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 deletion syndrome (del) suffering from heart defects. This retrospective cohort study included 125 patients with heart defects. T-cell receptor excision circles (TRECs), a measure for T-cell lymphopenia, were quantified by RT-PCR using stored newborn screening blood spots. Three patient groups were compared: non-conotruncal defects (n = 57), conotruncal defects (n = 42), and 22q11.2 del with conotruncal defects (n = 26). Significantly lower TREC values were detected in patients with 22q11.2 del and conotruncal heart defects compared to those with non-syndromic conotruncal (p < 0.001) and non-conotruncal (p < 0.001) defects. In contrast, no significant difference was found between patients with non-syndromic conotruncal and non-conotruncal heart defects (p = 0.152). Low TREC levels were obtained in neonates treated with heart surgery/intervention within 2 weeks after birth and in those with a fatal outcome (p = 0.02) independent of patient group. A correlation was found between low TREC numbers and oxygen saturation, SpO2 below 95% (p = 0.017). The SpO2 was significantly lower in the non-syndromic conotruncal group compared to non-conotruncal (p < 0.001) and 22q11.2 del group (p = 0.015). No correlation was found between low neonatal TRECs and infections needing hospitalization later in life (p = 0.135). Patients with 22q11.2 del and conotruncal defects have significantly lower TREC levels compared to patients with heart defects without this syndrome.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Gul KA,Strand J,Pettersen RD,Brun H,Abrahamsen TGdoi
10.1007/s00246-020-02317-ysubject
Has Abstractpub_date
2020-04-01 00:00:00pages
809-815issue
4eissn
0172-0643issn
1432-1971pii
10.1007/s00246-020-02317-yjournal_volume
41pub_type
杂志文章abstract：:A 12-year-old male presented with a 6-week history of apparent digital vasculitis with color changes of the distal fingers and erythematous macules of the palms and soles. Physical examination revealed skin findings of Carney complex and an abnormal cardiac examination. Echocardiography demonstrated a large left atria...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-005-1238-3
更新日期：2006-03-01 00:00:00
abstract：:Over the past decade, revolutionary advances in ultrasound imaging technology have allowed the study of the evolution of congenital heart disease during fetal life. The frustration arising from watching the prenatal progression of severe semilunar valve obstructions and therapy-refractory fetal arrhythmias has prompte...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-001-0197-6
更新日期：2002-05-01 00:00:00
abstract：:Heart failure (HF) has high morbidity and mortality in children. This study aimed to investigate the value of cardiac myosin binding protein-C (cMyBP-C) as a diagnostic and prognostic biomarker in children with heart failure. This study was a prospective case-control study that involved 50 children with acute HF and 2...
journal_title：Pediatric cardiology
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doi：10.1007/s00246-016-1532-2
更新日期：2017-02-01 00:00:00
abstract：:We report a case of a 12-year-old boy with severe congenital aortic stenosis in whom magnetic resonance imaging (MRI) with delayed contrast enhancement demonstrated extensive subendocardial hyperenhancement within the left ventricle. The hyperenhancement was confirmed to be subendocardial infarct and fibrosis by histo...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-005-5891-3
更新日期：2006-05-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0321-9
更新日期：2012-12-01 00:00:00
abstract：:The treatment of recurrent supraventricular tachycardia in a 3-week-old infant is described. Multiple doses of adenosine were used successfully to convert the dysrhythmia, without adverse effects or apparent tachyphylaxis. ...
journal_title：Pediatric cardiology
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doi：10.1007/s002469900222
更新日期：1997-11-01 00:00:00
abstract：:Recurrent congestive heart failure (CHF) attributable to myocarditis is a seldom-discussed entity in the scientific literature. This report describes the case of an 8-year-old girl who had three clinically identical episodes of CHF, beginning at the age of 5 years, with each episode preceded by a viral prodrome. The c...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-011-0109-3
更新日期：2012-01-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002460010218
更新日期：2001-05-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
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更新日期：2005-03-01 00:00:00
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journal_title：Pediatric cardiology
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doi：
更新日期：1986-01-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02082333
更新日期：1982-01-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/BF00798068
更新日期：1995-11-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02080-9
更新日期：2019-04-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002460010112
更新日期：2000-09-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-016-1543-z
更新日期：2017-03-01 00:00:00
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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
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journal_title：Pediatric cardiology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00246-007-9137-4
更新日期：2008-03-01 00:00:00
abstract：:Ruptured sinus Valsalva aneurysm (SVA) in a patient with tetralogy of Fallot is a very rare cardiac complication. This report describes the case of a 10-year-old Hispanic girl who presented to her cardiologist for a routine transthoracic echocardiography (TTE), which showed a ruptured sinus of Valsalva, with left-to-r...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0457-7
更新日期：2013-01-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章
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更新日期：2019-01-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF00801914
更新日期：1995-05-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章
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journal_title：Pediatric cardiology
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更新日期：2008-05-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-006-0064-6
更新日期：2007-05-01 00:00:00
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 (5...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0184-0
更新日期：2012-04-01 00:00:00
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journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0288-6
更新日期：2013-02-01 00:00:00
abstract：:Digoxin has been an effective treatment for fetal supraventricular tachycardia (SVT), but second-line therapy remains more controversial. Thirty-seven cases of fetal SVT were identified that received digoxin as first-line therapy. Seventeen fetuses (46%) converted to and maintained normal sinus rhythm. Flecainide was ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002460010279
更新日期：2001-11-01 00:00:00
abstract：:Congenitally corrected transposition of the great arteries is a rare anomaly that is thought to arise from a defect in looping of the primitive cardiac tube. Cor triatriatum is another rare congenital cardiac anomaly due to faulty incorporation of the common pulmonary vein into the left atrium. We present a rare case ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-011-9912-0
更新日期：2011-04-01 00:00:00
abstract：:Ventriculo-coronary arterial connections are frequently observed in pulmonary atresia with intact interventricular septum. Sustained right ventricular hypertension during cardiac morphogenesis is thought to be responsible for persistence of myocardial sinusoidal-coronary artery connections. Considering an unusual obse...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002469900305
更新日期：1998-05-01 00:00:00
abstract：:This study identified tracheobronchial cartilage calcification in children with congenital heart disease. Calcification of the tracheobronchial airways has been found previously in adults receiving warfarin and in children receiving warfarin after mitral valve replacement. A 9-year-old girl who had received a Fontan r...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-014-0880-z
更新日期：2014-08-01 00:00:00
abstract：:Branch pulmonary artery stenosis is a common problem in pediatric cardiology. Treatment has included surgery, balloon angioplasty, and balloon expandable stent placement. It was the purpose of this investigation to demonstrate the cost-effectiveness of each of these modes of treatment. From 1983 to 1994 there were 30 ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002469900195
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