Browsing by Subject "Adult congenital heart disease"
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Item COVID-19-Related Thrombotic and Bleeding Events in Adults With Congenital Heart Disease(Elsevier, 2023-11-21) Fusco, Flavia; Krasuski, Richard A.; Sadeghi, Soraya; Rosenbaum, Marlon S.; Lewis, Matthew J.; Carazo, Matthew R.; Rodriguez, Fred H.; Halpern, Dan G.; Feinberg, Jodi L.; Galilea, Francisca A.; Baraona, Fernando; Cedars, Ari M.; Ko, Jong M.; Porayette, Prashob; Maldonado, Jennifer R.; Frogoudaki, Alexandra A.; Nir, Amiram; Chaudhry, Anisa; John, Anitha S.; Karbassi, Arsha; Ganame, Javier; Hoskoppal, Arvind; Frischhertz, Benjamin P.; Hendrickson, Benjamin; Rodriguez-Monserrate, Carla P.; Broda, Christopher R.; Tobler, Daniel; Gregg, David; Martinez-Quintana, Efrén; Yeung, Elizabeth; Krieger, Eric V.; Ruperti-Repilado, Francisco J.; Giannakoulas, George; Lui, George K.; Ephrem, Georges; Singh, Harsimran S.; Hasan, Almeneisi; Bartlett, Heather L.; Lindsay, Ian; Grewal, Jasmine; Nicolarsen, Jeremy; Araujo, John J.; Cramer, Jonathan W.; Bouchardy, Judith; Al Najashi, Khalid; Ryan, Kristi; Alshawabkeh, Laith; Andrade, Lauren; Ladouceur, Magalie; Schwerzmann, Markus; Greutmann, Matthias; Merás, Pablo; Ferrero, Paolo; Dehghani, Payam; Tung, Poyee P.; Garcia-Orta, Rocio; Tompkins, Rose; Gendi, Salwa M.; Cohen, Scott; Klewer, Scott E.; Hascoet, Sebastien; Upadhyay, Shailendra; Fisher, Stacy D.; Cook, Stephen; Cotts, Timothy B.; Kovacs, Adrienne H.; Aboulhosn, Jamil A.; Scognamiglio, Giancarlo; Broberg, Craig S.; Sarubbi, Berardo; Medicine, School of MedicineBackground: Altered coagulation is a striking feature of COVID-19. Adult patients with congenital heart disease (ACHD) are prone to thromboembolic (TE) and bleeding complications. Objectives: The purpose of this study was to investigate the prevalence and risk factors for COVID-19 TE/bleeding complications in ACHD patients. Methods: COVID-19-positive ACHD patients were included between May 2020 and November 2021. TE events included ischemic cerebrovascular accident, systemic and pulmonary embolism, deep venous thrombosis, myocardial infarction, and intracardiac thrombosis. Major bleeding included cases with hemoglobin drop >2 g/dl, involvement of critical sites, or fatal bleeding. Severe infection was defined as need for intensive care unit, endotracheal intubation, renal replacement therapy, extracorporeal membrane oxygenation, or death. Patients with TE/bleeding were compared to those without events. Factors associated with TE/bleeding were determined using logistic regression. Results: Of 1,988 patients (age 32 [IQR: 25-42] years, 47% male, 59 ACHD centers), 30 (1.5%) had significant TE/bleeding: 12 TE events, 12 major bleeds, and 6 with both TE and bleeding. Patients with TE/bleeding had higher in-hospital mortality compared to the remainder cohort (33% vs 1.7%; P < 0.0001) and were in more advanced physiological stage (P = 0.032) and NYHA functional class (P = 0.01), had lower baseline oxygen saturation (P = 0.0001), and more frequently had a history of atrial arrhythmia (P < 0.0001), previous hospitalization for heart failure (P < 0.0007), and were more likely hospitalized for COVID-19 (P < 0.0001). By multivariable logistic regression, prior anticoagulation (OR: 4.92; 95% CI: 2-11.76; P = 0.0003), cardiac injury (OR: 5.34; 95% CI: 1.98-14.76; P = 0.0009), and severe COVID-19 (OR: 17.39; 95% CI: 6.67-45.32; P < 0.0001) were independently associated with increased risk of TE/bleeding complications. Conclusions: ACHD patients with TE/bleeding during COVID-19 infection have a higher in-hospital mortality from the illness. Risk of coagulation disorders is related to severe COVID-19, cardiac injury during infection, and use of anticoagulants.Item 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 MedicineDramatic 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.Item Heart Failure and Patient-Reported Outcomes in Adults With Congenital Heart Disease from 15 Countries(American Heart Association, 2022) Lu, Chun-Wei; Wang, Jou-Kou; Yang, Hsiao-Ling; Kovacs, Adrienne H.; Luyckx, Koen; Ruperti-Repilado, Francisco Javier; Van De Bruaene, Alexander; Enomoto, Junko; Sluman, Maayke A.; Jackson, Jamie L.; Khairy, Paul; Cook, Stephen C.; Chidambarathanu, Shanthi; Alday, Luis; Oechslin, Erwin; Eriksen, Katrine; Dellborg, Mikael; Berghammer, Malin; Johansson, Bengt; Mackie, Andrew S.; Menahem, Samuel; Caruana, Maryanne; Veldtman, Gruschen; Soufi, Alexandra; Fernandes, Susan M.; White, Kamila; Callus, Edward; Kutty, Shelby; Apers, Silke; Moons, Philip; APPROACH-IS consortium, the International Society for Adult Congenital Heart Disease (ISACHD); Medicine, School of MedicineBackground: Heart failure (HF) is the leading cause of mortality and associated with significant morbidity in adults with congenital heart disease. We sought to assess the association between HF and patient‐report outcomes in adults with congenital heart disease. Methods and Results: As part of the APPROACH‐IS (Assessment of Patterns of Patient‐Reported Outcomes in Adults with Congenital