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treatment of congenital heart disease

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https://www.readbyqxmd.com/read/28932552/chylothorax-after-pediatric-cardiac-surgery-complicates-short-term-but-not-long-term-outcomes-a-propensity-matched-analysis
#1
Nikoletta R Czobor, György Roth, Zsolt Prodán, Daniel J Lex, Erzsébet Sápi, László Ablonczy, Mihály Gergely, Edgar A Székely, János Gál, Andrea Székely
BACKGROUND: The occurrence of postoperative chylothorax in children with congenital heart disease is a rare and serious complication in cardiac intensive care units (ICUs). The aim of our study was to identify the perioperative characteristics, treatment options, resource utilization and long term complications of patients having chylothorax after a pediatric cardiac surgery. METHODS: Patients were retrospectively assessed for the presence of chylothorax between January 2002 and December 2012 in a tertiary national cardiac center...
August 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28923474/beyond-the-inhaled-nitric-oxide-in-persistent-pulmonary-hypertension-of-the-newborn
#2
REVIEW
Mei-Yin Lai, Shih-Ming Chu, Satyan Lakshminrusimha, Hung-Chih Lin
Persistent pulmonary hypertension (PPHN) is a consequence of failed pulmonary vascular transition at birth and leads to pulmonary hypertension with shunting of deoxygenated blood across the ductus arteriosus (DA) and foramen ovale (FO) resulting in severe hypoxemia, and it may eventually lead to life-threatening circulatory failure. PPHN is a serious event affecting both term and preterm infants in the neonatal intensive care unit. It is often associated with diseases such as congenital diaphragmatic hernia, meconium aspiration, sepsis, congenital pneumonia, birth asphyxia and respiratory distress syndrome...
August 10, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28915908/advances-in-paediatrics-in-2016-current-practices-and-challenges-in-allergy-autoimmune-diseases-cardiology-endocrinology-gastroenterology-infectious-diseases-neonatology-nephrology-neurology-nutrition-pulmonology
#3
REVIEW
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Silvia Montella, Sergio Bernasconi
This review reports main progresses in various pediatric issues published in Italian Journal of Pediatrics and in international journals in 2016. New insights in clinical features or complications of several disorders may be useful for our better understanding. They comprise severe asthma, changing features of lupus erythematosus from birth to adolescence, celiac disease, functional gastrointestinal disorders, Moebius syndrome, recurrent pneumonia. Risk factors for congenital heart defects, Kawasaki disease have been widely investigated...
September 16, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28914735/palliative-care-in-neuromuscular-diseases
#4
Marianne de Visser, David J Oliver
PURPOSE OF REVIEW: Palliative care is an approach that improves the quality of life of patients and their families facing the problem associated with life-threatening illness. Neuromuscular disorders (NMDs) are characterized by progressive muscle weakness, leading to pronounced and incapacitating physical disabilities. Most NMDs are not amenable to curative treatment and would thus qualify for palliative care. Amyotrophic lateral sclerosis is a relentlessly progressive disease, which leads to death about 2 years after onset due to respiratory muscle weakness...
September 13, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28914109/stem-cell-therapy-for-the-systemic-right-ventricle
#5
Ming-Sing Si, Richard G Ohye
In specific forms of congenital heart defects and pulmonary hypertension, the right ventricle (RV) is exposed to systemic levels of pressure overload. The RV is prone to failure in these patients because of its vulnerability to chronic pressure overload. As patients with a systemic RV reach adulthood, an emerging epidemic of RV failure has become evident. Medical therapies proven for LV failure are ineffective in treating RV failure. Areas covered: In this review, the pathophysiology of the failing RV under pressure overload is discussed, with specific emphasis on the pivotal roles of angiogenesis and oxidative stress...
