Eileen C. King

Improved Outcomes in a Quality Improvement Collaborative for Pediatric Inflammatory Bowel Disease

OBJECTIVES: Unintended variation in the care of patients with Crohn disease (CD) and ulcerative colitis (UC) may prevent achievement of optimal outcomes. We sought to improve chronic care delivery and outcomes for children with inflammatory bowel disease by using network-based quality improvement methods. METHODS: By using a modified Breakthrough Series collaborative structure, 6 ImproveCareNow Network care centers tested changes in chronic illness care and collected data monthly. We used an interrupted time series design to evaluate the impact of these changes. RESULTS: Data were available for 843 children with CD and 345 with UC. Changes in care delivery were associated with an increase in the proportion of visits with complete disease classification, measurement of thiopurine methyltransferase (TPMT) before initiation of thiopurines, and patients receiving an initial thiopurine dose appropriate to their TPMT status. These were significant in both populations for all process variables (P < .01) except for measurement of TPMT in CD patients (P = .12). There were significant increases in the proportion of CD (55%–68%) and UC (61%–72%) patients with inactive disease. There was also a significant increase in the proportion of CD patients not taking prednisone (86%–90%). Participating centers varied in the success of achieving these changes. CONCLUSIONS: Improvements in the outcomes of patients with CD and UC were associated with improvements in the process of chronic illness care. Variation in the success of implementing changes suggests the importance of overcoming organizational factors related to quality improvement success.

Abnormal Circumferential Strain is Present in Young Duchenne Muscular Dystrophy Patients

Advances in management of non-cardiac issues in Duchenne muscular dystrophy (DMD) have improved such that DMD-associated cardiac disease has become the leading cause of death for such patients. Cardiac dysfunction measured by standard transthoracic echocardiographic methods, e.g., fractional shortening (FS) and ejection fraction (EF), is rarely present during the first decade of life. The current study used transthoracic echocardiogram (TTE) to assess strain (ε), an indicator of regional ventricular function, in young DMD patients. A retrospective review of the TTE database was performed. TTE results from DMD patients <8xa0years (nxa0=xa063) performed during 2009 to 2010 were compared with TTE results from an unaffected control group (nxa0=xa061). Feature tracking analysis software was used to measure total circumferential strain (εcc) as well as segmental εcc based on the American Society of Echocardiography 16-segment model. Although there were no differences in FS, the absolute value for left-ventricular (LV) εcc at the mid-chamber level was decreased in DMD (−21.7 %xa0±xa03.8 % vs. −19.8 %xa0±xa04.2 %, pxa0<xa00.01; unaffected vs. DMD). Segmental εcc was similarly affected in the anteroseptal segment (−23.0 %xa0±xa06.1 % vs. −18.9 %xa0±xa07.0 %, pxa0=xa00.001; controls vs. DMD), the inferior segment (−20.7 %xa0±xa05.16 % vs. −17.7 %xa0±xa06.1 %, pxa0=xa00.003; controls vs. DMD), and the inferolateral segment (−18.3 %xa0±xa06.2 % vs. −15.9 %xa0±xa06.7 %, pxa0=xa00.04; controls vs. DMD). In the present study we demonstrate both total and segmental LV εcc (anteroseptal, inferior, and inferolateral segments) abnormalities at the mid-chamber level in a large group of young DMD patients with normal FS. These novel findings substantiate that the disease process is present and results in abnormal myocardial function before standard measures detect global dysfunction.

Meta-analysis of function after secondary shoulder surgery in neonatal brachial plexus palsy.

Background: Shoulder internal rotation contracture, active abduction, and external rotation deficits are common secondary problems in neonatal brachial plexus palsy (NBPP). Soft tissue shoulder operations are often utilized for treatment. The objective was to conduct a meta-analysis and systematic review analyzing the clinical outcomes of NBPP treated with a secondary soft-tissue shoulder operation. Methods: A literature search identified studies of NBPP treated with a soft-tissue shoulder operation. A meta-analysis evaluated success rates for the aggregate Mallet score (≥4 point increase), global abduction score (≥1 point increase), and external rotation score (≥1 point increase) using the Mallet scale. Subgroup analysis was performed to assess these success rates when the author chose arthroscopic release technique versus open release technique with or without tendon transfer. Results: Data from 17 studies and 405 patients were pooled for meta-analysis. The success rate for the global abduction score was significantly higher for the open technique (67.4%) relative to the arthroscopic technique (27.7%, P<0.0001). The success rates for the global abduction score were significantly different among sexes (P=0.01). The success rate for external rotation was not significantly different between the open (71.4%) and arthroscopic techniques (74.1%, P=0.86). No other variable was found to have significant impact on the external rotation outcomes. The success rate for the aggregate Mallet score was 57.9% for the open technique, a nonsignificant increase relative to the arthroscopic technique (53.5%, P=0.63). Data suggest a correlation between increasing age at the time of surgery and a decreasing likelihood of success with regards to aggregate Mallet with an odds ratio of 0.98 (P=0.04). Conclusions: Overall, the secondary soft-tissue shoulder operation is an effective treatment for improving shoulder function in NBPP in appropriately selected patients. The open technique had significantly higher success rates in improving global abduction. There were no significant differences in the success rates for improvement in the external rotation or aggregate Mallet score among these surgical techniques. Level of Evidence: Level IV—meta-analysis.

