Abstract:
:Endothelial dysfunction has been reported in hypoxaemic patients with the Eisenmenger syndrome, but a direct correlation between levels of endothelial markers and the severity of hypoxaemia has not been explored. With this in mind, we compared the levels in the plasma of tissue-type plasminogen activator, thrombomodulin, and von Willebrand factor in 25 patients with the Eisenmenger syndrome. They had a median age of 31 years, and were divided into 2 groups according to their recent clinical history. Thus, 18 patients were stable, being in functional class II or III, seen as outpatients, and having peripheral saturations of oxygen of 89 plus or minus 5 percent. In contrast, 7 patients were unstable, showing episodes of symptoms placing them in functional class IV, requiring care in hospital, and manifesting saturations of oxygen of 77 plus or minus 5 percent. We were able to follow 12 patients, 8 who were stable and 4 unstable, for 24 months. At baseline, levels of von Willebrand factor were higher in the unstable patients when compared to those who were stable, at 142 plus or minus 29 and 110 plus or minus 25 units per decilitre, respectively (p equal to 0.013). This correlated positively with oxygen desaturation (p less than 0.020). The structural abnormalities also correlated positively with the magnitude of hypoxaemia (p less than 0.020). Levels remained higher in the unstable patients throughout the period of follow-up (p equal to 0.006). Tissue-type plasminogen activator was also increased, at 14.3 plus or minus 8.4 versus 6.5 plus or minus 2.7 nanograms per millilitre in controls (p less than 0.001), whereas thrombomodulin was decreased, with values of 14.4 versus 34.6 nanograms per millilitre in controls (p for median values of less than 0.001). There was no correlation with saturations of oxygen. We conclude that measurement of von Willebrand factor, as compared with tissue-type plasminogen activator and thrombomodulin, will prove a better marker of endothelial response to hypoxaemia in patients with the Eisenmenger syndrome.
journal_name
Cardiol Youngjournal_title
Cardiology in the youngauthors
de P S Soares R,Maeda NY,Bydlowski SP,Lopes AAdoi
10.1017/S1047951105001381keywords:
subject
Has Abstractpub_date
2005-10-01 00:00:00pages
504-13issue
5eissn
1047-9511issn
1467-1107pii
S1047951105001381journal_volume
15pub_type
杂志文章abstract::We report a case of unroofed coronary sinus not associated with the persistent left superior vena cava. Definite diagnosis of the unroofed coronary sinus was obtained by trans-oesophageal echocardiography, which revealed the unroofed portion with left-to-right shunt. Real-time three-dimensional trans-oesophageal echoc...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951113002400
更新日期:2015-01-01 00:00:00
abstract::The persistence of so-called spongy myocardium was first reported in humans in the 1960s. Subsequently, a few reports described the condition, utilizing inconsistent facts and terminology. In 1990 the first report appeared using the term "noncompaction", and detailed its clinical implications. Following this report, m...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951108001972
更新日期:2008-06-01 00:00:00
abstract:BACKGROUND:Care of children with functionally univentricular hearts is resource-intensive. OBJECTIVES:To analyse pregnancy and early post-natal outcomes of fetuses with functionally univentricular hearts in the setting of a low-middle-income country. METHODS:A retrospective study was conducted during the period of Ja...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120002929
更新日期:2020-12-01 00:00:00
abstract::Communications between the pulmonary artery and left atrium cause cyanosis. The images document serial haemodynamic changes in such a fistula from fetal life to the postnatal period with a successful transcatheter intervention. ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951118000616
更新日期:2018-10-01 00:00:00
abstract:OBJECTIVE:Prognosis of the transposition of the great arteries has completely changed since the introduction of the arterial switch. Time limit to perform this intervention is still controversial. The aim of this study is to demonstrate the early and late outcome of primary arterial switch operation beyond the age of m...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120001456
更新日期:2020-07-01 00:00:00
abstract:OBJECTIVE:Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on cl...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120000864
更新日期:2020-05-01 00:00:00
