Christopher Statile

Estimated cardiac output and cardiovascular profile score in fetuses with high cardiac output lesions

High cardiac output lesions are associated with an increased risk of fetal death, largely as a result of cardiac failure and hydrops fetalis. The cardiovascular profile score (CVPS) has been used to characterize cardiovascular wellbeing, and has been linked to fetal outcomes in other conditions. We aimed to test the hypothesis that elevated combined cardiac output (CCO) in fetuses with high output lesions may be associated with worsening cardiovascular status, as evidenced by a lower CVPS.

Left ventricular noncompaction in Duchenne muscular dystrophy

BackgroundLeft ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective of this study is to define the prevalence of LVNC in the Duchenne Muscular Dystrophy (DMD) population and characterize its relationship to global LV function.MethodsCardiac magnetic resonance (CMR) was used to assess ventricular morphology and function in 151 subjects: DMD with ejection fraction (EF) > 55% (n = 66), DMD with EF < 55% (n = 30), primary LVNC (n = 15) and normal controls (n = 40). The non-compacted to compacted (NC/C) ratio was measured in each of the 16 standard myocardial segments. LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.ResultsLVNC criteria were met by 27/96 DMD patients (prevalence of 28%): 11 had an EF > 55% (prevalence of 16.7%), and 16 had an EF < 55% (prevalence of 53.3%). The median maximum NC/C ratio was 1.8 for DMD with EF > 55%, 2.46 for DMD with EF < 55%, 1.54 for the normal subjects, and 3.69 for primary LVNC patients. Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.ConclusionThe high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.

Anesthetic techniques for fetal surgery: effects of maternal anesthesia on intraoperative fetal outcomes in a sheep model.

Background:Use of high-dose inhalational anesthesia during open fetal surgery may induce maternal–fetal hemodynamic instability and fetal myocardial depression. The authors’ preliminary human retrospective study demonstrated less fetal bradycardia and left ventricular systolic dysfunction with lower dose desflurane supplemented with propofol and remifentanil IV anesthesia (SIVA). In this animal study, the authors compare maternal–fetal effects of high-dose desflurane anesthesia (HD-DES) and SIVA. Methods:Of 26 instrumented midgestational ewes, data from 11 animals exposed to both SIVA and HD-DES in random sequences and six animals exposed to HD-DES while maternal normotension was maintained were analyzed. Maternal electroencephalography was used to guide comparable depths of anesthesia in both techniques. Hemodynamic parameters, blood gas, and fetal cardiac function from echocardiography were recorded. Results:Compared with SIVA, HD-DES resulted in significant maternal hypotension (mean arterial pressure difference, 19.53 mmHg; 95% CI, 17.6–21.4; P < 0.0001), fetal acidosis (pH 7.11 vs. 7.24 at 150 min, P < 0.001), and decreased uterine blood flow. In the HD-DES group with maternal normotension, uterine blood flow still declined and fetal acidosis persisted, with no statistically significant difference from the group exposed to HD-DES that had maternal hypotension. There was no statistically significant difference in fetal cardiac function. Conclusion:In sheep, SIVA affects maternal hemodynamics less and provides better fetal acid/base status than high-dose desflurane. Fetal echocardiography did not reflect myocardial dysfunction in this model.

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.

Usefulness of Ventricular Premature Complexes in Asymptomatic Patients ≤21 Years as Predictors of Poor Left Ventricular Function

Although ventricular premature complexes (VPCs) have been shown to correlate with decreased cardiac function in adults, the correlation of left ventricular (LV) function to VPCs in asymptomatic children remains unclear. The aim of this study was to determine the correlation of VPC burden with LV function in asymptomatic pediatric patients with structurally normal hearts. This was a retrospective analysis of patients aged ≤21 years with echocardiograms and 24-hour Holter monitors with ≥0.5% VPCs completed within 60 days of each other. LV fractional shortening (FS) was compared with VPC burden and VPC characteristics. Normal LV function was defined as FS ≥28%. Correlation between VPC burden and LV function was determined by regression analysis. Wilcoxons rank-sum test was used to compare LV function with VPC characteristics. This study included 123 patients (77 male [63%]). The median age was 11.6 years (interquartile range 5.8 to 14.3). The median VPC burden was 11.2% (interquartile range 4.8% to 18.9%), and median FS was 36% (interquartile range 33% to 38%). There was no significant correlation between VPC burden and LV FS (p = 0.50). The presence of uniform versus multiform VPCs (p = 0.29), ventricular couplets (p = 0.37), or runs of ventricular ectopy (p = 0.19) were not associated with a decrease in LV FS. Twenty-two patients (18%) had VPC burden >24%, none of which had decreased LV FS. In conclusion, there was no significant relation between VPC burden or VPC characteristics and LV systolic function in this pediatric population with structurally normal hearts.

Factors Influencing Pediatric Outpatient Transthoracic Echocardiography Utilization Before Appropriate Use Criteria Release: A Multicenter Study

Background: Although pediatric appropriate use criteria (AUC) for outpatient transthoracic echocardiography (TTE) are available, little is known about TTE utilization patterns before their release. The aims of this study were to determine the relation between AUC and TTE utilization and to identify patient and physician factors associated with discordance between the AUC and clinical practice. Methods: A retrospective review of 3,000 initial outpatient pediatric cardiology encounters at six centers was performed. Investigator‐determined indications were classified using AUC definitions. Concordance between AUC and TTE utilization was determined. Multivariate analysis was performed to identify patient and physician factors associated with TTEs being performed for rarely appropriate and TTEs not being performed for appropriate indications. Results: Concordance between AUC and TTE utilization was 88%. TTE was performed for rarely appropriate indications in 9% and was associated with patient age < 3 months, indications of murmur, noninvasive imaging physician subspecialty, and physician volume. No TTE was ordered for appropriate indications in 3% and was associated with indications including prior test result (primarily abnormal electrocardiographic findings), older patients, and physician subspecialty other than generalist or imaging. There was high variability in TTE utilization among centers. Conclusions: There was a reasonable degree of concordance between AUC and clinical practice before AUC publication. Several patient and physician factors were associated with discordance with the AUC. These findings should be considered in efforts to disseminate the AUC and in the development of future iterations. The causes for variation among centers deserve further exploration. HighlightsLittle is known about baseline TTE utilization patterns and how well they align with the pediatric AUC.Concordance between pediatric AUC and TTE ordering was 88%.Among discordant ordering, the highest rates of TTE for rarely appropriate indications were in patients less than 3 months of age. An abnormal prior test result (primary abnormal electrocardiographic findings) was the indication most strongly associated with not ordering TTE for appropriate indications.There was high variability in concordance between AUC and TTE utilization between the different participating centers. Abbreviations: AUC = Appropriate use criteria; TTE = Transthoracic echocardiography.

STANDARDIZING THE CARE OF OUTPATIENT PEDIATRIC SYNCOPE AND DIZZINESS: A LESSON IN UTILIZATION AND CHARGE REDUCTION

Background: Variability in medical practice is associated with higher cost without improved outcome. Our aim was to standardize the evaluation and treatment of dizziness/syncope and track resultant cost savings. Methods: A multidisciplinary team developed a care algorithm using best evidence and

Congenital Cardiology SolutionsLEFT VENTRICULAR NON-COMPACTION IN DUCHENNE MUSCULAR DYSTROPHY

Left ventricular non-compaction (LVNC) is characterized by regions with prominent, deep trabeculations in the left ventricular (LV) endocardium. Anecdotal data suggest an increased prevalence of LVNC in neuromuscular diseases including Duchenne Muscular Dystrophy (DMD). This study aims to define the