Browsing by Author "Commean, Paul"

Now showing 1 - 2 of 2
  • Thumbnail Image
    Item
    Cardiac Imaging and Biomarkers for Assessing Myocardial Fibrosis in Children with Hypertrophic Cardiomyopathy
    (Elsevier, 2023) Kirmani, Sonya; Woodard, Pamela K.; Shi, Ling; Hamza, Taye H.; Canter, Charles E.; Colan, Steven D.; Pahl, Elfriede; Towbin, Jeffrey A.; Webber, Steven A.; Rossano, Joseph W.; Everitt, Melanie D.; Molina, Kimberly M.; Kantor, Paul F.; Jefferies, John L.; Feingold, Brian; Addonizio, Linda J.; Ware, Stephanie M.; Chung, Wendy K.; Ballweg, Jean A.; Lee, Teresa M.; Bansal, Neha; Razoky, Hiedy; Czachor, Jason; Lunze, Fatima I.; Marcus, Edward; Commean, Paul; Wilkinson, James D.; Lipshultz, Steven E.; Pediatrics, School of Medicine
    Background: Myocardial fibrosis, as diagnosed on cardiac magnetic resonance imaging (cMRI) by late gadolinium enhancement (LGE), is associated with adverse outcomes in adults with hypertrophic cardiomyopathy (HCM), but its prevalence and magnitude in children with HCM have not been established. We investigated: (1) the prevalence and extent of myocardial fibrosis as detected by LGE cMRI; (2) the agreement between echocardiographic and cMRI measurements of cardiac structure; and (3) whether serum concentrations of N-terminal pro hormone B-type natriuretic peptide (NT-proBNP) and cardiac troponin-T are associated with cMRI measurements. Methods: A cross-section of children with HCM from 9 tertiary-care pediatric heart centers in the U.S. and Canada were enrolled in this prospective NHLBI study of cardiac biomarkers in pediatric cardiomyopathy (ClinicalTrials.gov Identifier: NCT01873976). The median age of the 67 participants was 13.8 years (range 1-18 years). Core laboratories analyzed echocardiographic and cMRI measurements, and serum biomarker concentrations. Results: In 52 children with non-obstructive HCM undergoing cMRI, overall low levels of myocardial fibrosis with LGE >2% of left ventricular (LV) mass were detected in 37 (71%) (median %LGE, 9.0%; IQR: 6.0%, 13.0%; range, 0% to 57%). Echocardiographic and cMRI measurements of LV dimensions, LV mass, and interventricular septal thickness showed good agreement using the Bland-Altman method. NT-proBNP concentrations were strongly and positively associated with LV mass and interventricular septal thickness (P < .001), but not LGE. Conclusions: Low levels of myocardial fibrosis are common in pediatric patients with HCM seen at referral centers. Longitudinal studies of myocardial fibrosis and serum biomarkers are warranted to determine their predictive value for adverse outcomes in pediatric patients with HCM.
  • Thumbnail Image
    Item
    Extracorporeal photopheresis to attenuate decline in lung function due to refractory obstructive allograft dysfunction
    (Wiley, 2021) EPI Study Group; Hage, Chadi A.; Klesney-Tait, Julia; Wille, Keith; Arcasoy, Selim; Yung, Gordon; Hertz, Marshall; Chan, Kevin M.; Morrell, Matt; Goldberg, Hilary; Vedantham, Suresh; Derfler, Mary Clare; Commean, Paul; Berman, Keith; Spitznagel, Ed; Atkinson, Jeff; Despotis, George; Medicine, School of Medicine
    Background: This study was designed to prospectively evaluate the efficacy of extracorporeal photopheresis (ECP) to attenuate the rate of decline of FEV1 in lung transplant recipients with refractory bronchiolitis obliterans. Due to an observed higher than expected early mortality, a preliminary analysis was performed. Study design and methods: Subjects from 10 lung transplant centres were assigned to ECP treatment or to observation based on spirometric criteria, with potential crossover for those under observation. The primary endpoint of this study was to assess response to ECP (i.e., greater than a 50% decrease in the rate of FEV1 decline) before and 6 months after initiation of ECP. Mortality was also evaluated 6 and 12 months after enrolment as a secondary endpoint. Results: Of 44 enrolled subjects, 31 were assigned to ECP treatment while 13 were initially assigned to observation on a non-random basis using specific spirometric inclusion criteria (seven of the observation patients subsequently crossed over to receive ECP). Of evaluable patients, 95% of patients initially assigned to treatment responded to ECP with rates of FEV1 decline that were reduced by 93% in evaluable ECP-treated patients. Mortality rates (percentages) at 6 and 12 months after enrolment was 32% and 41%, respectively. The most common (92%) primary cause of death was respiratory or graft failure. Significantly (p = 0.002) higher rates of FEV1 decline were observed in the non-survivors (-212 ± 177 ml/month) when compared to the survivors (-95 ± 117 ml/month) 12 months after enrolment. In addition, 18 patients with bronchiolitis obliterans syndrome (BOS) diagnosis within 6 months of enrolment had lost 38% of their baseline lung function at BOS diagnosis and 50% of their lung function at enrolment. Conclusions: These analyses suggest that earlier detection and treatment of BOS should be considered to appreciate improved outcomes with ECP.