abstract:OBJECTIVE:The aim of this study was to determine developmental enamel defects and dental treatment conditions in children with congenital heart disease by comparing them with a control group of healthy children. METHODS:Children included in the study were referred to a paediatric dentistry for dental examination and t...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951113002308
更新日期:2015-02-01 00:00:00
abstract:OBJECTIVE:To evaluate the family psycho-social outcomes of children with Down syndrome and atrioventricular septal defect, and examine the impact of these variables on the child's neurodevelopmental outcome. METHODS:This was a cross-sectional study that consisted of 57 children with Down syndrome - 20 cases and 37 con...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951115000062
更新日期:2016-02-01 00:00:00
abstract::Introduction Neurodevelopmental abnormalities are common in children with CHD and are the highest-priority concerns for parents and professionals following cardiac surgery in childhood. There is no additional routine monitoring of development for children with CHD in the United Kingdom; hence, neurodevelopmental conce...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951117002918
更新日期:2018-04-01 00:00:00
abstract::Advances in biomedical engineering have led to three-dimensional (3D)-printed models being used for a broad range of different applications. Teaching medical personnel, communicating with patients and relatives, planning complex heart surgery, or designing new techniques for repair of CHD via cardiac catheterisation a...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951119000398
更新日期:2019-06-01 00:00:00
abstract::Improvements in diagnosis and surgical technique for correction have led to an increasing number of women with congenital heart disease reaching the child-bearing age. Pregnancy places considerable strain on the heart and circulation and necessitates marked cardiorespiratory adaptation. Today, with the exception of th...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/s1047951100007496
更新日期:1999-01-01 00:00:00
abstract::Supravalvular aortic stenosis is associated with the Williams-Beuren syndrome, but it also occurs in a non-syndromatic congenital form. An elastin gene mutation of chromosome 7q11.23 is responsible in both cases. The vascular features are identical. These patients have a higher risk of sudden death, particularly when ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951110001563
更新日期:2011-02-01 00:00:00
abstract::We report three cases of an abnormal finding of duplicated left pulmonary artery: two of these occurring in children with Kabuki syndrome and configuring the setting of a pseudo-pulmonary sling without any clinical or cardiac cross-sectional evidence of tracheal compression. The other case instead represents duplicate...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951115000281
更新日期:2016-02-01 00:00:00
abstract:INTRODUCTION:Dilatation of the ascending aorta is described in Turner's syndrome with variable prevalence (6.8-32%). Reported series typically include patients with associated cardiac anomalies. OBJECTIVE:To characterise the prevalence, age of onset, and the progress of dilatation of the ascending aorta in Turner's sy...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951115000682
更新日期:2016-03-01 00:00:00
abstract::We describe what is, to the best of our knowledge, a previously unreported association in patients with similar facial features, skin and joint laxity, of lengthening and tortuosity of systemic, pulmonary and coronary vessels. We evaluated 12 patients with similar phenotypes, from eight different families. Detailed ec...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/s1047951100008854
更新日期:2000-11-01 00:00:00
abstract::Cardiac anesthesia and critical care provide an important continuum of care for patients with congenital heart disease. Clinicians in both areas work in complex environments in which the interactions between humans and technology is critical. Understanding our contributions to outcomes (modifiable risk) and our abilit...
journal_title:Cardiology in the young
pub_type:
doi:10.1017/S104795111700213X
更新日期:2017-12-01 00:00:00
abstract:BACKGROUND:Risk for neurodevelopmental delay in infants and children with CHD is well established, but longer-term outcomes are equivocal. A meta-analysis was conducted to establish whether cognitive deficits remain beyond childhood - into teenage and young adult years. Methods and results A total of 18 unique samples,...
