Abstract：
:There is a higher incidence of sudden death in patients with Rett syndrome than individuals in the general population. Previous studies have implicated cardiac dysautonomia and a long QT interval as causative factors. Because carnitine plays a critical role in cellular metabolism and may have beneficial effects on cardiac and nerve function, we investigated the effects of long-term treatment with acetyl-L-carnitine on heart rate variability and electrocardiographic abnormalities in 10 girls with Rett syndrome and compared the results with 12 control patients (girls with Rett syndrome who were not treated). The age range of the subjects was 2-21 years. The study design called for the evaluation of heart rate variability, corrected QT interval, and QTc dispersion. In the 10 Rett girls treated with acetyl-L-carnitine, a significant increase in heart rate variability was observed. To explain these results, we hypothesize that acetyl-L-carnitine has a neurotrophic action on the cardiac autonomic nervous system. This effect may reduce the risk of sudden death in patients with this syndrome.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Guideri F,Acampa M,Hayek Y,Zappella Mdoi
10.1007/s00246-005-0784-zsubject
Has Abstractpub_date
2005-09-01 00:00:00pages
574-7issue
5eissn
0172-0643issn
1432-1971journal_volume
26pub_type
杂志文章,随机对照试验abstract：:This report aims to summarize and evaluate the efficacy and experience of reintervention for children with postoperative pulmonary venous restenosis. Clinical data from 61 patients with postoperative pulmonary venous restenosis who underwent reintervention at Shanghai Children's Medical Center (SCMC) from September 20...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02098-z
更新日期：2019-06-01 00:00:00
abstract：:Left ventricular hypertrophy is an adaptive mechanism in children undergoing chronic dialysis to improve contractility at rest. The aim of this study was to determine the left ventricular performance and contractility reserve by "dobutamine stress echocardiography" in children undergoing chronic dialysis. Thirty-five ...
journal_title：Pediatric cardiology
pub_type： 临床试验,杂志文章
doi：10.1007/s00246-010-9721-x
更新日期：2010-10-01 00:00:00
abstract：:Venous correction for complete transposition with intact ventricular septum remains the preferred surgical option in most centers (Mustard or Senning procedures). The long-term function of the morphologically right ventricle and tricuspid valve, which continue to sustain the systemic circulation thereafter, remains a ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：
更新日期：1986-01-01 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：:The objective of this study was to evaluate the incidence of pre-existing catheterization left pulmonary artery (LPA) gradients and correlation of these gradients with later LPA stenosis after successful patent ductus arteriosus (PDA) occlusion. We performed a single-center review of 130 patients with PDA closure from...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-009-9448-8
更新日期：2009-10-01 00:00:00
abstract：:This study aimed to assess the impact of integrating a simulation-based education module into an extracorporeal membrane oxygenation (ECMO) curriculum on novice learners and to test the duration of time that skills obtained during this training exercise were retained. The authors hypothesized that multidisciplinary, s...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0490-6
更新日期：2013-03-01 00:00:00
abstract：:A newborn infant with familial Wolff-Parkinson-White (WPW) syndrome presented with a supraventricular tachycardia of 300 beats/min, refractory to digoxin and flecainide administration. Serial electropharmacologic tests were performed via the esophagus before and during oral therapy with verapamil at 40, 80, and 60 mg ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02238370
更新日期：1990-10-01 00:00:00
abstract：:A rare form of endocardial cushion defect is an atrioventricular (AV) canal-type ventricular septal defect with cleft mitral valve in the absence of a primum atrial septal defect. In two infants with Down's syndrome, preoperative delineation of this entity was accomplished. M-mode echocardiographic features included p...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02426975
更新日期：1982-01-01 00:00:00
abstract：:The development of Leadless cardiac pacemakers avoids the inherent complications that may occur secondary to lead insertion. A large number of devices have been inserted in adult patients although data in pediatric patients are lacking. We aimed to assess our experience with the Leadless device in the pediatric popula...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02277-y
更新日期：2020-04-01 00:00:00
abstract：:Radiation exposure from pediatric cardiac catheterization may be substantial, although published estimates vary. We sought to report patient radiation dose across a range of diagnostic and interventional cases in a modern, high-volume pediatric catheterization laboratory. We retrospectively reviewed diagnostic and int...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-014-0869-7
更新日期：2014-06-01 00:00:00
abstract：:Adrenomedullin (ADM) is a vasoactive peptide with potent dilatory effects. We studied whether perioperative myocardial injury could be altered by the presence of ADM. Blood samples from 19 children with congenital heart disease undergoing surgical repair were collected at six time points: preoperative, on cardiopulmon...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002460010046
更新日期：2000-05-01 00:00:00
abstract：:B-type natriuretic peptide (BNP) reflects volume overload on left ventricle and pulmonary hypertension (PH) in patients with ventricular septal defect (VSD). Pulmonary vascular resistance (PVR) has been reported to correlate positively with BNP in VSD patients with various degrees of PH. We aimed to investigate the re...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-007-9037-7
更新日期：2008-01-01 00:00:00
abstract：:Acute myopericarditis is a major cause of acquired heart disease worldwide in pediatric patients. Various viruses have been reported as the etiology, of which varicella zoster virus was first reported in 1953. However, since nationwide administration of the varicella vaccine, reports of varicella-associated myopericar...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-009-9637-5
更新日期：2010-07-01 00:00:00
