Publications by authors named "Pei-Ni Jone"

58 Publications

IVIG Compared to IVIG Plus Infliximab in Multisystem Inflammatory Syndrome in Children.

Pediatrics 2021 Sep 22. Epub 2021 Sep 22.

Department of Pediatrics, Section of Infectious Diseases, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO.

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http://dx.doi.org/10.1542/peds.2021-052702DOI Listing
September 2021

Angiostatic Peptide, Endostatin, Predicts Severity in Pediatric Congenital Heart Disease-Associated Pulmonary Hypertension.

J Am Heart Assoc 2021 10 8;10(20):e021409. Epub 2021 Oct 8.

Division of Pediatric Cardiology Department of Pediatrics Johns Hopkins University Baltimore MD.

Background Endostatin, an angiogenic inhibitor, is associated with worse pulmonary arterial hypertension (PAH) outcomes in adults and poor lung growth in children. This study sought to assess whether endostatin is associated with disease severity and outcomes in pediatric PAH. Methods and Results Serum endostatin was measured in cross-sectional (N=160) and longitudinal cohorts (N=64) of pediatric subjects with PAH, healthy pediatric controls and pediatric controls with congenital heart disease (CHD) (N=54, N=15), and adults with CHD associated PAH (APAH-CHD, N=185). Outcomes, assessed by regression and Kaplan-Meier analysis, included hemodynamics, change in endostatin over time, and transplant-free survival. Endostatin secretion was evaluated in pulmonary artery endothelial and smooth muscle cells. Endostatin was higher in those with PAH compared with healthy controls and controls with CHD and was highest in those with APAH-CHD. In APAH-CHD, endostatin was associated with a shorter 6-minute walk distance and increased mean right atrial pressure. Over time, endostatin was associated with higher pulmonary artery pressure and pulmonary vascular resistance index, right ventricular dilation, and dysfunction. Endostatin decreased with improved hemodynamics over time. Endostatin was associated with worse transplant-free survival. Addition of endostatin to an NT-proBNP (N-terminal pro-B-type natriuretic peptide) based survival analysis improved risk stratification, reclassifying subjects with adverse outcomes. Endostatin was secreted primarily by pulmonary artery endothelial cells. Conclusions Endostatin is associated with disease severity, disease improvement, and worse survival in APAH-CHD. Endostatin with NT-proBNP improves risk stratification, better predicting adverse outcomes. The association of elevated endostatin with shunt lesions suggests that endostatin could be driven by both pulmonary artery flow and pressure. Endostatin could be studied as a noninvasive prognostic marker, particularly in APAH-CHD.
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http://dx.doi.org/10.1161/JAHA.120.021409DOI Listing
October 2021

IVIG Compared to IVIG Plus Infliximab in Multisystem Inflammatory Syndrome in Children.

Pediatrics 2021 Sep 21. Epub 2021 Sep 21.

Department of Pediatrics, Section of Infectious Diseases, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO;

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http://dx.doi.org/10.1542/peds.2021-052702DOI Listing
September 2021

A multicenter study of three-dimensional echocardiographic evaluation of normal pediatric left ventricular volumes and function.

Echocardiography 2021 04 8;38(4):641-645. Epub 2021 Mar 8.

Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Background: Three-dimensional echocardiography (3DE) evaluation of left ventricular (LV) volume and function in pediatrics compares favorably with cardiac magnetic resonance imaging. The aim of this study was to establish from a multicenter, normal pediatric z-score values of 3DE left ventricular volumes and function.

Methods: Six hundred and ninety-eight healthy children (ages 0-18 years) were recruited from five centers. LV 3DE was acquired from the 4-chamber view. A vendor-independent software analyzed end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), and ejection fraction (EF) using semi-automated quantification. Body surface area (BSA)-based z-scores were generated. Intraobserver and interobserver variability were calculated using intraclass correlation (ICC) and repeatability coefficient (RC).

Results: Z-scores were generated for ESV, EDV, and SV. The ICC for intraobserver variability for EDV, ESV, and SV was 0.99, 0.99, and 0.99, respectively. The ICC for interobserver variability for EDV, ESV, and SV was 0.98, 0.94, and 0.98, respectively. The RC for intraobserver and interobserver variability for LV EF was 4.39% (95% CI: 3.01, 5.59) and interobserver was 7.08% (95%CI: 5.51, 8.42).

Conclusions: We report pediatric z-scores for normal LV volumes using the semi-automated method from five centers, enhancing its generalizability. 3DE evaluation of LV volumes and EF in pediatric patients is highly reproducible.
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http://dx.doi.org/10.1111/echo.15026DOI Listing
April 2021

Transesophageal three-dimensional echocardiographic guidance for pacemaker lead extraction.

Pacing Clin Electrophysiol 2021 04 22;44(4):641-650. Epub 2021 Feb 22.

Department of Pediatric Cardiology, Division of Cardiology, Heart Institute, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado, USA.