September 15, 2017: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/28905004/gastroenteritis-aggressive-versus-slow-treatment-for-rehydration-gastro-a-pilot-rehydration-study-for-severe-dehydration-who-plan-c-versus-slower-rehydration
#6
Kirsty A Houston, Jack G Gibb, Ayub Mpoya, Nchafatso Obonyo, Peter Olupot-Olupot, Margeret Nakuya, Jennifer A Evans, Elizabeth C George, Diana M Gibb, Kathryn Maitland
BACKGROUND: The World Health Organization (WHO) rehydration management guidelines (Plan C) for children with acute gastroenteritis (AGE) and severe dehydration are widely practiced in resource-poor settings, yet have never been formally evaluated in a clinical trial. A recent audit of outcome of AGE at Kilifi County Hospital, Kenya noted that 10% of children required high dependency care (20% mortality) and a number developed fluid-related complications. The fluid resuscitation trial, FEAST, conducted in African children with severe febrile illness, demonstrated higher mortality with fluid bolus therapy and raised concerns regarding the safety of rapid intravenous rehydration therapy...
2017: Wellcome Open Research
https://www.readbyqxmd.com/read/28902362/histone-lysine-methylation-and-congenital-heart-disease-from-bench-to-bedside-review
#7
Xin Yi, Xuejun Jiang, Xiaoyan Li, Ding-Sheng Jiang
Histone post-translational modifications (PTM) as one of the key epigenetic regulatory mechanisms that plays critical role in various biological processes, including regulating chromatin structure dynamics and gene expression. Histone lysine methyltransferase contributes to the establishment and maintenance of differential histone methylation status, which can recognize histone methylated sites and build an association between these modifications and their downstream processes. Recently, it was found that abnormalities in the histone lysine methylation level or pattern may lead to the occurrence of many types of cardiovascular diseases, such as congenital heart disease (CHD)...
October 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28884218/use-of-levosimendan-in-postoperative-setting-after-surgical-repair-of-congenital-heart-disease-in-children
#8
Vivianne Amiet, Marie-Hélène Perez, David Longchamp, Tatiana Boulos Ksontini, Julia Natterer, Sonia Plaza Wuthrich, Jacques Cotting, Stefano Di Bernardo
Low cardiac output is one of the most common complications after cardiac surgery. Levosimendan, a new inotrope agent, has been demonstrated in adult patient to be an effective treatment for this purpose when classical therapy is not effective. The aim of this study was to evaluate the effect of Levosimendan on cardiac output parameters in cardiac children with low cardiac output syndrome (LCOS.). We carried out a retrospective analysis on 62 children hospitalized in our pediatric intensive care unit (PICU) after cardiac surgery, which demonstrated LCOS not responding to classical catecholamine therapy and who received levosimendan as rescue therapy...
September 7, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28882885/modeling-major-adverse-outcomes-of-pediatric-and-adult-patients-with-congenital-heart-disease-undergoing-cardiac-catheterization-observations-from-the-ncdr-impact-registry
#9
Natalie Jayaram, John A Spertus, Kevin F Kennedy, Robert Vincent, Gerard R Martin, Jeptha P Curtis, David G Nykanen, Phillip M Moore, Lisa Bergersen
Background -Risk-standardization for adverse events following congenital cardiac catheterization is needed to equitably compare patient outcomes among different hospitals as a foundation for quality improvement. The goal of this project was to develop a risk-standardization methodology to adjust for patient characteristics when comparing major adverse outcomes in the NCDR® IMPACT(TM) (Improving Pediatric and Adult Congenital Treatment) Registry. Methods -39,725 consecutive patients within IMPACT undergoing cardiac catheterization between January 2011 and March 2014 were identified...
September 7, 2017: Circulation
https://www.readbyqxmd.com/read/28879342/anesthetic-management-of-a-patient-with-chromosome-6p-duplication-a-case-report
#10
Saori Morinaga, Masanori Tsukamoto, Takeshi Yokoyama
Chromosome 6p duplication is very rare and clinically characterized by short stature, mental retardation, and congenital heart diseases. Patients with mental retardation may present with poor oral health conditions. Dental treatment may need to be performed under general anesthesia in such patients. Our case report deals with induction of general anesthesia to a patient with chromosome 6p duplication, for dental treatment. The selection of a nasotracheal tube of an appropriate size, because of the patient's short stature, was especially important for airway management...
