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https://www.readbyqxmd.com/read/29666709/anesthetic-considerations-for-an-adult-patient-with-freeman-sheldon-syndrome-undergoing-open-heart-surgery
#1
S Viehmeyer, P Gabriel, K Bauer, S Bauer, R Sodian, J N Hilberath
Freeman-Sheldon syndrome (FSS) or "whistling face" syndrome is a rare congenital disorder complicated by characteristic facial deformities and muscular contractures. We report on a 64-year-old male patient presenting for surgical replacement of his aortic valve and review the available literature on anesthetic considerations and perioperative management principles. FSS frequently poses a significant challenge to airway management and gaining vascular access. Moreover, these patients are reportedly at risk for developing malignant hyperthermia (MH) or neuroleptic malignant syndrome...
2018: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/29666178/causes-of-death-in-a-contemporary-adult-congenital-heart-disease-cohort
#2
Christopher Yu, Benjamin M Moore, Irina Kotchetkova, Rachael L Cordina, David S Celermajer
OBJECTIVE: The life expectancy of patients with congenital heart disease (CHD) has significantly improved with advances in their paediatric medical care. Mortality patterns are changing as a result. Our study aims to describe survival and causes of death in a contemporary cohort of adult patients with CHD. METHODS: We reviewed 3068 patients in our adult CHD database (age ≥16 years, seen at least once in our centre between 2000 and 2015), and documented the number and causes of death, via Australia's National Death Index...
April 17, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29666177/predicting-survival-in-adults-with-congenital-heart-disease-what-are-the-odds
#3
EDITORIAL
Jonathan Buber, Anne Marie Valente
No abstract text is available yet for this article.
April 17, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29663641/congenital-heart-diseases-and-cardiovascular-abnormalities-in-22q11-2-deletion-syndrome-from-well-established-knowledge-to-new-frontiers
#4
REVIEW
Marta Unolt, Paolo Versacci, Silvia Anaclerio, Caterina Lambiase, Giulio Calcagni, Matteo Trezzi, Adriano Carotti, Terrence Blaine Crowley, Elaine H Zackai, Elizabeth Goldmuntz, James William Gaynor, Maria Cristina Digilio, Donna M McDonald-McGinn, Bruno Marino
Congenital heart diseases (CHDs) and cardiovascular abnormalities are one of the pillars of clinical diagnosis of 22q11.2 deletion syndrome (22q11.2DS) and still represent the main cause of mortality in the affected children. In the past 30 years, much progress has been made in describing the anatomical patterns of CHD, in improving their diagnosis, medical treatment, and surgical procedures for these conditions, as well as in understanding the underlying genetic and developmental mechanisms. However, further studies are still needed to better determine the true prevalence of CHDs in 22q11...
April 16, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29661025/self-efficacy-as-a-predictor-of-patient-reported-outcomes-in-adults-with-congenital-heart-disease
#5
Corina Thomet, Philip Moons, Markus Schwerzmann, Silke Apers, Koen Luyckx, Erwin N Oechslin, Adrienne H Kovacs
OBJECTIVE: Self-efficacy is a known predictor of patient-reported outcomes in individuals with acquired diseases. With an overall objective of better understanding patient-reported outcomes in adults with congenital heart disease, this study aimed to: (i) assess self-efficacy in adults with congenital heart disease, (ii) explore potential demographic and medical correlates of self-efficacy and (iii) determine whether self-efficacy explains additional variance in patient-reported outcomes above and beyond known predictors...
April 1, 2018: European Journal of Cardiovascular Nursing
https://www.readbyqxmd.com/read/29660764/prognostic-utility-of-meld-xi-in-adult-congenital-heart-disease-patients-undergoing-cardiac-transplantation
#6
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/29658866/mechanical-valve-replacement-for-congenital-heart-disease-complicated-by-native-pulmonary-valve-endocarditis-a-case-report-and-literature-review
#7
Qing Wang, Yan Gao, Wang Xi, Hua Shen, Jing Wang, Jian Xiao, Zhinong Wang
Congenital heart disease (CHD) is one of the most common risk factors for infective endocarditis. However, it is rare to find a CHD patient complicated by isolated pulmonary valve endocarditis. Here, we report an adult patient with congenital heart disease complicated by native pulmonary valve endocarditis who underwent a mechanical valve replacement. We also review previous literature to examine key points in the treatment of such patients.
March 7, 2018: Heart Surgery Forum
https://www.readbyqxmd.com/read/29655882/incidence-and-mortality-of-adults-with-pulmonary-hypertension-and-congenital-heart-disease
#8
Sara Søndergaard Schwartz, Nicolas Madsen, Henning Bækgaard Laursen, Russel Hirsch, Morten Smærup Olsen
Reports on pulmonary hypertension (PH) in the aging congenital heart disease (CHD) population are few and focused on arterial PH and patients with systemic-to-pulmonary shunts. Our objective was to estimate incidence and mortality of adult-onset PH in the CHD population. Using Danish nationwide registries, we identified all patients diagnosed with CHD from 1963 to 1974 and 1977 to 2012. Patients were matched 1:10 by birth year and gender with general population subjects. Between 1977 and 2013 adults >18 years of age were followed up until PH diagnosis, death, or emigration, whichever came first, using data from the Danish National Registry of Patients...
