Abstract:
:Presented is a retrospective outcome study of a 15-year single institutional experience with a contemporary cohort of patients with hypoplastic left heart syndrome and complex that underwent a "Giessen Hybrid" stage I as initial palliation. Hybrid approach consisting of surgical bilateral pulmonary artery banding and percutaneous duct stenting with or without atrial septum manipulation was developed from a rescue approach to a first-line procedure. Comprehensive Aristotle score defined pre-operative condition. Fifteen-year follow-up mortality is reported as occurring within the staged univentricular palliation or before and after biventricular repair. Hybrid stage I was performed in 154 patients; 107 should be treated by single ventricle palliation, 33 by biventricular repair (BVR), 7 received heart transplantation, and 7 were treated by comfort care, respectively. Overall 34 children died. The Aristotle score (mean value 18.2 ± 3) classified for univentricular circulations in newborns did not have statistical impact on the outcome. Two patients died during stage I (1.2%), and the interstage I mortality was 6.7%, and stage II mortality 9%, respectively. Stage III was up to now performed in 57 patients without mortality. At 1 year, the overall unadjusted survival of HLHS and variants was 84% and following BVR 89%, respectively. The Fifteen-year survival rate for HLHS and variants was 77%, with no significant impact of birth weight of less than 2.5 kg. In conclusion, Hybrid stage I fulfilled the criteria of life-saving approach. In our institution, Hybrid procedure replaced Norwood-staged palliation with a considerable mid- and long-term survival rate. Considering interstage mortality close surveillance is mandatory.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Schranz D,Bauer A,Reich B,Steinbrenner B,Recla S,Schmidt D,Apitz C,Thul J,Valeske K,Bauer J,Müller M,Jux C,Michel-Behnke I,Akintürk Hdoi
10.1007/s00246-014-1015-2subject
Has Abstractpub_date
2015-02-01 00:00:00pages
365-73issue
2eissn
0172-0643issn
1432-1971journal_volume
36pub_type
杂志文章abstract::Eighteen of 25 survivors of aortic valvotomy in infancy were reinvestigated by cross-sectional echocardiography a mean of 7.5 (2.3-13.4) years after surgery. They had been operated at a median age of 38 (5-330) days. At the follow-up examination the gradient across the aortic valve was 41 +/- 19 (15-85) mmHg and the e...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF00788221
更新日期:1992-01-01 00:00:00
abstract::This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF02310569
更新日期:1991-10-01 00:00:00
abstract::A 6-year-old girl presented with respiratory distress. Chest radiographs exhibited calcifications in the mediastinum. Further imaging revealed extensive cardiac calcifications on computed tomography of the chest. The laboratory parameters were consistent with findings of secondary hyperparathyroidism. Detailed review ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-004-0765-7
更新日期:2005-07-01 00:00:00
abstract::Hammock mitral valve (MV), also known as anomalous mitral arcade, is a rare congenital anomaly. We report a case of a 10-month-old child who presented with congestive heart failure and was found to have severe mitral stenosis (MS) secondary to a hammock MV anomaly. Detailed advanced imaging with cardiac MRI and three-...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-017-1797-0
更新日期:2018-03-01 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::Using the Doppler technique, this study compared the prenatal and postnatal flow patterns of an infant with cerebral arteriovenous (AV) malformation. Fetal right ventricular end-diastolic dimension was 1.7 cm with right ventricular ejection equaling 66% of the combined cardiac output. Diastolic flow was reversed in th...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF00801914
更新日期:1995-05-01 00:00:00
abstract::Home international normalized ratio (INR) monitors are being increasingly used for monitoring the adequacy of anticoagulation in children on long-term warfarin. Their safety and effectiveness in the home setting has not been fully established. The purpose of this study was to explore the safety and effectiveness of ho...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-009-9535-x
更新日期:2010-01-01 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::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::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::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::The response to warfarin is highly variable among individuals and such variability is likely to have some genetic basis. We evaluted the effect of VKORC1 polymorphisms on warfarin response among Japanese, taking advantage of its unique population structure in which CYP2C9 *2 and *3 alleles are relatively rare. Thirty-...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-005-1150-x
更新日期:2006-11-01 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::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::There has been substantial controversy regarding treatment of congenital heart defects in infants with trisomies 13 and 18. Most reports have focused on surgical outcomes versus expectant treatment, and rarely there has been an effort to consolidate existing evidence into a more coherent way to help clinicians with de...
journal_title:Pediatric cardiology
pub_type: 杂志文章,评审
doi:10.1007/s00246-020-02444-6
更新日期:2020-10-01 00:00:00
abstract::Postoperative arrhythmia is a common complication after open heart surgery in children. JET is the most common and dangerous arrhythmia. We aimed to assess safety and efficacy of prophylactic amiodarone in preventing JET in children underwent cardiac surgery and to assess risk factors for JET among our patients. In to...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-016-1343-5
更新日期:2016-04-01 00:00:00
abstract::Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality among patients with congenital heart disease (CHD). This study was designed to determine biomarker levels in patients with PAH associated with CHD (PAH-CHD) and CHD patients without PAH and to investigate the relationship of these potent...