Background: The ability of transesophageal three-dimensional echocardiography (3DE) to aid in pacemaker lead extraction has not yet been evaluated. 3DE provides real-time evaluation of intracardiac anatomy and the location of pacemaker leads in greater detail than either fluoroscopy or -two-dimensional echocardiography (2DE), aiding in the extraction of such leads, which can be potentially dangerous. We sought to investigate the feasibility and utility of 3DE to visualize intracardiac anatomy and pacemaker leads, and to assist in lead extraction procedures.

Methods: We utilized 3DE in nine encounters for eight different patients, to visualize intracardiac anatomy and leads before, during, and after extraction to evaluate the feasibility and utility to aid in the procedure and evaluate for potential sequelae.

Results: 3DE was able to identify pertinent intracardiac anatomy and leads in all cases. 3DE detected procedural complications or altered management in five of nine encounters (five of eight patients); this included detection of an avulsed papillary muscle, tricuspid valve leaflet damage, and cast/thrombus after lead removal, as well as adjustment of excess lead slack to avoid future valve damage, or risk stratification of lead removal.

Conclusion: 3DE is feasible and adds utility to lead extraction cases by visualizing intracardiac anatomy and leads beyond fluoroscopy or 2DE, providing real-time information during extraction, and identifying potential complications.
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http://dx.doi.org/10.1111/pace.14191DOI Listing
April 2021

Longitudinal assessment of right atrial conduit fraction provides additional insight to predict adverse events in pediatric pulmonary hypertension.

Int J Cardiol 2021 04 2;329:242-245. Epub 2021 Jan 2.

University of Colorado, Department of Pediatrics, Section of Pediatric Cardiology, United States of America.

Background: Recent studies show adverse right atrial (RA) emptying pattern is prognostic for clinical worsening events in pediatric pulmonary arterial hypertension (PAH). No study has reported changes in RA emptying over time or evaluated whether serial measurements offer further prognostic information.

Methods: Prospective study of 32 children with idiopathic or heritable PAH undergoing echocardiogram at baseline and 1-year. RA conduit fraction percent (RA cF%) was measured as percentage of total diastolic RA area change prior to the electrical p wave. Clinical worsening was analyzed with a predefined composite adverse event outcome.

Results: Longitudinal subjects (median age 13.3 yr) had RA cF% 61% (IQR 32-68%) at baseline and 60% (IQR 35-73%) at 1-year (NS). 11 subjects had a qualifying event during median 21-month follow-up. Subjects with an event had 1-year RA cF% = 33% (IQR 20-40%) compared to 72% (IQR 63-75%) for those with no event (p < 0.001). Event rates were lowest for subjects with both echocardiograms showing RA cF% > 60% (0%), highest for subjects with neither (80%), and intermediate for those with one (38%, p = 0.003).

Conclusions: Changes in RA cF% inform risk of adverse events in pediatric PAH. This finding supports the role of RA cF% as both a prognostic biomarker and potential treatment target.
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http://dx.doi.org/10.1016/j.ijcard.2020.12.073DOI Listing
April 2021

Right ventricular area strain from 3-dimensional echocardiography: Mechanistic insight of right ventricular dysfunction in pediatric pulmonary hypertension.

J Heart Lung Transplant 2021 02 15;40(2):138-148. Epub 2020 Nov 15.

Universite Côte d'Azur UR2CA, Inria Epione Team, Department of Cardiology, Hôpital Pasteur, CHU de Nice, France.

Background: Right ventricular (RV) function is a major contributor to the outcome of pulmonary arterial hypertension (PAH). Adult studies demonstrated that regional and global changes in RV deformation are prognostic in PAH using 3-dimensional echocardiography (3DE). However, regional and global dynamic changes in RV mechanics have not been described in pediatric PAH. We compared 3DE RV regional and global deformation between pediatric patients who had associated PAH with congenital heart disease (APAH-CHD), pediatric patients who had idiopathic PAH (IPAH), and normal controls, and evaluated the clinical outcomes.

Methods: A total of 48 controls, 47 patients with APAH-CHD, and 45 patients with IPAH were evaluated. 3DE RV sequences were analyzed and post-processed to extract global and regional deformation (circumferential, longitudinal, and area strain). Statistical analyses compared the sub-groups on the basis of global and regional deformation, and outcome analysis was performed.

Results: Patients with PAH had significantl8y different global and regional deformation (p < 0.001) compared with controls. Patients with APAH-CHD and and those with IPAH significantly differed in global circumferential strain (p < 0.010), area strain (inlet septum, p = 0.041), and circumferential strain at the inlet septum (p < 0.019), apex free wall (p < 0.004), and inlet free wall (p < 0.004). Circumferential strain at the inlet free wall and circumferential, longitudinal, and area strain at the apex free wall were predictors of adverse events.

