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Congenital heart disease outcomes

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https://www.readbyqxmd.com/read/29664959/contemporary-survival-of-patients-with-pulmonary-arterial-hypertension-and-congenital-systemic-to-pulmonary-shunts
#1
Chodchanok Vijarnsorn, Kritvikrom Durongpisitkul, Paweena Chungsomprasong, Densiri Bositthipichet, Salisa Ketsara, Yuttapon Titaram, Prakul Chanthong, Supaluck Kanjanauthai, Jarupim Soongswang
OBJECTIVE: To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort. METHODS: All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded...
2018: PloS One
https://www.readbyqxmd.com/read/29661025/self-efficacy-as-a-predictor-of-patient-reported-outcomes-in-adults-with-congenital-heart-disease
#2
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
#3
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/29657908/a-rare-case-of-coronary-artery-embolism-in-a-patient-with-d-transposition-of-the-great-arteries-with-prior-mustard-repair
#4
Prince Sethi, Udit Bhatnagar, Kelly Steffen, Edgard Bendaly, Adam Stys
The dextro-transposition of great arteries (d-TGA) is a rare, congenital, cyanotic heart disease and there is a paucity of data regarding long-term cardiovascular outcomes. We present a rare case of non-ST-elevation myocardial infarction (NSTEMI) in a patient with surgically repaired d-TGA. A 43-year-old male who had previously undergone a Mustard atrial switch palliative procedure presented with chest pain and diaphoresis and was diagnosed with NSTEMI. A coronary angiogram revealed a small, underdeveloped, left anterior descending and a large, left circumflex coronary artery with an acute embolic lesion...
February 12, 2018: Curēus
https://www.readbyqxmd.com/read/29653251/probabilistic-guidance-for-catheter-tip-motion-in-cardiac-ablation-procedures
#5
Mihaela Am Constantinescu, Su-Lin Lee, Sabine Ernst, Apit Hemakom, Danilo Mandic, Guang-Zhong Yang
Radiofrequency catheter ablation is one of the commonly available therapeutic methods for patients suffering from cardiac arrhythmias. The prerequisite of successful ablation is sufficient energy delivery at the target site. However, cardiac and respiratory motion, coupled with endocardial irregularities, can cause catheter drift and dispersion of the radiofrequency energy, thus prolonging procedure time, damaging adjacent tissue, and leading to electrical reconnection of temporarily ablated regions. Therefore, positional accuracy and stability of the catheter tip during energy delivery is of great importance for the outcome of the procedure...
March 23, 2018: Medical Image Analysis
https://www.readbyqxmd.com/read/29652749/sera-from-children-after-cardiopulmonary-bypass-reduces-permeability-of-capillary-endothelial-cell-barriers
#6
Richard W Pierce, Riad Abou Zahr, Sarah Kandil, E Vincent S Faustino, Jordan S Pober
OBJECTIVES: Children undergoing cardiopulmonary bypass develop clinically impactful capillary leak of unclear etiology. A widely held hypothesis that exposure of circulating cells to the cardiopulmonary bypass circuit induces the release of inflammatory mediators that act to disrupt intercellular junctions of capillary endothelial cells inducing paracellular capillary leak either directly or through new gene expression. DESIGN: Cohort study. SETTING: Tertiary pediatric hospital...
April 12, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29625576/skeletal-cardiac-and-respiratory-muscle-function-and-histopathology-in-the-p448lneo-mouse-model-of-fkrp-deficient-muscular-dystrophy
#7
Qing Yu, Melissa Morales, Ning Li, Alexander G Fritz, Ren Ruobing, Anthony Blaeser, Ershia Francois, Qi-Long Lu, Kanneboyina Nagaraju, Christopher F Spurney
BACKGROUND: Fukutin-related protein (FKRP) mutations are the most common cause of dystroglycanopathies known to cause both limb girdle and congenital muscular dystrophy. The P448Lneo- mouse model has a knock-in mutation in the FKRP gene and develops skeletal, respiratory, and cardiac muscle disease. METHODS: We studied the natural history of the P448Lneo- mouse model over 9 months and the effects of twice weekly treadmill running. Forelimb and hindlimb grip strength (Columbus Instruments) and overall activity (Omnitech Electronics) assessed skeletal muscle function...
