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Congenital heart diseases

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https://www.readbyqxmd.com/read/29775814/composite-self-expanding-bioresorbable-prototype-stents-with-reinforced-compression-performance-for-congenital-heart-disease-application-computational-and-experimental-investigation
#1
Fan Zhao, Wen Xue, Fujun Wang, Laijun Liu, Haoqin Shi, Lu Wang
Stents are vital devices to treat vascular stenosis in pediatric patients with congenital heart disease. Bioresorbable stents (BRSs) have been applied to reduce challenging complications caused by permanent metal stents. However, it remains almost a total lack of BRSs with satisfactory compression performance specifically for children with congenital heart disease, leading to importantly suboptimal effects. In this work, composite bioresorbable prototype stents with superior compression resistance were designed by braiding and annealing technology, incorporating poly (p-dioxanone) (PPDO) monofilaments and polycaprolactone (PCL) multifilament...
May 8, 2018: Journal of the Mechanical Behavior of Biomedical Materials
https://www.readbyqxmd.com/read/29775406/phrase-mining-of-textual-data-to-analyze-extracellular-matrix-protein-patterns-across-cardiovascular-disease
#2
David Alexandre Liem, Sanjana Murali, Dibakar Sigdel, Yu Shi, Xuan Wang, Jiaming Shen, Howard Choi, J Harry Caufield, Wei Wang, Peipei Ping, Jiawei Han
Extracellular matrix (ECM) proteins have been shown to play important roles regulating multiple biological processes in an array of organ systems, including the cardiovascular system. By using a novel bioinformatics text-mining tool, we studied six categories of cardiovascular disease (CVD), namely ischemic heart disease (IHD), cardiomyopathies (CM), cerebrovascular accident (CVA), congenital heart disease (CHD), arrhythmias (ARR), and valve disease (VD), anticipating novel ECM protein-disease and protein-protein relationships hidden within vast quantities of textual data...
May 18, 2018: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/29774522/genome-wide-compound-heterozygosity-analysis-highlighted-four-novel-susceptibility-loci-for-congenital-heart-disease-in-chinese-population
#3
T Jiang, M Huang, T Jiang, Y Gu, Y Wang, Y Wu, H Ma, G Jin, J Dai, Z Hu
Genome-wide association studies (GWASs) have achieved great success in deciphering the genetic cause of congenital heart disease (CHD). However, the heritability of CHD remains to be clarified, and numerous genetic factors responsible for occurrence of CHD are yet unclear. In this study, we performed a genome-wide search for relaxed forms of compound heterozygosity (CH) in association with CHD using our existing GWAS data including 2,265 individuals (957 CHD cases and 1,308 controls). CollapsABEL was used to iteratively test the association between the CH genotype and CHD phenotype in a sliding window manner...
May 17, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29773284/the-role-of-cardiopulmonary-exercise-testing-in-decision-making-in-adults-with-congenital-heart-disease
#4
José Carlos Areias
No abstract text is available yet for this article.
May 14, 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29770299/regenerative-medicine-therapy-for-single-ventricle-congenital-heart-disease
#5
REVIEW
Chetan Ambastha, Gregory J Bittle, David Morales, Nathaniel Parchment, Progyaparamita Saha, Rachana Mishra, Sudhish Sharma, Alexander Vasilenko, Muthukumar Gunasekaran, Manal T Al-Suqi, Deqiang Li, Peixin Yang, Sunjay Kaushal
One of the most complex forms of congenital heart disease (CHD) involving single ventricle physiology is hypoplastic left heart syndrome (HLHS), characterized by underdevelopment of the left ventricle (LV), mitral and aortic valves, and narrowing of the ascending aorta. The underdeveloped LV is incapable of providing long-term systemic flow, and if left untreated, the condition is fatal. Current treatment for this condition consists of three consecutive staged palliative operations: the first is conducted within the first few weeks of birth, the second between 4 to 6 months, and the third and final surgery within the first 4 years...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29770298/mitochondrial-transplantation-applications-for-pediatric-patients-with-congenital-heart-disease
#6
REVIEW
Sitaram M Emani, James D McCully
Mitochondrial transplantation refers to transplantation of respiratory competent mitochondria from healthy tissue into tissues injured by ischemia and reperfusion. This technique has been utilized for recovery of myocardial dysfunction in pediatric patients. The preclinical experience and initial patient experience with this technique are reviewed in this article. Initial experience is with pediatric patients undergoing extracorporeal membrane oxygenation support following myocardial ischemia and reperfusion...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29770292/innovative-interventional-catheterization-techniques-for-congenital-heart-disease
#7
REVIEW
Jeffrey D Zampi, Wendy Whiteside
Since 1929, when the first cardiac catheterization was safely performed in a human by Dr. Werner Forssmann (on himself), there has been a rapid progression of cardiac catheterization techniques and technologies. Today, these advances allow us to treat a wide variety of patients with congenital heart disease using minimally invasive techniques; from fetus to infants to adults, and from simple to complex congenital cardiac lesions. In this article, we will explore some of the exciting advances in cardiac catheterization for the treatment of congenital heart disease, including transcatheter valve implantation, hybrid procedures, biodegradable technologies, and magnetic resonance imaging (MRI)-guided catheterization...
