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 practices for patients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low-risk vs high-risk Fontan patients across regional programs. RESULTS:Fifty-six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available within 50 miles of the patient's home institution with some limitations of cardiac catheterization and cardiac magnetic resonance imaging availability. Surveillance and screening were less frequent in low-risk Fontan patients compared to high-risk Fontan patients. Counseling practices were similar for both low-risk and high-risk Fontan patients. Aspirin monotherapy was recommended by 82% of providers for low-risk Fontan patients, while anticoagulation regimens were more varied for the high-risk population. Practitioners with ≤15 years of experience were more likely to provide quality of life testing in both low-risk and high-risk Fontan patients. There were no other major differences in testing frequencies by years of practice, quaternary vs nonquaternary care facility, or the number of Fontan patients in a practice. CONCLUSION:This survey provides insight into regional practices of screening and surveillance of Fontan patients. These data may be used to design future research studies and evidence-based guidelines to streamline the approach to manage these complex patients.
journal_name
Congenit Heart Disjournal_title
Congenital heart diseaseauthors
Davey BT,Toro-Salazar OH,Gauthier N,Valente AM,Elder RW,Wu FM,Berman N,Pollack P,Lee JH,Rathod RHdoi
10.1111/chd.12854subject
Has Abstractpub_date
2019-11-01 00:00:00pages
1013-1023issue
6eissn
1747-079Xissn
1747-0803journal_volume
14pub_type
杂志文章abstract:OBJECTIVE:Published case reports suggest that congenital portosystemic venous connections (PSVC) and other abdominal venous anomalies may be relatively frequent and potentially important in patients with polysplenia syndrome. Our objective was to investigate the frequency and range of portal and other abdominal systemi...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/j.1747-0803.2010.00478.x
更新日期:2011-01-01 00:00:00
abstract::Carditis is a common manifestation of adult patients with Lyme disease affecting 4-10% of Lyme patients in the United States. However, children with Lyme disease rarely present with acute carditis. The management of pediatric patients with complete heart block (CHB) secondary to Lyme carditis has not been well describ...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/j.1747-0803.2007.00122.x
更新日期:2007-09-01 00:00:00
abstract::The origin of pulmonary artery branches (particularly the left pulmonary artery) from the ascending aorta is a rare condition. We detected prominent hypoplasia of the main and right pulmonary arteries in a 3.5-month-old 3.7 kg female infant who had tetralogy of Fallot with origin of the left pulmonary artery in the as...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00542.x
更新日期:2012-05-01 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: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 surgical management of adults with congenital heart disease (ACHD) offers a great challenge, with a large number of anomalies with complex pathophysiology necessitating specific treatments. Pre- and postoperative morbidity has been relatively high, and the influencing factors are not completely identified...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12000
更新日期:2013-07-01 00:00:00
abstract:INTRODUCTION:Physician decisions drive most of the increases in health care expenditures, yet virtually no published literature has sought to understand the types of evidence used by physicians as they make decisions in real time. METHODS:Ten pediatric cardiologists recorded every clinically significant decision made ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2010.00433.x
更新日期:2010-07-01 00:00:00
abstract::Appropriate interpretation of a screening test's validity poses a challenge to the clinician. The purpose of this review is to revisit the terms sensitivity, specificity, likelihood ratio, and pre- and posttest probability and their application to the clinical setting. For illustration, we use a recently published art...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12083
更新日期:2013-09-01 00:00:00
abstract::Scimitar syndrome is characterized by anomalous pulmonary venous drainage of a part or the whole of the right lung into the inferior vena cava (IVC). It is extremely uncommon in scimitar syndrome for the pulmonary veins to drain anomalously to a site other than the IVC. The following case report describes an otherwise...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00513.x
更新日期:2012-01-01 00:00:00
abstract::Crisscross heart is a rare congenital cardiac anomaly in which systemic and pulmonary venous streams cross without mixing at atrioventricular level. We report a case of crisscross heart with tricuspid atresia, double outlet right ventricle, and pulmonary stenosis, which was diagnosed prenatally. ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2012.00716.x
