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 care center. The study aimed to evaluate the effect of SCD on left main coronary artery (LMCA) dimensions in the contemporary pediatric population. The echocardiography database was searched for studies of children with SCD from 2000 to 2009. The LMCA diameter was remeasured offline from digital images. Digital echocardiographic studies of 68 children (35 boys) 11.7 ± 4.6 years of age with SCD were analyzed. All the patients had normal systolic function as measured by fractional shortening. The left ventricular end-diastolic dimension (LVEDD) for 34 % of the pediatric SCD patients showed a dilated LV. In 24 % of the children, a LV mass index (LVMI) greater than 50 g/m(2.7) denoted LV hypertrophy. Application of sex-specific normal values showed that 60 % of the boys and 33 % of the girls had an increased LVMI. The LMCA diameter was enlarged in 43 % of the SCD children, including 26 % with an LMCA z-score higher than 3. This study found a higher incidence of LMCA dilation in a pediatric referral population with SCD. The findings also validated an increased LVMI in the contemporary SCD patient population, which was noted previously. This study adds the need to include assessment of coronary arteries in cardiac evaluation of SCD patients, and special attention should be paid to patients with a high-normal to high coronary z-score.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Doshi AR,Pauliks LBdoi
10.1007/s00246-012-0587-ysubject
Has Abstractpub_date
2013-04-01 00:00:00pages
954-8issue
4eissn
0172-0643issn
1432-1971journal_volume
34pub_type
杂志文章abstract：:A 10-month-old boy was referred for tachypnea and heart murmur. An echocardiogram showed unexplained left heart dilation without evidence of an intracardiac shunt. A 64-slice computed tomographic contrast-enhanced angiography showed a large tortuous anomalous artery arising from the descending thoracic aorta and suppl...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-008-9287-z
更新日期：2009-01-01 00:00:00
abstract：:Rho-kinase (ROCK) belongs to the AGC (protein kinase A/protein kinase G/protein kinase C, PKA/PKG/PKC) family of serine/threonine kinases and is a major downstream effector of small GTPase RhoA. Rho-kinase is involved in a wide range of fundamental cellular functions such as contraction, adhesion, migration, and proli...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-011-9920-0
更新日期：2011-03-01 00:00:00
abstract：:Maintaining patency of the ductus arteriosus pending surgical intervention can be critical to the survival of the neonate with ductal dependent congenital heart disease. Spontaneously delayed ductal closure has been observed clinically and experimentally in newborns with critical pulmonic stenosis. Infants with ductal...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02263445
更新日期：1987-01-01 00:00:00
abstract：:The slip ring and nutate/rotate configuration of the new, fourth-generation, computed tomography (CT) (TCT-900S, Toshiba Medical Company) makes it possible to scan consecutively 25 slices every 75 s. This scanner also rapidly provides hemodynamic and multiplanar reconstruction images. Studies were performed on 13 chil...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02309917
更新日期：1989-04-01 00:00:00
abstract：:We report the placement of an implantable cardiac defibrillator as preventative treatment in a 2-year-old with long QT syndrome. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-003-5230-5
更新日期：2004-07-01 00:00:00
abstract：:Unroofed coronary sinus is a rare cardiac anomaly in which communication occurs between the coronary sinus and the left atrium due to the partial or complete absence of the roof of the coronary sinus. It is usually associated with other cardiovascular anomalies, especially persistent left superior vena cava. It is oft...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002460010087
更新日期：2000-07-01 00:00:00
abstract：:Internationally, there have been isolated case reports published of children presenting with dilated cardiomyopathy (DCM) in the setting of undiagnosed rickets. Although there has been an increased prevalence of rickets in the United States, there has been only one documented case of associated DCM. At our institution...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-009-9444-z
更新日期：2009-08-01 00:00:00
abstract：:Systemic candidiasis developed in a seven-week-old premature baby after 6 weeks treatment with antibiotics for suspected septicemia. At that time the echocardiogram showed a dense layer of echoes posteriorly to the anterior tricuspid leaflet during atrial systole. The diagnosis of Candida endocarditis with vegetations...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02281007
更新日期：1983-01-01 00:00:00
abstract：:Atrial flutter/fibrillation (AFL/AF) is a late complication in adults with repaired tetralogy of Fallot (TOF). Its effects on long-term prognosis are not fully understood. We evaluate the impact of AFL/AF in adults with repaired TOF on global mortality and unplanned hospitalizations during follow-up, and the predictor...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02106-2
更新日期：2019-06-01 00:00:00
abstract：:When the common pulmonary vein fails to develop, the embryonic connections of the pulmonary veins to one or more of the systemic veins almost always persist. Anomalous pulmonary venous connections to the inferior vena cava (IVC) are typically characterized by hypoplasia of the involved pulmonary veins and pulmonary ar...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-005-0920-9
更新日期：2005-11-01 00:00:00
abstract：:Acute pulmonary vasodilator testing (AVT) is essential to determining the initial therapy for children with pulmonary arterial hypertension (PAH). This study aimed to report the initial experience with inhaled treprostinil used for AVT in children with PAH and to evaluate the hemodynamic change after inhaled treprosti...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0597-9
更新日期：2013-04-01 00:00:00
abstract：:A 6-year-old girl with evidence of a parahisian accessory pathway on a baseline electrocardiogram underwent successful catheter ablation using magnetic navigation. Magnetic remote controlled ablation eliminated the parahisian pathway with the first radiofrequency application. A second anterolaterally located concealed...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0540-0
