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https://www.readbyqxmd.com/read/29660764/prognostic-utility-of-meld-xi-in-adult-congenital-heart-disease-patients-undergoing-cardiac-transplantation
#1
Evan D Adams, Nicholas J Jackson, Tim Young, Eugene C DePasquale, Leigh C Reardon
BACKGROUND: MELD-XI (Model for End-Stage Liver Disease eXcluding INR) at cardiac transplant has demonstrated prognostic survival utility, but has not been specifically validated in adult congenital heart disease (ACHD) in a registry study. METHODS: Adults undergoing first time orthotopic heart transplant from 2005-2015 in the UNOS (United Network for Organ Sharing) registry were examined in parallel: ACHD (n=543), ischemic dilated cardiomyopathy (IDCM, n=6,954) and valvular heart disease (VHD, n=355)...
April 16, 2018: Clinical Transplantation
https://www.readbyqxmd.com/read/29603626/pulmonary-hypertension-barrier-or-just-a-bump-in-the-road-in-transplanting-adults-with-congenital-heart-disease
#2
Jonathan N Menachem, Edo Y Birati, Payman Zamani, Anjali T Owens, Pavan Atluri, Christian A Bermudez, David Drajpuch, Stephanie Fuller, Yuli Y Kim, Christopher E Mascio, Vikram Palanivel, J Eduardo Rame, Joyce Wald, Michael A Acker, Jeremy A Mazurek
BACKGROUND: Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Identification of disease progression and timing of referral for advanced therapies is often delayed. However, increased awareness and understanding of ACHD and improvements in the approach to treatment have led to improved outcomes. Pulmonary hypertension (PH) is a common barrier to HT. In ACHD, the approach to PH and HT is quite complicated, given the anatomic heterogeneity and lower prevalence and experience...
March 30, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29574895/-not-such-a-kid-thing-anymore-young-adults-perspectives-on-transfer-from-paediatric-to-adult-cardiology-care
#3
G Catena, G R Rempel, A H Kovacs, K N Rankin, I V Muhll, A S Mackie
BACKGROUND: Transfer of adolescents with congenital heart disease from paediatric cardiology providers to specialized adult congenital heart disease (ACHD) care providers is becoming a standard practice. However, some paediatric cardiologists continue to provide care for their patients into adult life. Little is known about the perspectives of young adult patients who have been transferred to ACHD clinics versus those who continue to receive their cardiology care in paediatric settings...
March 25, 2018: Child: Care, Health and Development
https://www.readbyqxmd.com/read/29561310/mechanical-circulatory-support-challenges-in-pediatric-and-adult-congenital-heart-disease
#4
Martin Schweiger, Angela Lorts, Jennifer Conway
PURPOSE OF REVIEW: Increased miniaturization of ventricular assist devices (VADs) and new mechanical support strategies (MCS) has increased the use of MCS in the pediatric and congenital heart disease (CHD) population. This comes with the need for care providers specialized in this field to determine optimal patient and device selection, and to improve outcomes and decrease complication rates for new innovative strategies. A review of the published literature in this field is timely and relevant...
March 20, 2018: Current Opinion in Organ Transplantation
https://www.readbyqxmd.com/read/29542247/functional-classification-of-heart-failure-before-and-after-implementing-a-healthcare-transition-program-for-youth-and-young-adults-transferring-from-a-pediatric-to-an-adult-congenital-heart-disease-clinics
#5
Albert C Hergenroeder, Douglas S Moodie, Daniel J Penny, Constance M Wiemann, Blanca Sanchez-Fournier, Lauren K Moore, Jane Head
OBJECTIVE: To describe changes in functional status between the last pediatric and first adult congenital heart disease (CHD) clinic visits in patients with moderate to severe CHD after implementing a healthcare transition (HCT) planning program. DESIGN: Quasi-experimental design. Patients were followed prospectively following the implementation of the intervention; Control patients transitioned from the Pediatric CHD Clinic into Adult CHD Clinic before the intervention...