Conclusions: RV regional and global strain differ between controls and pediatric patients with PAH. RV apical free-wall area strain provides insight into the mechanism of RV dysfunction in pediatric patients with PAH, with regional strain emerging as outcome predictors, suggesting that this novel measure may be considered as a future measure of RV function.
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http://dx.doi.org/10.1016/j.healun.2020.11.005DOI Listing
February 2021

Update on noninvasive imaging of right ventricle dysfunction in pulmonary hypertension.

Cardiovasc Diagn Ther 2020 Oct;10(5):1604-1624

Division of Pediatric Cardiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado, USA.

Pulmonary hypertension (PH) is a progressive disease affecting patients across the life span. The pathophysiology primarily involves the pulmonary vasculature and right ventricle (RV), but eventually affects the left ventricular (LV) function as well. Safe, accurate imaging modalities are critical for diagnosis, serial monitoring, and tailored therapy. While cardiac catheterization remains the conventional modality for establishing diagnosis and serial monitoring, noninvasive imaging has gained considerable momentum in providing accurate assessment of the entire RV-pulmonary axis. In this state-of-the-art review, we will discuss the most recent developments in echocardiography, magnetic resonance imaging, and computed tomography in PH evaluation from pediatric to adult population.
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http://dx.doi.org/10.21037/cdt-20-272DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7666960PMC
October 2020

Pediatric Cardiology: From Basics to Innovation.

Pediatr Clin North Am 2020 10 10;67(5):xvii-xviii. Epub 2020 Aug 10.

Children's Hospital Colorado, 13123 East 16th Avenue, Aurora, CO 80045, USA. Electronic address:

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http://dx.doi.org/10.1016/j.pcl.2020.07.009DOI Listing
October 2020

Update on the Management of Kawasaki Disease.

Pediatr Clin North Am 2020 10 11;67(5):811-819. Epub 2020 Aug 11.

Department of Pediatrics, Pediatric Cardiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA.

Treatment of Kawasaki disease (KD) with intravenous immunoglobulin (IVIG) administered within the initial 10 days of fever onset decreases the risk of coronary artery aneurysms (CAAs) from ∼ 25% to less than 5%. However, patients with IVIG resistance, young infants, men, highly inflamed patients, and/or those with coronary changes at diagnosis remain at high risk for CAA. High-risk patients may benefit from acute, adjunctive antiinflammatory treatment in addition to IVIG. Optimal therapy remains unknown. This article reviews the acute pharmacologic management of patients with KD, focusing on adjunctive primary therapy options and treatment of patients with IVIG resistance.
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http://dx.doi.org/10.1016/j.pcl.2020.06.002DOI Listing
October 2020

Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From the International Kawasaki Disease Registry.

J Am Heart Assoc 2020 08 28;9(15):e016440. Epub 2020 Jul 28.

Division of Pediatric Cardiology Centre Hospitalier Universitaire Ste-Justine University of Montreal Quebec Canada.

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD. Methods and Results A 34-institution international registry of 1651 patients with KD who had CAAs (maximum CAA score ≥2.5) was used. Time-to-event analyses were performed using the Kaplan-Meier method and Cox proportional hazard models for risk factor analysis. In patients with CAA scores ≥10, the cumulative incidence of luminal narrowing (>50% of lumen diameter), coronary artery thrombosis, and composite major adverse cardiovascular complications at 10 years was 20±3%, 18±2%, and 14±2%, respectively. No complications were observed in patients with a CAA score <10. Higher CAA score and a greater number of coronary artery branches affected were associated with increased risk of all types of complications. At 10 years, normalization of luminal diameter was noted in 99±4% of patients with small (2.5≤<5.0), 92±1% with medium (5.0≤<10), and 57±3% with large CAAs (≥10). CAAs in the left anterior descending and circumflex coronary artery branches were more likely to normalize. Risk factor analysis of coronary artery branch level outcomes was performed with a total of 893 affected branches with score ≥10 in 440 patients. In multivariable regression models, hazards of luminal narrowing and thrombosis were higher for patients with CAAs of the right coronary artery and left anterior descending branches, those with CAAs that had complex architecture (other than isolated aneurysms), and those with CAAs with scores ≥20. Conclusions For patients with CAA after KD, medium-term risk of complications is confined to those with maximum CAA scores ≥10. Further risk stratification and close follow-up, including advanced imaging, in patients with large CAAs is warranted.
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http://dx.doi.org/10.1161/JAHA.119.016440DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7792232PMC
August 2020

Circulating microRNAs differentiate Kawasaki Disease from infectious febrile illnesses in childhood.

J Mol Cell Cardiol 2020 09 4;146:12-18. Epub 2020 Jul 4.

Pediatric Cardiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA.

Background: Kawasaki Disease (KD) is an acute vasculitis of unknown etiology in children that can lead to coronary artery lesions (CAL) in 25% of untreated patients. There is currently no diagnostic test for KD, and the clinical presentation is often difficult to differentiate from other febrile childhood illnesses. Circulating microRNAs (miRNAs) are small noncoding RNA molecules that control gene expression by inducing transcript degradation or by blocking translation. We hypothesize that the expression of circulating miRNAs will differentiate KD from non-KD febrile illnesses in children.

