Abstract:
:Cognitive, functional, educational achievement and behavioural measures were employed to assess neurobehavioral status in 57 of 60 participants who were initially enrolled in the Baltimore-Washington Infant Study, and who survived surgical correction of complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections). Charts were reviewed to investigate the relationship between birth variables, surgical strategy and developmental outcomes. Higher preoperative weight was associated with better outcomes on the Stanford-Binet Short-term Memory subtest, while lower preoperative oxygen tension was associated with better outcomes on the Abstract/Visual Reasoning subtest and a test of Visual-Motor Integration. Longer total bypass time was associated with poor outcomes on the Short-term Memory subtests. Higher average flow rates during cooling and rewarming were associated with higher scores in the test of short term memory but poorer outcomes on a test for visual motor integration. Longer cooling times were associated with higher scores on the test for Visual-Motor Integration. Patients suffering seizures scored lower on the Stanford-Binet Composite, as well as in their tests of achievement. The data indicate that non-verbal skills may be particularly sensitive to variations in surgical strategies employed to correct complete transposition. Overt neurological events, such as seizures, were related to global deficits in intellectual functioning. Prospective studies evaluating systemic variations in surgical procedures and attempts to prevent and manage perioperative neurological events are important for further investigation of neurodevelopmental outcomes in children surviving surgical correction.
journal_name
Cardiol Youngjournal_title
Cardiology in the youngauthors
Gomelsky A,Holden EW,Ellerbeck KA,Brenner JIdoi
10.1017/s1047951100006867subject
Has Abstractpub_date
1998-07-01 00:00:00pages
352-7issue
3eissn
1047-9511issn
1467-1107journal_volume
8pub_type
杂志文章,多中心研究abstract:BACKGROUND:SCN5A encodes sodium-channel α-subunit Nav1.5. The mutations of SCN5A can lead to hereditary cardiac arrhythmias such as the long-QT syndrome type 3 and Brugada syndrome. Here we sought to identify novel mutations in a family with arrhythmia. METHODS:Genomic DNA was isolated from blood of the proband, who w...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951117002980
更新日期:2018-05-01 00:00:00
abstract::We present two cases of percutaneous Sapien XT valve-in-valve implantation in the tricuspid position: a 20-year-old man with severe congenital pulmonary stenosis and percutaneous valvuloplasty, who required surgical implantation of two protheses, pulmonary and tricuspid, and a 12-year-old boy with CHD and a degenerate...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951117000786
更新日期:2017-10-01 00:00:00
abstract:AIM:In present study, we aimed to evaluate the changes in valvular regurgitations in mid-term follow-up of children with first attack acute rheumatic fever diagnosed after updated Jones criteria. MATERIALS AND METHODS:The medical records of the children diagnosed with acute rheumatic fever between June 2015 and Novemb...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951119003317
更新日期:2020-03-01 00:00:00
abstract::A newborn presented with an aorto-right ventricular tunnel, a defect connecting the left aortic sinus to the right ventricle. The patient underwent repair on 4th day of life. ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951115001572
更新日期:2016-01-01 00:00:00
abstract:OBJECTIVES:The purpose of our study was to assess the prevalence and progression, during childhood and adolescence, of dilation of the neo-aortic root, and neo-aortic valvar regurgitation, and to identify risk factors for such dilation and regurgitation, after the arterial switch operation. METHODS:We included all pat...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951106000953
更新日期:2006-10-01 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::A bicuspid aortic valve is not only a common congenital heart defect but also an enigmatic condition that can cause a large spectrum of diseases, such as aortic valve stenosis and severe heart failure in newborns whereas aortic dissection in adults. On the contrary, a bicuspid aortic valve can also occur with normal f...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951118001658
