiomyopathy, Stage IIIA, Stage IIIB, and Stage IIIC. Spearman correlation (rs ) was calculated between the Children's Hospital of Philadelphia (CHOP) score, cardiovascular profile score (CVPS), Cincinnati Stage and myocardial performance index (MPI). There was low correlation between the Cincinnati Stage and the CHOP (rs = 0.36) and CVPS scores (rs = -0.39), while correlation was high between the CHOP and CVPS scores (rs = -0.72). MPI elevation was more frequently concordant with Cincinnati stage (82% of cases) than were ventricular hypertrophy (43%) or AV valve regurgitation (28%). 51% of fetuses with minimally elevated CHOP score (0-1) and 48% of fetuses with minimally depressed CVPS (9-10) scores had significant elevation (z-score >+3) in RV or LV MPI. The MPI has a strong influence on grading severity of fetal cardiomyopathy using the Cincinnati Stage among fetuses with mild TTTS. Furthermore, significant elevation of the MPI is common among fetuses with mild disease as assessed by the CHOP score and CVPS. These differences should be understood when assessing and grading cardiomyopathy in TTTS, particularly in early (Quintero stage I and II) disease.
    Ultrasound in Obstetrics and Gynecology 10/2013; · 3.56 Impact Factor
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    ABSTRACT: Diagnostic ultrasound is widespread in obstetric practice, yet many babies with major congenital heart disease remain undiagnosed. Factors affecting prenatal diagnosis of major congenital heart disease are not well understood. This study aims to document prenatal detection rates for major congenital heart disease in the Greater Cincinnati area, and identify factors associated with lack of prenatal diagnosis. Methods All living infants diagnosed with major congenital heart disease by 4 months of age at our centre were prospectively identified. Prenatal care data were obtained by parent interview. Neonatal records were reviewed for postnatal data. Obstetricians were contacted for diagnostic ultrasound data. A total of 100 infants met the inclusion criteria. In all, 95 infants were analysed, of whom 94 were offered diagnostic ultrasound. In all, 41 had a prenatal diagnosis of major congenital heart disease. The rate of prenatal detection varied by cardiac lesion, with aortic arch abnormalities, semilunar valve abnormalities, and venous anomalies going undetected in this sample. Among subjects without prenatal detection, the highest proportion consisted of those having Level 1 diagnostic ultrasound only (66%). Prenatal detection was not significantly influenced by maternal race, education level, income, or insurance type. Despite nearly universal diagnostic ultrasound, detection rates of major congenital heart disease remain low in southwest Ohio. An educational outreach programme including outflow tract sweeps for community-level obstetrical personnel may improve detection rates.
    Cardiology in the Young 10/2013; · 0.95 Impact Factor
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    ABSTRACT: -The Pediatric Heart Network's (PHN) Single Ventricle Reconstruction Trial (SVR) randomized infants with single right ventricles (RV) undergoing a Norwood procedure to a modified Blalock-Taussig or RV-to-pulmonary artery shunt. This report compares RV parameters in the two groups using 3-dimensional echocardiography (3DE). -3DE studies were obtained at 10/15 SVR centers. Of the 549 subjects, 314 underwent 3DE studies at one to four time points (pre-Norwood, post-Norwood, pre-stage II, and 14 months) for a total of 757 3DEs. Of these, 565 (75%) were acceptable for analysis. RV volume, mass, mass:volume ratio, ejection fraction (EF), and severity of tricuspid regurgitation did not differ by shunt type. RV volumes and mass did not change after the Norwood, but increased from pre-Norwood to pre-stage II (end-diastolic volume [EDV, ml]/body surface area [BSA](1.3), end-systolic volume [ESV, ml]/BSA(1.3) and mass[g]/BSA(1.3) mean difference [95% confidence interval] = 25.0 [8.7, 41.3], 19.3 [8.3, 30.4], and 17.9 [7.3, 28.5], then decreased by 14 months (EDV/BSA(1.3), ESV/BSA(1.3) and mass/BSA(1.3) mean difference [95% confidence interval] = -24.4 [-35.0, -13.7], -9.8 [-17.9, -1.7], and -15.3 [-22.0, -8.6]. EF decreased from pre-Norwood to pre-stage II (mean difference [95% confidence interval] = -3.7% [-6.9%, -0.5%]), but did not decrease further by 14 months. -We found no statistically significant differences between study groups in 3DE measures of RV size and function, or magnitude of tricuspid regurgitation. Volume unloading was seen after stage II, as expected, but EF did not improve. This study provides insights into the remodeling of the operated univentricular RV in infancy. Clinical Trial Registration-URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934.
