Abstract:
BACKGROUND:Transient elastography (TE) offers a noninvasive correlate with the degree of hepatic fibrosis. However, factors other than fibrosis affect liver stiffness. We sought to determine whether hepatic congestion related to hemodynamics in Fontan circulation influences liver stiffness measurement (LSM) assessed by TE. METHODS:We studied 45 subjects with Fontan circulation undergoing cardiac catheterization with or without simultaneous liver biopsy. Subjects underwent TE within 5 days before catheterization. Clinical history, hemodynamic and biopsy data, and hepatic biomarkers were collected. Five subjects who had previously undergone liver biopsy and TE were also included. RESULTS:Median age was 13.1 years (range 2.4-57.8); median time since Fontan was 9.9 years (range 0.1-32.5). No subject had known hepatitis C. Mean LSM for the entire cohort was 21.4 ± 10.8 kPa. Univariate regression analysis using LSM as a continuous outcome variable shows significant correlations with age (R = 0.35, P = .01), time since Fontan (R = 0.41, P = .003), Fontan pressure (R = 0.31, P = .04), cardiac index (R = 0.33, P = .03), pulmonary vascular resistance (R = 0.34, P = .03), systemic arterial oxygen saturation (R = 0.31, P = .04), and platelet count (R = 0.29, P = .05). On multiple regression analysis, Fontan pressure (β = 0.901, P = .03) and cardiac index (β = 2.703, P = .02) were significant predictors of LSM with overall model R(2) = 0.206. Univariate analysis shows LSM to be associated with more severe centrilobular fibrosis (P = .05). CONCLUSIONS:Higher LSM is associated with unfavorable Fontan hemodynamics and advanced centrilobular hepatic fibrosis. TE may be a useful tool for identifying Fontan patients who warrant invasive testing.
journal_name
Congenit Heart Disjournal_title
Congenital heart diseaseauthors
Wu FM,Opotowsky AR,Raza R,Harney S,Ukomadu C,Landzberg MJ,Valente AM,Breitbart RE,Singh MN,Gauvreau K,Jonas MMdoi
10.1111/chd.12159subject
Has Abstractpub_date
2014-09-01 00:00:00pages
438-47issue
5eissn
1747-079Xissn
1747-0803journal_volume
9pub_type
杂志文章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: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:To evaluate the efficacy of using fetal heart size measurements derived from axial echocardiography to predict homozygous α-thalassemia-1. DESIGN:Prospective diagnostic study. SETTING:The carrier rate of α-thalassemia-1 (-/αα) in China's Guangxi Zhuang Autonomous Region is approximately 15%. If both parents...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12568
更新日期:2018-03-01 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::This review synthesizes the empirical literature on outcomes and experiences of transfer and transition from pediatric to adult care for young people with congenital heart disease. A systematic review of papers published between January 2001 and May 2013 that examined outcomes or experiences of transfer and transition...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12251
更新日期:2015-09-01 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: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::Isolated left ventricular (LV) apical hypoplasia is a recently described congenital abnormality characterized by: (1) a truncated and spherical LV configuration with rightward bulging of the interventricular septum, (2) deficiency of the myocardium within the LV apex with adipose tissue infiltrating the apex, (3) orig...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00489.x
更新日期:2011-11-01 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:INTRODUCTION:With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring. METHODS:In 2013 our Heart Center beg...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12459
更新日期:2017-07-01 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:OBJECTIVE: The objective of this study is to evaluate the safety and feasibility of the AZUR hydrogel-polymer coated platinum coil (hydrocoil) for vascular occlusion in patients with congenital cardiovascular disease (CCVD). DESIGN: Retrospective case review. PATIENTS: Eight patients with CCVD who underwent attemp...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00583.x
更新日期:2012-05-01 00:00:00
abstract:OBJECTIVE:Intravascular stents are now routinely used to treat young patients with vascular stenoses. Future stent redilations are often necessary to account for somatic growth. The purpose of this study was to compile a database of characteristics for stents commonly used in the treatment of congenital heart disease p...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12399
更新日期:2016-12-01 00:00:00
abstract::A male infant presented at birth with severe coarctation of the aorta and marginal left ventricular and mitral valve dimensions associated with a large secundum atrial septal defect. Following successful arch repair, the left ventricle remained small with preferential left-to-right atrial shunting and a dilated right ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2007.00140.x
更新日期:2007-11-01 00:00:00
abstract:INTRODUCTION:Cardiovascular diseases in pediatric pathologies have emerged in the recent years in sub-Saharan Africa (SSA), with congenital heart diseases (CHDs) being the most frequent. Unfortunately, their diagnosis is usually delayed, thereby increasing childhood morbidity and mortality. OBJECTIVES:Describe the cli...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12529
更新日期:2018-01-01 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:OBJECTIVE:Patients with tetralogy of Fallot (TOF) undergoing surgery in adulthood represent a challenge. We report our experience with such patients in or beyond the fourth decade of life. DESIGN:Retrospective cohort. SETTING:Multispeciality tertiary level referral center PATIENTS:Forty-one (age 30-52 years) with TO...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12162
更新日期:2014-09-01 00:00:00
abstract:OBJECTIVE:The purpose of this study was to identify factors that influence postoperative intensive care unit length of stay (ICULOS) in infants less than 6 months of age undergoing congenital heart surgery. METHODS:Records from a single institution, from January 2000 to December 2000, were reviewed. For analysis, surg...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2006.00027.x
更新日期:2006-07-01 00:00:00
abstract:OBJECTIVE:Congenital cardiac defects represent the most common group of birth defects, affecting an estimated six per 1000 births. Genetic characterization of patients and families with cardiac defects has identified a number of genes required for heart development. Yet, despite the rapid pace of these advances, mutati...
journal_title:Congenital heart disease
pub_type: 杂志文章,meta分析
doi:10.1111/chd.12179
更新日期:2015-05-01 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: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: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::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: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::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::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:Following congenital heart surgery, pediatric patients may experience persistent respiratory failure that requires tracheostomy placement. Currently, definitive knowledge of the optimal timing for tracheostomy placement in this patient population is lacking. METHODS:An 8-year retrospective review of 17 pe...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12192
更新日期:2015-01-01 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:OBJECTIVE:Atrial septal defect (ASD) is the second most common congenital heart defect (CHD) and is observed in families as an autosomal dominant trait as well as in nonfamilial CHD. Mutations in the NKX2-5 gene, located on chromosome 5, are associated with ASD, often combined with conduction disturbances, cardiomyopat...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12317
更新日期:2016-05-01 00:00:00
abstract::To evaluate the influence of echocardiographic examination in the clinical management of the sick neonate, 241 patients, admitted to the neonatal intensive care unit of a tertiary referral center that had echocardiograms with data available for review, were enrolled in a retrospective study. Asymptomatic murmurs (45%)...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2008.00209.x
更新日期:2008-09-01 00:00:00