Abstract:
BACKGROUND:Patients with a Fontan circulation are at risk of renal dysfunction. We analyzed cross-sectional data in pediatric and adult Fontan patients in order to assess the accuracy of commonly used serum creatinine-based methods in estimating glomerular filtration rate (GFR). METHODS:A total of 124 Fontan patients (58 children, 66 adults) were enrolled across three study centers. Measurement of GFR (mGFR) using in vivo 99m Tc-DTPA clearance was performed. Various serum creatinine-based equations were used to calculate estimated GFR (eGFR). RESULTS:Mean mGFR was 108 ± 28 mL/min/1.73 m2 in children and 92 ± 20 mL/min/1.73 m2 in adults. Fourteen children (25%) and 28 adults (45%) had an mGFR <90 mL/min/1.73 m2 . There was no significant correlation between mGFR and eGFR (Schwartz) in children (r = 0.22, P = .1), which substantially overestimated mGFR (bias 50.8, 95%CI: 41.1-60.5 mL/min/1.73 m2 , P < .0001). The Bedside Schwartz equation also performed poorly in the children (r = 0.08, P = .5; bias 5.9, 95%CI: -2.9-14.6 mL/min/1.73 m2 , P < .0001). There was a strong correlation between mGFR and both eGFR (CKD-EPI) and eGFR (MDRD) in adults (r = 0.67, P < .0001 in both cases), however, both methods overestimated mGFR (eGFR(CKD-EPI):bias 23.8, 95%CI: 20-27.6 mL/min/1.73 m2 , P < .0001; eGFR (MDRD):bias 16.1, 95%CI: 11.8-20.4 mL/min/1.73 m2 , P < .0001). None of the children with an mGFR <90 mL/min/1.73 m2 had an eGFR (Schwartz) <90 mL/min/1.73 m2 . Sensitivity and specificity of eGFR (CKD-EPI) and eGFR (MDRD) for mGFR <90 mL/min/1.73 m2 in adults were 25% and 92% and 39% and 100%, respectively. CONCLUSIONS:This study identifies the unreliability of using creatinine-based equations to estimate GFR in children with a Fontan circulation. The accuracy of formulas incorporating cystatin C should be further investigated and may aid noninvasive surveillance of renal function in this population.
journal_name
Congenit Heart Disjournal_title
Congenital heart diseaseauthors
Wilson TG,d'Udekem Y,Winlaw DS,Cordina RL,Ayer J,Gentles TL,Weintraub RG,Grigg LE,Cheung M,Cain TM,Rao P,Verrall C,Plessis KD,Rice K,Iyengar AJdoi
10.1111/chd.12746subject
Has Abstractpub_date
2019-05-01 00:00:00pages
454-463issue
3eissn
1747-079Xissn
1747-0803journal_volume
14pub_type
杂志文章,多中心研究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::We describe a simple technique for chest wall reconstruction in pentalogy of Cantrell using split-thickness cartilage grafts. This technique provides a robust reconstruction, gives immediate and excellent cosmetic results, allows for skeletal and tissue growth, and avoids the use of synthetic material. ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2012.00697.x
更新日期:2013-01-01 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::Singleton pregnancy in patients with single ventricle after the Fontan operation has been reported with significant offspring and maternal complications. We report a twin pregnancy and premature delivery, in a patient following the Fontan operation. ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12054
更新日期:2013-11-01 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::Down syndrome (DS) patients are prone to pulmonary hypertension (PHTN) due to various cardiopulmonary causes. However, the association of DS with pulmonary vein stenosis (PVS) is not adequately described. We illustrate three cases from our center and an additional 13 cases from an extensive review of the literature of...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12088
更新日期:2014-05-01 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:OBJECTIVE:Management of the patients with transposition of the great arteries and intact ventricular septum may be challenging beyond the newborn period. Herein, we would like to present our alternative strategy for training the left ventricle in these patients. METHODS:Six patients with transposition of the great art...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12749
更新日期:2019-05-01 00:00:00
abstract:INTRODUCTION:Pectus excavatum is commonly viewed as a benign condition. Associated alterations in hemodynamics are rare. We present an unusual case of right ventricular inflow obstruction and hemodynamic compromise as a consequence of pectus excavatum encountered during surgical intervention. CASE:a 15-year-old male w...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2008.00231.x