更新日期:2013-09-01 00:00:00
abstract:OBJECTIVE:Arterial switch operation has become the treatment of choice for neonates with transposition of the great arteries. The most important step of the procedure is transferring the coronary arteries to the neoaorta successfully. This study shows the impact of coronary anatomy on early mortality and morbidity afte...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12295
更新日期:2016-03-01 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::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: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: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::We report the use of the Amplatzer Duct Occluder for transcatheter closure of a rare subclavian artery to innominate vein fistula in a neonate. ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2006.00008.x
更新日期:2006-01-01 00:00:00
abstract::So-called heterotaxy affects lateralization of the thoracic and abdominal organs. Congenital malformations may be present in one of several organ systems. Cardiac involvement includes both structural and conduction abnormalities. Data regarding arrhythmias in heterotaxy come from case reports and small case series. We...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12288
更新日期:2016-01-01 00:00:00
abstract::A 22-year-old female with no medical history presented to the emergency room with 2 weeks of rapidly worsening dyspnea on exertion, orthopnea, and cough. On cardiac auscultation, she was noted to have to-and-fro murmurs and a continuous murmur with signs of right heart failure. Echocardiographic images obtained showed...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/j.1747-0803.2010.00467.x
更新日期:2011-03-01 00:00:00
abstract:OBJECTIVE:Intraatrial Mustard baffle repair of dextro-transposition of the great arteries (d-TGA) is vulnerable to complications, typically obstruction and leaks. Because patients often require pacemakers or intracardiac electrophysiology studies (EPS)/ablation for arrhythmias, narrowed or obstructed baffles restrict c...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00532.x
更新日期:2011-09-01 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:Surgical management of tetralogy of Fallot (TOF) is increasingly moving toward valve-sparing approaches rather than transannular patch (TAP). We evaluate whether fetal pulmonary valve (PV) size is predictive of postnatal course and surgical approach in TOF. METHODS:In this retrospective study, fetal and pos...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12120
更新日期:2014-05-01 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: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:OBJECTIVE:Clinical genetic testing is expanding rapidly, but the application of new testing has not been reported in an unselected, comprehensive congenital heart disease (CHD) patient population. This study aims to identify cytogenetic testing practices and diagnostic yield in infants with CHD as an important first st...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12112
更新日期:2014-03-01 00:00:00
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:Eight...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12810
更新日期:2019-09-01 00:00:00
abstract:OBJECTIVE:We studied, as a physiological benchmark, acute effects of right ventricular (RV) apical, RV outflow, and left ventricular (LV) pacing in children with normal cardiac function on LV and RV function and ventricular-ventricular interactions. DESIGN:The design of the study was a prospective, acute intervention....
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2009.00327.x
更新日期:2009-09-01 00:00:00
abstract::A 24-year-old woman presented with a recent increase in dyspnea on exertion and development of presyncope. The patient stated that she has reproducible episodes of dizziness and near fainting when she climbs a flight of stairs and activity is limited to a slow gait. ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2007.00110.x
更新日期:2007-07-01 00:00:00
abstract:OBJECTIVE:The aim is to present current clinical practice of thoracic aorta screening in a tertiary referral center. We identified how often imaging techniques were used for screening and established the value of transthoracic echocardiography (TTE) in comparison with computed tomography (CT) to detect aortic dilation....
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12663
更新日期:2018-11-01 00:00:00
abstract::There has been a steady rise in the prevalence of severe congenital heart disease (CHD) in adults because of improved treatment and survival during childhood. This has resulted in a shift in CHD morbidity and mortality beyond 18 years of age. The healthcare community must be prepared to meet this new challenge. Adult ...
journal_title:Congenital heart disease
pub_type: 历史文章,杂志文章,评审
doi:10.1111/j.1747-0803.2010.00402.x
更新日期:2010-05-01 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