Abstract:
:This study aimed to clarify the characteristics of pulmonary arterial resistance (Rp)-compliance (Cp) coupling in individuals with Down syndrome (DS), who have increased risks of pulmonary arterial hypertension (PAH). We performed cardiac catheterization before and after corrective surgery in 85 DS infants and 85 controls with congenital heart disease and PAH. We retrospectively collected hemodynamic data and compared Rp and Cp between the groups. Age at surgery was 3.5 (2.6-4.6) months. The first and second catheterizations were performed 1 month before and after corrective surgery in both groups. Preoperative Cp in DS patients was significantly lower than that in controls [2.27 (1.62-3.0) vs. 2.50 (1.86-3.31) mL/mmHg/m2, p = 0.039], although there was no significant difference in mean pulmonary arterial pressure and Rp between the groups. Analysis of covariance revealed that the slopes of the preoperative regression lines for the logarithmic transformations of Rp and Cp were identical in DS patients and controls (p = 0.299). However, the postoperative regression line was shifted downward in DS patients after corrective surgery. Postoperative home oxygen therapy (HOT) was performed in 39 patients (36 DS patients) and multivariate logistic regression analysis revealed that postoperative HOT was significantly related to low preoperative Cp (p = 0.039) and DS (p = 0.0001). Individuals with DS have the unique pulmonary vasculature characterized with low Cp that is related to postoperative HOT.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Iwaya Y,Muneuchi J,Inoue Y,Watanabe M,Okada S,Ochiai Ydoi
10.1007/s00246-019-02080-9subject
Has Abstractpub_date
2019-04-01 00:00:00pages
841-847issue
4eissn
0172-0643issn
1432-1971pii
10.1007/s00246-019-02080-9journal_volume
40pub_type
杂志文章abstract::Different devices have been used for percutaneous closure of ventricular septal defects (VSDs). Over the past 6 months, we have used the Amplatzer duct occluder for VSDs associated with tunnel-shaped aneurysm in three patients here at our institution. Due to the anatomic resemblance between such VSDs and the duct occl...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-007-9092-0
更新日期:2008-03-01 00:00:00
abstract::Intrathoracic ventricular aneurysms and diverticula can be differentiated by several criteria. Contractility is the only reliable parameter: aneurysms expand, whereas diverticula contract during ventricular systole. ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s002460010264
更新日期:2001-09-01 00:00:00
abstract::The most common modes of medical education for congenital heart disease (CHD) rely heavily on 2-dimensional imaging. Three-dimensional (3D) printing technology allows for the creation of physical cardiac models that can be used for teaching trainees. 3D printed cardiac models were created for the following lesions: pu...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-019-02146-8
更新日期:2019-08-01 00:00:00
abstract::Kawasaki disease (KD) is an acute febrile disease of unknown etiology that develops in children and is sometimes accompanied by myocardial dysfunction and systemic vasculitis. However, myocardial repolarization lability has not yet been fully investigated. Thus, the objective of this study was to evaluate myocardial r...
journal_title:Pediatric cardiology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00246-011-9908-9
更新日期:2011-04-01 00:00:00
abstract::To reveal the association between DNMT1 polymorphisms and congenital heart disease (CHD) in child patients, a total of 224 CHD child patients as well as 199 healthy individuals were enrolled in the present study. The DNA was extracted from whole blood, and four SNPs including rs16999593, rs2228612, rs2288349 and rs104...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-015-1093-9
更新日期:2015-06-01 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::Congenital heart defects (CHDs) are the most prevalent of all birth defects and the leading cause of death in the first year of life. The molecular causes of most CHDs remain largely unknown. The LIM homeodomain transcriptor factor ISL1 is a marker for undifferentiated cardiac progenitor cells that give rise to both t...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-012-0578-z
更新日期:2013-04-01 00:00:00
abstract::Use of extracorporeal membrane oxygenation (ECMO) in infants with congenital heart disease is becoming more frequent. We present the first reported use of balloon atrial septostomy during ECMO support and describe possible complications of such procedures unique to ECMO therapy. ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF00795647
更新日期:1993-07-01 00:00:00
abstract::Congenital heart defect (CHD) represents the most prevalent birth defect, and accounts for substantial morbidity and mortality in humans. Aggregating evidence demonstrates the genetic basis for CHD. However, CHD is a heterogeneous disease, and the genetic determinants underlying CHD in most patients remain unknown. In...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-018-1822-y
更新日期:2018-04-01 00:00:00
abstract::Protein-losing enteropathy (PLE) and plastic bronchitis (PB) are major causes of long-term mortality after Fontan operation. The objective of this study was to determine early clinical risk factors before the onset of PLE and PB. In a cohort study, 106 Fontan patients between 2005 and 2013 were examined. A median of 5...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-017-1632-7
更新日期:2017-08-01 00:00:00
abstract::Sleep-disordered breathing (SDB) is described in patients with acquired heart failure but its prevalence in adults with congenital heart disease is not well documented. It is likely that single-ventricle patients who have undergone Fontan palliation poorly tolerate the additional stress of SDB on their fragile cardiac...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-020-02403-1
更新日期:2020-10-01 00:00:00
abstract::In the classic scimitar syndrome, a pulmonary vein draining all or part of the right lung enters the inferior vena cava. A variant is described with the same roentgenographic appearance, but with drainage of the anomalous pulmonary vein into both the inferior vena cava and the left atrium; the atrial septum was intact...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF02079472
更新日期:1987-01-01 00:00:00
abstract::Cardiopulmonary bypass (CPB) reduces coagulation factor levels through hemodilution and consumption. Differences in CPB-induced alterations of factor XIII (FXIII) levels in children with cyanotic and acyanotic congenital heart defects (CHDs) are not well characterized. FXIII activity (determined by Berichrom assay), p...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-014-0903-9
更新日期:2014-10-01 00:00:00
abstract::The HOXA1 gene plays a fundamental role in embryonic morphogenesis. Recent studies in humans and mice have indicated that HOXA1 plays a previously unrecognized role in cardiovascular system development. Congenital heart disease (CHD), particularly ventricular septal defect (VSD), might be a clinically isolated manifes...