Methods: Circulating miRNA profiles from 84 KD patients and 29 non-KD febrile controls (7 viral and 22 bacterial infections) were evaluated. 3 ul of serum from each subject was submitted to 3 freeze/heat cycles to ensure miRNA release from microvesicles or interaction with serum proteins. miRNAs were reverse transcribed using a pool of primers specific for each miRNA. Real-time PCR reactions were performed in a 384 well plate containing sequence-specific primers and TaqMan probes in the ABI7900. '.

Results: KD patients (3.6 ± 2.2 yrs., 58% male) were found to have a unique circulating miRNA profile, including upregulation of miRNA-210-3p, -184, and -19a-3p (p < .0001), compared to non-KD febrile controls (8.5 ± 6.1 yrs., 72% male).

Conclusions: Circulating miRNAs can differentiate KD from infectious febrile childhood diseases, supporting their potential as a diagnostic biomarker for KD.
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http://dx.doi.org/10.1016/j.yjmcc.2020.06.011DOI Listing
September 2020

Applications of three-dimensional transesophageal echocardiography in congenital heart disease.

Authors:
Pei-Ni Jone

Echocardiography 2020 10 28;37(10):1665-1672. Epub 2020 Jun 28.

Pediatric Cardiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA.

Three-dimensional echocardiography allows for presurgical planning for congenital heart disease, reduces radiation using fusion imaging in catheter interventions, and provides guidance during catheter interventions and lead placements or extractions. The purpose of this review is to detail applications of three-dimensional transesophageal echocardiography in presurgical planning of congenital heart disease, guidance of catheter interventions such as fusion imaging, and guidance in electrophysiology lead extractions or placements.
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http://dx.doi.org/10.1111/echo.14780DOI Listing
October 2020

Echocardiography-fluoroscopy fusion imaging: The essential features used in congenital and structural heart disease interventional guidance.

Echocardiography 2020 05 27;37(5):769-780. Epub 2020 Apr 27.

Division of Cardiology, University of Colorado School of Medicine, Aurora, Colorado.

Increased catheter-based interventions in congenital and structural heart disease require imaging modalities to be oriented in the same visual perspective. The use of echocardiography-fluoroscopy fusion (EFF) imaging has been developed for better characterization of complex anatomy and to facilitate key steps in interventional procedures. This review will detail the technology behind EFF, the differences between the two ultrasound fusion systems, and essential features of EFF imaging in congenital and structural heart disease interventions.
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http://dx.doi.org/10.1111/echo.14670DOI Listing
May 2020

Right Atrial Conduit Phase Emptying Predicts Risk of Adverse Events in Pediatric Pulmonary Arterial Hypertension.

J Am Soc Echocardiogr 2020 08 24;33(8):1006-1013. Epub 2020 Apr 24.

Section of Cardiology, Department of Pediatrics, University of Colorado Denver School of Medicine, Denver, Colorado.

Background: Idiopathic pulmonary arterial hypertension (PAH) is a severe disease associated with a 20% 5-year mortality, often due to right heart failure. Recent studies suggest that compensatory changes in right atrial (RA) function may precede other clinical and echocardiographic evidence of right ventricular dysfunction. No prior prospective study has evaluated the role of RA emptying pattern as a prognostic marker of adverse clinical events in pediatric PAH.

Objective: To demonstrate whether RA fractional emptying indices will prospectively predict risk of adverse clinical outcomes in pediatric PAH patients.

Methods: Single-center, prospective cohort analysis of 41 patients with idiopathic or heritable PAH and 1:1 age and sex-matched controls with normal echo and electrocardiogram. Right atrial area (RAA) was measured just prior to tricuspid valve opening (RAAmax), at electrical p wave (RAAp), and just after tricuspid valve closing (RAAmin). Right atrial conduit fraction percent (RA cF%) was defined as the percentage of total RAA change happening prior to the electrical p wave = (RAAmax - RAAp)/(RAAmax - RAAmin) ∗ 100. Clinical worsening was analyzed with a predefined composite adverse event outcome.

Results: RA measurements were technically feasible in all study participants. The PAH patients (median age 11.9 years) had decreased RA cF% compared with controls (P < .0001), and PAH subjects with lower RA cF% demonstrated higher right ventricular systolic (R = -0.49, P = .019) and end-diastolic (R = -0.52, P = .012) pressure than those with higher RA cF%. Sixteen subjects had a clinical event. Right atrial cF% (hazard ratio = 0.09; P < .001) was highly prognostic for risk of adverse clinical event with area under the curve = 0.90 on receiver operating characteristic curve analysis (median 3.2-year follow-up).