    Circulation Cardiovascular Imaging 10/2013; · 5.80 Impact Factor
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    ABSTRACT: Background:Fetal growth abnormalities in hypoplastic left heart syndrome (HLHS) have been documented primarily by birth measurements. Fetal growth trajectory has not been described. We hypothesized that fetal growth trajectory declines across late gestation in this population.Methods:Infants with a prenatal diagnosis of HLHS and no history of prematurity or a genetic syndromewere identified. Fetal ultrasound measurements and birth anthropometrics were obtained from clinical records. Z-scores for estimated fetal weight (EFWz) and birth weight (BWTz) were compared. BWTz for three neonatal standards were compared.Results:Paired fetal and neonatal data was identified in 33 cases of HLHS. Mean gestation age at ultrasound and birth were 27 and 38 weeks, respectively. BWTz was lower than EFWz by a mean of 0.82 (SD = 0.72, p< 0.0001) with 64% of subjects demonstrating a decrease in z-score of >0.5. Umbilical artery Doppler found no evidence of significant placental insufficiency. Modest differences in BWTz were seen across birth weight standards in this cohort.Conclusions:The majority of fetuses with HLHS demonstrate decreased growth velocity during later pregnancy, suggesting growth abnormalities manifest in utero. The potential relationship to future clinical outcomes warrants further study.Pediatric Research (2013); doi:10.1038/pr.2013.100.
    Pediatric Research 06/2013; · 2.67 Impact Factor
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    ABSTRACT: Background:Hypoplastic left heart syndrome (HLHS) is associated with significant mortality and morbidity. Fetal head growth abnormalities have been identified in a subset of HLHS fetuses, but it is unclear whether specific patterns of maladaptive growth affect clinical outcomes. Wehypothesized that poor fetal head growth is associated with an increased frequency of adverse clinical outcomes.Methods:We retrospectively examined a cohort of HLHS patients from midgestation to one year of age. Fetal and birth anthropometric measurements were analyzed using the Olsen standard, and clinical outcomes were obtained.Results:A total of 104 HLHS patients were identified over a 12-year period; fetal data were available in 38 cases. HLHS neonates demonstrated a high incidence of microcephaly (12%), small head size (27%), and poor head growth(32%). All-cause mortality was 31% at 30 days and 43% at one year. Neurologic outcomes were observed in 12% of patients, and were significantly increased with microcephaly (43% vs. 4%, p = 0.02). The average hospital length of stay following stage I palliationwas 33.4 ± 33 days correcting for early death.Conclusions:In term nonsyndromic HLHS,fetal and neonatal microcephalyisassociated with early adverse neurologic outcomes, but not mortality.Pediatric Research (2013); doi:10.1038/pr.2013.61.
    Pediatric Research 04/2013; · 2.67 Impact Factor
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    ABSTRACT: Staged surgical palliation has revolutionized the care of patients with hypoplastic left heart syndrome (HLHS), although the outcomes of survival and cost at a national level remain unclear. This study sought to evaluate (1) trends in HLHS surgical outcomes including in-hospital mortality, length of stay (LOS), and cost, and (2) patient and hospital risk factors associated with these outcomes. Hospitalizations for patients with HLHS, including stage I, II, and III palliations, were analyzed using the Kids' Inpatient Database from 2000 through 2009. Trends in mortality, LOS, and cost were analyzed and chi-squared tests were used to test association between categorical variables. Patient and hospital characteristics associated with death were analyzed using logistic regression and associations with LOS were analyzed using ordinary least squared regression. There were 16,923 hospital admissions in patients with HLHS of which 5,672 (34%) included surgical intervention. Total (3,201-5,102) and surgery-specific admissions (1,165-1,618) increased from 2000 to 2009. Mortality decreased 14% per year in stage III palliations (odds ratio [OR] 0.86; 95% confidence interval [CI]: 0.79-0.94) and 6% per year for stage I palliations (OR 0.94; 95% CI 0.90-0.99) but not for stage II palliations (OR 1.01; 95% CI; 0.89-1.14). Length of stay increased for stage I and II palliations; however, per-patient hospital cost decreased in 2009. In conclusion, recent decrease in per patient cost for staged surgical palliation for HLHS has correlated temporally with improved mortality.