April 6, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29625509/adult-congenital-heart-disease-with-pregnancy
#8
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/29624879/financial-burdens-and-mental-health-needs-in-families-of-children-with-congenital-heart-disease
#9
Nancy McClung, Jill Glidewell, Sherry L Farr
OBJECTIVE: To examine the financial burdens and mental health needs of families of children with special healthcare needs (CSHCN) with congenital heart disease (CHD). METHODS: Data from the 2009-2010 National Survey of Children with Special Health Care Needs (NS-CSHCN) were used to examine parent-reported financial burdens (out-of-pocket expenses, financial problems, employment impact, caregiving hours) and family members' need for mental health services in families of CSHCN with CHD...
April 6, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29618066/pulmonary-vein-obstruction-after-primary-sutureless-pericardial-repair-of-a-total-anomalous-pulmonary-venous-connection
#10
Kota Agematsu, Toru Okamura, Yoji Takiguchi, Yorikazu Harada
Total anomalous pulmonary venous connection is a rare congenital heart disease. The development of pulmonary venous obstruction is one of the major risk factors for poor outcomes after surgical repair. Sutureless pericardial repair for a total anomalous pulmonary venous connection was introduced to decrease the risk of pulmonary venous obstruction after surgical repair, and favourable outcomes have been reported. Herein, we report the case of an infant with a total anomalous pulmonary venous connection who developed pulmonary venous obstruction after primary sutureless pericardial repair...
April 2, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29607544/outcome-of-pediatric-heart-transplantation-in-blood-culture-positive-donors-in-the-united-states
#11
Shahnawaz M Amdani, Wei Du, Sanjeev Aggarwal
Active donor infection at time of organ procurement poses a potential infection risk and may increase post-transplant morbidity and mortality in recipients. Our hypothesis was that pediatric heart transplant recipients from blood culture positive donors (BCPD) would have increased morbidity and mortality compared to non-blood culture positive donors (NBCPD). A retrospective analyses of pediatric heart transplant recipients using the Organ Procurement and Transplant Network (OPTN) between 1987 and 2015 were conducted...
April 1, 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
#12
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/29603246/elevated-free-hemoglobin-and-decreased-haptoglobin-levels-are-associated-with-adverse-clinical-outcomes-unfavorable-physiologic-measures-and-altered-inflammatory-markers-in-pediatric-cardiac-surgery-patients
#13
Jill M Cholette, Anthony P Pietropaoli, Kelly F Henrichs, George M Alfieris, Karen S Powers, Francisco Gensini, Jeffrey S Rubenstein, Dawn Sweeney, Richard Phipps, Sherry L Spinelli, Majed A Refaai, Michael P Eaton, Neil Blumberg
BACKGROUND: There are data suggesting that free hemoglobin (Hb), heme, and iron contribute to infection, thrombosis, multiorgan failure, and death in critically ill patients. These outcomes may be mitigated by haptoglobin. STUDY DESIGN AND METHODS: 164 consecutively treated children undergoing surgery for congenital heart disease were evaluated for associations between free Hb and haptoglobin and clinical outcomes, physiologic metrics, and biomarkers of inflammation RESULTS: Higher perioperative free Hb levels (and lower haptoglobin levels) were associated with mortality, nosocomial infection, thrombosis, hours of intubation and inotropes, increased interleukin-6, peak serum lactate levels, and lower nadir mean arterial pressures...
