Abstract：
:We report our findings in the microscopic examination of the conduction system in four infants with glycogen storage disease, one of whom had adequate electrophysiologic studies. The electrophysiologic studies in the latter case showed P-A and A-H intervals at the lower limits of normal, but the H-V interval was just above the normal mean. This suggests that the rapid conduction was not localized in the anatomic counterpart of the H-V interval. The short P-R interval in the ECG may be related to the enlargement of cells, which may in turn be related to increased glycogen content. The relationship of glycogen per se to the speed of conduction is unknown. We found that the summit of the ventricular septum bulged, probably because of the generally increased cell size, and that the topography of the atrioventricular conducting system was different from normal. This is possibly related both to an increase in the cell sizes of the specialized conducting tissue itself and to deforming effects of this bulging summit of the ventricular septum. New microscopic details of the components of the conducting system are described in these cases.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Bharati S,Serratto M,DuBrow I,Paul MH,Swiryn S,Miller RA,Rosen K,Lev Mdoi
10.1007/BF02265613subject
Has Abstractpub_date
1982-01-01 00:00:00pages
25-32issue
1eissn
0172-0643issn
1432-1971journal_volume
2pub_type
杂志文章abstract：:A 9-year-old girl was admitted because she had had palpitations for 2 years. Her electrocardiogram revealed multifocal ectopic beats with left bundle branch morphology, sinoatrial block, and first- and second-degree atrioventricular blocks. Cross-sectional echocardiography demonstrated an enlarged left ventricle and f...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02505810
更新日期：1996-01-01 00:00:00
abstract：:This prospective observational study conducted in a neonatal intensive care unit aimed to evaluate echocardiographic changes provoked by anemia and transfusion of packed red blood cells (pRBCs) in premature infants. In this study, 32 anemic premature infants had serial echocardiographic assessment of left ventricular ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0154-6
更新日期：2012-04-01 00:00:00
abstract：:Neo-aortic arch obstruction (NAAO) is a common complication following the Norwood/Sano procedure (NP) for hypoplastic left heart syndrome (HLHS) and is associated with increased morbidity and mortality. However, there is currently no objective method for predicting which patients will develop NAAO. This study was desi...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0713-5
更新日期：2013-01-01 00:00:00
abstract：:A cohort of 52 French unrelated infant cases who died unexpectedly before they reached 12 months of age was blindly investigated to better quantify the contribution of long-QT syndrome (LQTS) genetic variants in French cases of sudden infant death syndrome (SIDS). After a standardized autopsy protocol, a blinded molec...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-009-9417-2
更新日期：2009-05-01 00:00:00
abstract：:This report describes the case of two pediatric patients who demonstrated echocardiographic evidence of pulmonary hypertension (PH) during the acute phase of Kawasaki disease. The etiology of PH development in this setting is currently unknown, but the authors hypothesize that pulmonary vasculitis may play a significa...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-012-0510-6
更新日期：2013-01-01 00:00:00
abstract：:Aortic atresia and interrupted aortic arch is a rare cardiac combination. Review of the literature revealed nine cases. We present two patients with this combination and the additional finding of quadricuspid pulmonary valves, one of which was severely stenotic. In the latter patient, an aortopulmonary window was pres...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-004-0754-x
更新日期：2005-07-01 00:00:00
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 ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-006-0066-4
更新日期：2007-09-01 00:00:00
abstract：:The COVID-19 pandemic has had devastating direct consequences on the health of affected patients. It has also had a significant impact on the ability of unaffected children to be physically active. We evaluated the effect of deconditioning from social distancing and school shutdowns implemented during the COVID-19 pan...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-020-02513-w
更新日期：2021-01-04 00:00:00
abstract：:Although transthoracic echocardiography (TTE) is the first-line examination for the study of coronary lesions in Kawasaki disease, CT coronary angiography (CTCA) is increasingly used and showed good results. Our aim is to evaluate the contribution of CTCA in the detection of coronary lesions and to compare its results...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-018-2044-z
更新日期：2019-02-01 00:00:00
abstract：:Two cases of arrhythmogenic right ventricular dysplasia (ARVD) in siblings are reported. In the boy, 14 years old, the clinical history, ECG, echocardiography, and histopathological findings were consistent with ARVD. Premature ventricular contractions of left bundle branch block (LBBB) pattern were recorded but no ve...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02239576
更新日期：1990-04-01 00:00:00
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：:We sought to characterize the shifting epidemiology and resource utilization of Lyme disease and associated carditis in US children's hospitals. We hypothesized that the Lyme carditis burden has increased and that hospitalizations for Lyme carditis are costlier than those for Lyme disease without carditis. The PHIS da...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02250-9