    The American journal of cardiology 03/2013; · 3.58 Impact Factor
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    Ultrasound in Obstetrics and Gynecology 01/2013; 41(1):54-58. · 3.56 Impact Factor
  • Cardiology in the Young 10/2012; · 0.95 Impact Factor
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    ABSTRACT: OBJECTIVES: We sought to identify factors associated with the timing and surgical outcomes of the superior cavopulmonary anastomosis. METHODS: The Pediatric Heart Network's Infant Single Ventricle trial database identified participants who underwent superior cavopulmonary anastomosis. Factors potentially associated with age at superior cavopulmonary anastomosis, length of stay and death by 14 months of age were evaluated. Factors included subject demographics, cardiac anatomy, measures from neonatal hospitalization and pre-superior cavopulmonary anastomosis visit, adverse events, echocardiographic variables, intraoperative variables, superior cavopulmonary anastomosis type, and number of concurrent cardiac surgical procedures. Age at superior cavopulmonary anastomosis was analyzed using Cox proportional hazards regression. Natural log length of stay was analyzed by multiple linear regression. RESULTS: Superior cavopulmonary anastomosis was performed in 193 subjects at 5.2 months of age (interquartile range, 4.2, 6.2) and weight of 5.9 kg (interquartile range, 5.3, 6.6). The median length of stay was 7 days (interquartile range, 6, 10). There were 3 deaths and 1 transplant during the superior cavopulmonary anastomosis hospitalization, and 3 deaths and 3 transplants between discharge and 14 months of age. Age at superior cavopulmonary anastomosis was associated with center and interstage adverse events. A longer length of stay was associated with younger age and greater case complexity. Superior cavopulmonary anastomosis type, valve regurgitation, ventricular ejection fraction, and ventricular end-diastolic pressure were not independently associated with age at superior cavopulmonary anastomosis or the length of stay. CONCLUSIONS: Greater case complexity and more frequent interstage adverse events are associated with an earlier age at superior cavopulmonary anastomosis. Significant variation in age at superior cavopulmonary anastomosis among centers, independent of subject factors, highlights a lack of consensus regarding the optimal timing. Factors associated with length of stay could offer insights for improving presuperior cavopulmonary anastomosis care and surgical outcome.
    The Journal of thoracic and cardiovascular surgery 08/2012; · 3.41 Impact Factor
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    ABSTRACT: OBJECTIVES: Identify trends of enrolment and key challenges when recruiting infants with complex cardiac diseases into a multi-centre, randomised, placebo-controlled drug trial and assess the impact of efforts to share successful strategies on enrolment of subjects. METHODS: Rates of screening, eligibility, consent, and randomisation were determined for three consecutive periods of time. Sites collectively addressed barriers to recruitment and shared successful strategies resulting in the Inventory of Best Recruiting Practices. Study teams detailed institutional practices of recruitment in post-trial surveys that were compared with strategies of enrolment initially proposed in the Inventory. RESULTS: The number of screened patients increased by 30% between the Initial Period and the Intermediate Period (p = 0.007), whereas eligibility decreased slightly by 7%. Of those eligible for entry into the study, the rate of consent increased by 42% (p = 0.025) and randomisation increased by 71% (p = 0.10). During the Final Period, after launch of a competing trial, fewer patients were screened (-14%, p = 0.06), consented (-19%, p = 0.12), and randomised (-34%, p = 0.012). Practices of recruitment in the post-trial survey closely mirrored those in the Inventory. CONCLUSIONS: Early identification and sharing of best strategies of recruitment among all recruiting sites can be effective in increasing recruitment of critically ill infants with congenital cardiac disease and possibly other populations. Strategies of recruitment should focus on those that build relationships with families and create partnerships with the medical providers who care for them. Competing studies pose challenges for enrolment in trials, but fostering trusting relationships with families can result in successful enrolment into multiple studies.
    Cardiology in the Young 07/2012; · 0.95 Impact Factor
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    ABSTRACT: Syncope is a common problem in children and adolescents. The diagnostic yield for most tests commonly used in the evaluation of pediatric patients with syncope is low. To examine the epidemiology of pediatric patients presenting to United States (US) emergency departments (EDs) with a complaint of syncope and compare their initial management to published guidelines. ED visits from the National Hospital Ambulatory Medical Care Survey for 2003-2007 for patients aged 7-18 years were analyzed. Outcome variables were diagnostic tests and management of patients presenting with syncope. There were 627,489 (95% confidence interval [CI] 527,237-727,722) ED visits for syncope (0.9% of all ED visits for patients aged 7-18 years). Patients presenting to the ED for syncope were more commonly female (p<0.01), adolescent (13-18 years) (p<0.01), covered by private insurance (p=0.01), and more likely to arrive to the ED by ambulance (p<0.01), compared to those presenting with other complaints. Only 58.1% (95% CI 50.3-66.0%) of syncope patients received an electrocardiogram, and 26.5% (95% CI 18.2-34.7%) received a computed tomography (CT) or magnetic resonance imaging (MRI) scan as part of their diagnostic work-up. When evaluating pediatric patients presenting with syncope, there should be an increased use of the electrocardiogram to screen for underlying cardiac abnormalities. There should also be a tempered use of CT/ MRI imaging in this population.