Heart disease—International Study), we collected data on HF status and patient‐reported outcomes in 3959 patients from 15 countries across 5 continents. Patient‐report outcomes were: perceived health status (12‐item Short Form Health Survey), quality of life (Linear Analogue Scale and Satisfaction with Life Scale), sense of coherence‐13, psychological distress (Hospital Anxiety and Depression Scale), and illness perception (Brief Illness Perception Questionnaire). In this sample, 137 (3.5%) had HF at the time of investigation, 298 (7.5%) had a history of HF, and 3524 (89.0%) had no current or past episode of HF. Patients with current or past HF were older and had a higher prevalence of complex congenital heart disease, arrhythmias, implantable cardioverter‐defibrillators, other clinical comorbidities, and mood disorders than those who never had HF. Patients with HF had worse physical functioning, mental functioning, quality of life, satisfaction with life, sense of coherence, depressive symptoms, and illness perception scores. Magnitudes of differences were large for physical functioning and illness perception and moderate for mental functioning, quality of life, and depressive symptoms. Conclusions: HF in adults with congenital heart disease is associated with poorer patient‐reported outcomes, with large effect sizes for physical functioning and illness perception.Item Isolated Coarctation of the Aorta: Current Concepts and Perspectives(Frontiers Media, 2022-05-25) Bhatt, Ami B.; Lantin-Hermoso, Maria R.; Daniels, Curt J.; Jaquiss, Robert; Landis, Benjamin John; Marino, Bradley S.; Rathod, Rahul H.; Vincent, Robert N.; Keller, Bradley B.; Villafane, Juan; Pediatrics, School of MedicineCurrent management of isolated CoA, localized narrowing of the aortic arch in the absence of other congenital heart disease, is a success story with improved prenatal diagnosis, high survival and improved understanding of long-term complication. Isolated CoA has heterogenous presentations, complex etiologic mechanisms, and progressive pathophysiologic changes that influence outcome. End-to-end or extended end-to-end anastomosis are the favored surgical approaches for isolated CoA in infants and transcatheter intervention is favored for children and adults. Primary stent placement is the procedure of choice in larger children and adults. Most adults with treated isolated CoA thrive, have normal daily activities, and undergo successful childbirth. Fetal echocardiography is the cornerstone of prenatal counseling and genetic testing is recommended. Advanced 3D imaging identifies aortic complications and myocardial dysfunction and guides individualized therapies including re-intervention. Adult CHD program enrollment is recommended. Longer follow-up data are needed to determine the frequency and severity of aneurysm formation, myocardial dysfunction, and whether childhood lifestyle modifications reduce late-onset complications.Item Kidney disease and congenital heart disease: Partnership for life(Frontiers Media, 2022-08-19) El Sayegh, Skye; Ephrem, Georges; Wish, Jay B.; Moe, Sharon; Lim, Kenneth; Medicine, School of MedicineThe literature on the relationship between kidney and cardiovascular diseases is continuously expanding. Scientists have elucidated many of the neurohormonal and hemodynamic pathways involved in cardiorenal disease. However, little is known about kidney disease in patients with congenital heart disease. Given advances in the medical and surgical care of this highly complex patient population, survival rates have dramatically improved leading to a higher percentage of adults living with congenital heart disease. Accordingly, a noticeable increase in the prevalence of kidney disease is appreciated in these patients. Some of the main risk factors for developing chronic kidney disease in the adult congenital heart disease population include chronic hypoxia, neurohormonal derangements, intraglomerular hemodynamic changes, prior cardiac surgeries from minimally invasive to open heart surgeries with ischemia, and nephrotoxins. Unfortunately, data regarding the prevalence, pathophysiology, and prognosis of chronic kidney disease in the adult congenital heart disease population remain scarce. This has led to a lack of clear recommendations for evaluating and managing kidney disease in these patients. In this review, we discuss contemporary data on kidney disease in adults with congenital heart disease in addition to some of the gaps in knowledge we face. The article highlights the delicate interaction between disease of the heart and kidneys in these patients, and offers the practitioner tools to more effectively manage this vulnerable population.Item Pain in adults with congenital heart disease - An international perspective(Elsevier, 2021) Leibold, Allison; Eichlera, Erin; Chung, Sukyung; Moons, Philip; Kovacs, Adrienne H.; Luyckx, Koen; Apers, Silke; Thomet, Corina; Budts, Werner; Enomoto, Junko; Sluman, Maayke A.; Wang, Jou-Kou; Jackson, Jamie L.; Khairy, Paul; Cook, Stephen