更新日期：2013-03-01 00:00:00
abstract：:The American Academy of Pediatrics (AAP) recommends that any child diagnosed with hypertension have an echocardiogram to evaluate for the presence of left-ventricular (LV) hypertrophy (LVH) and advocates that LVH is an indication to initiate or intensify antihypertensive therapy. However, there is no consensus on the ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0829-7
更新日期：2014-04-01 00:00:00
abstract：:Long-term neurodevelopmental sequelae are commonly detectable in children after open-heart surgery with cardiopulmonary bypass (CPB). The objective of the study was to determine the neurodevelopmental outcome in these children in relation to postoperative inflammatory reaction. This is a prospective, observational stu...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-008-9354-5
更新日期：2009-04-01 00:00:00
abstract：:An aneurysm of the fossa ovalis was identified at autopsy in 17 infants. In each case the aneurysm of the fossa ovalis was considered to have resulted from excessive elevation of pressure in that atrium contralateral to the side into which the aneurysm bulged. The aneurysms were divided into two types: intrinsic (thre...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02282740
更新日期：1985-01-01 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：:DiGeorge syndrome (DGS) is commonly associated with both congenital heart disease (CHD) and immunologic abnormalities. While CHD may prompt consideration for heart transplantation (HTx), little is known about HTx management or outcomes in this group. The aim of this study was to describe the spectrum of patients with ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02063-w
更新日期：2019-04-01 00:00:00
abstract：:A case of cerebral hemorrhage associated with sildenafil (Revatio) use in an infant is presented. Sildenafil is increasingly used in the treatment of primary and secondary pulmonary arterial hypertension and pulmonary arteriovenous fistula. In the reported case, sildenafil used to treat pulmonary arteriovenous fistula...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-009-9460-z
更新日期：2009-10-01 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：:Anomalous origin of left pulmonary artery (ALPA) from aorta with atrial septal defect (ASD) is a rare congenital cardiac malformation. We report a case of ALPA from the aorta with ASD in a 1.8-kg baby. Repair was needed at an early age due to progressive congestive heart failure. Surgical correction was performed with...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-008-9234-z
更新日期：2008-07-01 00:00:00
abstract：:The hybrid pathway for the management of patients with hypoplastic left heart syndrome was pioneered at our institution and is the preferred approach compared with the traditional Norwood pathway. Patients undergoing this surgery are generally <6 months of age, and pain management in this age group after surgery for c...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0233-8
更新日期：2012-10-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00246-017-1756-9
更新日期：2018-02-01 00:00:00
abstract：:Increased risk of perioperative hemorrhage has been described in patients with Turner syndrome and coarctation of the aorta. We have operated on 11 patients with Turner syndrome and coarctation of the aorta, two of whom developed bleeding. One patient died. End-to-end anastomosis was performed in all patients. Histolo...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02427041
更新日期：1984-07-01 00:00:00
abstract：:Chylothorax is a well-described complication after cardiothoracic surgery in children. Medical nutritional therapy for chylothorax includes medium-chain triglyceride (MCT) formulas and reduction in enteral long-chain triglyceride intake to reduce chyle production. Human milk is usually eliminated from the diet of infa...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-016-1393-8
更新日期：2016-08-01 00:00:00
abstract：:Cardiac surgery for congenital heart disease often necessitates a period of myocardial ischemia during cardiopulmonary bypass and cardioplegic arrest, followed by reperfusion after aortic cross-clamp removal. In experimental models, myocardial ischemia-reperfusion is associated with significant oxidative stress and ve...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-016-1345-3
更新日期：2016-04-01 00:00:00
abstract：BACKGROUND:Cardiac magnetic resonance imaging (MRI) is an important diagnostic tool for congenital heart disease (CHD), as reflected by class 1 recommendations for the use of cardiac MRI by various consensus panels. However, little is known about the safety and clinical utility of cardiac MRI for these critically ill i...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-008-9295-z
更新日期：2009-02-01 00:00:00
abstract：:The slope of the relation between the unadjusted QT interval and heart rate during the face immersion test has been reported to be useful as an index for predicting an abnormal lengthening of the QT interval for children with nonfamilial long QT syndrome. Our goals were to determine whether we can replace the slope of...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-001-0034-y
更新日期：2002-03-01 00:00:00
abstract：:Ventricular arrhythmias (VAs) are common pediatric rhythm disorders requiring comprehensive laboratory evaluation. Although usually idiopathic, implying a benign character and favorable prognosis, the initial clinical approach is still not established in all cases. Considering their prognostic significance, exercise-i...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-009-9582-3
更新日期：2010-02-01 00:00:00
abstract：:There is often a diagnostic dilemma in pediatric patients presenting with depressed ventricular function, as myocarditis and dilated cardiomyopathy (DCM) of other etiologies can appear very similar. Accurate identification is critical to guide treatment and to provide families with the most accurate expectation of lon...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-018-1867-y
更新日期：2018-08-01 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