June 2017: J Dent Anesth Pain Med
https://www.readbyqxmd.com/read/28878122/wnt11-regulates-cardiac-chamber-development-and-disease-during-perinatal-maturation
#11
Marlin Touma, Xuedong Kang, Fuying Gao, Yan Zhao, Ashley A Cass, Reshma Biniwale, Xinshu Xiao, Mansuoreh Eghbali, Giovanni Coppola, Brian Reemtsen, Yibin Wang
Ventricular chamber growth and development during perinatal circulatory transition is critical for functional adaptation of the heart. However, the chamber-specific programs of neonatal heart growth are poorly understood. We used integrated systems genomic and functional biology analyses of the perinatal chamber specific transcriptome and we identified Wnt11 as a prominent regulator of chamber-specific proliferation. Importantly, downregulation of Wnt11 expression was associated with cyanotic congenital heart defect (CHD) phenotypes and correlated with O2 saturation levels in hypoxemic infants with Tetralogy of Fallot (TOF)...
September 7, 2017: JCI Insight
https://www.readbyqxmd.com/read/28871660/clinical-echocardiographic-and-therapeutic-aspects-of-congenital-heart-diseases-of-children-at-douala-general-hospital-a-cross-sectional-study-in-sub-saharan-africa
#12
Félicité Kamdem, Danielle Kedy Koum, Ba Hamadou, Mélanie Yemdji, Henry Luma, Marie Solange Doualla, Diomède Noukeu, Esther Barla, Christophe Akazong, Anastase Dzudie, Henry Ngote, Yves Monkam, Sidiki Mouliom, Samuel Kingue
INTRODUCTION: Cardiovascular diseases in pediatric pathologies have emerged in the recent years in sub-Saharan Africa (SSA), with congenital heart diseases (CHDs) being the most frequent. Unfortunately, their diagnosis is usually delayed, thereby increasing childhood morbidity and mortality. OBJECTIVES: Describe the clinical, echocardiographic, and therapeutic aspects of CHDs of children at Douala General Hospital. METHODS: We carried out a cross-sectional descriptive study over a 10-year period, from January 2006 to December 2015...
September 4, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28858551/the-anesthetic-management-for-a-patient-with-trisomy-13
#13
Masanori Tsukamoto, Takashi Hitosugi, Kanako Esaki, Takeshi Yokoyama
Trisomy 13 is a chromosomal disorder that occurs in complete or partial mosaic forms. It is characterized by central apnea, mental retardation, seizure and congenital heart disease. The survival of the patients with trisomy 13 is the majority dying before one month. Trisomy 13 is the worst life prognosis among all trisomy syndromes. It is reported the cause of death is central apnea. Special needs patients with mental retardation are recognized to have poorer oral health condition. Oral health related quality of life reflects daily activity and well-being...
2017: Anesthesia Progress
https://www.readbyqxmd.com/read/28852106/the-role-of-na-ca2-exchanger-1-in-maintaining-ductus-arteriosus-patency
#14
Minghui Li, Chuan Jiang, Lincai Ye, Shoubao Wang, Haibo Zhang, Jinfen Liu, Haifa Hong
Patency of the ductus arteriosus (DA) is crucial for both fetal circulation and patients with DA-dependent congenital heart diseases (CHD). The Na(+)/Ca(2+) exchanger 1 (NCX1) protein has been shown to play a key role in the regulation of vascular tone and is elevated in DA-dependent CHD. This current study was conducted to investigate the mechanisms underpinning the role of NCX1 in DA patency. Our data showed NCX1 expression was up-regulated in the DA of fetal mice. Up-regulation of NCX1 expression resulted in a concomitant decrease in cytosolic Ca(2+) levels in human DA smooth muscle cells (DASMCs) and an inhibition of the proliferation and migration capacities of human DASMCs...