Impaired cerebral autoregulation in preoperative newborn infants with congenital heart disease

Objectives: To characterize cerebral autoregulation (CA) in preoperative newborn infants with congenital heart disease (CHD). Methods: This was a prospective, pilot study of term newborns with CHD who required intensive care. Continuous mean arterial blood pressure (MAP), cerebral tissue oxygen saturation (SCTO2) via near‐infrared spectroscopy, and arterial oxygen saturation (SaO2) were collected. Significant low‐frequency coherence between MAP and SCTO2 was used to define impaired CA in 20‐minute epochs. Cerebral fractional tissue oxygen extraction (FTOE) = (SaO2 − SCTO2)/SaO2 was calculated. Spearmans and rank bi‐serial correlations and logistic linear models accounting for multiple measures were used to identify associations with impaired CA and coherence. Results: Twenty‐four term neonates were evaluated for 23.4 ± 1.8 hours starting the first day of life. Periods of SaO2 variability >5% were excluded, leaving 63 ± 10 epochs per subject, 1515 total for analysis. All subjects demonstrated periods of abnormal CA, mean 15.3% ± 12.8% of time studied. Significant associations with impaired CA per epoch included greater FTOE (P = .02) and lack of sedation (P = .02), and associations with coherence included greater FTOE (P = .03), lack of sedation (P = .03), lower MAP (P = .006), and lower hemoglobin (P = .02). Conclusions: Term newborns with CHD display time‐varying CA abnormalities. Associations seen between abnormal CA and greater FTOE, lack of sedation, and lower hemoglobin suggest that impaired oxygen delivery and increased cerebral metabolic demand may overwhelm autoregulatory capacity in these infants. Further studies are needed to determine the significance of impaired CA in this population.

Correlation of Precordial Voltages to Left Ventricular Mass on Echocardiogram in Adolescent Patients With Hypertrophic Cardiomyopathy Compared With that in Adolescent Athletes

Electrocardiograms continue to be part of screening programs for athletes and familial hypertrophic cardiomyopathy (HC). Whether electrocardiographic (ECG) findings of left ventricular (LV) hypertrophy can distinguish between healthy populations and those with HC remains unclear. We sought to (1) analyze the relation between ECG voltage and LV mass in patients with HC and (2) evaluate ECG characteristics of patients with phenotypical HC. Retrospective cohort of patients with HC aged 13 to 18 years. Relation between ECG voltages (RV6, SV1, and RV6 + SV1) and echocardiogram measurements of LV mass was investigated using smoothing splines to display relations and compared with those in a prospectively obtained population of adolescents. Frequency of abnormal LV voltages and nonvoltage ECG changes (Q waves, T-wave changes, and ST changes) were analyzed for association with HC. Fifty-three patients with HC (72% men) were age and gender matched to 104 control patients. Smoothing splines demonstrated that parabolic rather than linear relations existed between LV mass and SV1, RV6, and RV6 + SV1 in patients with HC and not the control cohort. LV hypertrophy by ECG voltage criteria was present in 34% of patients with HC and associated with poor sensitivity (29%). In patients with HC, 56% demonstrated nonvoltage ECG abnormalities and were associated with improved sensitivity (68%) and high specificity (94%). In conclusion, there is a parabolic relation between LV voltages and LV mass in adolescents with HC that may lead to pseudonormalization. Voltage abnormalities were associated with poor sensitivity, whereas nonvoltage criteria were associated with improved sensitivity with high specificity.

