Abstract：
:Left atrial isomerism includes a complex spectrum of cardiac and extracardiac anomalies. The records of all patients with left isomerism born during the period of 1973-2010 and treated at the Children's Hospital, Helsinki were reviewed. The short- and long-term outcomes were studied. The review included 38 patients (50% females). The overall survival with left atrial isomerism was 63% during a median follow-up time of 16 years (range, 4-30 years). Extracardiac anomalies were noted in 14 (37%) of 38 cases. Cardiac defects included dextrocardia in 26%, partially or totally anomalous pulmonary venous return in 29%, common atrium in 50%, atrioventriculoseptal defect in 73%, single ventricle in 40%, ventriculoseptal defect without atrioventricular defect in 11%, transposition in 21%, double outlet of the right ventricle in 26%, pulmonary stenosis or atresia in 61%, and left ventricular outflow obstruction in 24% of the cases. Cardiac arrhythmias were presented in 71% and pacemaker treatment in 29% of the cases. Of the 38 patients, 33 had cardiac surgery. Simple palliative methods were used in 11 cases, single-ventricle palliation in 12 cases, and operation with a biventricular track in 10 cases. In the groups that had surgery, 3 of 11 patients, 3 of 12 patients, and 3 of 10 patients died, respectively. In this review, 14 deaths occurred, associated with extracardiac anomalies in five cases and with cardiac arrhythmia in four cases. Five postoperative deaths occurred. At this writing, all three patients who had heart transplantation are alive. Complicated heart defects associated with severe arrhythmias and extracardiac anomalies contribute to a high mortality rate with left isomerism. Cardiac transplantation was considered a good option for selected patients.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Eronen MP,Aittomäki KA,Kajantie EO,Sairanen HIdoi
10.1007/s00246-012-0184-0subject
Has Abstractpub_date
2012-04-01 00:00:00pages
596-600issue
4eissn
0172-0643issn
1432-1971journal_volume
33pub_type
杂志文章abstract：:This review examines the mechanism of estrogen signaling in cardiomyocytes, with an emphasis on mechanisms that might be important in cardioprotection. It investigates estrogen signaling mediated by the nuclear estrogen receptors alpha and beta and the G-protein-coupled receptor (GPR 30/GPER). Estrogen signaling via n...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-010-9877-4
更新日期：2011-03-01 00:00:00
abstract：:The single right ventricle (RV) in hypoplastic left heart syndrome (HLHS) often develops systolic dysfunction with time and this affects prognosis. Mechanical dyssynchrony has been reported in HLHS but has not consistently correlated with systolic function or electrical dyssynchrony. The aims of this study were to ass...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02091-6
更新日期：2019-06-01 00:00:00
abstract：:Circulating progenitor cells have been extensively studied in the context of heart disease in adults. In these patients, they have been demonstrated to be markers of myocardial injury and recovery as well as potential therapeutic agents. However, studies in children are much more limited. Here we review current knowle...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-015-1300-8
更新日期：2016-02-01 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：:Mammalian cardiomyocytes withdraw from the cell cycle soon after birth. This process is called terminal differentiation. The c-kit, a receptor tyrosine kinase, is expressed on cardiomyocytes immediately after birth but for only a few days. In mice with genetic c-kit dysfunction, adult cardiomyocytes are phenotypically...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-008-9366-1
更新日期：2009-07-01 00:00:00
abstract：:Balloon angioplasty for native coarctation of the aorta in infants and children is gaining acceptance as an alternative to surgery in discrete membranous obstruction. The aim of this study was to assess the immediate and intermediate-term effectiveness and safety of balloon angioplasty in infants and children with dis...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-007-9029-7
更新日期：2008-05-01 00:00:00
abstract：:Although liver fibrosis causes significant morbidity in the late postoperative period of the Fontan procedure, the diagnostic value of hyaluronic acid (HA), a serum marker of liver fibrosis, has not been established in Fontan patients. The purpose of this study was to determine whether increased serum HA concentration...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0827-9
更新日期：2014-04-01 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：:In this report, we describe a case of coarctation of the aorta, congenital aortic stenosis with bicuspid valve, and supravalvar aortic stenosis with left coronary artery ostial stenosis. The child underwent staged treatment using a hybrid approach employing both surgical and interventional cardiology methods. A single...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002460010164
更新日期：2001-01-01 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 original version of this article unfortunately contained a mistake. The presentation of Table 2 was incorrect .The corrected table is given below. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章,已发布勘误
doi：10.1007/s00246-018-1816-9
更新日期：2018-03-01 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：:In 19 consecutive children with aortic valve stenosis, the left ventricle was entered retrogradely with a Gensini catheter guided by a tip-deflector guidewire which could produce any desirable degree of curvature at its tip. In all patients, the aortic valve was retrogradely traversed for measurement of the pressure g...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02328955
更新日期：1986-01-01 00:00:00