March 15, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29541749/association-of-albuminuria-with-major-adverse-outcomes-in-adults-with-congenital-heart-disease-results-from-the-boston-adult-congenital-heart-biobank
#6
Saurabh Rajpal, Laith Alshawabkeh, Nureddin Almaddah, Caroline M Joyce, Keri Shafer, Michelle Gurvitz, Sushrut S Waikar, Finnian R Mc Causland, Michael J Landzberg, Alexander R Opotowsky
Importance: Albuminuria is associated with adverse outcomes in diverse groups of patients, but the importance of albuminuria in the emerging population of increasingly complex adults with congenital heart disease (ACHD) remains unknown. Objective: To assess the prevalence, risk factors, and prognostic implications of albuminuria in ACHD. Design, Setting, and Participants: This prospective study assessed a cohort of ambulatory patients aged 18 years and older who were examined at an ACHD referral center and enrolled in the Boston ACHD Biobank between May 17, 2012, to August 5, 2016...
April 1, 2018: JAMA Cardiology
https://www.readbyqxmd.com/read/29537971/current-state-of-risk-stratification-for-sudden-cardiac-death-in-adults-with-congenital-heart-disease
#7
Jim T Vehmeijer, Barbara Jm Mulder, Joris R de Groot
Sudden cardiac death (SCD), mainly caused by ventricular arrhythmias, is one of the leading causes of mortality in adult congenital heart disease (ACHD) patients. An implantable cardioverter defibrillator (ICD) may prevent SCD, but risk stratification remains challenging. In this review, we will address the current guideline recommendations for ICD implantation in ACHD patients, as well as review a recent study in which the discriminative ability for SCD of these guidelines is evaluated. In this study, the guideline recommendations were applied to patients who died of SCD and living controls...
March 13, 2018: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/29525133/long-term-follow-up-of-adults-following-the-atrial-switch-operation-for-transposition-of-the-great-arteries-a-contemporary-cohort
#8
Mark Dennis, Irina Kotchetkova, Rachael Cordina, David S Celermajer
BACKGROUND: The atrial switch operation for transposition of the Great Arteries (TGA) (Mustard or Senning Procedure) provides excellent short-term survival. Significant long-term concerns exist for these patients, however, including the ability of the right ventricle to maintain systemic perfusion and the risk of arrhythmia. We seek to describe long-term mortality and morbidity of this group of adult patients. METHODS: Consecutive patients who had undergone an atrial switch procedure, who were aged over 16 years and who were followed up at our tertiary level adult congenital heart disease (ACHD) service in Sydney, Australia since 2000 were included...
October 31, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/29525069/red-cell-distribution-width-in-adults-with-congenital-heart-disease-a-worldwide-available-and-low-cost-predictor-of-cardiovascular-events
#9
Vivan J M Baggen, Annemien E van den Bosch, Roland R van Kimmenade, Jannet A Eindhoven, Maarten Witsenburg, Judith A A E Cuypers, Frank W G Leebeek, Eric Boersma, Jolien W Roos-Hesselink
BACKGROUND: Red cell distribution width (RDW) is a standard component of the automated blood count, and is of prognostic value in heart failure and coronary heart disease. We investigated the association between RDW and cardiovascular events in patients with adult congenital heart disease (ACHD). METHODS AND RESULTS: In this prospective cohort study, 602 consecutive patients with ACHD who routinely visited the outpatient clinic were enrolled between 2011 and 2013...
March 2, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29506740/oral-anticoagulant-therapy-in-adults-with-congenital-heart-disease-and-atrial-arrhythmias-implementation-of-guidelines
#10
H Yang, J F Heidendael, J R de Groot, T C Konings, G Veen, A P J van Dijk, F J Meijboom, G Tj Sieswerda, M C Post, M M Winter, B J M Mulder, B J Bouma
BACKGROUND: Current guidelines on oral anticoagulation (OAC) in adults with congenital heart disease (ACHD) and atrial arrhythmias (AA) consist of heterogeneous and divergent recommendations with limited level of evidence, possibly leading to diverse OAC management and different outcomes. Therefore, we aimed to evaluate real-world implementation and outcome of three guidelines on OAC management in ACHD patients with AA. METHODS: The ESC GUCH 2010, PACES/HRS 2014 and ESC atrial fibrillation (AF) 2016 guidelines were assessed for implementation...