journal_title:Cardiology in the young
pub_type: 杂志文章,meta分析
doi:10.1017/S104795111700230X
更新日期:2018-03-01 00:00:00
abstract::CHD is associated with poor growth, delayed motor and language skills development, and increased length of hospital stay; 28.2% of infants were stunted, with z-scores<-2. The severity of surgery score was not associated with an increased length of stay, suggesting that a low weight-for-age z-score at the time of surge...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951115001778
更新日期:2016-04-01 00:00:00
abstract:OBJECTIVE:The objective of the study was to identify incidence, aetiology, and outcomes of extubation failure in infants with shunt-dependent pulmonary blood flow at a single tertiary care, academic children's hospital. The second objective of this study was to determine the haemodynamic effects of transition of positi...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951112002181
更新日期:2014-02-01 00:00:00
abstract::Milrinone is a drug frequently used for hemodynamic support in children during critical illness. Although the hemodynamic changes induced by milrinone in children may appear similar to those of adults, the physiologic contributors of these changes remain vastly unknown. A systematic review was conducted to identify st...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951119002865
更新日期:2020-01-01 00:00:00
abstract::The neuro-cutaneous syndrome tuberous sclerosis is commonly associated with rhabdomyomas in various organs including the heart. We are reporting a rare case of a 7-month old male child with congenitally corrected transposition of the great arteries associated with tuberous sclerosis and cardiac rhabdomyomas. To our kn...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951113002047
更新日期:2014-10-01 00:00:00
abstract::Intercostal aneurysms are associated with aortic coarctation. Their aetiology is not well-understood but may be related to intrinsic vascular pathology and altered flow dynamics through the intercostal artery. We present the cases of two patients with coarctation and intercostal aneurysms. The aneurysms were recognise...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S104795111500116X
更新日期:2016-02-01 00:00:00
abstract::In many parts of the world, mostly low- and middle-income countries, timely diagnosis and repair of congenital heart diseases (CHDs) is not feasible for a variety of reasons. In these regions, economic growth has enabled the development of cardiac units that manage patients with CHD presenting later than would be idea...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951117002591
更新日期:2017-12-01 00:00:00
abstract::To determine whether cardiac catheterisation procedures for low birth weight neonatesr < or = 2.5 kg carries a greater risk of complications compared with neonates > 2.5 kg, we conducted a single-centre retrospective case–control study. From 01/03 to 01/09, 46 consecutive neonates < 2.5 kg at the time of cardiac cathe...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951112002247
更新日期:2013-10-01 00:00:00
abstract:OBJECTIVE:To describe the gross motor development of infants who had undergone cardiac surgery in the neonatal or early infant period. METHODS:Gross motor performance was assessed when infants were 4, 8, 12, and 16 months of age with the Alberta Infant Motor Scale. This scale is a discriminative gross motor outcome me...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951112000121
更新日期:2012-10-01 00:00:00
abstract::The Holt-Oram syndrome was confirmed in an asymptomatic 36-year-old man by a novel TBX5-gene mutation (exon 8 acceptor splicing site, c.663-1G greater than A). Computed tomography showed an atrial septal defect and an anomalous right coronary artery crossing between the aorta and pulmonary arteries. Surgery corrected ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951111000072
更新日期:2011-06-01 00:00:00
abstract:BACKGROUND:Anomalous left coronary artery from the pulmonary artery is a rare congenital heart disease (CHD) with diverse clinical presentations despite the same anatomy. Factors determining this heterogeneous presentation are not well understood. METHOD AND RESULTS:We retrospectively investigated 14 patients (12 fema...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951119003299
更新日期:2020-03-01 00:00:00
abstract:OBJECTIVES:It is accepted treatment to give vasopressin to adults in postcardiotomy shock, but such use in children is controversial. Cardiopulmonary bypass is presumed to attenuate the normal endogenous vasopressin response to shock. We hypothesized that levels of vasopressin in children are altered by bypass, and tha...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951108001881
更新日期:2008-04-01 00:00:00
abstract::The Fellowship Program of the Department of Cardiology at Boston Children's Hospital seeks to train academically oriented leaders in clinical care and laboratory and clinical investigation of cardiovascular disease in the young. The core clinical fellowship involves 3 years in training, comprising 24 months of clinica...
journal_title:Cardiology in the young
pub_type: 历史文章,杂志文章
doi:10.1017/S1047951116001712
更新日期:2016-12-01 00:00:00
abstract:OBJECTIVE:To assess haemodynamic patterns in head-up tilt testing on 400 paediatric cases with unexplained syncope. METHODS:Medical records of 520 children who underwent head-up tilt testing in the preceding year were retrospectively evaluated, and 400 children, 264 (66%) girls and 136 (34%) boys, aged 12.6±2.6 years ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951114001176
更新日期:2015-06-01 00:00:00