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treatment of congenital heart disease

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https://www.readbyqxmd.com/read/28643420/impact-on-survival-of-warfarin-in-patients-with-pulmonary-arterial-hypertension-receiving-subcutaneous-treprostinil
#1
Mona Ascha, Xuan Zhou, Youlan Rao, Omar A Minai, Adriano R Tonelli
INTRODUCTION: Anticoagulation is a common treatment modality in patients with pulmonary arterial hypertension (PAH). Further studies are needed to appropriately assess the risk / benefit ratio of anticoagulation, particularly in PAH patients receiving PAH-specific therapies. AIMS: We use observational long-term data on PAH patients treated with subcutaneous (SQ) treprostinil from a large open-label study. Patients were followed for up to four years. The use of warfarin and bleeding events were recorded...
June 23, 2017: Cardiovascular Therapeutics
https://www.readbyqxmd.com/read/28632651/imaging-in-adult-congenital-heart-disease
#2
Stephanie S Gaydos, Akos Varga-Szemes, Rochelle N Judd, Pal Suranyi, David Gregg
There has been tremendous growth in the population of adults with congenital heart disease (CHD) over the last few decades because of advances in medical care. Whereas some cases are cured during childhood, most patients instead undergo palliation, which leaves them at risk for late complications. Lifelong clinical follow-up involving serial multimodality imaging is helpful to monitor and guide the treatment of late complications. Imaging of these individuals is challenging because of their unique anatomy and therefore requires careful consideration on a case-by-case basis...
July 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28628748/does-young-age-really-put-the-heart-at-risk
#3
Michael P Belanger, Luke Yang Tan, Carin Wittnich
Despite significant advances in the management and treatment of heart disease in children, there continue to be patients who have worse outcomes than might be expected. A number of risk factors that could be responsible have been identified. Evidence based findings will be reviewed including whether young age and/or reduced body weight exacerbate these responses. For example, newborn children undergoing congenital cardiac surgery are known to have worse outcomes than older children. Evidence exists that newborn hearts do not tolerate ischemia as well as adults; developing irreversible injury sooner and exhibiting at risk metabolic profiles...
June 19, 2017: Canadian Journal of Physiology and Pharmacology
https://www.readbyqxmd.com/read/28627006/parents-perceptions-during-the-transition-to-home-for-their-child-with-a-congenital-heart-defect-how-can-we-support-families-of-children-with-hypoplastic-left-heart-syndrome
#4
Sarita March
PURPOSE: The aim of the study was to explore the literature related to transitions in healthcare between the hospital and home that caregivers experience with a child who has a congenital heart defect (CHD), specifically related to hypoplastic left heart syndrome (HLHS). DESIGN AND METHODS: A systematic literature review was conducted searching OVID Medline, CINAHL, and PubMed to discover the caregivers' perceptions on their transitions between hospital care and home care of their child with a CHD...
June 18, 2017: Journal for Specialists in Pediatric Nursing: JSPN
https://www.readbyqxmd.com/read/28624838/dental-management-of-pediatric-patients-affected-by-pulmonary-atresia-with-ventricular-septal-defect-a-scoping-review
#5
A Garrocho-Rangel, A-C Echavarría-García, M-A Rosales-Bérber, J Flores-Velázquez, A Pozos-Guillén
BACKGROUND: Congenital Heart Diseases (CHD) involves a wide range of pathological conditions, such as Pulmonary Atresia with Ventricle Septal Defect (PA/VSD). This disorder leads to the systemic circulation of oxygen-poor blood (cyanosis), with associated features and consequences in the oral cavity. MATERIAL AND METHODS: Using scoping review methodology for screening and article selection, the primary objectives of this paper were as follows: first, to pose a research question; second, to identify relevant studies in order to answer the research question; third, to select and retrieve the studies; fourth, to chart the critical data, and finally, to collate, summarize, and report the results from the most important articles on the dental management of children affected with PA/VSD...