April 2018: Translational pediatrics
https://www.readbyqxmd.com/read/29769443/acetylation-contributes-to-hypertrophy-caused-maturational-delay-of-cardiac-energy-metabolism
#8
Arata Fukushima, Liyan Zhang, Alda Huqi, Victoria H Lam, Sonia Rawat, Tariq Altamimi, Cory S Wagg, Khushmol K Dhaliwal, Lisa K Hornberger, Paul F Kantor, Ivan M Rebeyka, Gary D Lopaschuk
A dramatic increase in cardiac fatty acid oxidation occurs following birth. However, cardiac hypertrophy secondary to congenital heart diseases (CHDs) delays this process, thereby decreasing cardiac energetic capacity and function. Cardiac lysine acetylation is involved in modulating fatty acid oxidation. We thus investigated what effect cardiac hypertrophy has on protein acetylation during maturation. Eighty-four right ventricular biopsies were collected from CHD patients and stratified according to age and the absence (n = 44) or presence of hypertrophy (n = 40)...
May 17, 2018: JCI Insight
https://www.readbyqxmd.com/read/29767458/overlapping-but-distinct-roles-for-notch-receptors-in-human-cardiovascular-disease
#9
REVIEW
J A N Meester, A Verstraeten, M Alaerts, D Schepers, L Van Laer, B L Loeys
The NOTCH signalling pathway is an essential pathway, involved in many cellular processes, including cell fate decision, cell proliferation, and cell death and important in the development of most organs. Mutations in genes encoding components of the NOTCH signalling pathway lead to a spectrum of congenital disorders. Over the past decades mutations in human NOTCH signalling genes have been identified in several diseases with cardiovascular involvement. NOTCH1 mutations have been described in bicuspid aortic valve disease, left-sided congenital heart disease, and Adams-Oliver syndrome...
May 16, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29765932/fluid-overload-and-renal-angina-index-at-admission-are-associated-with-worse-outcomes-in-critically-ill-children
#10
Sidharth K Sethi, Veena Raghunathan, Shilpi Shah, Maninder Dhaliwal, Pranaw Jha, Maneesh Kumar, Sravanthi Paluri, Shyam Bansal, Maroun J Mhanna, Rupesh Raina
Objectives: We investigated the association of fluid overload and oxygenation in critically sick children, and their correlation with various outcomes (duration of ventilation, ICU stay, and mortality). We also assessed whether renal angina index (RAI) at admission can predict mortality or acute kidney injury (AKI) on day 3 after admission. Design and setting: Prospective study, pediatric intensive care in a tertiary hospital. Duration: June 2013-June 2014. Patients: Patients were included if they needed invasive mechanical ventilation for >24 h and had an indwelling arterial catheter...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29764576/-nipbl-gene-mutations-in-two-children-with-cornelia-de-lange-syndrome
#11
Yun-Jing Zhao, Hong-Wei Ma
Both children (one boy and one girl) experienced disease onset in infancy and visited the hospital due to growth retardation. They had unusual facies including thick hair, arched and confluent eyebrows, long and curly eyelashes, short nose, and micrognathia. Patient 1 had congenital heart disease (atrial septal defect and pulmonary stenosis) and special dermatoglyph (a single palmar crease). Patient 2 had cleft palate and moderate-to-severe deafness. Clinical features suggested Cornelia de Lange syndrome in both children...
May 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29764528/symptomatic-myocardial-bridging-a-frequently-occurring-coronary-variation-can-cause-severe-myocardial-ischaemia-in-affected-children-with-underlying-cardiac-conditions
#12
Alexandra Kiess, Marcel Vollroth, Farhad Bakhtiary, Hiroshi Seki, Martin Kostelka, Milan Djukic, Ingo Daehnert, Robert Wagner
Myocardial bridging is a congenital coronary artery anomaly in which the coronary artery has a partly "tunnelled" intramyocardial course. This tunnelling leads to compression of the affected vessel segment during ventricular systole. It is considered to be a benign variation of the norm in about 25% of the population caused by an aberrancy of embryologic coronary development. The bridging is also thought to cause severe cardiac conditions in a few of those affected. The series of six young patients presented here is the largest series so far to report on symptomatic myocardial bridging in children with different underlying heart diseases...