August 29, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28843326/valve-interventions-in-utero-understanding-the-timing-indications-and-approaches
#15
REVIEW
Aleksander Sizarov, Younes Boudjemline
Efficient use of fetal echocardiography has enabled early detection of congenital heart disease and of its often irreversible complications, such as ventricular hypoplasia in case of severe stenosis of the semilunar valves. Experience of the past 25 years has proved that balloon dilatation of the severely stenotic or atretic valve in fetuses as early as the 23rd week of gestation is technically feasible with a learning curve. Reported results regarding the ultimate biventricular circulation outcome after fetal valve intervention are at best controversial, with the desired improvements in the quality of life and cost-benefits of the postnatal treatment being as yet unconfirmed...
September 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28840327/the-safety-and-effectiveness-of-flecainide-in-children-in-the-current-era
#16
Taylor Cunningham, Orhan Uzun, Rachel Morris, Sonia Franciosi, Amos Wong, Ida Jeremiasen, Elizabeth Sherwin, Shubhayan Sanatani
This retrospective study sought to determine the safety and effectiveness of flecainide in children with normal hearts and those with congenital heart disease (CHD) or cardiomyopathy (CMO). Baseline and follow-up data at two pediatric cardiology sites were queried (2000-2015); a total of 175 patients (20 with CHD and two with CMO) receiving flecainide were assessed. When comparing patients with CHD to those with normal hearts, patients with CHD were younger at diagnosis (median age 19 days; IQR 3-157.5 days vs normal heart patients median age 21 days; IQR 7-172 days, p = 0...
August 24, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28828325/evaluation-of-death-among-the-patients-undergoing-permanent-pacemaker-implantation-a-competing-risks-analysis
#17
Haleh Ghaem, Mohammad Ghorbani, Samira Zare Dorniani
BACKGROUND: Permanent artificial pacemaker is one of the important therapies for treatment of cardiac conduction system problems. The present study aimed to determine the association between some predictive variables and all-cause and cause-specific mortality in the patients who had undergone pacemaker implantation. METHODS: This study was conducted on 1207 patients who had undergone permanent pacemaker implantation in the hospitals affiliated with Shiraz University of Medical Sciences, Iran, from Mar 2002 to Mar 2012...
June 2017: Iranian Journal of Public Health
https://www.readbyqxmd.com/read/28794135/pulmonary-vasodilator-therapy-is-associated-with-greater-survival-in-eisenmenger-syndrome
#18
Clare Arnott, Geoff Strange, Andrew Bullock, Adrienne C Kirby, Clare O'Donnell, Dorothy J Radford, Leeanne E Grigg, David S Celermajer
OBJECTIVE: Eisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT). METHODS: From January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand...
August 9, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28790229/autoimmune-disease-with-cardiac-valves-involvement-libman-sacks-endocarditis
#19
Eka Ginanjar, Yulianto Yulianto
This case study aim to evaluate the response of steroid treatment for autoimmune endocarditis. Valvular heart disease is relatively rising in both congenital and acquired cases, but the autoimmune endocarditis remains rare. In this case, a 34 year old woman with clinical manifestation resembling systemic lupus erythematosus (SLE) is diagnosed with Libman-sacks Endocarditis. After six months of steroid treatment, her clinical manifestations and heart structure improved.
April 2017: Acta Medica Indonesiana
https://www.readbyqxmd.com/read/28783102/the-pulmonary-circulation-in-the-single-ventricle-patient
#20
REVIEW
Amanda Hauck, Nicolas Porta, Steven Lestrud, Stuart Berger
In recent decades, survival of children with complex congenital heart disease has improved considerably. Specifically, children with a variety of congenital heart defects resulting in 'single ventricle' physiology can now undergo palliative surgery that allows survival beyond the neonatal period, and in many cases into adulthood, despite having a single functional ventricular pumping chamber supplying both the pulmonary and systemic circulation. Our growing understanding of the functionally univentricular heart has resulted in freedom from Fontan failure of >50% at 25 years post-Fontan...
August 7, 2017: Children
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