June 18, 2017: Medicina Oral, Patología Oral y Cirugía Bucal
https://www.readbyqxmd.com/read/28619193/protein-losing-enteropathy-in-patients-with-congenital-heart-disease
#6
Maxim Itkin, David A Piccoli, Gregory Nadolski, Jack Rychik, Aaron DeWitt, Erin Pinto, Jonathan Rome, Yoav Dori
BACKGROUND: Protein-losing enteropathy (PLE), characterized by loss of proteins in the intestine, is a devastating complication in patients with congenital heart disease. The cause of PLE is unknown, but lymphatic involvement has been suspected. OBJECTIVES: The authors evaluated the use of lymphangiographic imaging and liver lymphatic embolization as a treatment for PLE. METHODS: This was a single-center, retrospective review of imaging and interventions used in 8 consecutive patients with liver lymphatic embolization and congenital heart disease with elevated central venous pressure complicated by PLE...
June 20, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28616089/severe-pulmonary-arterial-hypertension-associated-with-congenital-cardiac-shunts-evolution-under-specific-treatment
#7
R I Negoi, I Ghiorghiu, F Filipoiu, M Hostiuc, I Negoi, C Ginghina
Objective: The aim of this study was to compare the effects of Sildenafil, Bosentan and combined therapy in patients with congenital cardiac shunts associated pulmonary artery hypertension (CCS-PAH). Design: Prospective observational study (February 2011 - January 2014) with a historical control group (January 2009 - January 2011). Setting: "CC Iliescu" Institute for Emergency Cardiovascular Diseases of Bucharest, a tertiary university-affiliated center. Patients: All cases with CCS-PAH. Interventions: Specific vasodilatory therapy: Sildenafil, Bosentan or combined therapy...
April 2017: Journal of Medicine and Life
https://www.readbyqxmd.com/read/28609765/transcatheter-treatment-of-aortopulmonary-window-with-a-symmetrical-membranous-ventricular-septal-occluder
#8
Hai-Xia Xu, Dong-Dong Zheng, Min Pan, Xiao-Fei Li
Aortopulmonary window (APW), the presence of a communication between aorta and pulmonary artery, is a rare congenital heart disease, and surgical intervention is the standard for closure. Recently, several cases have been treated with transcatheter device occluders. Here, we report an APW patient treated successfully using a transcatheter closure with a symmetrical membranous ventricular septal occluder. We are the first to report on a case treated with this type of occluder for APW.
June 14, 2017: Cardiology
https://www.readbyqxmd.com/read/28606655/mortality-in-pulmonary-arterial-hypertension-due-to-congenital-heart-disease-serial-changes-improve-prognostication
#9
M T U Schuijt, I M Blok, A H Zwinderman, A C M J van Riel, M J Schuuring, R J de Winter, A L Duijnhouwer, A P J van Dijk, B J M Mulder, B J Bouma
BACKGROUND: Adult patients with pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) suffer from high mortality. This underlines the importance of adequate risk stratification to guide treatment decisions. Several baseline parameters are associated with mortality, however, their prognostic value may weaken after years of follow-up. Therefore we investigated the prognostic value of serial changes in standard clinical parameters in PAH-CHD. METHODS: In this prospective observational cohort study we included consecutive PAH-CHD adults, between 2005 and 2016...
June 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28600095/adult-congenital-interventions-in-heart-failure
#10
REVIEW
Hussam S Suradi, Ziyad M Hijazi
Congenital heart disease (CHD) is the most common birth defect, occurring in approximately 0.8% to 1.0% of neonates. Advances in medical and surgical therapies for children with CHD have resulted in a growing population of patients reaching adulthood, with survival rates exceeding 85%. Many of these patients, especially if managed inappropriately, face the prospect of future complications including heart failure and premature death. For adults with uncorrected or previously palliated CHD, percutaneous therapies have become the primary treatment for many forms of CHD...
