Abstract：
:Marfan syndrome (MS) is a connective tissue disease involving the cardiovascular, ocular, and the musculoskeletal systems. MS has variable phenotypic expression and is most often diagnosed in adult life. Infantile-onset MS is rare and is associated with severe cardiovascular manifestations; there is an extremely high mortality during the first 2 years of life. We present a case of a child with severe infantile MS who, during the course of infancy and early childhood, developed aortic root dilatation and polyvalvar insufficiency requiring subsequent successful replacement of the aortic root and of all cardiac valves. To our knowledge, this is the first reported case of quadrivalvar replacement in the pediatric age group.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Strigl S,Quagebeur JM,Gersony WMdoi
10.1007/s00246-006-0066-4subject
Has Abstractpub_date
2007-09-01 00:00:00pages
403-5issue
5eissn
0172-0643issn
1432-1971journal_volume
28pub_type
杂志文章abstract：BACKGROUND:This study applied tissue Doppler imaging and color tissue Doppler imaging to study atrial function changes in patients with hypertrophic cardiomyopathy (HCM). The profile of the segmental atrial velocities and the strain rate were determined and compared with those of normal matched control subjects. METHO...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-007-9112-0
更新日期：2008-03-01 00:00:00
abstract：:Ebstein anomaly of mitral valve (MV) is an extremely rare congenital heart disease. In the current report, we present a case of Ebstein of MV that was diagnosed prenatally. Fetal echocardiogram showed that the posterior leaflet of MV was tethered to the lateral wall of left ventricle (LV) with downward displacement in...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-018-1903-y
更新日期：2018-08-01 00:00:00
abstract：:We present a 2-month-old male infant with thrombosis in the superior vena cava and pericardium due to transient protein C deficiency. Protein C deficiency was related to sepsis and hepatitis-induced liver function impairment. The patient's cardiac anatomy was otherwise normal. The patient was referred to us with signs...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-006-1265-8
更新日期：2006-07-01 00:00:00
abstract：:Various echocardiographic parameters are determined in the assessment and evaluation of a patent ductus arteriosus. In isolation, many of these parameters have a low sensitivity and specificity for ductal hemodynamic significance compared with ductal size. This study aimed to correlate various echocardiographic parame...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0640-5
更新日期：2013-06-01 00:00:00
abstract：:This report describes an unusual case with tortuosity of the great vessels in a neonate who presented at birth with cyanosis. The diagnosis was made with magnetic resonance imaging (MRI), then confirmed by genetic analysis. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-009-9486-2
更新日期：2009-11-01 00:00:00
abstract：:Thrombus formation in the native aortic root is a rare but potentially life-threatening complication in patients with hypoplastic left heart syndrome. The native aortic root in these patients serves as a conduit for the retrograde filling of the coronary arteries. Thrombus in this location could result in myocardial i...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-005-1274-z
更新日期：2006-05-01 00:00:00
abstract：:The longer survival of patients with Duchenne muscular dystrophy due to advances in clinical care has increased the incidence of Duchenne muscular dystrophy-associated cardiomyopathy, a nearly consistent feature in the third decade of life. A 26-year-old patient with Duchenne muscular dystrophy experienced severe acut...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02072-9
更新日期：2019-03-01 00:00:00
abstract：:The mechanism of heart failure in patients with enterovirus 71 rhombencephalitis (brain stem encephalitis) remains unknown. Our previous reports hypothesized that a catecholamine storm induced by rhombencephalitis may account for the heart failure. The aim of this study was to develop a novel feline model of norepinep...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-005-0915-6
更新日期：2006-09-01 00:00:00
abstract：:Interruption of the aortic arch and hypoplastic left heart syndrome in the same patient is exceptional. In the combined collections of the Registry of Cardiovascular Disease (St. Paul, Minnesota, U.S.A.) and the Registry of Congenital Heart Defects of the Rijksuniversiteit Gent (Gent, Belgium) three specimens were fou...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/BF00798068
更新日期：1995-11-01 00:00:00
abstract：:This study aimed to evaluate the effects of washed cardiopulmonary (CPB) circuit residual blood reinfusion on the postoperative clinical outcome for pediatric patients undergoing cardiac surgery. A total of 309 consecutive Chinese cardiac patients receiving CPB between October 2010 and April 2011 were prospectively an...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0606-z
更新日期：2013-06-01 00:00:00
abstract：:Although the Fontan operation can improve outcomes, surviving patients still face Fontan-associated liver disease (FALD). The aim of this study was to determine the associated factors of FALD in relation to ultrasound liver elastography. A cross-sectional study was conducted for all patients on whom an ultrasound uppe...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-020-02422-y
更新日期：2020-12-01 00:00:00
abstract：:In adult heart failure (HF) patients, a higher ventricular arterial (VA) coupling ratio measured non-invasively is associated with worse HF prognosis and response to treatment. There are no data regarding the relationship of VA coupling to outcome in pediatric dilated cardiomyopathy (DCM) patients. We investigated the...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-018-2021-6
更新日期：2019-02-01 00:00:00
abstract：:Studies of larger patient groups for systematic assessment of the anatomical accuracy of magnetic resonance imaging (MRI) for partial anomalous pulmonary venous drainage (PAPVD) have been performed so far only in adults. This study was undertaken to evaluate whether MRI can precisely depict pulmonary venous anatomy in...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-008-9367-0
更新日期：2009-05-01 00:00:00
abstract：:Magnesium sulfate was given to pediatric cardiac surgical patients during cardiopulmonary bypass period in an attempt to reduce the occurrence of postoperative junctional ectopic tachycardia (PO JET). We reviewed our data to evaluate the effect of magnesium on the occurrence of JET and assess a possible relationship b...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-015-1141-5
