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 multicenter retrospective/cross-sectional study included 232 patients (140 females, age 25.6 ± 10.8 years) after Fontan palliation (19.8% atrioventricular connection; 20.3% atriopulmonary connection; 59.9% total cavopulmonary connection). Resting spirometry, cardiopulmonary exercise tests, and quality-of-life assessment (SF-36 questionnaire) were performed between 2003 and 2015. RESULTS:Overall, mean forced expiratory volume in one second (FEV1 ) was 74.7 ± 17.8%predicted (%pred). In 59.5% of the patients, FEV1 was <80%pred., and all of these patients had FEV1 /forced vital capacity (FVC) > 80%, suggestive of a restrictive ventilatory pattern. Reduced FEV1 was associated with a reduced peakVO2 of 67.0 ± 17.6%pred. (r = 0.43, P < .0001), even if analyzed together with possible confounding factors (sex, BMI, age, years after palliation, number of interventions, scoliosis, diaphragmatic paralysis). Synergistically to exercise capacity, FEV1 was associated to quality of life in terms of physical component summary (r = 0.30, P = .002), physical functioning (r = 0.25, P = .008), bodily pain (r = 0.22, P = .02), and general health (r = 0.16, P = .024). Lower FEV1 was associated with diaphragmatic paralysis (P = .001), scoliosis (P = .001), higher number of interventions (P = .002), and lower BMI (P = .01). No correlation was found to ventricular morphology, type of surgeries, or other perioperative/long-term complications. CONCLUSIONS:This study shows that the common restrictive ventilatory pattern in Fontan patients is associated with lower exercise capacity and quality of life. Risk factors are diaphragmatic paralysis, scoliosis, a high total number of interventions and low BMI.
journal_name
Congenit Heart Disjournal_title
Congenital heart diseaseauthors
Callegari A,Neidenbach R,Milanesi O,Castaldi B,Christmann M,Ono M,Müller J,Ewert P,Hager Adoi
10.1111/chd.12694subject
Has Abstractpub_date
2019-03-01 00:00:00pages
147-155issue
2eissn
1747-079Xissn
1747-0803journal_volume
14pub_type
杂志文章,多中心研究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: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: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: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: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::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:The study aims to assess whether the increasing use of cardiovascular magnetic resonance imaging in place of diagnostic cardiac catheterization in the management of pediatric patients with congenital heart disease has had an impact on pediatric cardiac care. DESIGN:Retrospective analysis of data was used. S...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12032
更新日期:2013-07-01 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: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: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 current study aims to identify the rates of lapses in care and loss to follow-up before age one through age five for white and nonwhite congenital heart disease (CHD) survivors. Nonwhite CHD survivors were hypothesized to experience an earlier lapse in care and be lost to follow-up than whites. DESIGN:Pa...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12732
更新日期:2019-03-01 00:00:00
abstract:OBJECTIVE:It is unclear whether neonates with interrupted aortic arch (IAA) and a smaller left ventricular outflow tract may have improved outcomes with a Yasui operation (ventricular outflow bypass procedure) over a primary complete repair. This study sought to identify preoperative echocardiographic parameters to dif...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12599
更新日期:2018-05-01 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: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::Brugada syndrome is an arrhythmogenic disease characterized by a pattern of ST segment elevation in the right precordial leads on an electrocardiogram with an increased risk of sudden cardiac death. It is primarily reported in adults with limited data about the prevalence, prognosis, and long-term management in childr...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2010.00382.x
更新日期:2010-11-01 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
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::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: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::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:INTRODUCTION:The neurodevelopmental outcome of children with repaired congenital heart defect has risen in importance with improved survival. This study compares neurodevelopmental outcomes of children who had CHD with single ventricle physiology with those who had CHD with two-ventricle physiology. PATIENTS AND METHO...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2009.00373.x
更新日期:2010-03-01 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: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:Deficient retro-aortic rim has been identified as a risk factor for device erosion following trans-catheter closure of atrial septal defects (ASDs). Transthoracic echocardiography (TTE) is the primary screening method for subjects for possible device closure of ASD, but its reliability in measuring retro-aort...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12226
更新日期:2015-07-01 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: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, th...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2008.00200.x
更新日期:2008-07-01 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::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: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::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