Browsing by Author "Ermis, Peter"

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    Design and Harmonization Approach for the Multi-Institutional Neurocognitive Discovery Study (MINDS) of Adult Congenital Heart Disease (ACHD) Neuroimaging Ancillary Study: A Technical Note
    (MDPI, 2023-09-06) Panigrahy, Ashok; Schmithorst, Vanessa; Ceschin, Rafael; Lee, Vince; Beluk, Nancy; Wallace, Julia; Wheaton, Olivia; Chenevert, Thomas; Qiu, Deqiang; Lee, James N.; Nencka, Andrew; Gagoski, Borjan; Berman, Jeffrey I.; Yuan, Weihong; Macgowan, Christopher; Coatsworth, James; Fleysher, Lazar; Cannistraci, Christopher; Sleeper, Lynn A.; Hoskoppal, Arvind; Silversides, Candice; Radhakrishnan, Rupa; Markham, Larry; Rhodes, John F.; Dugan, Lauryn M.; Brown, Nicole; Ermis, Peter; Fuller, Stephanie; Cotts, Timothy Brett; Rodriguez, Fred Henry; Lindsay, Ian; Beers, Sue; Aizenstein, Howard; Bellinger, David C.; Newburger, Jane W.; Glass Umfleet, Laura; Cohen, Scott; Zaidi, Ali; Gurvitz, Michelle; Pediatric Heart Network MINDS Neuroimaging Ancillary Study Investigators; Radiology and Imaging Sciences, School of Medicine
    Dramatic advances in the management of congenital heart disease (CHD) have improved survival to adulthood from less than 10% in the 1960s to over 90% in the current era, such that adult CHD (ACHD) patients now outnumber their pediatric counterparts. ACHD patients demonstrate domain-specific neurocognitive deficits associated with reduced quality of life that include deficits in educational attainment and social interaction. Our hypothesis is that ACHD patients exhibit vascular brain injury and structural/physiological brain alterations that are predictive of specific neurocognitive deficits modified by behavioral and environmental enrichment proxies of cognitive reserve (e.g., level of education and lifestyle/social habits). This technical note describes an ancillary study to the National Heart, Lung, and Blood Institute (NHLBI)-funded Pediatric Heart Network (PHN) “Multi-Institutional Neurocognitive Discovery Study (MINDS) in Adult Congenital Heart Disease (ACHD)”. Leveraging clinical, neuropsychological, and biospecimen data from the parent study, our study will provide structural–physiological correlates of neurocognitive outcomes, representing the first multi-center neuroimaging initiative to be performed in ACHD patients. Limitations of the study include recruitment challenges inherent to an ancillary study, implantable cardiac devices, and harmonization of neuroimaging biomarkers. Results from this research will help shape the care of ACHD patients and further our understanding of the interplay between brain injury and cognitive reserve.
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    The Effect of Udenafil on Heart Rate and Blood Pressure in Adolescents With the Fontan Circulation
    (Elsevier, 2024) Edelson, Jonathan B.; Zak, Victor; Goldberg, David; Fleming, Greg; Mackie, Andrew S.; Patel, Jyoti K.; Files, Matthew; Downing, Tacy; Richmond, Marc; Acheampong, Ben; Cartoski, Mark; Detterich, Jon; McCrindle, Brian; McHugh, Kimberly; Hansen, Jesse E.; Wagner, Jonathan; Di Maria, Michael; Weingarten, Angela; Nowlen, Todd; Yoon, Ja Kyoung; Kim, Gi Beom; Williams, Richard; Whitehill, Robert; Kirkpatrick, Edward; Yin, Suellen; Ermis, Peter; Lubert, Adam M.; Stylianou, Mario; Freemon, D'Andrea; Hu, Chenwei; Garuba, Olukayode D.; Frommelt, Peter; Goldstein, Bryan H.; Paridon, Stephen; Garg, Ruchira; Pediatric Heart Network Investigators; Pediatrics, School of Medicine
    The Fontan Udenafil Exercise Longitudinal (FUEL) trial showed that treatment with udenafil was associated with improved exercise performance at the ventilatory anaerobic threshold in children with Fontan physiology. However, it is not known how the initiation of phosphodiesterase 5 inhibitor therapy affects heart rate and blood pressure in this population. These data may help inform patient selection and monitoring after the initiation of udenafil therapy. The purpose of this study is to evaluate the effects of udenafil on vital signs in the cohort of patients enrolled in the FUEL trial. This international, multicenter, randomized, double-blind, placebo-controlled trial of udenafil included adolescents with single ventricle congenital heart disease who had undergone Fontan palliation. Changes in vital signs (heart rate [HR], systolic [SBP] and diastolic blood pressure [DBP]) were compared both to subject baseline and between the treatment and the placebo groups. Additional exploratory analyses were performed to evaluate changes in vital signs for prespecified subpopulations believed to be most sensitive to udenafil initiation. Baseline characteristics were similar between the treatment and placebo cohorts (n = 200 for each). The groups demonstrated a decrease in HR, SBP, and DBP 2 hours after drug/placebo administration, except SBP in the placebo group. There was an increase in SBP from baseline to after 6-min walk test in the treatment and placebo groups, and the treatment group showed an increase in HR (87.4 ± 15.0 to 93.1 ± 19.4 beats/min, p <0.01) after exercise. When comparing changes from baseline to the 26-week study visit, small decreases in both SBP (-1.9 ± 12.3 mm Hg, p = 0.03) and DBP (-3.0 ± 9.6 mm Hg, p <0.01) were seen in the treatment group. There were no clinically significant differences between treatment and placebo group in change in HR or blood pressure in the youngest age quartile, lightest weight quartile, or those on afterload-reducing agents. In conclusion, initiation of treatment with udenafil in patients with Fontan circulation was not associated with clinically significant changes in vital signs, implying that for patients similar to those enrolled in the FUEL trial, udenafil can be started without the requirement for additional monitoring after initial administration.