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      Risk Factors for Major Early Adverse Events Related to Cardiac Catheterization in Children and Young Adults With Pulmonary Hypertension: An Analysis of Data From the IMPACT (Improving Adult and Congenital Treatment) Registry


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          Cardiac catheterization is the gold standard for assessment and follow‐up of patients with pulmonary hypertension ( PH). To date, there are limited data about the factors that influence the risk of catastrophic adverse events after catheterization in this population.

          Methods and Results

          A retrospective multicenter cohort study was performed to measure risk of catastrophic adverse outcomes after catheterization in children and young adults with PH and identify risk factors for these outcomes. All catheterizations in children and young adults, aged 0 to 21 years, with PH at hospitals submitting data to the IMPACT (Improving Adult and Congenital Treatment) registry between January 1, 2011, and December 31, 2015, were studied. Using mixed‐effects multivariable regression, we assessed the association between prespecified subject‐, procedure‐, and center‐level covariates and the risk of death, cardiac arrest, or mechanical circulatory support during or after cardiac catheterization. A total of 8111 procedures performed in 7729 subjects at 77 centers were studied. The observed risk of the composite outcome was 1.4%, and the risk of death before discharge was 5.2%. Catheterization in prematurely born neonates and nonpremature infants was associated with increased risk of catastrophic adverse event, as was precatheterization treatment with inotropes and lower systemic arterial saturation. Secondary analyses demonstrated the following: (1) increasing volumes of catheterization in patients with PH were associated with reduced risk of composite outcome (odds ratio, 0.8 per 10 procedures; P=0.002) and (2) increasing pulmonary vascular resistance and pulmonary artery pressures were associated with increased risk ( P<0.0001 for both).


          Young patients with PH are a high‐risk population for diagnostic and interventional cardiac catheterization. Hospital experience with PH is associated with reduced risk, independent of total catheterization case volume.

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          Most cited references27

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          The National Cardiovascular Data Registry (NCDR) Data Quality Brief: the NCDR Data Quality Program in 2012.

          The National Cardiovascular Data Registry (NCDR) developed the Data Quality Program to meet the objectives of ensuring the completeness, consistency, and accuracy of data submitted to the observational clinical registries. The Data Quality Program consists of 3 main components: 1) a data quality report; 2) a set of internal quality assurance protocols; and 3) a yearly data audit program. Since its inception in 1997, the NCDR has been the basis for the development of performance and quality metrics, site-level quality improvement programs, and peer-reviewed health outcomes research. Before inclusion in the registry, data are filtered through the registry-specific algorithms that require predetermined levels of completeness and consistency for submitted data fields as part of the data quality report. Internal quality assurance protocols enforce data standards before reporting. Within each registry, 300 to 625 records are audited annually in 25 randomly identified sites (i.e., 12 to 25 records per audited site). In the 2010 audits, the participant average raw accuracy of data abstraction for the CathPCI Registry, ICD Registry, and ACTION Registry-GWTG were, respectively, 93.1% (range, 89.4% minimum, 97.4% maximum), 91.2% (range, 83.7% minimum, 95.7% maximum), and 89.7.% (range, 85% minimum, 95% maximum). The 2010 audits provided evidence that many fields in the NCDR accurately represent the data from the medical charts. The American College of Cardiology Foundation is undertaking a series of initiatives aimed at creating a quality assurance rapid learning system, which, when complete, will monitor, evaluate, and improve data quality. Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
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            Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005.

            Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are unknown. This study describes the nationwide epidemiological features of pediatric PH in the Netherlands during a 15-year period and the clinical course of pediatric PAH. Two registries were used to retrospectively identify children (0-17 years) with PH. Overall, 3263 pediatric patients were identified with PH due to left heart disease (n=160; 5%), lung disease/hypoxemia (n=253; 8%), thromboembolic disease (n=5; <1%), and transient (n=2691; 82%) and progressive (n=154; 5%) PAH. Transient PAH included persistent PH of the newborn and children with congenital heart defects (CHD) and systemic-to-pulmonary shunt, in whom PAH resolved after successful shunt correction. Progressive PAH mainly included idiopathic PAH (n=36; iPAH) and PAH associated with CHD (n=111; PAH-CHD). Pulmonary arterial hypertension associated with CHD represented highly heterogeneous subgroups. Syndromes were frequently present, especially in progressive PAH (n=60; 39%). Survival for PAH-CHD varied depending on the subgroups, some showing better and others showing worse survival than for iPAH. Survival of children with Eisenmenger syndrome appeared worse than reported in adults. For iPAH and PAH-CHD, annual incidence and point prevalence averaged, respectively, 0.7 and 4.4 (iPAH) and 2.2 and 15.6 (PAH-CHD) cases per million children. Compared to studies in adults, iPAH occurred less whereas PAH-CHD occurred more frequently. Pediatric PH is characterized by various age-specific diagnoses, the majority of which comprise transient forms of PAH. Incidence of pediatric iPAH is lower whereas incidence of pediatric PAH-CHD is higher than reported in adults. Pediatric PAH-CHD represents a heterogeneous group with highly variable clinical courses.
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              Childhood idiopathic pulmonary arterial hypertension: a national cohort study.

              To clarify the clinical characteristics and epidemiology of idiopathic pulmonary arterial hypertension (IPAH) in childhood, a rare condition with a bad prognosis, poorly documented in children. Also, to describe the long-term outcome. A retrospective study of 7 years' experience. UK Service for Pulmonary Hypertension in Children based at a tertiary referral centre. 64 children. Patients were initially treated with prostanoids (n=15), bosentan (n=23), sildenafil (n=9), combination therapy (n=11) or calcium channel antagonists (n=6). WHO functional class, distance walked in 6 minutes, escalation of therapy, survival, transplant-free survival. Incidence of IPAH was 0.48 cases per million children per year and the prevalence was 2.1 cases per million. 31% presented with syncope. Oedema was rare. During the first year of follow-up WHO functional class and 6-minute walk distance improved significantly. Survival at 1, 3 and 5 years was 89%, 84% and 75%, respectively; while transplant-free survival was 89% 76% and 57%, respectively. Factors predicting worse survival were WHO functional class (HR 2.4, p=0.04) and poor height and weight z-score (p<0.05 for both) at presentation. We showed, for the first time, that the incidence of IPAH is lower in children than adults and that the clinical features can be different. Most children present with clinical evidence of advanced disease and clinical status at presentation is predictive of outcome. This 7-year experience confirms the significant improvement in survival over historical controls.

                Author and article information

                J Am Heart Assoc
                J Am Heart Assoc
                Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
                John Wiley and Sons Inc. (Hoboken )
                28 February 2018
                06 March 2018
                : 7
                : 5 ( doiID: 10.1002/jah3.2018.7.issue-5 )
                [ 1 ] Division of Cardiology and Center for Pediatric Clinical Effectiveness The Children's Hospital of Philadelphia PA
                [ 2 ] Department of Pediatrics Perelman School of Medicine at the University of Pennsylvania Philadelphia PA
                [ 3 ] Leonard Davis Institute at the University of Pennsylvania Philadelphia PA
                [ 4 ] Mid America Heart Institute, St Luke's Health System Kansas City MO
                [ 5 ] Division of Cardiology Children's National Health System Washington DC
                [ 6 ] Department of Pediatrics George Washington University School of Health Sciences Washington DC
                Author notes
                [*] [* ] Correspondence to: Michael L. O'Byrne, MD, MSCE, The Children's Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104. E‐mail: obyrnem@ 123456email.chop.edu
                © 2018 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

                This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial‐NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                Page count
                Figures: 3, Tables: 4, Pages: 12, Words: 8658
                Funded by: National Institutes of Health
                Funded by: National Heart, Lung, and Blood Institute
                Award ID: K23 HL130420‐01
                Funded by: American College of Cardiology
                Funded by: National Cardiovascular Data Registry
                Original Research
                Original Research
                Pediatric Cardiology
                Custom metadata
                06 March 2018
                Converter:WILEY_ML3GV2_TO_NLMPMC version:version= mode:remove_FC converted:06.03.2018


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