更新日期:2019-01-01 00:00:00
abstract:BACKGROUND:We previously reported four heterozygous missense mutations of MYH7, KCNQ1, MYLK2, and TMEM70 in a single three-generation Chinese family with dual Long QT and hypertrophic cardiomyopathy phenotypes for the first time. However, the clinical course among the family members was various, and the potential myoca...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951118000860
更新日期:2018-09-01 00:00:00
abstract::We describe a patient with the rare association of the heart in the left chest, congenitally corrected transposition, pulmonary atresia and an intact ventricular septum. There were associated fistulous communications between the morphologically left ventricle and the coronary arteries. Diagnosis was made by echocardio...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/s1047951100009227
更新日期:2000-05-01 00:00:00
abstract::We present a case of circumferential fracture of aortic coarctation stent with severe re-stentosis presenting 16 years after initial stent implantation with end-stage renal disease. The patient was treated with a covered stent using the stent-in-stent technique. The use of an ultra-high-pressure balloon was proved nec...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951115001079
更新日期:2016-02-01 00:00:00
abstract:BACKGROUND:Kawasaki disease, which is characterised by systemic vasculitides accompanied by acute fever, is regularly treated by intravenous immunoglobulin to avoid lesion formation in the coronary artery; however, the mechanism of intravenous immunoglobulin therapy is unclear. Hence, we aimed to analyse the global exp...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951117001433
更新日期:2017-11-01 00:00:00
abstract::Transcatheter pulmonary valve replacement is fast becoming an accepted alternative to repeat surgical pulmonary valve replacement for selected patients and therefore a complementary strategy in the long-term management of those requiring surgical pulmonary valve replacement. With a combined surgical and percutaneous a...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951112001527
更新日期:2012-12-01 00:00:00
abstract::We present a case of a 31-year-old male with a large atrial septal defect, who was found to have interrupted inferior caval vein with azygous continuation to the superior caval vein, which precluded transcutaneous closure by device. The defect was successfully closed with a 33 mm Occlutech Figula septal occluder using...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S104795112000092X
更新日期:2020-06-01 00:00:00
abstract::A non-dysmorphic 10 month old female was discovered at surgery to have severe vasculopathy of both the systemic and pulmonary arteries. These findings were confirmed by pathologic examination. Follow-up angiography has confirmed multiple sites of vascular obstruction which appear to be worsening. Angioplasty has only ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/s104795110200063x
更新日期:2002-05-01 00:00:00
abstract::Gaucher disease is an autosomal-recessive lysosomal storage disease characterised by the accumulation of glucocerebroside in macrophages; it is caused by mutations in glucocerebrosidase gene-1 in many organ tissues such as the liver, spleen, and bone marrow. Its different clinical subtypes, according to the presence a...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951117000579
更新日期:2017-09-01 00:00:00
abstract::Congenital complete tracheal rings are usually associated with pulmonary slings. We report a rare association of congenital complete tracheal rings with hypoplastic left heart variant. A term infant with diagnosis of a mildly hypoplastic mitral valve, unicuspid aortic valve, and moderately hypoplastic aortic arch with...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951118002391
更新日期:2019-03-01 00:00:00
abstract:OBJECTIVES:The objective of this study was to demonstrate the safety and feasibility of using the new Cardia Ultrasept II™ device with interposed Goretex patch referring to the perforation of polyvinyl alcohol membrane. BACKGROUND:Great advances have been made in the development of devices for closure of atrial septal...