Conclusions: Right atrial conduit phase emptying is significantly altered in pediatric PAH. Within the PAH population, decreased RA cF% was prognostic for risk of clinical worsening. The combination of accuracy and ease of measurement could make RA cF% a clinically useful, noninvasive biomarker of early right heart failure and risk of disease progression in pediatric PAH.
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http://dx.doi.org/10.1016/j.echo.2020.02.015DOI Listing
August 2020

Multicentre validation of a computer-based tool for differentiation of acute Kawasaki disease from clinically similar febrile illnesses.

Arch Dis Child 2020 08 5;105(8):772-777. Epub 2020 Mar 5.

Department of Pediatrics, University of California San Diego, La Jolla, California, USA

Background: The clinical features of Kawasaki disease (KD) overlap with those of other paediatric febrile illnesses. A missed or delayed diagnosis increases the risk of coronary artery damage. Our computer algorithm for KD and febrile illness differentiation had a sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of 94.8%, 70.8%, 93.7% and 98.3%, respectively, in a single-centre validation study. We sought to determine the performance of this algorithm with febrile children from multiple institutions across the USA.

Methods: We used our previously published 18-variable panel that includes illness day, the five KD clinical criteria and readily available laboratory values. We applied this two-step algorithm using a linear discriminant analysis-based clinical model followed by a random forest-based algorithm to a cohort of 1059 acute KD and 282 febrile control patients from five children's hospitals across the USA.

Results: The algorithm correctly classified 970 of 1059 patients with KD and 163 of 282 febrile controls resulting in a sensitivity of 91.6%, specificity of 57.8% and PPV and NPV of 95.4% and 93.1%, respectively. The algorithm also correctly identified 218 of the 232 KD patients (94.0%) with abnormal echocardiograms.

Interpretation: The expectation is that the predictive accuracy of the algorithm will be reduced in a real-world setting in which patients with KD are rare and febrile controls are common. However, the results of the current analysis suggest that this algorithm warrants a prospective, multicentre study to evaluate its potential utility as a physician support tool.
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http://dx.doi.org/10.1136/archdischild-2019-317980DOI Listing
August 2020

Development and Utility of Quality Metrics for Ambulatory Pediatric Cardiology in Kawasaki Disease.

Clin Pediatr (Phila) 2020 03 3;59(3):245-251. Epub 2020 Jan 3.

Seattle Children's Hospital, Seattle, WA, USA.

The Adult Congenital and Pediatric Cardiology (ACPC) Section of the American College of Cardiology sought to develop quality indicators/metrics for ambulatory pediatric cardiology practice. The objective of this study was to report the creation of metrics for patients with Kawasaki disease. Over a period of 5 months, 12 pediatric cardiologists developed 24 quality metrics based on the most relevant statements, guidelines, and research studies pertaining to Kawasaki disease. Of the 24 metrics, the 8 metrics deemed the most important, feasible, and valid were sent on to the ACPC for consideration. Seven of the 8 metrics were approved using the RAND method by an expert panel. All 7 metrics approved by the ACPC council were accepted by ACPC membership after an "open comments" process. They have been disseminated to the pediatric cardiology community for implementation by the ACPC Quality Network.
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http://dx.doi.org/10.1177/0009922819896098DOI Listing
March 2020

Congenital and Structural Heart Disease Interventions Using Echocardiography-Fluoroscopy Fusion Imaging.

J Am Soc Echocardiogr 2019 12 6;32(12):1495-1504. Epub 2019 Oct 6.

Division of Cardiology, University of Colorado School of Medicine, Aurora, Colorado.

With the increasing frequency of catheter-based interventions in congenital heart disease and structural heart disease, the use of fusion imaging has become a major enhancement for understanding complex anatomy and facilitating key steps in interventional procedures. Because transesophageal echocardiography and fluoroscopy are displayed in different visual perspectives, the interventional cardiologist must mentally reregister the images from the two modalities during the procedure. Echocardiography-fluoroscopy fusion (EFF) imaging displays the x-ray and ultrasound overlay images in the same visual perspective. This new technology allows for enhanced team communication, improved visual guidance, and more efficient navigation. The purpose of this review is to describe the EFF imaging technology, current uses of EFF imaging in congenital and structural heart disease, and future directions that will enhance this unique imaging technology to guide interventional procedures.
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http://dx.doi.org/10.1016/j.echo.2019.07.023DOI Listing
December 2019

Phase I/IIa Trial of Atorvastatin in Patients with Acute Kawasaki Disease with Coronary Artery Aneurysm.

J Pediatr 2019 12 24;215:107-117.e12. Epub 2019 Sep 24.

Kawasaki Disease Research Center, Department of Pediatrics, University of California San Diego, La Jolla, CA; Rady Children's Hospital-San Diego, San Diego, CA.

Objectives: To determine the safety, tolerability, pharmacokinetics, and immunomodulatory effects of a 6-week course of atorvastatin in patients with acute Kawasaki disease with coronary artery (CA) aneurysm (CAA).