March 14, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29650544/evaluation-and-management-of-right-sided-heart-failure-a-scientific-statement-from-the-american-heart-association
#9
REVIEW
Marvin A Konstam, Michael S Kiernan, Daniel Bernstein, Biykem Bozkurt, Miriam Jacob, Navin K Kapur, Robb D Kociol, Eldrin F Lewis, Mandeep R Mehra, Francis D Pagani, Amish N Raval, Carey Ward
BACKGROUND AND PURPOSE: The diverse causes of right-sided heart failure (RHF) include, among others, primary cardiomyopathies with right ventricular (RV) involvement, RV ischemia and infarction, volume loading caused by cardiac lesions associated with congenital heart disease and valvular pathologies, and pressure loading resulting from pulmonic stenosis or pulmonary hypertension from a variety of causes, including left-sided heart disease. Progressive RV dysfunction in these disease states is associated with increased morbidity and mortality...
April 12, 2018: Circulation
https://www.readbyqxmd.com/read/29644235/endograft-rescue-of-compromised-interposition-aortic-graft-in-an-adult-patient-with-congenital-heart-disease
#10
Jesse W Lee, Kanishka Ratnayaka, Howaida G El-Said, John W Moore
In a 19-year-old male with interrupted aortic arch and complex congenital heart disease, we report percutaneous repair of a compromised aortic conduit. The patient had aortic arch repair in childhood utilizing a 12 mm Hemashield Dacron conduit. CT angiography showed multiple segments of this conduit were dilated to 16 mm suggesting conduit degeneration and failure with pseudoaneurysm formation. We utilized a self-expanding aortic endograft supported by internal placement of bare metal stents to repair the conduit...
March 14, 2018: Global Cardiology Science & Practice
https://www.readbyqxmd.com/read/29626133/long-term-risk-of-hemorrhagic-stroke-in-young-patients-with-congenital-heart-disease
#11
Kok Wai Giang, Zacharias Mandalenakis, Mikael Dellborg, Georgios Lappas, Peter Eriksson, Per-Olof Hansson, Annika Rosengren
BACKGROUND AND PURPOSE: The risk of ischemic stroke is increased in patients with congenital heart disease (CHD); however, data on the risk of hemorrhagic stroke, including intracerebral hemorrhage (ICH) and subarachnoid hemorrhage (SAH), are lacking. METHODS: The Swedish Patient Register was used to identify all patients who were born with a diagnosis of CHD between 1970 and 1993. Each patient was compared with 10 randomly selected controls from the general population, matched for age, sex, and county...
April 6, 2018: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/29625509/adult-congenital-heart-disease-with-pregnancy
#12
REVIEW
Koichiro Niwa
The number of women with congenital heart disease (CHD) at risk of pregnancy is growing because over 90% of them are grown-up into adulthood. The outcome of pregnancy and delivery is favorable in most of them provided that functional class and systemic ventricular function are good. Women with CHD such as pulmonary hypertension (Eisenmenger syndrome), severe left ventricular outflow stenosis, cyanotic CHD, aortopathy, Fontan procedure and systemic right ventricle (complete transposition of the great arteries [TGA] after atrial switch, congenitally corrected TGA) carry a high-risk...
April 2018: Korean Circulation Journal
https://www.readbyqxmd.com/read/29619223/respiratory-exacerbation-in-a-young-adult-with-cystic-fibrosis-and-tricuspid-atresia
#13
Jamie Wood, Abbey Sawyer, Siobhain Mulrennan, Andrew Bullock
Tricuspid atresia (TAt) is a complex congenital heart defect (CHD) characterized by the absence of the tricuspid valve and right ventricular hypoplasia requiring surgery in childhood, the Fontan procedure. We present a case of a 21-year-old male with TAt and cystic fibrosis (CF), who underwent a Fontan procedure in childhood, presenting to an adult CF clinic with severe deterioration in his respiratory status and multi-organ dysfunction associated with CF. This report describes problems associated with the management of a CF respiratory exacerbation and extrapulmonary manifestations of CF in the unique situation of a Fontan circulation, a circulation with absence of a subpulmonary ventricle and pulsatile pulmonary arterial blood flow where maintenance of systemic cardiac output is totally dependent on good respiratory function and low pulmonary artery pressures...