journal_title:Cardiology in the young
pub_type: 杂志文章,多中心研究
doi:10.1017/S1047951118000100
更新日期:2018-05-01 00:00:00
abstract:PURPOSE:The aim of this study was to compare associations between generic versus disease-specific functional health status assessments and patient and clinical characteristics for patients with severe congenital heart disease. METHODS:This was a cross-sectional observational study involving 325 single ventricle patien...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951113000632
更新日期:2014-06-01 00:00:00
abstract::An acute injury to a coronary artery was recognized during the surgical construction of the Fontan circulation. Surgical manipulation of the site of injury was not successful in restoring normal myocardial blood flow. A stent was therefore placed intraoperatively under direct vision, with restoration of normal coronar...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/s1047951104001180
更新日期:2004-02-01 00:00:00
abstract::Percutaneous treatment of aortic coarctation is a widely used option. Covered stents have increased the profile of efficacy and safety of this procedure. Here we report on a 32-year-old woman with significant aortic recoarctation associated with aortic wall aneurysm and close proximity of both lesions to the origin of...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951114001061
更新日期:2015-04-01 00:00:00
abstract::Innocent murmurs are common in childhood. They require accurate diagnosis to avoid unnecessary anxiety and/or restrictions. With this in mind, we studied prospectively children diagnosed by a paediatric cardiologist as having innocent murmurs to review the necessity of investigations in excluding organic abnormalities...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/s104795110000963x
更新日期:2000-10-01 00:00:00
abstract:BACKGROUND:Acute rheumatic carditis is still an important cause of cardiac failure in developing countries. B-type natriuretic peptides, especially N-terminal segment of its prohormone are now recognised as essential parts of cardiologic evaluation. Increased plasma concentrations of B-type natriuretic peptide and its ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951110000089
更新日期:2010-06-01 00:00:00
abstract::An 8-year-old girl, without any previous medical history, presented with a first short syncope. Physical examination was unremarkable. Transthoracic echocardiography revealed a thin-walled, echo-free cystic structure adjacent to the posterior wall of the left ventricle, and compressing it moderately. Other echocardiog...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/s1047951101001056
更新日期:2001-11-01 00:00:00
abstract:OBJECTIVE:To examine the clinical utility of the Pediatric Symptom Checklist 17 for identifying psychosocial concerns and improving access to psychology services within a paediatric cardiology clinic. METHOD:Parents of 561 children (aged 4-17 years) presenting for follow-up of CHD, acquired heart disease, or arrhythmi...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951115001900
更新日期:2016-08-01 00:00:00
abstract::This review includes a brief discussion, from the perspective of cardiac surgeons, of the rationale for creation and maintenance of multi-institutional databases of outcomes of congenital heart surgery, together with a history of the evolution of such databases, a description of the current state of the art, and a dis...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951108002813
更新日期:2008-12-01 00:00:00
abstract::A percutaneous transcatheter balloon dilation of a pulmonary venous pathway obstruction was successfully performed in a 40-year-old patient after a Mustard procedure. During the procedure, real-time three-dimensional trans-oesophageal echocardiography demonstrated the morphology of the obstruction. Our case highlights...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951119001045
更新日期:2019-07-01 00:00:00
abstract::A 29-year-old gravida1 para1 woman presented with increasing fatigue. Multi-modality imaging demonstrated the left coronary artery arising from the main pulmonary artery with large collateral vessels in the interventricular septum, in keeping with unrepaired anomalous left coronary artery from the pulmonary artery. Ca...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951117000932
更新日期:2017-11-01 00:00:00
abstract:BACKGROUND:The relationship between sildenafil dosing, exposure, and systemic hypotension in infants is incompletely understood. OBJECTIVES:The aim of this study was to characterise the relationship between predicted sildenafil exposure and hypotension in hospitalised infants. METHODS:We extracted information on sild...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951117001639
更新日期:2018-01-01 00:00:00
abstract:BACKGROUND:Cardiopulmonary bypass is associated with systemic inflammatory response. Steroids suppress this response, although the therapeutic evidence remains controversial. We hypothesised that intravenous steroids in children undergoing open-heart surgery would decrease inflammation leading to better early post-oper...
journal_title:Cardiology in the young
pub_type: 杂志文章,随机对照试验
doi:10.1017/S1047951115000566
更新日期:2016-03-01 00:00:00
abstract:BACKGROUND:Adverse influences arising in fetal life or immediately after birth have a permanent effect on body structure, physiology and metabolism. Evidence is now accumulating that programming of bone growth might be an important contributor to the later risk of osteoporosis. Long-term morbidity and mortality associa...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/s1047951102000963
更新日期:2002-12-01 00:00:00