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 DS patients with PHTN and PVS. In DS patients PVS is rare, they were diagnosed at a young age (<7.2 months), had high mean pulmonary artery pressures (38 mm Hg), and had rapid progression of the stenosis with an increased mortality (91%) in patients with two or more vein involvement. In DS patients, PVS may be missed by echocardiography; thus, any DS patients with persistent PHTN should undergo cardiac catheterization to assess hemodynamics and to evaluate all four pulmonary veins.
journal_name
Congenit Heart Disjournal_title
Congenital heart diseaseauthors
Gowda S,Bhat D,Feng Z,Chang CH,Ross RDdoi
10.1111/chd.12088subject
Has Abstractpub_date
2014-05-01 00:00:00pages
E90-7issue
3eissn
1747-079Xissn
1747-0803journal_volume
9pub_type
杂志文章,评审abstract:OBJECTIVE:Assess the early and intermediate results with respect to blood pressure control in older children undergoing endovascular stenting for native coarctation of the aorta. DESIGN:Eleven hypertensive patients (10 +/- 3 years of age) underwent endovascular stenting via standard techniques for native coarctation o...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2007.00148.x
更新日期:2008-01-01 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::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:INTRODUCTION:Perturbations in the CACNA1C-encoded L-type calcium channel α-subunit have been linked recently to heritable arrhythmia syndromes, including Timothy syndrome, Brugada syndrome, early repolarization syndrome, and long QT syndrome. These heritable arrhythmia syndromes may serve as a pathogenic basis for auto...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12371
更新日期:2016-12-01 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:OBJECTIVE:The use of video-assisted thoracoscopic surgery (VATS) is becoming increasingly common in the treatment of congenital heart defects, particularly for the division of vascular rings. We compare the short-term outcomes of vascular ring division by VATS as opposed to open thoracotomy and discuss new issues raise...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2007.00086.x
更新日期:2007-03-01 00:00:00
abstract:OBJECTIVE:We hypothesized that a right aortic arch in situs solitus, with or without an associated cardiovascular malformation, is often associated with a vascular ring. METHODS:From those born in Southern Nevada between March 2012 and March 2017, we identified 50 (3.6 per 10,000 live births) with a right aortic arch ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12487
更新日期:2017-09-01 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: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: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::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:OBJECTIVE:The aims of this study were to compare the level of posttraumatic stress disorder between adults with and without congenital heart disease, and to examine the correlates of posttraumatic stress disorder (e.g., sociodemographics). DESIGN:Cross-sectional. SETTING:Two university-affiliated heart hospitals in T...
journal_title:Congenital heart disease
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1111/chd.12452
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::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: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: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...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12159
更新日期:2014-09-01 00:00:00
abstract:BACKGROUND:As the prevalence of obesity continues to increase, it now includes the growing number of patients with congenital heart disease (CHD). This particular obese patient population may pose additional intraoperative as well as postoperative challenges that may contribute to poor outcomes. Our aims were to determ...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00522.x
更新日期:2011-05-01 00:00:00
abstract::We report the case of a 30 year-old male with congenitally corrected transposition of the great arteries, atrial, and ventricular septal defects (VSD), and pulmonary stenosis. He previously underwent three palliative surgical procedures before undergoing intracardiac repair at age 20 with a left ventricular to pulmona...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2009.00339.x
更新日期:2010-01-01 00:00:00
abstract::We report a 51-year-old patient with platypnea-orthodeoxia syndrome after percutaneous closure of a secundum atrial septal defect, an unusual complication of this modality of treatment. Echocardiography, the main diagnostic technique in the present case, showed that one of the percutaneous device's rims was fixed to t...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2012.00639.x
更新日期:2012-09-01 00:00:00
abstract::Dexmedetomidine is a selective alpha-2 adrenergic agonist that is used frequently for short-term sedation in children. It has been noted to cause hypertension, hypotension, bradycardia, and sinus pauses; however, QTc prolongation has not been reported with dexmedetomidine administration. We describe a case of marked Q...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12055
更新日期:2014-01-01 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::Pulmonary hypertension (PH) is a complex condition with a broad range of etiologies that result in a common outcome--elevated pulmonary arterial pressure. For the pediatric cardiologist, this entity provides a manifest demonstration of the interrelationship of cardiac and pulmonary physiology. A thoughtful approach mu...
journal_title:Congenital heart disease
pub_type: 杂志文章,评审
doi:10.1111/chd.12012
更新日期:2012-11-01 00:00:00
abstract:PURPOSE:Right ventricular (RV) failure and ventricular dyssynchrony are strong determinants of prognosis in patients with adult congenital heart disease (ACHD). The aim of this study was to investigate the relationship between interventricular dyssynchrony (IVD) using cine-tagged magnetic resonance imaging (MRI) and RV...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/chd.12217
更新日期:2015-05-01 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: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: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: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: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::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::Iron deficiency state in patients with cyanotic congenital heart disease can mimic as well as aggravate hyperviscosity symptoms. Correction of iron deficiency in these cases is expected to improve symptoms. We report an unexpected occurrence of refractory cyanotic spell in a child with tetralogy of Fallot due to throm...
journal_title:Congenital heart disease
pub_type: 杂志文章
doi:10.1111/j.1747-0803.2011.00608.x
更新日期:2012-07-01 00:00:00