更新日期：2015-08-01 00:00:00
abstract：:We report on a 4-kg infant with Alagille syndrome and congenital heart disease consisting of atrial septal defect (ASD), patent ductus arteriosus (PDA), and severe peripheral pulmonic stenosis. He underwent successful orthotopic liver transplant along with catheter closure of the ASD and PDA using the Amplatzer device...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：
更新日期：2002-11-01 00:00:00
abstract：:This study examined the age-related criteria of signal-averaged electrocardiographic (SA-ECG) parameters in children. SA-ECGs were obtained in 82 healthy volunteers in six groups depending on age (group 1: 1 day to < 1 month; group 2: 1 month to < 1 year; group 3: 1 to < 6 years; group 4: 6 to < 12 years; group 5: 12 ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF00796320
更新日期：1994-05-01 00:00:00
abstract：:Fifty infants with interrupted aortic arch (IAA), admitted between 1979 and 1988, were reviewed. They usually presented early in severe cardiac failure or shock. In the initial 5-year period, 17 of the 21 infants underwent diagnostic or confirmatory cardiac catheterization, in contrast with the latter 5 years when onl...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF00838779
更新日期：1992-10-01 00:00:00
abstract：:The inauguration of Sequential Segmental Analysis (SSA) a few decades ago provides basic conditions to establish a universally applicable classification system for cardiac malformations. To gain practical experience, we used this method to classify a collection of 292 congenitally malformed human hearts. Our aims were...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/pl00021002
更新日期：2002-09-01 00:00:00
abstract：:A reduced exercise capacity is a common finding in adult congenital heart disease and is associated with cardiovascular morbidity and mortality. However, data on exercise capacity in patients after repair of coarctation of the aorta (CoA) are scarce. Furthermore, a high rate of exercise-induced hypertension has been d...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02173-5
更新日期：2019-10-01 00:00:00
abstract：:A primary rhabdomyosarcoma of the heart observed in a 3-week-old infant, provides an opportunity to review this rare clinical entity; 61 reported cases are collated. The more common sites are right ventricle, left atrium, and right atrium; the tumour may protrude into the cavity and cause obstruction. Echocardiography...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02265621
更新日期：1982-01-01 00:00:00
abstract：:The timing of pulmonary valve replacement (PVR) in asymptomatic patients with repaired tetralogy of Fallot (TOF) is typically based on cardiac magnetic resonance imaging-derived ventricular volume measurements. Current criteria do not account for sex-based differences in chamber size. The purpose of this study was to ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02181-5
更新日期：2019-10-01 00:00:00
abstract：:For adults with sickle cell disease (SCD), cardiac changes are well described and may account for up to one-fourth of disease-related mortality. Much less is known about heart disease in pediatric SCD patients. This retrospective study analyzed a contemporary cohort of children with SCD followed at a single tertiary c...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0587-y
更新日期：2013-04-01 00:00:00
abstract：:The objective of this study was to compare histopathological changes in hypoplastic left heart syndrome right ventricles (RV) of patients undergoing Sano and modified Blalock-Taussig (MBT) shunt and correlate them with echocardiographic findings. Myocardial tissue samples were obtained from hearts with Sano or MBT shu...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0462-x
更新日期：2013-02-01 00:00:00
abstract：:Calculating pulmonary vascular resistance is important in many fields of medicine. Although the influence of hematocrit on calculated resistance has been known for many years, it is rare to find the appropriate corrections in published articles. This review discusses the relationship between viscosity and resistance a...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-011-9954-3
更新日期：2011-06-01 00:00:00
abstract：:This study aimed to assess the technical aspects of atrial septal defect (ASD) closure using the Amplatzer septal occluder (ASO) and the Gore Helex septal occluder (GHSO) for infants weighing less than 8 kg and to determine the safety, effectiveness, and near-to-intermediate-term outcome of the closure. The Mid-Atlant...
journal_title：Pediatric cardiology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00246-014-0905-7
更新日期：2014-10-01 00:00:00
abstract：:Color-coded two-dimensional (2D) echocardiography confirmed the presence of severe congenital mitral regurgitation in an 8-month-old infant. Intraoperative inspection revealed an isolated perforation in the anterior leaflet. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02239570
更新日期：1990-04-01 00:00:00
abstract：:A 2100-g neonate underwent a two-ventricular surgical repair of a right ventricle-dominant unbalanced atrioventricular septal defect associated with the heterotaxy syndrome and sinus node dysfunction. Postoperative congestive heart failure persisted despite bradycardia management by temporary ventricular pacing. Spect...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002469900446
更新日期：1999-05-01 00:00:00
abstract：:That apoptosis is mediated by specific pathways has long been established. However, more recent data have begun to suggest that necrosis may in fact be "programmed" and not a default "accidental" pathway as previously thought. The mitochondrial permeability transition pore, a known contributor to the development of ma...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-010-9880-9
更新日期：2011-03-01 00:00:00
abstract：BACKGROUND:The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. Staged repair may be associated with a higher risk of inter-stage mortality, while primary repair may lead to frequent post-repair re-interventions. METHODS:From 2004 t...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-020-02352-9
更新日期：2020-06-01 00:00:00
abstract：:An atrial septal aneurysm (ASA) is an increasingly recognized entity that involves septal tissue significantly bulging into either atria instead of remaining in a relatively neutral position. ASAs may be described based on the length of the segment of atrial septal tissue involved as well as the distance and direction...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-018-2017-2
更新日期：2019-01-01 00:00:00
