Abstract:
OBJECTIVE:We sought to define what clinical parameters were related to the ultimate outcome in Fontan patients who had developed protein-losing enteropathy (PLE). BACKGROUND:PLE is a serious complication of the Fontan operation. Several preoperative and perioperative findings are associated with later PLE. However, there is limited information regarding postoperative abnormalities contributing to or influencing outcome in PLE. METHODS:We evaluated 44 consecutive Fontan patients with PLE. A matched control group of Fontan patients without PLE was used for comparison (matched-pair analyses). Kaplan-Meier and Cox proportional hazard methods were used for survival analyses. RESULTS:Median age was 18 years (range 4-48 years). Short-axis and apical fractional area change (Delta FA) were less in PLE patients than in controls (50 vs. 57% [P < .0001] and 49 vs. 54% [P = .01]). Five and 10-year actuarial survival rates for the PLE group were 49 +/- 9% and 30 +/- 11%. Deceleration time <120 milliseconds (hazard ratio [HR] = 9.2, P = .04), New York Heart Association classification III or IV (HR = 4.0, P = .01), and lower serum albumin (HR = 0.30, P = .04) were independent predictors of mortality in those with PLE. CONCLUSION:Mild reduction in Delta FA was the only late echocardiographic finding associated with development of PLE. However, short deceleration time, poor New York Heart Association (NYHA) class, and low serum albumin identify a group of patients at the greatest risk for death. Presence of these findings in a PLE patient should lead to aggressive management strategies and may warrant early consideration of transplantation.
journal_name
Congenit Heart Disjournal_title
Congenital heart diseaseauthors
Silvilairat S,Cabalka AK,Cetta F,Grogan M,Hagler DJ,O'Leary PWdoi
10.1111/j.1747-0803.2008.00200.xsubject
Has Abstractpub_date
2008-07-01 00:00:00pages
262-8issue
4eissn
1747-079Xissn
1747-0803pii
CHD200journal_volume
3pub_type
杂志文章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::In patients with Kawasaki disease (KD) and coronary artery aneurysms (CAAs), serial evaluation with multiple imaging modalities is recommended to guide risk stratification and management. We present the case of a 7-year-old boy with a history of KD and multiple giant CAAs who developed thrombosis in one of the aneurys...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12131
更新日期:2014-11-01 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: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
abstract:OBJECTIVE:The study examined parent stress and health-related quality of life (HRQOL) among families of children with congenital heart disease (CHD) referred for psychological services. METHODS:Parents of 54 children (85% boys) aged 3 to 13 (Mage = 7.48, SD = 2.38) completed measures to assess parenting stress (Paren...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12547
更新日期:2018-01-01 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::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: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:AIMS:To outline the etiology, clinical course, short-term survival to discharge and neurological outcome of infants (<1 yr) with new cardiac diagnoses presenting to a pediatric intensive care (PICU) unit with acute cardiac compromise. METHODS:Retrospective search of a computerized database and medical case notes for a...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2010.00430.x
更新日期:2010-09-01 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:OBJECTIVE:We previously identified a pathogenic germline DICER1 variant in a child with transposition of the great arteries who was a member of a family with DICER1 syndrome. In view of a report linking Dicer1 knockout in murine cardiomyocytes to cardiac outflow defects, we investigated the involvement of DICER1 in tra...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12578
更新日期:2018-05-01 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: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: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:UNLABELLED:BACKGROUND; Several studies have demonstrated the utility of B-type natriuretic peptide (BNP) in the months following cardiac transplant. The purpose of this study was to analyze longitudinal BNP data in pediatric cardiac transplant patients and determine the efficacy of BNP in routine follow-up of transplan...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2010.00417.x
更新日期:2010-05-01 00:00:00
abstract:OBJECTIVE:Pulmonary autograft aortic root replacement (Ross' operation) is now associated with low operative risk. Recent series suggest that patients with primary aortic insufficiency have diminished autograft durability and that patients with large discrepancies between pulmonary and aortic valve sizes have a low but...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2008.00221.x
更新日期:2008-11-01 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: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:OBJECTIVES:To determine if patients evaluated using the pediatric chest pain standardized clinical assessment and management plan (SCAMP) in cardiology clinic were later diagnosed with unrecognized cardiac pathology, and to determine if other patients with cardiac pathology not enrolled in the SCAMP would have been ide...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12335
更新日期:2016-09-01 00:00:00
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::A 52-year-old male patient, with a medical history of surgically repaired double outlet right ventricle presented with severe aortic stenosis (AS) and hepatitis C with cirrhosis, presented with New York Heart Association Class IV heart failure. During evaluation for a liver transplant, he was deemed a poor surgical ca...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12114
更新日期:2014-09-01 00:00:00
abstract:OBJECTIVE:To describe treatment, survival, and morbidity for liveborn infants with isolated transposition of great arteries (TGA). DESIGN:Population-based data from 7 European registries of congenital malformations (EUROCAT). RESULTS:Ninety-seven infants were diagnosed with isolated TGA and livebirth prevalence was 2...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2007.00093.x
更新日期:2007-05-01 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: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::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::This case report demonstrated an apical muscular ventricular septal defect (VSD) that was a large defect but behaved like a small defect because of the restrictive flow across the anomalous muscle bundles in the right ventricular (RV) apex. The anomalous muscle bundles separated the RV sinus into two parts: the RV ape...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2007.00075.x
更新日期:2007-01-01 00:00:00
abstract:OBJECTIVE:Obesity affects adults with congenital heart disease (CHD). The effect of an increased body mass index (BMI) with respect to morbidity and mortality has not been evaluated in adults with complex CHD. Our objective was to evaluate the effects of increased BMI on heart failure and mortality in univentricular pa...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12296
更新日期:2016-01-01 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: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: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