abstract：:Long-term ECG is widely used in diagnosis and assessment of many cardiac symptoms which may be caused by dangerous arrhythmias that sometimes can be difficult to document. The PocketECG system is a new technological solution for a long-term, noninvasive, continuous and real-time ECG monitoring that provides automatic ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-016-1534-0
更新日期：2017-03-01 00:00:00
abstract：:Little data are published about right ventricular (RV) growth and function in infants and neonates after balloon pulmonary valvuloplasty (BPV) for critical valvular pulmonary stenosis (PS). We aimed to assess the RV growth and function during 1 year after BPV for critical valvular PS in neonates and infants. A total o...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-020-02314-1
更新日期：2020-04-01 00:00:00
abstract：:We report a rare case of tetralogy of Fallot with total anomalous pulmonary venous return, left heart hypoplasia, right lung hypoplasia, and left ocular-mandibular synchinesia (Marcus-Gunn phenomenon), correctly diagnosed by cardiovascular magnetic resonance imaging and successfully operated by modified Glenn anastomo...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-004-0769-3
更新日期：2005-07-01 00:00:00
abstract：:A 6-year-old girl with type 3 long QT syndrome was safely and successfully implanted with an implantable cardioverter-defibrillator (ICD) system. Prior to implantation, she had experienced uncontrollable life-threatening arrhythmia in spite of high-dose administration of mexiletine. An ICD coil lead for transvenous us...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-011-0065-y
更新日期：2011-10-01 00:00:00
abstract：:Device closure of atrial septal defects (ASDs) is rapidly becoming routine in children. Comparisons are often made to older surgical series with higher morbidities. However outcomes including reintervention, late failure, and the need for long-term follow-up must be considered and compared to those of a current surgic...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-002-0131-6
更新日期：2003-09-01 00:00:00
abstract：BACKGROUND:This study aimed to measure and compare right ventricular (RV) function in normal infants and those with pulmonary hypertension (PHT) using the myocardial performance index (RVMPI) and to investigate the relationship between RV function and pulmonary artery pressure. METHODS:A case-control study measured RV...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-008-9285-1
更新日期：2009-02-01 00:00:00
abstract：:Patency of the ductus arteriosus (PDA) is a common finding in small premature infants. Recently pulsed-Doppler-cross sectional echocardiography (PD-CSE) has been successfully used in these patients. We report a case of a premature infant with an unusual PD-CSE pattern. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02279882
更新日期：1988-01-01 00:00:00
abstract：:Two-dimensional echocardiography was performed on a patient with criss-cross atrioventricular (AV) relationship and ventriculo-arterial discordance. A systematic approach from the subcostal area revealed the complex cardiac pathology including the criss-cross AV relationship of right and left heart, indicating that th...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02427034
更新日期：1982-01-01 00:00:00
abstract：:Two patients (one girl, one boy) with Kartagener syndrome (situs inversus, bronchiectasis, sinusitis), despite pulmonary problems and associated congenital cardiac anomalies, were operated on at the ages of 4 years and 7 years, respectively. They had had previous palliative treatment at the age of 3 months and 1.3 yea...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002469900115
更新日期：1997-01-01 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：:Human pluripotent stem cells (hPSCs) offer a multifaceted platform to study cardiac developmental biology, understand disease mechanisms, and develop novel therapies. Remarkable progress over the last two decades has led to methods to obtain highly pure hPSC-derived cardiomyocytes (hPSC-CMs) with reasonable ease and s...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-019-02165-5
更新日期：2019-10-01 00:00:00
abstract：:The epidemiologic data for pediatric hypertrophic cardiomyopathy (HCM) needs to be periodically updated as diagnostic techniques and management strategies improve. Herein, the incidence, prevalence, and mortality rates of pediatric HCM in a population-based treatment system are described. Patients aged ≤ 17 years and ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-020-02472-2
更新日期：2020-10-03 00:00:00
abstract：:Children requiring reimplantation of a branch pulmonary artery (PA) are at risk for postoperative stenosis and impaired growth of the reimplanted PA. Outcomes and risk factors for reintervention and impaired growth are incompletely described. We reviewed data on patients who underwent reimplantation of a branch PA bet...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-017-1767-6
更新日期：2018-02-01 00:00:00
abstract：:The relationship between pulmonary function and right ventricle (RV) in Duchenne muscular dystrophy (DMD) has not been evaluated. Using cardiac magnetic resonance (CMR), we describe the relationship of RV size and function with spirometry in a DMD cohort. Fifty-seven boys undergoing CMR and pulmonary function testing ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-016-1362-2
更新日期：2016-06-01 00:00:00
abstract：:A 16-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery, Bland-White-Garland syndrome, underwent a mammary artery bypass grafting to the left coronary artery (LCA) together with closure of the stenosed origin of the left coronary artery. A residual LCA to pulmonary artery fistula ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002469910028
更新日期：2000-03-01 00:00:00
abstract：:In chronic aortic insufficiency (AI), left-ventricular (LV) dysfunction must be detected early to allow timely surgery. Strain and strain rate have been used for this purpose in adults, but the value of this method in pediatric AI has not been established. Forty patients with moderate to severe AI were included in thi...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0646-z
更新日期：2013-08-01 00:00:00
abstract：:We present a case of congenital pulmonary systemic collateral vein associated with truncus arteriosus. Pulmonary systemic collateral vein with nonobstructed left atrial egress is different from those with obstructed left atrial egress in that it is functionally redundant. Including this case, 8 patients among 33 repor...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-002-0439-2
更新日期：2004-01-01 00:00:00