Study Design: This was a Phase I/IIa 2-center dose-escalation study of atorvastatin (0.125-0.75 mg/kg/day) in 34 patients with Kawasaki disease (aged 2-17 years) with echocardiographic evidence of CAA. We measured levels of the brain metabolite 24(S)-hydroxycholesterol (24-OHC), serum lipids, acute-phase reactants, liver enzymes, and creatine phosphokinase; peripheral blood mononuclear cell populations; and CA internal diameter normalized for body surface area before atorvastatin treatment and at 2 and 6 weeks after initiation of atorvastatin treatment.

Results: A 6-week course of up to 0.75 mg/kg/day of atorvastatin was well tolerated by the 34 subjects (median age, 5.3 years; IQR, 2.6-6.4 years), with no serious adverse events attributable to the study drug. The areas under the curve for atorvastatin and its metabolite were larger in the study subjects compared with those reported in adults, suggesting a slower rate of metabolism in children. The 24-OHC levels were similar between the atorvastatin-treated subjects and matched controls.

Conclusions: Atorvastatin was safe and well tolerated in our cohort of children with acute Kawasaki disease and CAA. A Phase III efficacy trial is warranted in this patient population, which may benefit from the known anti-inflammatory and immunomodulatory effects of this drug.
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http://dx.doi.org/10.1016/j.jpeds.2019.07.064DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6878161PMC
December 2019

Transesophageal 3-Dimensional Echocardiographic Guidance for Pacemaker Lead Placement.

JACC Case Rep 2019 Oct 4;1(3):391-393. Epub 2019 Sep 4.

Division of Cardiology, Heart Institute, Children's Hospital Colorado, University of Colorado, Aurora, Colorado.

The feasibility of transesophageal 3-dimensional echocardiography to reduce fluoroscopy in pacemaker lead placement has not yet been evaluated. This clinical vignette demonstrates the ability of 3-dimensional echocardiography to visualize intracardiac anatomy and the pacemaker lead and to guide positioning of the lead into the right atrial appendage, thus reducing fluoroscopy by nearly 50%. ().
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http://dx.doi.org/10.1016/j.jaccas.2019.06.028DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8288660PMC
October 2019

Diagnostic and Treatment Trends in Children With Kawasaki Disease in the United States, 2006-2015.

Pediatr Infect Dis J 2019 10;38(10):1010-1014

From the Department of Pediatrics, University of Colorado School of Medicine.

Objective: To evaluate variations in treatment practice and compliance with national guidelines for the diagnostic evaluation of children with Kawasaki disease (KD).

Study Design: We used the Pediatric Hospital Information System database to analyze demographic, laboratory and treatment data from patients admitted with KD between January 1, 2006, and December 31, 2015.

Results: During the study period, 12,089 children with KD were diagnosed. Nearly all patients had a complete blood cell count, erythrocyte sedimentation rate, and C-reactive protein ordered. Fewer patients had alanine aminotransferase (48.6%) or a urinalysis (75.3%). A small percentage of children had abdominal imaging (11.5%), neck imaging (5.9%), and lumbar punctures (4.5%), and 36.0% of patients received antibiotic therapy. Obtaining echocardiograms pretreatment and the use of steroids and infliximab significantly increased over the study period (P < 0.001). For patients who failed initial intravenous immunoglobulin (IVIG) monotherapy, 82.0% received a second dose of IVIG, 7.7% received steroids, 6.5% received infliximab, and 3.9% received combination therapy. Patients receiving infliximab or steroids as second therapy had a higher response rate than those who received only a second IVIG dose (87.9% versus 83.0% versus 73.3%, P < 0.001).

Conclusions: KD remains a challenging diagnosis. Opportunities exist for earlier use of echocardiograms in the evaluation of children with potential KD. Significant variations in practice exist surrounding second-line therapy. Our data suggest superiority of second-line therapy use of infliximab or steroids over IVIG in terms of reducing need for additional therapies. Prospective, controlled studies are needed to confirm this finding.
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http://dx.doi.org/10.1097/INF.0000000000002422DOI Listing
October 2019

Aortic Coarctation: A Comprehensive Analysis of Shape, Size, and Contractility of the Fetal Heart.

Fetal Diagn Ther 2020 27;47(5):429-439. Epub 2019 May 27.

Division of Cardiology, Department of Pediatrics, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado, USA.

Background: An integrated assessment of the size and shape of the 4-chamber view (4-CV) and right and left ventricles (RV and LV) as well as the function of the RV and LV in fetuses with coarctation of the aorta (CoA) has not yet been conducted.

Objectives: We evaluated the size and shape of the 4-CV, RV, and LV, and function of the RV and LV, to identify a profile for fetuses with CoA when compared to a control population.

Methods: 50 CoA fetuses were compared to 200 controls. This was a retrospective case series comparing the 4-CV of CoA fetuses and controls. The 4-CV end-diastolic area, length, width, and sphericity index were measured to determine the configuration of the 4-CV. Speckle-tracking analysis was used to compute the RV and LV end-diastolic area, length, 24-segment sphericity index, 24-segment transverse width, and the following functional parameters: (1) fractional area change; (2) global, lateral, and septal strain; (3) basal-apical, lateral, and septal annular displacement and fractional shortening; and (4) 24-segment transverse width fractional shortening. Using 5 and 95% reference intervals, the CoA fetal measurements were classified; from these, the odds ratio was computed between the fetuses with CoA and the controls. p < 0.05 was considered significant.