July 2018: Respirology Case Reports
https://www.readbyqxmd.com/read/29603633/a-translational-model-of-incomplete-catch-up-growth-early-life-hypoxia-and-the-effect-of-physical-activity
#14
Shlomit Radom-Aizik, Frank P Zaldivar, Dwight M Nance, Fadia Haddad, Dan M Cooper, Gregory R Adams
Advances in therapies have led to prolonged survival from many previously lethal health threats in children, notably among prematurely born babies and those with congenital heart disease. Evidence for catch-up growth is common in these children, but in many cases the adult phenotype is never achieved. A translational animal model is required in which specific tissues can be studied over a reasonable time interval. We investigated the impact of postnatal hypoxia (HY) (12%O2 (HY12) or 10% O2 (HY10)) on growth in rats relative to animals raised in room air...
March 30, 2018: Clinical and Translational Science
https://www.readbyqxmd.com/read/29603626/pulmonary-hypertension-barrier-or-just-a-bump-in-the-road-in-transplanting-adults-with-congenital-heart-disease
#15
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/29601372/illness-uncertainty-and-posttraumatic-stress-in-young-adults-with-congenital-heart-disease
#16
Patricia Moreland, Sheila Judge Santacroce
BACKGROUND: Young adults with congenital heart disease (CHD) are at risk for chronic illness uncertainty in 4 domains: ambiguity about the state of their illness; lack of information about the disease, its treatment, and comorbidities; complexity of the healthcare system and relationship with healthcare providers; and unpredictability of the illness course and outcome. Chronic uncertainty has been associated with posttraumatic stress symptoms (PTSS) and posttraumatic stress disorder (PTSD)...
March 29, 2018: Journal of Cardiovascular Nursing
https://www.readbyqxmd.com/read/29581963/current-knowledge-and-recent-advances-of-right-ventricular-molecular-biology-and-metabolism-from-congenital-heart-disease-to-chronic-pulmonary-hypertension
#17
REVIEW
Samantha Guimaron, Julien Guihaire, Myriam Amsallem, François Haddad, Elie Fadel, Olaf Mercier
Studies about pulmonary hypertension and congenital heart diseases have introduced the concept of right ventricular remodeling leading these pathologies to a similar outcome: right ventricular failure. However right ventricular remodeling is also a physiological process that enables the normal fetal right ventricle to adapt at birth and gain its adult phenotype. The healthy mature right ventricle is exposed to low pulmonary vascular resistances and is compliant. However, in the setting of chronic pressure overload, as in pulmonary hypertension, or volume overload, as in congenital heart diseases, the right ventricle reverts back to a fetal phenotype to sustain its function...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29581364/adult-congenital-heart-disease-and-radiation-exposure-the-malignant-price-of-cardiac-care
#18
EDITORIAL
Ninian N Lang, Niki L Walker
No abstract text is available yet for this article.
March 27, 2018: Circulation
https://www.readbyqxmd.com/read/29581225/prognostic-value-of-serial-n-terminal-pro-b-type-natriuretic-peptide-measurements-in-adults-with-congenital-heart-disease
#19
Vivan J M Baggen, Sara J Baart, Annemien E van den Bosch, Jannet A Eindhoven, Maarten Witsenburg, Judith A A E Cuypers, Jolien W Roos-Hesselink, Eric Boersma
BACKGROUND: A single NT-proBNP (N-terminal pro-B-type natriuretic peptide) measurement is a strong prognostic factor in adult congenital heart disease. This study investigates NT-proBNP profiles within patients with adult congenital heart disease and relates these to cardiovascular events. METHODS AND RESULTS: In this prospective cohort, 602 patients with adult congenital heart disease were enrolled at the outpatient clinic (years 2011-2013). NT-proBNP was measured at study inclusion in 595 patients (median age 33 [IQR 25-41] years, 58% male, 90% NYHA I) and at subsequent annual visits...
March 26, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29579186/arrhythmias-in-congenital-heart-disease-a-position-paper-of-the-european-heart-rhythm-association-ehra-association-for-european-paediatric-and-congenital-cardiology-aepc-and-the-european-society-of-cardiology-esc-working-group-on-grown-up-congenital-heart-disease
#20
Antonio Hernández-Madrid, Thomas Paul, Dominic Abrams, Peter F Aziz, Nico A Blom, Jian Chen, Massimo Chessa, Nicolas Combes, Nikolaos Dagres, Gerhard Diller, Sabine Ernst, Alessandro Giamberti, Joachim Hebe, Jan Janousek, Thomas Kriebel, Jose Moltedo, Javier Moreno, Rafael Peinado, Laurent Pison, Eric Rosenthal, Jonathan R Skinner, Katja Zeppenfeld
The population of patients with congenital heart disease (CHD) is continuously increasing with more and more patients reaching adulthood. A significant portion of these young adults will suffer from arrhythmias due to the underlying congenital heart defect itself or as a sequela of interventional or surgical treatment. The medical community will encounter an increasing challenge as even most of the individuals with complex congenital heart defects nowadays become young adults. Within the past 20 years, management of patients with arrhythmias has gained remarkable progress including pharmacological treatment, catheter ablation, and device therapy...
March 20, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
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