    Journal of Emergency Medicine 03/2012; 43(4):575-83. · 1.33 Impact Factor
  • David Kwiatkowski, Yu Wang, James Cnota
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    ABSTRACT:   The purpose of this study is to review sedated outpatient echocardiograms performed to evaluate asymptomatic murmurs in children between the ages of 1 month and 4 years and describe outcomes of tests done to determine if utility varies among age of study and referral type (primary care physician vs. pediatric cardiologist.) We aim to describe the yield in a contemporary cohort which has increased availability and quality of diagnostic aids such as fetal ultrasound, newborn pulse oximetry, and neonatal echocardiography.   Retrospective cohort study.   Cincinnati Children's Hospital Medical Center: Outpatient Echocardiography Laboratory.   Children between 1 month and 4 years of age with asymptomatic murmurs who are referred for outpatient echocardiogram for evaluation of murmur.   Primary diagnosis of echocardiography studies, classified into severity score. Results.  Four hundred sixty-two sedated echocardiograms were studied. Six (1%) echocardiograms showed severe pathology, and no severe pathology was shown in the echocardiograms ordered at the age of over 6 months old. The yield of studies decreased as age increased. The incidence of abnormal pathology was higher among tests ordered by cardiologists, across all severity levels (P < .0001).   Among echocardiograms ordered for children over 1 year of age with an asymptomatic murmur, there was no severe and little moderate disease. Cardiac disease is significantly less likely when echocardiograms are ordered without referral to a pediatric cardiologist. The workup for asymptomatic murmurs does not require an echocardiogram, and these results may aid clinicians when deciding whether evaluation of a child should include this study.
    Congenital Heart Disease 02/2012; 7(3):283-8. · 1.01 Impact Factor
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    ABSTRACT: The study objective was to identify factors associated with death and cardiac transplantation in infants undergoing the Norwood procedure and to determine differences in associations that might favor the modified Blalock-Taussig shunt or a right ventricle-to-pulmonary artery shunt. We used competing risks methodology to analyze death without transplantation, cardiac transplantation, and survival without transplantation. Parametric time-to-event modeling and bootstrapping were used to identify independent predictors. Data from 549 subjects (follow-up, 2.7 ± 0.9 years) were analyzed. Mortality risk was characterized by early and constant phases; transplant was characterized by only a constant phase. Early phase factors associated with death included lower socioeconomic status (P = .01), obstructed pulmonary venous return (P < .001), smaller ascending aorta (P = .02), and anatomic subtype. Constant phase factors associated with death included genetic syndrome (P < .001) and lower gestational age (P < .001). The right ventricle-to-pulmonary artery shunt demonstrated better survival in the 51% of subjects who were full term with aortic atresia (P < .001). The modified Blalock-Taussig shunt was better among the 4% of subjects who were preterm with a patent aortic valve (P = .003). Lower pre-Norwood right ventricular fractional area change, pre-Norwood surgery, and anatomy other than hypoplastic left heart syndrome were independently associated with transplantation (all P < .03), but shunt type was not (P = .43). Independent risk factors for intermediate-term mortality include lower socioeconomic status, anatomy, genetic syndrome, and lower gestational age. Term infants with aortic atresia benefited from a right ventricle-to-pulmonary artery shunt, and preterm infants with a patent aortic valve benefited from a modified Blalock-Taussig shunt. Right ventricular function and anatomy, but not shunt type, were associated with transplantation.