Results: In fetuses with CoA, the 4-CV was spherical in shape, increased in area and width, and decreased in length. Abnormal CoA sphericity indices reflected a flatter LV and a more spherical RV. The LV area, length, and width, and RV length were decreased. The transverse width of the RV was increased. RV and LV global, longitudinal, and transverse contractility were depressed.

Conclusions: The results demonstrate previously unappreciated differences in the shape, size, and function of the heart in fetuses with CoA. These differences may assist examiners in identifying fetuses with CoA.
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http://dx.doi.org/10.1159/000500022DOI Listing
March 2021

Left ventricular outflow tract pseudoaneurysm occlusion with fusion of live 3‑dimensional transesophageal echocardiography and fluoroscopy.

Kardiol Pol 2019 06 25;77(6):647-648. Epub 2019 May 25.

Department of Cardiology, Colorado Children’s Hospital, Aurora, Colorado, United States

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http://dx.doi.org/10.33963/KP.14841DOI Listing
June 2019

Right atrial function in pediatric heart transplant patients by echocardiographic strain measurements.

Pediatr Transplant 2019 05 13;23(3):e13383. Epub 2019 Mar 13.

Division of Cardiology, Department of Pediatrics, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, Colorado.

Background: CAV is a major cause of mortality in PHTx patients. Research on echocardiographic indices to detect CAV focuses primarily on ventricular function and less is known about RAF. Thus, we primarily sought to evaluate RAF in PHTx patients with CAV. For secondary analysis, we compared RAF between PHTx patients and control patients and evaluated RAF with respect to rejection and surgical type.

Methods: We retrospectively evaluated echocardiography derived RA strain indices in recipients <18 years old and >1 year from time of transplant. The RA strain phases included, reservoir (εs), conduit (εe), pump (εa), and respective strain rate indices (SRs, SRe, SRa).

Results: There were 36 PHTx patients and 14 age-, sex-matched control patients. There was a significant reduction in εs, εe, SRs, and SRe (P < 0.001) in the PHTx patients when compared to controls. There was no difference between the CAV (+) and CAV (-) patients with respect to RAF indices. Furthermore, εs, εe, and SRe (P < 0.05) were lower in patients with acute rejection (n = 7) compared to those without (n = 26). Patients with a bi-atrial anastomosis (n = 14) had decreased εs, εa, SRs, SRa (P < 0.05), compared to bi-caval anastomosis (n = 24).

Conclusion: PHTx patients have decreased RAF compared to healthy children. RAF does not differentiate PHTx patients based on the presence of CAV. RAF is also decreased in PHTx patients with rejection and in those transplanted with a bi-atrial anastomosis.
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http://dx.doi.org/10.1111/petr.13383DOI Listing
May 2019

The Kawasaki Disease Comparative Effectiveness (KIDCARE) trial: A phase III, randomized trial of second intravenous immunoglobulin versus infliximab for resistant Kawasaki disease.

Contemp Clin Trials 2019 04 3;79:98-103. Epub 2019 Mar 3.

Intuit, San Diego, CA, United States.

Background: Although intravenous immunoglobulin (IVIG) is effective therapy for Kawasaki disease (KD), the most common cause of acquired heart disease in children, 10-20% of patients are IVIG-resistant and require additional therapy. This group has an increased risk of coronary artery aneurysms (CAA) and there has been no adequately powered, randomized clinical trial in a multi-ethnic population to determine the optimal therapy for IVIG-resistant patients.

Objectives: The primary outcome is duration of fever in IVIG-resistant patients randomized to treatment with either infliximab or a second IVIG infusion. Secondary outcomes include comparison of inflammatory markers, duration of hospitalization, and coronary artery outcome. An exploratory aim records parent-reported outcomes including signs, symptoms and treatment experience.

Methods: The KIDCARE trial is a 30-site randomized Phase III comparative effectiveness trial in KD patients with fever ≥36 h after the completion of their first IVIG treatment. Eligible patients will be randomized to receive either a second dose of IVIG (2 g/kg) or infliximab (10 mg/kg). Subjects with persistent or recrudescent fever at 24 h following completion of the first study treatment will cross-over to the other treatment arm. Subjects will exit the study after their first outpatient visit (5-18 days following last study treatment). The parent-reported outcomes, collected daily during hospitalization and at home, will be compared by study arm.

Conclusion: This trial will contribute to the management of IVIG-resistant patients by establishing the relative efficacy of a second dose of IVIG compared to infliximab and will provide data regarding the patient/parent experience of these treatments.
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http://dx.doi.org/10.1016/j.cct.2019.02.008DOI Listing
April 2019

Comprehensive Noninvasive Evaluation of Right Ventricle-Pulmonary Circulation Axis in Pediatric Patients with Pulmonary Hypertension.