abstract：:A 2-year-old girl with cor triatriatum with unroofed coronary sinus and persistent left superior vena cava underwent preoperative radiofrequency catheter ablation for atrial tachycardia attack that developed after admission to our hospital. The procedure was not successful. Cryoablation was successfully performed duri...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-002-0376-0
更新日期：2003-09-01 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
abstract：:This report describes a case involving the rare combination of persistent truncus arteriosus type A2, double aortic arch, and mitral stenosis. At the age of 26 days, the patient underwent division of the right-sided aortic arch with tracheal compression and bilateral pulmonary banding. Fontan completion was successful...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0572-5
更新日期：2013-01-01 00:00:00
abstract：:Right ventricular (RV) remodeling in hypoplastic left heart syndrome (HLHS) begins prenatally and continues through staged palliations. However, it is unclear if the most marked observed remodeling post-Norwood is secondary to cardiopulmonary bypass (CPB) exposure or if it is an adaptation intrinsic to the systemic RV...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-020-02482-0
更新日期：2020-10-10 00:00:00
abstract：:Various surgical techniques have been proposed for the repair of supravalvular pulmonary stenosis (SVPS) in pediatric populations. Whereas growth potential should be promised, excessive expansion under the presence of undiminished high pulmonary arterial pressure should be avoided. The authors applied all-autologous t...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0698-0
更新日期：2013-10-01 00:00:00
abstract：:Acute respiratory distress syndrome (ARDS) in children after open heart surgery, although uncommon, can be a significant source of morbidity. Because high-frequency oscillatory ventilation (HFOV) had been used successfully with pediatric patients who had no congenital heart defects, this therapy was used in our unit. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0655-y
更新日期：2013-08-01 00:00:00
abstract：:The aim of this study was to evaluate by Doppler echocardiography whether administration of transdermal nitroglycerin (NTG) to children with congestive heart failure could modify mitral flow velocity profile with redistribution of left ventricular filling to late diastole, suggesting preload reduction of the left vent...
journal_title：Pediatric cardiology
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1007/s002460010144
更新日期：2001-01-01 00:00:00
abstract：:A two-year-old child who had had palliative surgery as a neonate for an absent right atrioventricular connection and double-inlet ventricle was shown to be developmentally retarded. Trisomy for the short arm of chromosome 8 was demonstrated, resulting from a familial 8:13 translocation, with a high risk of recurrence....
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02306750
更新日期：1984-01-01 00:00:00
abstract：:Surgical closure of the secundum type of atrial septal defect (ASD) in childhood leads to excellent survival. However, relevant morbidity has been reported. Transcatheter closure of these defects has now become an alternative approach. To compare the results of the two different interventions, reliable data are needed...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-005-0838-2
更新日期：2005-09-01 00:00:00
abstract：:We sought to describe the use and outcomes of small, medium and large premounted stents in patients with congenital heart disease, including incidence of and risk factors for re-intervention and development of in-stent stenosis. Premounted stents offer several advantages over traditional manually crimped bare-metal st...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-016-1466-8
更新日期：2016-12-01 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：:A case report of a neonate with cardiac tumors in association with tuberous sclerosis is presented. The rare complication of pulmonary embolism and its successful treatment by anticoagulation and thrombolytic therapy is described. The treatment of these cardiac tumors is conservative, because the tumors regress sponta...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002460010247
更新日期：2001-07-01 00:00:00
abstract：:We analyzed early and late results of surgical treatment of 100 consecutive children with Down's syndrome (DS) and congenital heart defect (CHD) who were operated on between 1990 and 1997. Fifty had common atrioventricular canal (CAVC), 24 ventricular septal defect, 8 the ostium primum atrial septal defect, 8 tetralog...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002469900483
更新日期：1999-09-01 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：BACKGROUND:The purpose of this study was to investigate the associations between clinical factors and cardiac function as measured by pressure-volume loops (PVLs) in a pediatric heart transplant cohort. METHODS:Patients (age < 20 years) who underwent heart transplantation presenting for a clinically indicated catheter...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-017-1758-7
更新日期：2018-02-01 00:00:00
abstract：:Common arterial trunk is relatively a straightforward diagnosis on echocardiography. We describe a neonate who was referred to our centre as a case of common arterial trunk but on evaluation was found to have pulmonary atresia with ventricular septal defect and aortopulmonary window, for which he underwent repair with...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0240-9
更新日期：2012-10-01 00:00:00
abstract：:Exercise testing can highlight repolarisation abnormalities in adults with coronary artery disease. Late after Kawasaki disease (KD), increased QT dispersion (QTd) has been reported on resting ECG in children, but there are no reported studies of QTd during exercise. Our objective was to determine the pattern of QTd r...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0201-3
更新日期：2012-06-01 00:00:00