July 2017: Interventional cardiology clinics
https://www.readbyqxmd.com/read/28599967/challenging-surgical-dogma-in-the-management-of-proximal-esophageal-atresia-with-distal-tracheoesophageal-fistula-outcomes-from-the-midwest-pediatric-surgery-consortium
#11
Dave R Lal, Samir K Gadepalli, Cynthia D Downard, Daniel J Ostlie, Peter C Minneci, Ruth M Swedler, Thomas H Chelius, Laura Cassidy, Cooper T Rapp, Deborah Billmire, Steven Bruch, R Carland Burns, Katherine J Deans, Mary E Fallat, Jason D Fraser, Julia Grabowski, Ferdynand Hebel, Michael A Helmrath, Ronald B Hirschl, Rashmi Kabre, Jonathan Kohler, Matthew P Landman, Charles M Leys, Grace Z Mak, Jessica Raque, Beth Rymeski, Jacqueline M Saito, Shawn D St Peter, Daniel von Allmen, Brad W Warner, Thomas T Sato
PURPOSE: Perioperative management of infants with esophageal atresia and tracheoesophageal fistula (EA/TEF) is frequently based on surgeon experience and dogma rather than evidence-based guidelines. This study examines whether commonly perceived important aspects of practice affect outcome in a contemporary multi-institutional cohort of patients undergoing primary repair for the most common type of esophageal atresia anomaly, proximal EA with distal TEF. METHODS: The Midwest Pediatric Surgery Consortium conducted a multicenter, retrospective study examining selected outcomes on infants diagnosed with proximal EA with distal TEF who underwent primary repair over a 5-year period (2009-2014), with a minimum 1-year follow up, across 11 centers...
June 1, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28599384/adolescent-and-parent-perspectives-prior-to-involvement-in-a-fontan-transition-program
#12
Karin du Plessis, Evelyn Culnane, Rebecca Peters, Yves d'Udekem
Background Successful transition to adult healthcare is particularly important for congenital heart disease (CHD) patients who have undergone palliative surgery, as they risk adverse events if not followed closely. This study examines young people at the worst end of the CHD spectrum who are born with a single ventricle (pumping heart chamber), and who undergo a series of operations that culminate in the Fontan surgical procedure. Purpose To explore adolescents with a Fontan circulation, and their parents', readiness for transition to adult care and pre-implementation of a transition program...
May 24, 2017: International Journal of Adolescent Medicine and Health
https://www.readbyqxmd.com/read/28582432/in-vivo-efficacy-of-the-akt-inhibitor-arq-092-in-noonan-syndrome-with-multiple-lentigines-associated-hypertrophic-cardiomyopathy
#13
Jianxun Wang, Vasanth Chandrasekhar, Giovanni Abbadessa, Yi Yu, Brian Schwartz, Maria I Kontaridis
Noonan Syndrome with Multiple Lentigines (NSML, formerly LEOPARD syndrome) is an autosomal dominant "RASopathy" disorder manifesting in congenital heart disease. Most cases of NSML are caused by catalytically inactivating mutations in the protein tyrosine phosphatase (PTP), non-receptor type 11 (PTPN11), encoding the SH2 domain-containing PTP-2 (SHP2) protein. We previously generated knock-in mice harboring the PTPN11 mutation Y279C, one of the most common NSML alleles; these now-termed SHP2Y279C/+ mice recapitulate the human disorder and develop hypertrophic cardiomyopathy (HCM) by 12 weeks of age...
2017: PloS One
https://www.readbyqxmd.com/read/28580658/improvement-in-perioperative-care-in-pediatric-cardiac-surgery-by-shifting-the-primary-focus-of-treatment-from-cardiac-output-to-perfusion-pressure-are-beta-stimulants-still-needed
#14
Amir-Reza Hosseinpour, Mathieu van Steenberghe, Marc-André Bernath, Stefano Di Bernardo, Marie-Hélène Pérez, David Longchamp, Mirko Dolci, Yann Boegli, Nicole Sekarski, Javier Orrit, Michel Hurni, René Prêtre, Jacques Cotting
OBJECTIVE: An important aspect of perioperative care in pediatric cardiac surgery is maintenance of optimal hemodynamic status using vasoactive/inotropic agents. Conventionally, this has focused on maintenance of cardiac output rather than perfusion pressure. However, this approach has been abandoned in our center in favor of one focusing primarily on perfusion pressure, which is presented here and compared to the conventional approach. DESIGN: A retrospective study...
June 5, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28580573/fetal-neck-tumors-antenatal-and-intrapartum-management
#15
Jakub Kornacki, Jana Skrzypczak
In this review article we describe the ante- and perinatal management of fetal neck tumors. Although there are rare congenital anomalies, the clinical consequences for the fetus may be fatal and include airways obstruction, heart insufficiency, pulmonary hypoplasia and cosmetic effect. The right management allows to decrease the fetal and neonatal mortality and morbidity associated with the disease. It includes intrauterine therapy in some cases, mostly in a goitrus hypothyroidism of the fetus, but firstly, an assessment of the fetal airways patency with a subsequent, eventual Ex-Utero Intrapartum Treatment (EXIT)...