Curr Treat Options Cardiovasc Med 2019 Feb 9;21(1). Epub 2019 Feb 9.

Pediatric Cardiology, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, B100, Aurora, CO, 80045, USA.

Purpose Of Review: The purpose of this review is to detail the advanced echocardiographic studies that evaluated outcomes in pediatric PH using these advanced imaging techniques: (1) right heart size and function (using 2D and 3D echocardiography), (2) myocardial mechanics, and (3) right ventricular (RV) to pulmonary arterial coupling.

Recent Findings: Advanced echocardiographic imaging tools in pediatric PH include RV/left ventricular ratio, 3D echocardiographic assessment of RV volumes and ejection fraction, right atrial and RV strain, and RV-pulmonary arterial coupling ratio that allow for evaluation of clinical outcomes in this patient population. The right atrial to RV axis provides insights to RV diastolic function that has been poorly understood in pediatrics. The RV-pulmonary arterial coupling ratio helps us understand the mechanics of the RV response to afterload. The advanced imaging tools provide clinical outcome evaluation in pediatric PH patients and provide insight into the RA-RV axis and the RV-pulmonary circulatory unit.
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http://dx.doi.org/10.1007/s11936-019-0710-yDOI Listing
February 2019

Echocardiography-Fluoroscopy Fusion Imaging for Guidance of Congenital and Structural Heart Disease Interventions.

JACC Cardiovasc Imaging 2019 07 16;12(7 Pt 1):1279-1282. Epub 2019 Jan 16.

Division of Cardiology, University of Colorado School of Medicine, Aurora, Colorado.

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http://dx.doi.org/10.1016/j.jcmg.2018.11.010DOI Listing
July 2019

Right Ventricular-Arterial Coupling Ratio Derived From 3-Dimensional Echocardiography Predicts Outcomes in Pediatric Pulmonary Hypertension.

Circ Cardiovasc Imaging 2019 12;12(1):e008176

Department of Pediatrics, Pediatric Cardiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora (P.-N.J., M.S., D.D.I.).

Background: Right ventricular (RV) function is an important determinant of outcomes in pulmonary hypertension (PH). RV-arterial coupling ratio using stroke volume (SV) to end-systolic volume (ESV) has been shown to be an independent predictor of outcome in adults with PH. SV/ESV has not been used in pediatrics to predict outcomes. We compared SV/ESV between pediatric patients with PH, controls, and among groups based on disease severity. We correlated SV/ESV to RV strain and evaluated SV/ESV as a predictor of outcomes in pediatric PH.

Methods: One hundred and twenty-five children with PH (8 years [3-12 years]) underwent 3-dimensional echocardiography from 2014 to 2017 and compared with 65 controls (9 years [7-13 years]). Offline analysis generated 3-dimensional end-diastolic volume, ESV, SV, and free-wall RV longitudinal strain. SV/ESV ratios were compared between patients with PH, controls, and disease severity. Correlations between SV/ESV to free-wall RV longitudinal strain were assessed using general linear mixed models. Cox proportional hazards analysis assessed the predictive ability of SV/ESV.

Results: Patients with PH had lower SV/ESV compared with controls (0.88±0.18 versus 1.24±0.23; P<0.0001). There were significant associations between SV/ESV to free-wall RV longitudinal strain (r=-0.53; P<0.001). SV/ESV emerged as a strong predictor of adverse clinical event (hazard ratio [CI], 0.52 [0.38-0.69] per 0.1 increase in SV/ESV; P<0.0001).

Conclusions: SV/ESV as a volume estimate of RV-arterial coupling ratio correlates with RV strain and is a strong predictor of adverse clinical events in pediatric PH.
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http://dx.doi.org/10.1161/CIRCIMAGING.118.008176DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6336104PMC
December 2019

Vascular anatomical considerations and clinical decision making during insertion of the Avalon Elite Dual Lumen single-site veno-venous ECMO cannula in children weighing less than 20 kg.

Perfusion 2019 05 11;34(4):267-271. Epub 2018 Dec 11.

4 Anesthesiology, Heart Institute, Children's Hospital Colorado, Anschutz Medical Campus, University of Colorado School of Medicine, Aurora, CO, USA.

The correct selection and placement of a single-site Avalon Elite Dual Lumen Cannula for veno-venous extracorporeal membrane oxygenation (ECMO) in children weighing less than 20 kg is dependent on both the mechanical characteristics of the catheter, including length and diameter, as well as the unique vascular anatomic considerations of the patient. This manuscript describes the development of a clinical strategy, over a four-year period from 2012-2016, for cannula selection to reduce the risk of migration and malposition in 20 critically ill children weighing less than 20 kg who presented for veno-venous ECMO.
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http://dx.doi.org/10.1177/0267659118815104DOI Listing
May 2019
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