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. OBJECTIVE:To explore the hypothesis that LCSD improves EMW most favorably in patients with LQT1. DESIGN:From September 2006 to July 2015, 44 LQT1 and 25 LQT2 patients underwent LCSD. Subset analysis was performed on the six LQT1 and seven LQT2 patients who had echocardiograms both pre-LCSD and ≥3 months post-LCSD. EMW is defined as the time difference (ms) between aortic valve closure and the end of the QT interval, measured from an ECG on the concurrent echocardiogram. RESULTS:Compared to published normal EMW values of 22 ± 19 ms, pre-LCSD EMW mean values were -78 ± 36 ms in LQT1 and -71 ± 35 ms in LQT2 (P < .001). Following LCSD, there was a 57 ± 35 ms decrease in QTc in LQT1 (P = .16) and 23 ± 21 ms decrease in QTc in LQT2 (P = .3). Overall, there was a 35 ± 57 ms mean improvement in EMW post-LCSD (P = .04). Five of the 6 (83%) LQT1 subjects had a favorable EMW change post-LCSD (mean improvement 56 ± 25 ms, P = .04). Five of the 7 (71%) LQT2 subjects had a favorable EMW change post-LCSD (mean improvement 18 ± 19 ms, P = .2). CONCLUSIONS:The precise mechanism of the LCSD therapeutic effect in LQTS patients is not fully understood. This pilot study raises the possibility that LCSD's antitorsadogenic effect in patients with LQT1 could be conferred in part by restoration of electromechanical order, evidenced by normalization of the EMW.
journal_name
Congenit Heart Disjournal_title
Congenital heart diseaseauthors
Schneider AE,Bos JM,Ackerman MJdoi
10.1111/chd.12332subject
Has Abstractpub_date
2016-09-01 00:00:00pages
437-443issue
5eissn
1747-079Xissn
1747-0803journal_volume
11pub_type
杂志文章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
abstract:OBJECTIVE:Approximately one-third of adult congenital heart disease (ACHD) patients have mood or anxiety disorders, the majority of which go untreated. The extent to which this group of patients is interested in psychological services is, however, unknown. This study investigated the perceived psychological needs of pa...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2009.00280.x
更新日期:2009-05-01 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::A genetic basis of congenital heart disease (CHD) has been known for decades. In addition to the sequence of the genome, the contribution of epigenetics to pediatric cardiology is increasingly recognized. Multiple epigenetic mechanisms, including DNA methylation, histone modification, and RNA-based regulation, are kno...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12543
更新日期:2017-12-01 00:00:00
abstract:OBJECTIVE:Nurse practitioners (NPs) have an established role for delivering competent care to patients in the primary care setting. The aim of this study was to compare satisfaction of patients managed by NPs vs. physicians in the outpatient adult congenital heart disease (CHD) clinic. DESIGN:A prospective study condu...
journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究
doi:10.1111/chd.12273
更新日期:2015-09-01 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: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:BACKGROUND & AIMS:Fontan surgery for single ventricle congenital heart disease leads to Fontan-associated liver disease (FALD). Typical laboratory tests, imaging, and histopathology cannot predict clinical severity in FALD. HepQuant SHUNT is a proprietary serum test of hepatic function and physiology that has not yet b...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12831
更新日期:2019-11-01 00:00:00
abstract::We present a rare case of spontaneous closure of a fistula between the left coronary artery and the right ventricle (RV) within a few days of newborn period. A 14-day-old male newborn was referred to our clinic for investigation of tachypnea and cardiac murmur. A color flow Doppler echocardiography revealed turbulent ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12060
更新日期:2014-01-01 00:00:00
abstract::Dexmedetomidine is a selective alpha-2 adrenergic agonist that is used frequently for short-term sedation in children. It has been noted to cause hypertension, hypotension, bradycardia, and sinus pauses; however, QTc prolongation has not been reported with dexmedetomidine administration. We describe a case of marked Q...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12055
更新日期:2014-01-01 00:00:00
abstract:BACKGROUND:It has been reported that gender differences in cardiovascular outcomes found in adults also are present in children who undergo surgical repair for congenital heart disease. METHODS:California statewide hospital discharge data 1989-99 were used to study outcomes in children <18 years undergoing cardiac sur...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2006.00013.x
更新日期:2006-05-01 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:Patients undergoing surgical repair of aortic coarctation have a 50% risk of pathologic left ventricular remodeling (increased left ventricular mass or relative wall thickness). Endothelin 1, ST2, galectin 3, norepinephrine and B-natriuretic peptide are biomarkers that have been associated with pathologic LV ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12744
更新日期:2019-05-01 00:00:00
abstract::Williams-Beuren syndrome (WBS) is a multisystem genetic disorder comprising of craniofacial, developmental, and cardiac malformations. The most common cardiac defects found are supravalvar aortic stenosis and peripheral pulmonary stenosis. However, WBS should be regarded as a general arteriopathy consisting of stenose...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12087
更新日期:2014-05-01 00:00:00
abstract:BACKGROUND/OBJECTIVE:Cardiopulmonary exercise capacity is often reduced in patients with transposition of the great arteries after atrial switch operation. Reduced exercise capacity may be caused by deterioration of systemic right ventricular function over time. This study analyzed serial changes in systemic right vent...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12137