April 15, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29492392/overview-of-adult-congenital-heart-transplants
#11
Roosevelt Bryant, David Morales
Transplantation for adult patients with congenital heart disease (ACHD) is a growing clinical endeavor in the transplant community. Understanding the results and defining potential high-risk patient subsets will allow optimization of patient outcomes. This review summarizes the scope of ACHD transplantation, the mechanisms of late ventricular dysfunction, the ACHD population at risk of developing heart failure, the indications and potential contraindications for transplant, surgical considerations, and post-transplant outcomes...
January 2018: Annals of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29480975/insurance-access-in-adults-with-congenital-heart-disease-in-the-affordable-care-act-era
#12
Chien-Jung Lin, Eric Novak, Michael W Rich, Joseph J Billadello
BACKGROUND: Adults with congenital heart disease (ACHD) have traditionally been viewed as an underinsured population. Whether this is true in the Affordable Care Act era is unknown. We determined insurance patterns in ACHD patients compared to the non-ACHD cardiology population in a contemporary cohort. METHODS: All cardiology outpatient visits between July 2016 and February 2017 to a large referral center in the United States were reviewed. The primary payer was categorized as health maintenance organization (HMO), preferred provider organization (PPO), Medicare, Medicaid, self-pay, or other...
February 26, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29437602/adult-congenital-heart-disease-in-a-nationwide-population-2000-2014-epidemiological-trends-arrhythmia-and-standardized-mortality-ratio
#13
Mei-Hwan Wu, Chun-Wei Lu, Hui-Chi Chen, Feng-Yu Kao, San-Kuei Huang
BACKGROUND: The adult congenital heart disease (ACHD) population will grow with medical advances, but data are limited. We investigated the epidemiological profile of ACHD in Taiwan, a country with a congenital heart disease program since 1955, population of 23 million, and easily accessible high-standard medical care. METHODS AND RESULTS: ACHD patients, born after 1954, were identified from the nationwide database 2000-2014. The ACHD prevalence in the population aged 18 to 59 was 140...
February 8, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29433351/transcatheter-pulmonary-valve-implantation-will-it-replace-surgical-pulmonary-valve-replacement
#14
Ahmed Kheiwa, Punag Divanji, Vaikom S Mahadevan
Right ventricular outflow tract (RVOT) dysfunction is a common hemodynamic challenge for adults with congenital heart disease (ACHD), including patients with repaired tetralogy of Fallot (TOF), truncus arteriosus (TA), and those who have undergone the Ross procedure for congenital aortic stenosis and the Rastelli repair for transposition of great vessels. Pulmonary valve replacement (PVR) has become one of the most common procedures performed for ACHD patients. Areas covered: Given the advances in transcatheter technology, we conducted a detailed review of the available studies addressing the indications for PVR, historical background, evolving technology, procedural aspects, and the future direction, with an emphasis on ACHD patients...
March 2018: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/29410185/evaluation-of-the-adult-congenital-heart-surgery-mortality-score-at-two-european-centers
#15
Jürgen Hörer, Emre Belli, Régine Roussin, Emanuel LeBret, Mohamed Ly, Jarrah Abdullah, Raffaella Marzullo, Martina Strbad, Julie Cleuziou, Jelena Pabst von Ohain, Rüdiger Lange
BACKGROUND: The adult congenital heart surgery (ACHS) score was derived from The Society of Thoracic Surgeons Congenital Heart Surgery Database. The score was validated with data for 1,603 operations and reached a good predictive power. We sought to evaluate its predictive power for 1,654 operations performed in two European centers. METHODS: Data of all consecutive patients aged 18 years or more who underwent surgery for congenital heart disease between 2004 and 2013 at center 1 (n = 830) and between 2005 and 2016 at center 2 (n = 824) were collected...