2017: Ginekologia Polska
https://www.readbyqxmd.com/read/28580351/transition-in-patients-with-congenital-heart-disease-in-germany-results-of-a-nationwide-patient-survey
#16
Paul C Helm, Harald Kaemmerer, Günter Breithardt, Elisabeth J Sticker, Roland Keuchen, Rhoia Neidenbach, Gerhard-Paul Diller, Oktay Tutarel, Ulrike M M Bauer
BACKGROUND: A growing number of adults with congenital heart disease (ACHD) pose a particular challenge for health care systems across the world. Upon turning into 18 years, under the German national health care system, ACHD patients are required to switch from a pediatric to an adult cardiologist or an ACHD-certified provider. To date, reliable data investigating the treatment situation of ACHD patients in Germany are not available. MATERIALS AND METHODS: An online survey was conducted in collaboration with patient organizations to address the life situation and the conditions of health care provision for ACHD patients in Germany...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28571637/interventional-treatment-of-patients-with%C3%A2-congenital-heart-disease-nationwide-danish-experience-over-39-years
#17
Signe H Larsen, Morten Olsen, Kristian Emmertsen, Vibeke E Hjortdal
BACKGROUND: The treatment of congenital heart (CHD) has changed rapidly. OBJECTIVES: The authors reviewed CHD treatment through a 39-year nationwide population-based study on congenital heart surgery and catheter-based interventions, unbiased by referral patterns. METHODS: Using medical registries, the authors identified children (<18 years of age) treated for CHD in Denmark from 1977 to 2015, their need for reinterventions, and their long-term survival...
June 6, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28560817/outcomes-of-pulmonary-arterial-hypertension-therapy-in-australia-is-monotherapy-adequate
#18
Avalon Moonen, Roger Garsia, Peter Youssef, Paul Torzillo, Tamera Corte, Christiane Boehm, Rachael Cordina, David Celermajer, Edmund Lau
BACKGROUND: In Australia, government-subsidised treatment of pulmonary arterial hypertension (PAH) is limited to monotherapy. Recent international guidelines advocate that initial combination therapy be considered for all symptomatic PAH patients. We sought to characterise "real-life" outcomes in PAH patients initiated on monotherapy. METHODS: We performed a retrospective analysis of 100 consecutive PAH patients at a single centre, who were commenced on monotherapy for PAH between 2004 and 2015...
May 31, 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/28558084/stent-placement-in-a-neonate-with-sano-modification-of-the-norwood-using-semi-elective-extracorporeal-membrane-oxygenation
#19
Mustafa Gulgun, Michael Slack
Extracorporeal membrane oxygenation (ECMO) is a well-established tool of cardiopulmonary circulatory support for cardiopulmonary failure in children and adults. It has been used as a supportive strategy during interventional procedures in neonates with congenital heart disease. Herein, we describe a neonate with hypoplastic left heart syndrome who underwent stenting of the Sano shunt and left pulmonary artery after Norwood Sano operation using intra-procedural ECMO support. The use of ECMO as a bridge to recovery might be a feasible and reasonably safe adjunctive approach in the treatment of complications in selective case of neonates having undergone the Norwood Sano procedure...
December 2016: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28555455/-vagus-nerve-stimulation-treatment-of-158-pediatric-patients-with-a-long-term-follow-up
#20
S Flesler, G Reyes, S Fortini, B Ramos, R Cersosimo, M Bartuluchi, R Caraballo
AIM: To describe a series of patients with drug resistant epilepsy treated with vagus nerve stimulation in a national pediatric hospital, evaluating efficacy, safety and tolerability. PATIENTS AND METHODS: A retrospective analysis of 158 pediatric patients with epilepsy resistant to pharmacological and non pharmacological treatment including surgery that were treated with vagus nerve stimulation between 2001-2015. Patients with progressive encephalopathies, and congenital heart disease were excluded...
June 1, 2017: Revista de Neurologia
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