更新日期:2009-05-01 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::This is a case report reviewing the presentation and clinical course of a patient diagnosed with abdominal aortic atresia at the level caudal to the superior mesenteric artery. Patients with abdominal aortic atresia need to be evaluated for associated underlying syndromes or diseases. Although the prognosis for this d...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/j.1747-0803.2010.00454.x
更新日期:2011-05-01 00:00:00
abstract:BACKGROUND:Physician burnout has many undesirable consequences, including negative impact on patient care delivery and physician career satisfaction. Electronic health records (EHRs) may exacerbate burnout by increasing physician workload. OBJECTIVE:To determine burnout in adult congenital heart disease (ACHD) special...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12745
更新日期:2019-05-01 00:00:00
abstract::In medical literature, few cases of Rasmussen's aneurysm have been reported until now, and to the best of our knowledge, there has been no description of a case of Rasmussen's aneurysm in childhood. The child described in the case report had a restrictive cardiomyopathy, complicated by pulmonary hypertension, in assoc...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00609.x
更新日期:2013-03-01 00:00:00
abstract:OBJECTIVE:The aim of this study was to compare the effects of anesthesia by dexmedetomidine and propofol on the hemodynamics and oxygen balance in children with complex congenital heart disease who were undergoing cardiac surgery. METHODS:Fifty-seven children were randomized to receive either a continuous infusion of ...
journal_title:Congenital heart disease
pub_type: 杂志文章,随机对照试验
doi:10.1111/chd.12228
更新日期:2015-05-01 00:00:00
abstract:OBJECTIVE:Traditionally, karyotype and fluorescence in situ hybridization (FISH) were used for cytogenetic testing of infants with congenital heart disease (CHD) who underwent cardiac surgery at our institution. Recently, chromosome microarray analysis (CMA) has been performed in lieu of the traditional tests. A standa...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12241
更新日期:2015-05-01 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:BACKGROUND:Hyponatremia (HN) is relatively common in adults with congenital heart disease and is a powerful predictor of mortality. However, the precise relationship of HN to the Fontan pathophysiology remains unknown. PURPOSE:Our study aimed to clarify the association of HN to the Fontan pathophysiology. METHODS AND...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00503.x
更新日期:2011-07-01 00:00:00
abstract:OBJECTIVE:To examine the knowledge and attitudes of anesthesia providers in relation to the care of adult congenital heart disease (ACHD) patients presenting for noncardiac surgery. DESIGN/SETTING:A novel survey was designed and administered to 168 anesthesiologists across a single academic department in a range of pr...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12076
更新日期:2014-01-01 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: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:OBJECTIVE:Enteral feeding is associated with decreased infection rates, decreased mechanical ventilation, decreased hospital length of stay, and improved wound healing. Enteral feeding difficulties are common in congenital heart disease. Our objective was to develop experience-based newborn feeding guidelines for the i...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12435
更新日期:2017-05-01 00:00:00
abstract:INTRODUCTION:The right ventricle (RV) supports the systemic circulation in patients who have had an intraatrial repair of transposition of the great arteries or have congenitally corrected transposition. There is concern about the ability of a systemic RV to support the additional volume load of pregnancy, and previous...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12001
更新日期:2013-07-01 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::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::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: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: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: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:INTRODUCTION:Heart transplant recipients are at risk for developing left ventricular (LV) dysfunction. While traditional echocardiographic parameters have limitations, speckle tracking echocardiography (STE) is a novel technique shown to be more sensitive and accurate in adult studies for evaluating ventricular systoli...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12263
更新日期:2015-07-01 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