Fetal somatic growth trajectory differs by type of congenital heart disease

BackgroundThe growth trajectories of common measurements, including estimated fetal weight (EFW), head circumference (HC), and abdominal circumference (AC), in fetuses with congenital heart disease (CHD) have not been described for different cardiac lesions. We hypothesized that (i) fetuses with CHD have differential growth in utero, and (ii) different categories of CHD demonstrate different in utero growth curves.MethodsWe performed a retrospective observational cohort study of pregnancies with known fetal CHD seen from January 2000 to June 2013. For analysis, the infants were divided into single ventricle (SV), biventricular conotruncal, d-transposition of great arteries (d-TGA), biventricular septal defects (SD; including atrial, ventricular, and atrioventricular SD), and all others (Other).ResultsA total of 194 newborns met inclusion criteria. There was significant differential growth of EFW in all CHD types, except d-TGA, starting with low z-scores before 25 weeks gestation, improving toward normal around 30–32 weeks gestation, and then again differential growth with advancing gestation. SV and SD groups had significant differential growth of HC starting early in gestation and linearly progressing negative z-scores with advancing gestation.ConclusionWe observed differences in the fetal growth curves throughout gestation for the major categories of CHD, including significant differential growth in even “simple” CHD, such as SD.

The Use of Concomitant Immunomodulators with Adalimumab Therapy in Pediatric Crohn’s Disease

Objectives: Adalimumab is an effective treatment for Crohn’s disease but antibody development may cause loss of response. Concomitant use of an immunomodulator reduces the development of antibodies. We performed a 5- year cross-sectional study of variation in use of adalimumab and concomitant therapy in a large pediatric population.Methods: We identified patients with Crohn’s disesae aged <18 years in the ImproveCareNow registry who received adalimumab between June 2010 through May 2015, and determined the rates of treatment with adalimumab and concomitant therapy with thiopurine or methotrexate, including variation by age, sex, geographical region and annual change. Chi-square tests compared percentages and the Cochran Armitage Trend Test tested percentages over time and across age groups.Results: Of 7,271 patients, adalimumab treatment occurred in 1,009 (14%), more likely with increasing age (p<0.001), in females (p<0.001), and in the West than the Northeast US (p<0.001). From year 1 to year 5, the use of adalimumab increased from 7% to 13% (p<0.001) and concomitant therapy increased from 25% to 47% (p<0.001). Of patients treated with adalimumab, 47% received concomitant therapy with thiopourine (19%) or methotrexate (28%). Concomitant therapy occurred more commonly in younger patients (p<0.01) but frequencies by sex were not significantly different (p=0.17).Conclusions: In pediatric Crohn’s disease there is increasing use of both adalimumab and concomitant therapy, including both thiopurine and methotrexate, with significant variation by age, sex and region of the US. Further study is needed to determine the effectiveness of and indications for concomitant therapy with adalimumab treatment.

Utility of Echocardiography in the Assessment of Left Ventricular Diastolic Function and Restrictive Physiology in Children and Young Adults with Restrictive Cardiomyopathy: A Comparative Echocardiography-Catheterization Study

The aim of the study is to determine the utility of echocardiography in the assessment of diastolic function in children and young adults with restrictive cardiomyopathy (RCM). RCM is a rare disease with high mortality requiring frequent surveillance. Accurate, noninvasive echocardiographic measures of diastolic function may reduce the need for invasive catheterization. Single-center, prospective, observational study of pediatric and young adult RCM patients undergoing assessment of diastolic parameters by simultaneous transthoracic echocardiogram (TTE) and invasive catheterization. Twenty-one studies in 15 subjects [median (IQR)xa0=xa013.8xa0years (7.0–19.2), 60% female] were acquired with median left ventricular end-diastolic pressure (LVEDP) 21 (IQR 18–25) mmHg. TTE parameters of diastolic function, including pulmonary vein A wave duration (rsxa0=xa00.79) and indexed left atrial volume (rsxa0=xa00.49), demonstrated significant positive correlation, while mitral valve A (rsxa0=xa0−0.44), lateral e′ (rsxa0=xa0−0.61) and lateral a′ (rsxa0=xa0−0.61) velocities showed significant negative correlation with LVEDP. Lateral a′ velocity (≤0.042xa0m/s) and pulmonary vein A wave duration (≥156xa0m/s) both had sensitivity and specificity ≥80% for LVEDPxa0≥xa020xa0mmHg. In pediatric and young adult patients with RCM, lateral a′ velocity and pulmonary vein A wave duration predicted elevated LVEDP with high sensitivity and specificity; however, due to technical limitations the latter was reliably measured in 12/21 patients. These noninvasive parameters may have utility in identifying patients that require further assessment with invasive testing. These findings require validation in a multicenter prospective cohort prior to widespread clinical implementation.