更新日期：2020-02-01 00:00:00
abstract：:With the advent of cardiac magnetic resonance imaging and high-resolution echocardiography, cardiac catheterization is unnecessary in clinical protocols in the "routine" single ventricle patient. Catheterization adds little to clinical care in these cases, and there are significant risks and costs associated with it. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-004-0960-6
更新日期：2005-03-01 00:00:00
abstract：:Natriuretic peptides (NP) are released from the heart in response to pressure and volume overload. The biologic properties of NPs include counterregulation of the rennin-angiotensin-aldosterone pathway and a decrease in sympathetic tone resulting in diuresis, natriuresis, and vasodilation. Natriuretic peptides help to...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-010-9758-x
更新日期：2010-11-01 00:00:00
abstract：:The purpose of this study was to test the hypothesis that the incidence of crochetage on the R wave in inferior limb leads can be used for the diagnosis of pediatric secundum atrial septal defect (ASD). Two hundred fifty-six children with secundum ASD (case cohort) and 256 age- and gender-matched children without hear...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-018-1857-0
更新日期：2018-06-01 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 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：:Apelin is an endogenous inotrope that decreased in heart failure (HF). We aimed to evaluate the prognostic value of its level in children with HF due to congenital heart disease (CHD). Sixty children with HF due to CHD were included as a patient group. Sixty healthy children matched for age, sex, and weight served as ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-018-1879-7
更新日期：2018-08-01 00:00:00
abstract：:Noninvasive Doppler-derived cardiac output was measured with the pulsed Doppler method in 22 healthy newborns during their first four days of life. Maximal blood flow velocity in the aorta was measured with the Doppler ultrasound method. The mean Doppler-derived cardiac output was 273 +/- 59 ml/min/kg. Ductal left-to-...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02083704
更新日期：1988-01-01 00:00:00
abstract：:Recurrent congenital left atrial aneurysm presenting in a newborn as cardiac tamponade is described. Such a presentation has not been previously reported. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002469900269
更新日期：1998-03-01 00:00:00
abstract：:Congenital aneurysms of the thoracic venous system are rare. In particular, innominate venous aneurysms are extremely rare. We describe a 16-year-old girl whose chest x-ray suggested a mediastinal tumor. Three-dimensional contrast-enhancement magnetic resonance venography showed a giant sacciform aneurysm of the left ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-003-0499-y
更新日期：2004-07-01 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：:In the fetus, the cardiac neural crest gives rise to both the thymus and the conotruncus of the heart. In newborn screening for severe T-cell lymphopenia neonates with congenital heart defects may be detected. In this study, we investigated the occurrence of T-cell lymphopenia in neonates with or without 22q11.2 delet...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-020-02317-y
更新日期：2020-04-01 00:00:00
abstract：:Intravenous immunoglobulin (IVIG) administered in the acute stage of Kawasaki disease (KD) is the standard therapy. Few reports describe nonresponders to initial treatment with IVIG in KD, which remains the most consistent risk factor for coronary artery lesions (CALs). This study aimed to investigate whether the seru...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0724-2
更新日期：2013-01-01 00:00:00
abstract：:In the very young child (less than eight years of age), transient loss of consciousness represents a diagnostic and management dilemma for clinicians. While most commonly benign, syncope may be due to cardiac dysfunction which can be life-threatening. It can be secondary to an underlying ion channelopathy, cardiac inf...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-020-02498-6
更新日期：2021-01-02 00:00:00
abstract：:Although recognized by pediatric cardiac surgeons, aortic insufficiency as a technical complication after tetralogy of Fallot repair is poorly documented, especially if it occurs late. The case of a boy with aortic insufficiency 10 years after complete tetralogy repair is described. No documentation in the literature ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF00817618
更新日期：1994-03-01 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：:Congenital diverticulum of the ventricle is a rare disease, but most cases of congenital left ventricular diverticula are asymptomatic. We present a child with congenital left ventricular diverticulum whose routine electrocardiographic examination showed T-wave inversion in inferior and V4 to V6 leads. He was successf...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-010-9719-4
更新日期：2010-08-01 00:00:00
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：:Left ventricular (LV) function is impaired by increased afterload in neonates with severe coarctation of the aorta, which may result in endocardial fibroelastosis. Repair of the coarctation usually solves the problem, with LV function normalizing after a few weeks. This report describes a patient who underwent success...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-009-9595-y
更新日期：2010-02-01 00:00:00