    The Journal of thoracic and cardiovascular surgery 02/2012; 144(1):152-9. · 3.41 Impact Factor
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    ABSTRACT: The management of twin-twin transfusion syndrome (TTTS) in its early stages (Quintero Stages I and II) is controversial. We describe the prevalence, severity, incidence and rate of progression of recipient-twin cardiomyopathy in Stages I and II TTTS. Among 451 cases of TTTS evaluated between 2004 and 2009, 123 (27.3%) cases of Stages I and II were reviewed. Echocardiography was used to 'upstage' cases based on the presence or absence of mild (IIIA), moderate (IIIB), or severe (IIIC) recipient cardiomyopathy. Progression was defined by worsening in the degree of recipient-twin cardiomyopathy from initial presentation or failure to respond to amnioreduction. Outcome data included progression of recipient-twin cardiomyopathy, treatment and survival to birth. Data were compared by the chi-square, Fisher's exact test or t-test as appropriate. Seventy-seven of 123 (62.6%) cases were Quintero Stage I and 46/123 (37.4%) Quintero Stage II. Eighty (65.0%) were upstaged to Cincinnati Stage IIIA (n = 25), IIIB (n = 23) or IIIC (n = 32). Management included observation in 11 (8.9%), amnioreduction in 26 (21.1%), amnioreduction followed by selective fetoscopic laser photocoagulation (SFLP) in 43 (35.0%) and primary SFLP in 43 (35.0%). Of 80 cases managed by observation or amnioreduction initially, 43 (53.8%) progressed within a mean duration of 1.4 ± 1.5 weeks. The incidence of progression increased significantly as degree of recipient-twin cardiomyopathy at presentation worsened: Stage I, 9/27 (33.3%); Stage II, 8/15 (53.3%); Stage IIIA, 8/16 (50.0%); Stage IIIB, 10/10 (100%); and Stage IIIC, 8/12 (66.7%) (χ(2) = 14, P < 0.01). Overall fetal survival was 205 out of 244 (84.0%). Fetal survival with observation only was 81.8% (18/22), with amnioreduction only it was 92.3% (48/52), with initial observation or amnioreduction followed by SFLP it was 86.9% (73/84) and with primary SFLP it was 76.7% (66/86). Echocardiography demonstrates a high incidence of recipient-twin cardiomyopathy in early-stage TTTS. The more advanced the recipient-twin cardiomyopathy is, the more likely is progression to occur during observation or following amnioreduction.
    Ultrasound in Obstetrics and Gynecology 01/2012; 39(1):63-8. · 3.56 Impact Factor
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    ABSTRACT: Cardiovascular disease affects >50% of Turner syndrome (TS) patients. With newer imaging modalities, this prevalence increases and the spectrum of recognized anomalies broadens. To determine the prevalence and hemodynamic significance of partial anomalous pulmonary venous return (PAPVR) in adolescents and young adults with TS using transthoracic echocardiogram (TTE) and cardiac magnetic resonance (CMR), and to study the association with phenotype. The records of 39 young TS patients who had received TTE and CMR were reviewed. PAPVR was diagnosed in seven (18%) patients; six were newly diagnosed by CMR after normal TTE. In one subject, PAPVR was associated with right ventricular enlargement and a pulmonic blood flow (Qp) to systemic blood flow (Qs) ratio of 1.9:1.0, necessitating surgical repair. In other subjects with and without PAPVR, Qp:Qs and the right ventricle to left ventricle end-diastolic volume ratio were statistically different. Other clinical features were not predictive of PAPVR. The prevalence of PAPVR is high in TS, and it may be hemodynamically significant. Diagnosis by TTE can be difficult. Appropriate screening and management are indicated.
    Journal of pediatric endocrinology & metabolism: JPEM 01/2012; 25(5-6):435-40. · 0.75 Impact Factor
  • Ultrasound in Obstetrics and Gynecology 10/2011; 38(S1). · 3.56 Impact Factor
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    ABSTRACT: To describe congenital heart disease death rates in infants born between 34 and 40 weeks, estimate the relationship between gestational age and congenital heart disease infant death rates, and compare congenital heart disease death rates across 1- and 2-week intervals in gestational age. The 2000 to 2003 national linked birth/infant death cohort datasets were obtained. Congenital heart disease deaths were identified by using International Statistical Classification of Diseases, 10th Revision codes. Proportional death rates were calculated by using congenital heart disease deaths and all live births. The relationship between congenital heart disease death rates and gestational age was determined. Death rates were compared across intervals. A total of 14.9 million records were analyzed. Congenital heart disease deaths occurred in 4736 infants (0.04%) born between 34 and 40 weeks. There was a significant, negative linear relationship between congenital heart disease death rate and gestational age (R(2) = 0.97). Comparisons across 1-week intervals varied (P = .02-.23). All 2-week intervals were statistically significant (P < .01). Congenital heart disease death rates decrease as gestational age approaches 40 weeks. These results should be considered before elective delivery for the sole indication of prenatally diagnosed congenital heart disease.