C.; Chidambarathanu, Shanthi; Alday, Luis; Oechslin, Erwin; Eriksen, Katrine; Dellborg, Mikael; Berghammer, Malin; Johansson, Bengt; Mackie, Andrew S.; Menahem, Samuel; Caruana, Maryanne; Veldtman, Gruschen; Soufi, Alexandra; White, Kamila; Callus, Edward; Kutty, Shelby; Fernandes, Susan M.; APPROACH-IS consortium; International Society for Adult Congenital Heart Disease (ISACHD); Medicine, School of MedicineBackground: Patients with adult congenital heart disease (ACHD) have many risk factors for chronic pain such as prior cardiac interventions and adult comorbidities. However, the prevalence of chronic pain has not been well described in this population. We sought to determine the prevalence of pain in a large international cohort of patients with ACHD. Methods: Data from the APPROACH-IS dataset was utilized for this study which includes 4028 patients with ACHD from 15 different countries. The prevalence of pain was assessed under the health status patient reported outcome domain utilizing the EuroQol-5D 3 level version tool. Multivariable logistic regression was used to assess differences across countries in pain, taking into account country-level random effects for clustering across observations within each country. Results: A total of 3832 patients with ACHD met the study criteria, median age 32 years [IQR 25, 42], 52.6% females. The prevalence of at least moderate pain was reported by 28.9% (95% CO 27.5 = 30.3%) of participants. Pain was associated with country of origin, age, gender, background, education and marital status as well as several clinical variables including disease complexity, cardiac device presence, history of heart failure, psychiatric conditions and presence of other medical conditions. Those with pain had lower levels of perceived health and a lower quality of life score. Conclusion: Pain in patients with ACHD is common, impacting nearly one-third of patients. Given the far reaching implications of pain in patients with ACHD, further study of pain characteristics and treatment management appear warranted.Item Permanent His-bundle Pacing in Pediatrics and Congenital Heart Disease(MediaSphere Medical LLC, 2020-02-15) Lyon, Shannon; Dandamudi, Gopi; Kean, Adam C.; Pediatrics, School of MedicinePermanent His-bundle pacing has been gaining popularity in the adult population requiring cardiac resynchronization therapy. Initial procedural challenges are being overcome, and this method of pacing has been shown to improve left ventricular function and heart failure symptoms secondary to ventricular dyssynchrony. Though the etiologies of ventricular dyssynchrony may differ in children and those with congenital heart disease than in adults with structurally normal hearts, His-bundle pacing may also be a preferred option in these groups to restore more physiologic electric conduction and improve ventricular function. We present a review of the current literature and suggested directions involving deploying permanent His-bundle pacing in the pediatric and congenital heart disease population.Item Preprocedural Anxiety in Adults With Congenital Heart Disease: The PANIC Study(Elsevier, 2023-09-01) Cook, Stephen C.; Saidi, Arwa; Singh, Harsimran S.; Madder, Ryan D.; Cohen, Scott B.; Van Oosterhout, Stacie; Samuel, Bennet P.; Finn, Michael T. M.; Medicine, School of MedicineBackground: Preprocedural anxiety may have detrimental effects both cognitively and physiologically. Objectives: The objective of this study was to determine the association between state (situational) and trait (persistent in everyday life) anxiety and differences between the adult congenital heart disease (ACHD) and acquired heart disease populations. Methods: The State-Trait Anxiety Inventory and financial stress scale were administered to adults with acquired and CHD at 4 tertiary referral centers in the United States prior to cardiac catheterization. Student's t-test and least absolute shrinkage and selection operator regression analyses were used to assess differences in anxiety between groups and identify the optimal model of predictors of anxiety. Results: Of the 291 patients enrolled, those with CHD (n = 91) were younger (age 41.3 ± 16.3 years vs 64.7 ± 11.3 years, P < 0.001), underwent more cardiac surgeries (P < 0.001), and had higher levels of trait anxiety (t[171] = 2.62, P = 0.001, d = 0.33). There was no difference in state anxiety between groups (t[158.65] = 1.37, P = 0.17, d = 0.18). State anxiety was singularly associated with trait anxiety. Trait anxiety was negatively associated with age and positively associated with state anxiety and financial stress. Patients with CHD of great complexity were more trait (F[2,88] = 4.21, P = 0.02) and state anxious (F[2,87] = 4.59, P = 0.01), though with relatively small effect size. Conclusions: Trait anxiety levels are higher in the ACHD population and directly associated with state anxiety. Specialists caring for ACHD patients should not only recognize the frequency of trait anxiety but also high-risk subgroups that may benefit from psychological or social interventions to reduce preprocedural anxiety.