更新日期:2014-05-01 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:OBJECTIVES:We aimed (1) determine if systemic right ventricle filling parameters influence systemic right ventricle stroke volume in adult patients with D-transposition of the great arteries (D-TGA) palliated by atrial switch, using cardiac magnetic resonance imaging and echocardiography, and (2) to study relationship ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12472
更新日期:2017-07-01 00:00:00
abstract::Tetralogy of Fallot is the most common cyanotic congenital heart defect. Advances in surgical technique and postoperative care have improved survival which is now very good. Patients now face long-term morbidities such as reduced exercise tolerance and arrthymias. Cardiologists caring for these patients are confronted...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12042
更新日期:2013-03-01 00:00:00
abstract:PURPOSE:Right ventricular (RV) failure and ventricular dyssynchrony are strong determinants of prognosis in patients with adult congenital heart disease (ACHD). The aim of this study was to investigate the relationship between interventricular dyssynchrony (IVD) using cine-tagged magnetic resonance imaging (MRI) and RV...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12217
更新日期:2015-05-01 00:00:00
abstract:OBJECTIVE:The need for conduit replacement in the growing child remains a major problem after right ventricular outflow tract reconstruction. We compared two diverse surgical centers with considerable practice variation in Europe and the United States to identify modifiable risk factors that can increase conduit longev...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2008.00190.x
更新日期:2008-05-01 00:00:00
abstract:OBJECTIVE:The objective of this study was to characterize the natural history of metabolic uncoupling (type B hyperlactemia and hyperglycemia) following cardiopulmonary bypass (CPB), and to determine the impact of insulin therapy on time to lactate normalization in patients without low cardiac output. DESIGN:The desig...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12285
更新日期:2015-11-01 00:00:00
abstract::Pulmonary hypertension (PH) is a complex condition with a broad range of etiologies that result in a common outcome--elevated pulmonary arterial pressure. For the pediatric cardiologist, this entity provides a manifest demonstration of the interrelationship of cardiac and pulmonary physiology. A thoughtful approach mu...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12012
更新日期:2012-11-01 00:00:00
abstract:BACKGROUND:Traveling to high altitude has become more popular. High-altitude exposure causes hypobaric hypoxia. Exposure to acute high altitude, during air travel or mountain stays, seems to be safe for most patients with congenital heart disorders (CHD). Still, current guidelines for CHD patients express concerns rega...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12850
更新日期:2019-11-01 00:00:00
abstract:OBJECTIVES:To investigate functional health status among adults previously operated for aortic coarctation (CoA) compared with healthy subjects; to assess the influence of medication and exercise capacity on patients' functional health. DESIGN:Questionnaire-based investigation among 119 patients who underwent surgical...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00595.x
更新日期:2011-11-01 00:00:00
abstract::We report on a 3-year-old male who underwent transcatheter stent fenestration of the inferior portion of an extracardiac total cavopulmonary connection in the setting of hypoplastic left heart syndrome. Transhepatic approach, following an unsuccessful attempt from the femoral vein facilitated delivery of a diabolo-sha...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00488.x
更新日期:2011-05-01 00:00:00
abstract:AIM:The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and quality of life is unknown and objective of this study. METHODS:This mu...
journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究
doi:10.1111/chd.12694
更新日期:2019-03-01 00:00:00
abstract::Dilated cardiomyopathy and ventricular noncompaction have been reported in association with deletion 1p36 syndrome. Previous descriptions include echocardiographic and/or gross pathologic descriptions. There are no previous reports of microscopic findings. We report a case with descriptions of echocardiographic, gross...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00514.x
更新日期:2012-01-01 00:00:00
abstract::Protein-losing enteropathy (PLE) is a rare, but serious complication in single ventricle patients after Fontan palliation, and is associated with a 5-year mortality of 46%. We describe a patient with PLE after Fontan palliation who achieved remission with high-dose spironolactone (an aldosterone antagonist), but had t...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2009.00320.x
更新日期:2010-03-01 00:00:00
abstract::Absence of the aortic valve is a rare congenital heart defect that is detectable in the prenatal period. In this condition, functional aortic valve leaflets are absent; in their place are rudimentary, immobile noncoapting plate-like structures at the level of the annulus resulting in severe aortic regurgitation. We re...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12089
更新日期:2014-05-01 00:00:00
abstract:OBJECTIVE:This study aims to assess and compare the early and long-term effects of extracardiac conduit (EC) and lateral tunnel (LT) in patients with a functional single ventricle through meta-analysis. DESIGN:A systematic search was performed in PubMed, Embase, Cochrane Library, CNKI, VIP, CBM, and WanFang databases ...
journal_title:Congenital heart disease
pub_type: 杂志文章,meta分析,评审
doi:10.1111/chd.12503
更新日期:2017-12-01 00:00:00