March 30, 2018: Transfusion
https://www.readbyqxmd.com/read/29601372/illness-uncertainty-and-posttraumatic-stress-in-young-adults-with-congenital-heart-disease
#14
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/29599167/quality-of-life-in-young-people-with-congenital-heart-disease-is-better-than-expected
#15
Barbara Reiner, Renate Oberhoffer, Peter Ewert, Jan Müller
OBJECTIVES: Improved treatments for patients with congenital heart disease (CHD) have led to a growing interest in long-term functional outcomes such as health-related quality of life (HRQoL). Studies on HRQoL in children with CHD have contradicting results. Therefore, we compared HRQoL of children with CHD with that of current healthy peers and stratify CHD cases by severity and diagnostic subgroups. METHODS: We included 514 patients (191 girls) aged 7-17 (12.9±3...
March 29, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29594089/the-need-for-specialized-pediatric-cardiac-critical-care-training-program-in-limited-resource-settings
#16
REVIEW
Indah K Murni, Ndidiamaka L Musa
Congenital heart disease (CHD) is one of the major global health problems with the highest birth prevalence in low- and middle-income countries. In these populous countries, basic health services for the children with CHD, including surgery, are lacking. Even though surgery is performed, outcome after cardiac surgery is influenced by the quality of the postoperative management with a reported high morbidity and mortality. Henceforth, there is an urgent need for comprehensive interventions to provide high quality cardiac intensive care programs to improve the quality of pediatric cardiac surgery services in order to address high morbidity and mortality after cardiac surgery...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29589379/results-and-factors-associated-with-adverse-outcome-after-tricuspid-valve-replacement
#17
Paweł Litwiński, Piotr Kołsut, Tadeusz Sitko, Tomasz Zieliński, Piotr Hoffman, Tomasz Hryniewiecki, Jacek Różański, Mariusz Kuśmierczyk
BACKGROUND: This retrospective analysis of patients with severe tricuspid valve disease, who underwent tricuspid valve replacement (TVR) for either tricuspid regurgitation or stenosis, represents an attempt to determine the factors that predict poor hospital and long-term survival. METHODS: The study population consisted of 86 patients, 65 female and 21 male, who underwent TVR with or without concomitant surgical procedures between 2000 and 2010 at our institution...
March 28, 2018: Kardiologia Polska
https://www.readbyqxmd.com/read/29587521/orthotopic-heart-transplantation-in-children
#18
Nagarajan Muthialu
Heart transplantation is a relatively infrequent but technically demanding procedure in pediatric cardiac surgery. Orthotopic heart transplantation is often offered to older children or adolescents following multiple previous operations, but with advances in intensive care, diagnostics, and management, more infants with complex congenital heart diseases are being listed for transplantation. Primary cardiomyopathies remain the most common indication. Outcomes following cardiothoracic transplantation have improved steadily in recent times...
January 1, 2018: Asian Cardiovascular & Thoracic Annals
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
#19
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/29576325/adjunct-targeted-biologic-inhibition-agents-to-treat-aggressive-multivessel-intraluminal-pediatric-pulmonary-vein-stenosis
#20
Ryan Callahan, Mark W Kieran, Christopher W Baird, Steven D Colan, Kimberlee Gauvreau, Christina M Ireland, Audrey C Marshall, Laureen M Sena, Sara O Vargas, Kathy J Jenkins
OBJECTIVE: To evaluate the use of imatinib mesylate with or without bevacizumab targeting neoproliferative myofibroblast-like cells with tyrosine kinase receptor expression, as adjuncts to modern interventional therapies for the treatment of multivessel intraluminal pulmonary vein stenosis (PVS). We describe the 48- and 72-week outcomes among patients receiving imatinib mesylate with or without bevacizumab for multivessel intraluminal PVS. STUDY DESIGN: This single-arm, prospective, open-label US Food and Drug Administration approved trial enrolled patients with ≥2 affected pulmonary veins after surgical or catheter-based relief of obstruction between March 2009 and December 2014...
March 23, 2018: Journal of Pediatrics
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