journal_title:Pediatric cardiology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00246-012-0418-1
更新日期:2013-02-01 00:00:00
abstract::Aortopulmonary window coexisting with tetralogy of Fallot (TF) was prospectively diagnosed by two-dimensional (2D) echocardiography and Doppler in an 18-month-old boy; the diagnosis was confirmed by cardiac catheterization and angiocardiography. Surgical correction was performed, but the patient died in the operating ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF02239546
更新日期:1990-01-01 00:00:00
abstract::Double outlet right ventricle (DORV) was produced experimentally in the chick embryo by preventing the incorporation of the posteromedial conus into the left ventricle. This was accomplished by placing a ligature around the caudal end of the conus at a stage in which it is exclusively continuous with the right ventric...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF02240456
更新日期:1982-01-01 00:00:00
abstract::The response to warfarin is highly variable among individuals and such variability is likely to have some genetic basis. We evaluted the effect of VKORC1 polymorphisms on warfarin response among Japanese, taking advantage of its unique population structure in which CYP2C9 *2 and *3 alleles are relatively rare. Thirty-...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-005-1150-x
更新日期:2006-11-01 00:00:00
abstract::We present an infant with unusual anatomy and physiology of a persistent left superior vena cava (LSVC). The adverse physiologic consequences of the LSVC were corrected with percutaneous placement of vascular plugs. ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-009-9617-9
更新日期:2010-05-01 00:00:00
abstract::Pulmonary artery thrombosis in neonates occurs rarely. This report describes the case of a term infant with a pulmonary artery thrombosis presenting as persistent pulmonary hypertension of the newborn. The risk factors identified in the case included maternal diabetes and heterozygous factor V Leiden deficiency. The p...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-008-9349-2
更新日期:2009-05-01 00:00:00
abstract::A fetal echocardiographic scan was performed when routine prenatal ultrasound screening failed to identify four cardiac chambers. The scan showed a single ventricle with an associated circoid varicosity. Because of these anomalies, amniocentesis was suggested and trisomy 18 confirmed. The presence of major cardiac str...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF00794843
更新日期:1993-01-01 00:00:00
abstract::Right-ventricular diverticula (RVD), although rare, have particular characteristics. Our study describes the morphology of anterosuperior diverticula of the right ventricle (RV) and patient outcome for this anomaly. Retrospective analysis was performed on all patients with anterosuperior RVD diagnosed in two tertiary ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-014-0885-7
更新日期:2014-08-01 00:00:00
abstract::Plastic bronchitis (PB) is a poorly understood disease that can complicate any underlying pulmonary disease. However, it appears to most often occur in patients with surgically palliated congenital heart disease, particularly after the Fontan procedure. Few data exist about the prevalence and etiology of PB in this po...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-012-0430-5
更新日期:2013-02-01 00:00:00
abstract::The case report describes a 9-year-old boy who presented with an acute cerebrovascular accident and was found to have cardiac tamponade caused by cardiac rhabdomyosarcoma. Symptoms of rhabdomyosarcoma can be indolent and nonspecific, even with metastatic disease. Echocardiography and cardiac magnetic resonance imaging...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-012-0549-4
更新日期:2013-03-01 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::A large ductus arteriosus aneurysm can lead to potentially serious and life-threatening complications. We report a case of aneurysmal dilatation of the ductus arteriosus in a premature infant that was successfully treated with indomethacin. Thus, surgical intervention with general anesthesia could be avoided. ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-005-1164-4
更新日期:2006-01-01 00:00:00
abstract::Ultrasound Doppler was used to establish time of ductal closure, normal values for blood flow velocity in the pulmonary artery (PA), and time interval between pulmonary valve closure (Pc) and tricuspid valve opening (To) in 37 healthy neonates. Ductal closure had occurred in 23% of the children within 12 h after deliv...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:
更新日期:1986-01-01 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::Successful balloon valvuloplasty for critical pulmonary valve stenosis is described in an 800-g infant. A modified catheter was required to cross the valve in the smallest child known to undergo this procedure. ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s002460010288
更新日期:2001-11-01 00:00:00
abstract::Very young mammals have an impressive cardiac regeneration capacity. In contrast, cardiac regeneration is very limited in adult humans. The hearts of young children have a higher regenerative capacity compared with adults, as, for example, seen after surgical correction of an anomalous left coronary artery arising fro...
journal_title:Pediatric cardiology
pub_type: 杂志文章,评审
doi:10.1007/s00246-015-1120-x
更新日期:2015-04-01 00:00:00
abstract::Noonan syndrome is an autosomal dominant disease that manifests a wide variety of clinical characteristics. The syndrome is also associated with some cardiac defects. Half of all Noonan syndrome cases are caused by mutations in the PTPN11 gene, but only limited data are available regarding aortic involvement in these ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-009-9537-8
更新日期:2010-01-01 00:00:00