    The Journal of pediatrics 06/2011; 159(5):761-5. · 4.02 Impact Factor
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    ABSTRACT: Twin-twin transfusion syndrome (TTTS) complicates 10% to 15% of monochorionic twin pregnancies. Cardiovascular changes of variable severity, such as ventricular hypertrophy, atrioventricular valve regurgitation, and systolic dysfunction, occur predominantly in recipient twins (RTs). It was the purpose of this study to perform a detailed assessment of ventricular geometry and diastolic function between controls, donor twins (DTs), and RTs. In this prospective, case-control study, two-dimensional, pulsed-wave, and Doppler tissue imaging were used to evaluate biventricular geometry and diastolic function in controls, DTs, and RTs. RTs were divided into two groups, severe and mild, on the basis of evidence of high central venous pressure. Specific variables evaluated included relative wall thickness, mitral valve and tricuspid valve E/A velocities, diastolic filling time corrected for heart rate, isovolumic relaxation time, and early (E') and late (A') diastolic myocardial velocities. A total of 120 fetuses (39 TTTS twin pairs and 42 controls) were compared. Increases in relative wall thickness and isovolumic relaxation time were seen in the mild group. In the severe group, further increases in relative wall thickness and isovolumic relaxation time as well as decreased diastolic filling time corrected for heart rate were accompanied by the appearance of a monophasic Doppler inflow profile and elevations in the E/E' ratio, consistent with elevated ventricular filling pressures. Concentric hypertrophy is observed in RTs affected by TTTS and is associated with impaired ventricular relaxation and shortened filling time. In severe cases, further decreases in diastolic filling time and Doppler signs of elevated ventricular filling pressures are present.
    Journal of the American Society of Echocardiography: official publication of the American Society of Echocardiography 06/2011; 24(8):834-40. · 2.98 Impact Factor
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    ABSTRACT: We investigated the effect of polymorphisms in the renin-angiotensin-aldosterone system (RAAS) genes on ventricular remodeling, growth, renal function, and response to enalapril in infants with single ventricle. Single ventricle infants enrolled in a randomized trial of enalapril were genotyped for polymorphisms in 5 genes: angiotensinogen, angiotensin-converting enzyme, angiotensin II type 1 receptor, aldosterone synthase, and chymase. Alleles associated with renin-angiotensin-aldosterone system upregulation were classified as risk alleles. Ventricular mass, volume, somatic growth, renal function using estimated glomerular filtration rate, and response to enalapril were compared between patients with ≥2 homozygous risk genotypes (high risk), and those with <2 homozygous risk genotypes (low risk) at 2 time points: before the superior cavopulmonary connection (pre-SCPC) and at age 14 months. Of 230 trial subjects, 154 were genotyped: Thirty-eight were high risk, and 116 were low risk. Ventricular mass and volume were elevated in both groups pre-SCPC. Ventricular mass and volume decreased and estimated glomerular filtration rate increased after SCPC in the low-risk (P<0.05), but not the high-risk group. These responses were independent of enalapril treatment. Weight and height z-scores were lower at baseline, and height remained lower in the high-risk group at 14 months, especially in those receiving enalapril (P<0.05). Renin-angiotensin-aldosterone system-upregulation genotypes were associated with failure of reverse remodeling after SCPC surgery, less improvement in renal function, and impaired somatic growth, the latter especially in patients receiving enalapril. Renin-angiotensin-aldosterone system genotype may identify a high-risk subgroup of single ventricle patients who fail to fully benefit from volume-unloading surgery. Follow-up is warranted to assess long-term impact. http://www.clinicaltrials.gov. Unique identifier: NCT00113087.
    Circulation 05/2011; 123(21):2353-62. · 15.20 Impact Factor

Publication Stats

112 Citations
118.76 Total Impact Points


  • 2013
    • Boston Children's Hospital
      Boston, Massachusetts, United States
  • 2012
    • University of California, Los Angeles
      • School of Nursing
      Los Angeles, California, United States
  • 2011
    • SickKids
      Toronto, Ontario, Canada
  • 2009–2011
    • Cincinnati Children's Hospital Medical Center
      • Division of Cardiology
      Cincinnati, OH, United States
    • Society for Maternal-Fetal Medicine
      Cincinnati, Ohio, United States
    • Columbia University
      • Division of Cardiology
      New York City, NY, United States
  • 2003–2009
    • Wake Forest School of Medicine
      • Department of Pediatrics
      Winston-Salem, North Carolina, United States