May 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29327893/heart-failure-in-grown-up-congenital-heart-disease
#16
Alessia Faccini, Angelo Micheletti, Diana G Negura, Luca Giugno, Gianfranco Butera, Mario Carminati, Alessandro Giamberti, Massimo Chessa
The increasing survival in the adulthood of patients with congenital heart disease (CHD) has changed the epidemiology of adult CHD (ACHD) patients and has led to an increment in hospitalization rates due to heart failure (HF). ACHD patients hospitalized for HF have a five-fold higher risk of death HF than those compensated. HF occurs predominantly in patients with tetralogy of Fallot, single ventricles, and after the Mustard operation for transposition of the great arteries. Diagnostic strategies applied in acquired HF patients are usually used to evaluate ACHD patients, but sometimes this can postpone the identification of HF that can become manifest with unusual and peculiar signs or symptoms...
January 10, 2018: Minerva Cardioangiologica
https://www.readbyqxmd.com/read/29310808/frequency-and-consequences-of-influenza-vaccination-in-adults-with-congenital-heart-disease
#17
Idris Bare, Jennifer Crawford, Kendell Pon, Negeen Farida, Payam Dehghani
Immunization against influenza is a critical, but perhaps underappreciated prevention of morbidity and mortality in the cardiac population. The purpose of the present study is to examine influenza vaccination rates in adults with congenital heart disease (ACHD). A secondary purpose is to explore whether there is an association between demographic, medical, and behavioral variables and receipt of the influenza vaccination. Of the 183 consecutive ACHD patients who were contacted, 123 responded to our telephone survey...
February 15, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29277859/management-of-acute-heart-failure-in-adult-patients-with-congenital-heart-disease
#18
REVIEW
Alexander Van De Bruaene, Lukas Meier, Walter Droogne, Pieter De Meester, Els Troost, Marc Gewillig, Werner Budts
Heart failure is an increasing reason for hospitalization and the leading cause of death in patients with adult congenital heart disease (ACHD). Recently, the European Society of Cardiology and the American Heart Association published consensus documents on the management of chronic heart failure in ACHD patients. However, little data and/or guidelines are available for the management of (sub)acute heart failure. The ACHD population is heterogeneous by definition and often has complex underlying anatomy, which could pose a challenge to the physician confronted with the ACHD patient in (sub)acute heart failure...
January 2018: Heart Failure Reviews
https://www.readbyqxmd.com/read/29236965/management-errors-in-adults-with-congenital-heart-disease-prevalence-sources-and-consequences
#19
Rachael Cordina, Subha Nasir Ahmad, Irina Kotchetkova, Gry Eveborn, Lynne Pressley, Julian Ayer, Richard Chard, David Tanous, Peter Robinson, Jens Kilian, John E Deanfield, David S Celermajer
Aims: Improved survival has resulted in increasing numbers and complexity of adults with congenital heart disease (ACHD). International guidelines recommend specialized care but many patients are still not managed at dedicated ACHD centres. This study analysed referral sources and appropriateness of management for patients referred to our tertiary ACHD Centre over the past 3 years. Methods and results: We compared differences in care between patients referred from paediatric/ACHD-trained vs...
March 21, 2018: European Heart Journal
https://www.readbyqxmd.com/read/29198985/clinical-and-psychological-drivers-of-perceived-health-status-in-adults-with-congenital-heart-disease
#20
Jong Mi Ko, Kristen M Tecson, Vanessa Al Rashida, Sandeep Sodhi, Josh Saef, Mehwish Mufti, Kamila S White, Philip A Ludbrook, Ari M Cedars
The factors having the greatest impact on self-reported health status in adults with congenital heart disease (ACHD) remain incompletely studied. We conducted a single-site, cross-sectional study of ACHD patients followed at the Center for ACHD at Washington University School of Medicine, including retrospectively gathered clinical data and psychometric and health status assessments completed at the time of enrollment. To identify primary drivers of perceived health status, we investigated the impact of the demographic, clinical, and psychological variables on self-reported health status as assessed using the Rand 36-Item Short Form Health Survey...
February 1, 2018: American Journal of Cardiology
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