May 16, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29762234/quality-and-safety-in-health-care-part-xli-the-impact-registry
#13
Jay A Harolds
The IMPACT Registry is a repository of information for heart catheterizations for congenital heart disease regardless of age and also the catheterizations for acquired heart disease in children. The registry collects information on outcomes, provides quality improvement opportunities for participants, provides reports to participants comparing their results with national results, compares the volume of catheter work done at an institution with the frequency of adverse events, and provides information that may be helpful in evaluating the use of medical devices and treatment options...
May 14, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29761681/milestones-in-the-development-of-fetal-cardiac-interventions
#14
REVIEW
Edward G Abinader
Cardiac patients of all ages were managed in the past by internists who specialized in cardiology. During the past 50 years, the medical field has witnessed great strides in the management of congenital heart disease, and thus pediatric cardiology has become a subspecialty in many countries. This review article focuses on the advances in fetal cardiac interventions (FCI) since its inception by our group in 1975. Three major modes of FCI have evolved during the past 42 years: pharmacologic, closed FCI, and open FCI...
May 2018: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/29761422/computational-pre-surgical-planning-of-arterial-patch-reconstruction-parametric-limits-and-in-vitro-validation
#15
S Samaneh Lashkarinia, Senol Piskin, Tijen A Bozkaya, Ece Salihoglu, Can Yerebakan, Kerem Pekkan
Surgical treatment of congenital heart disease (CHD) involves complex vascular reconstructions utilizing artificial and native surgical materials. A successful surgical reconstruction achieves an optimal hemodynamic profile through the graft in spite of the complex post-operative vessel growth pattern and the altered pressure loading. This paper proposes a new in silico patient-specific pre-surgical planning framework for patch reconstruction and investigates its computational feasibility. The proposed protocol is applied to the patch repair of main pulmonary artery (MPA) stenosis in the Tetralogy of Fallot CHD template...
May 14, 2018: Annals of Biomedical Engineering
https://www.readbyqxmd.com/read/29761130/spinal-muscular-atrophy-with-respiratory-distress-type-1-a-child-with-atypical-presentation
#16
Annie Ting Gee Chiu, Sophelia Hoi Shan Chan, Shun Ping Wu, Shun Hin Ting, Brian Hon Yin Chung, Angel On Kei Chan, Virginia Chun Nei Wong
The authors report a child with spinal muscular atrophy with respiratory distress type 1 (SMARD1). She presented atypically with hypothyroidism and heart failure due to septal defects that required early heart surgery and microcephaly in association with cerebral atrophy and thin corpus collosum. The subsequent asymmetrical onset of diaphragmatic paralysis, persistent hypotonia, and generalized muscle weakness led to the suspicion of spinal muscular atrophy with respiratory distress type 1. Sanger sequencing confirmed a compound heterozygous mutation in the Immunoglobulin Mu Binding Protein 2 (IGHMBP2) gene, with a known mutation c...
2018: Child Neurology Open
https://www.readbyqxmd.com/read/29760348/what-causes-pulmonary-arterial-hypertension-in-down-syndrome-with-congenital-heart-disease
#17
Susumu Hosokawa, Rebecca R Vanderpool, Taku Ishii, Mitsunori Nishiyama, Shozaburo Doi
No abstract text is available yet for this article.
May 12, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/29759780/screening-for-optimal-cardiac-resynchronization-therapy-indication-in-congenital-heart-disease
#18
EDITORIAL
Jan Janoušek
No abstract text is available yet for this article.
August 2017: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/29759779/16-years-of-cardiac-resynchronization-pacing-among-congenital-heart-disease-patients-direct-contractility-dp-dt-max-screening-when-the-guidelines-do-not-apply
#19
Peter P Karpawich, Neha Bansal, Sharmeen Samuel, Yamuna Sanil, Kathleen Zelin
OBJECTIVES: The purpose of this study was to use direct cardiac resynchronization therapy (CRT)-paced contractility (dP/dt-max) response as a pre-implantation evaluation among patients with congenital heart disease (CHD) and follow clinical parameters and contractility indexes after CRT implantation. BACKGROUND: Patients with CHD often develop early heart failure with few therapeutic options, leading to heart transplantation (HT). Unfortunately, guidelines for CRT do not apply, and function evaluations by cardiac ultrasound are often inaccurate among CHD anatomies...
August 2017: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/29757759/mechanical-circulatory-support-of-the-right-ventricle-for-adult-and-pediatric-patients-with-heart-failure
#20
Steven G Chopski, Nohra M Murad, Carson S Fox, Randy M Stevens, Amy L Throckmorton
The clinical implementation of mechanical circulatory assistance for a significantly dysfunctional or failing left ventricle as a bridge-to-transplant or bridge-to-recovery is on the rise. Thousands of patients with left-sided heart failure are readily benefitting from these life-saving technologies, and left ventricular failure often leads to severe right ventricular dysfunction or failure. Right ventricular failure (RVF) has a high rate of mortality caused by the risk of multisystem organ failure and prolonged hospitalization for patients after treatment...
May 10, 2018: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
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