Abstract:
OBJECTIVE:Cardiovascular lesions are the leading cause of morbidity and mortality in patients with Williams syndrome. Recent studies have rebutted conventional reports about the natural course of cardiovascular anomalies in Williams syndrome. DESIGN:Retrospective study. SETTING:Single tertiary center. PATIENTS:Eighty patients with Williams syndrome followed up for more than 5 years. INTERVENTIONS:Not applicable. OUTCOME MEASURES:Long-term outcome of cardiovascular lesions, peak velocity change in obstructive cardiovascular lesions over time, post-interventional courses of disease-specific intervention, and intervention-free survival of obstructive cardiovascular lesions. RESULTS:The median follow-up duration was 11.0 (5.1-28.3) years. Among 80 patients, supravalvular aortic stenosis (87.5%) was the most common cardiovascular lesion, followed by branch pulmonary stenosis (53.8%), mitral valve prolapse (22.5%), and aortic arch hypoplasia/coarctation (5.0%). During the follow-up period, the peak flow velocity of supravalvular aortic stenosis did not change on peak Doppler echocardiography. Initially, severe supravalvular aortic stenosis was aggravated (P < .027). Conversely, the peak velocity of branch pulmonary stenosis decreased (from 3.08 to 1.65 m/s; P < .001) within age 3.2 (0.4-6.9) years. Even the group with severe branch PS improved over time. Twenty-two patients (27.5%) with Williams syndrome underwent disease-specific interventions without mortality, mostly for supravalvular aortic stenosis or mitral valve prolapse. No patient in the late-onset and initially mild supravalvular aortic stenosis group needed intervention and 37.5%, 48.4%, and 65.1% in initially moderate and severe supravalvular aortic stenosis groups needed intervention at age 5, 10, and 20 years, respectively. Unlike the conventional therapeutic concept, the intervention for branch pulmonary stenosis was almost unnecessary. CONCLUSIONS:In Williams syndrome, initially severe supravalvular aortic stenosis worsened over time and most branch pulmonary stenoses, including those in the severe group, improved spontaneously. Most patients with branch pulmonary stenosis did not require disease-specific intervention. Surgical repairs for cardiovascular abnormalities in Williams syndrome showed favorable results.
journal_name
Congenit Heart Disjournal_title
Congenital heart diseaseauthors
Cha SG,Song MK,Lee SY,Kim GB,Kwak JG,Kim WH,Bae EJdoi
10.1111/chd.12810subject
Has Abstractpub_date
2019-09-01 00:00:00pages
684-690issue
5eissn
1747-079Xissn
1747-0803journal_volume
14pub_type
杂志文章abstract:OBJECTIVE:It was examined whether women and men (17-45 years) with operated congenital heart disease differ with respect to chances of employment. Patients were compared with the general population. DESIGN:Patients (n = 314) were classified by type of surgery (curative, reparative, palliative) as indicator of initial ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2008.00239.x
更新日期:2009-01-01 00:00:00
abstract:OBJECTIVE:The purpose of this study was to compare neurological development of children with cyanotic or hemodynamically impaired congenital heart disease (CHD) and healthy controls by using "Bayley Scales of Infant and Toddler Development Screening Test, Third Edition" (Bayley-III). PATIENTS:Children with CHD (n = 37...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12269
更新日期:2015-11-01 00:00:00
abstract:INTRODUCTION:Health care-associated infections (HAIs) increase mortality, length of stay, and cost in hospitalized patients. The incidence of and risk factors for developing HAIs in the pediatric population after cardiac surgery have been studied. This study evaluates the impact of HAIs on length of stay, inpatient mor...
journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究
doi:10.1111/chd.12779
更新日期:2019-09-01 00:00:00
abstract:OBJECTIVE:It is unclear whether neonates with interrupted aortic arch (IAA) and a smaller left ventricular outflow tract may have improved outcomes with a Yasui operation (ventricular outflow bypass procedure) over a primary complete repair. This study sought to identify preoperative echocardiographic parameters to dif...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12599
更新日期:2018-05-01 00:00:00
abstract:BACKGROUND:Children and adolescents with congenital heart disease (CHD) and their families require qualified combined medical and psychosocial information, care, and counseling. This study aimed to analyze CHD patients' and parents' perception of disease-specific knowledge, state of health, and impairments experienced ...
journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究
doi:10.1111/chd.12581
更新日期:2018-05-01 00:00:00
abstract::Iron deficiency state in patients with cyanotic congenital heart disease can mimic as well as aggravate hyperviscosity symptoms. Correction of iron deficiency in these cases is expected to improve symptoms. We report an unexpected occurrence of refractory cyanotic spell in a child with tetralogy of Fallot due to throm...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00608.x
更新日期:2012-07-01 00:00:00
abstract::Clinically significant patent ductus arteriosus (PDA) has been associated with significant morbidity in extremely low birth weight (ELBW) infants. Current management of ELBW infants with hemodynamically significant PDA includes supportive treatment, pharmacological therapy, and surgical ligation. All of these therapeu...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12712
更新日期:2019-01-01 00:00:00
abstract:BACKGROUND:Deficits in swallowing physiology are a leading morbidity for infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliation. Despite the high prevalence of this condition, the underlying deficits that cause this post-operative impairment remain poorly understoo...
journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究
doi:10.1111/chd.12456
更新日期:2017-05-01 00:00:00
abstract:OBJECTIVE:The aims of this study were to compare the level of posttraumatic stress disorder between adults with and without congenital heart disease, and to examine the correlates of posttraumatic stress disorder (e.g., sociodemographics). DESIGN:Cross-sectional. SETTING:Two university-affiliated heart hospitals in T...
journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1111/chd.12452
更新日期:2017-05-01 00:00:00
abstract:OBJECTIVE:This study aimed to quantitatively compare findings of emotional functioning across studies of adolescents and adults with congenital heart disease (CHD) through meta-analysis. DESIGN:The current meta-analysis included 22 studies of adolescent and adult survivors of CHD who completed measures of emotional fu...
journal_title:Congenital heart disease
pub_type: 杂志文章,meta分析,评审
doi:10.1111/chd.12178
更新日期:2015-01-01 00:00:00
abstract:OBJECTIVE:We aimed to study the efficiency and safety of once-a-week outpatient rehabilitation followed by home program with tele-monitoring in patients with complex cyanotic congenital heart disease. DESIGN:Prospective nonrandomized study. METHOD:Patients who have been diagnosed either Eisenmenger's syndrome or inop...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12659
更新日期:2018-11-01 00:00:00
abstract:OBJECTIVE:Atypical development, behavioral difficulties, and academic underachievement are common morbidities in children with a history of congenital heart defects and impact quality of life. Language and social-cognitive deficits have been described, which are associated with autism spectrum disorders. The current st...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12461
更新日期:2017-07-01 00:00:00
abstract:OBJECTIVE:The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) was established in 2008 to improve outcomes of hypoplastic left heart syndrome (HLHS) during the interstage period. They evaluated changes in patient variables and practice variation between early and late eras. DESIGN:Data includi...
journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究
doi:10.1111/chd.12442
更新日期:2017-05-01 00:00:00
abstract:BACKGROUND:Mean platelet volume (MPV), platecrit, and platelet distribution width (PDW) are markers of platelet activation. Previous studies have found that platelet activation occurs in patients with pulmonary arterial hypertension. Platelet indices including MPV, PDW, and platecrit have not been studied in children w...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12616
更新日期:2018-07-01 00:00:00
abstract:BACKGROUND:Interatrial shunts, caused by either atrial septal defect (ASD) or patent foramen ovale, have been reported to have a familial association. We sought to examine the familial risk of isolated interatrial shunt and explore associated comorbidities of stroke, transient ischemic attack (TIA), and migraine using ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2009.00340.x
更新日期:2009-11-01 00:00:00
abstract:OBJECTIVE:To provide data on the value of fused cardiac hybrid imaging with coronary computed tomography angiography (CCTA) and positron emission tomography myocardial perfusion imaging (PET-MPI) in patients with complex coronary artery anomalies (CCAA). DESIGN/SETTING:This is a retrospective, single-center study. PA...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12402
更新日期:2017-01-01 00:00:00
abstract:BACKGROUND:Pulmonary atresia with intact ventricular septum is a unique congenital malformation of the heart in which patients may undergo single-, two- or 1½- ventricle repair. Size of the tricuspid valve annulus, morphology of the right ventricle and presence of ventriculocoronary connections can all impact the selec...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12510
更新日期:2018-01-01 00:00:00
abstract::Currently, the role of stress echocardiography primarily resides in diagnosing acquired coronary artery disease (CAD) in adults. Besides an increasing concern for traditional CAD in young patients due to obesity and other chronic pediatric diseases, there is also a growing population of adolescents and young adults wi...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12495
更新日期:2017-09-01 00:00:00
abstract:BACKGROUND:Left cardiac sympathetic denervation (LCSD) exerts significant antifibrillatory effects in patients with long QT syndrome (LQTS). Recently, electromechanical window (EMW) has emerged as a novel torsadogenic marker in LQTS, superior to QT interval (QTc) in distinguishing symptomatic from asymptomatic patients...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12332
更新日期:2016-09-01 00:00:00
abstract:INTRODUCTION:Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures. METHODS:The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12854
更新日期:2019-11-01 00:00:00
abstract:OBJECTIVES:The primary objective of this study was to demonstrate that pulmonary artery (PA) debanding via cardiac catheterization using balloon angioplasty is feasible and safe in swine. The secondary objectives were to determine the acute and long-term effects of this therapy. DESIGN:This is a chronic survival exper...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12449
更新日期:2017-05-01 00:00:00
abstract:BACKGROUND:Robust risk-adjustment algorithms are often necessary if data from clinical registries is to be used to compare rates of important clinical outcomes between participating centers. Although such algorithms have been successfully developed for surgical and catheter-based cardiac interventions in children, outc...
journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究
doi:10.1111/chd.12050
更新日期:2013-09-01 00:00:00
abstract:OBJECTIVE:To assess how obesity or diabetes mellitus impacts outcomes in patients undergoing cardiac surgery in pediatric hospitals. DESIGN:A multi-institutional, matched case-control study of the Pediatric Health Information System database was performed. SETTING:Tertiary children's hospitals in the United States. ...
journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究
doi:10.1111/chd.12325
更新日期:2016-09-01 00:00:00
abstract:OBJECTIVE:Inappropriate implantable cardioverter defibrillator (ICD) shocks in children and patients with congenital heart disease (CHD) remain a major complication of device therapy, occurring in as many as 50% of children with ICDs. New generation devices include algorithms designed to minimize inappropriate shocks. ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12585
更新日期:2018-05-01 00:00:00
abstract::Transcatheter management of native coarctation of the aorta in 3 children with Turner syndrome is reported. Two children, 12 and 17 years of age, underwent stent implantation and 1 child, 3 years old, underwent balloon angioplasty alone. In all children the procedures were successful, both hemodynamically and angiogra...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/j.1747-0803.2006.00054.x
更新日期:2006-11-01 00:00:00
abstract:OBJECTIVE:The study aims to assess whether the increasing use of cardiovascular magnetic resonance imaging in place of diagnostic cardiac catheterization in the management of pediatric patients with congenital heart disease has had an impact on pediatric cardiac care. DESIGN:Retrospective analysis of data was used. S...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12032
更新日期:2013-07-01 00:00:00
abstract::While right ventricle to pulmonary artery homograft is the surgical procedure of choice for relieving right ventricle outflow tract obstruction; it is limited by the need for multiple surgical replacements owing to progressive conduit obstruction, valve dysfunction, or patient growth. Since January 2010, percutaneous ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12013
更新日期:2013-11-01 00:00:00
abstract:OBJECTIVE:We hypothesized that a right aortic arch in situs solitus, with or without an associated cardiovascular malformation, is often associated with a vascular ring. METHODS:From those born in Southern Nevada between March 2012 and March 2017, we identified 50 (3.6 per 10,000 live births) with a right aortic arch ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12487
更新日期:2017-09-01 00:00:00
abstract::The Fontan operation has gone through multiple incarnations since Fontan and Baudet's initial description in 1971. Through the medical dossier of a patient with a single ventricle, we plot the history of medical, surgical, and percutaneous interventions over the past 40 years, specifically focusing on the Fontan proce...
journal_title:Congenital heart disease
pub_type: 历史文章,杂志文章,评审
doi:10.1111/j.1747-0803.2007.00065.x
更新日期:2007-01-01 00:00:00
abstract:INTRODUCTION:Perturbations in the CACNA1C-encoded L-type calcium channel α-subunit have been linked recently to heritable arrhythmia syndromes, including Timothy syndrome, Brugada syndrome, early repolarization syndrome, and long QT syndrome. These heritable arrhythmia syndromes may serve as a pathogenic basis for auto...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12371
更新日期:2016-12-01 00:00:00