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::During the development of so-called aneurysmal transformation of perimembranous ventricular septal defects (pmVSD), tricuspid valve (TV) morphology and function may be altered resulting in left ventricular (LV) to right atrial (RA) shunting. The feasibility and outcome of interventional closure of these pmVSD has not ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-015-1170-0
更新日期:2015-10-01 00:00:00
abstract::We present a 2-month-old male infant with thrombosis in the superior vena cava and pericardium due to transient protein C deficiency. Protein C deficiency was related to sepsis and hepatitis-induced liver function impairment. The patient's cardiac anatomy was otherwise normal. The patient was referred to us with signs...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-006-1265-8
更新日期:2006-07-01 00:00:00
abstract::Controversy exists as to whether a hemodynamically significant left-to-right shunt due to a patent ductus arteriosus (PDA) affects ventricular contractility. Load-dependent indices such as ejection fraction and shortening fraction have traditionally been used to assess contractility, but the relationship between the r...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-003-0452-0
更新日期:2004-03-01 00:00:00
abstract::The etiology and pathogenesis of Kawasaki disease (KD) is largely unknown. Certain demographic factors and laboratory findings are predictive of the development of coronary artery (CA) aneurysms. The objectives of this study were to determine the epidemiology of KD patients in an urban hospital and determine risk fact...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-005-0916-5
更新日期:2005-11-01 00:00:00
abstract::Various agents have been suggested as causal or associated factors in the pathogenesis of Kawasaki disease (KD); however, the underlying factors of KD remain unknown. Plasma exchange is one of the most effective treatments for the acute phase of KD. This indicates that plasma may contain factors associated with the pa...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-012-0329-1
更新日期:2012-12-01 00:00:00
abstract::Paroxysmal non-reentrant supraventricular tachycardia due to double ventricular response through antegrade dual atrioventricular nodal pathways by a single atrial excitation has been reported in limited adult cases but not in pediatric patients with structurally normal hearts or with congenital heart defects. We repor...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-012-0326-4
更新日期:2013-03-01 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::Fetal echocardiography has impacted the fetus with congenital heart disease in many important ways. Advances in fetal echocardiography have allowed for more accurate and earlier detection of cardiac abnormalities. In turn, the prenatal diagnosis of cardiac abnormalities has improved the care and outcome of selected fe...
journal_title:Pediatric cardiology
pub_type: 杂志文章,评审
doi:10.1007/s00246-003-0593-1
更新日期:2004-05-01 00:00:00
abstract::Thrombocytopenia can be a real challenge during cardiac surgery in children with cyanotic congenital heart disease. This report describes a 7-year-old girl with d-transposition of the great arteries, ventricular septal defect, pulmonary hypertension, chronic thrombocytopenia, polycythemia, and chronic renal impairment...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-012-0570-7
更新日期:2013-01-01 00:00:00
abstract::In a patient with single ventricle, mitral atresia, common atrium, pulmonary stenosis, azygos continuation of the inferior vena cava and bilateral systemic artery--pulmonary artery anastomoses, the systemic veins were connected directly to the pulmonary arteries to achieve physiologic repair; the child was discharged ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:
更新日期:1986-01-01 00:00:00
abstract::The morphology and natural history of anomalous right ventricular muscle bundles (ARVMB) have been described in a number of postnatal studies. Whether this is a congenital or acquired cardiac lesion remains obscure. A fetal echocardiogram performed in a 32-week gestation mother showed a large ventricular septal defect...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF00795736
更新日期:1994-09-01 00:00:00
abstract::Pseudohypertension has often been reported in elderly subjects, but is an unusual phenomenon in children. We report the case of a 5-year-old child who presented with features of Williams syndrome (characterized by elfin facies, supravalvar aortic stenosis, and peripheral pulmonary artery stenosis). Repeated blood pres...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF00796994
更新日期:1993-03-01 00:00:00
abstract::Identification of heart transplant recipients at highest risk for a poor outcome could lead to improved posttransplantation survival. A chart review of primary heart transplantations from 1993 to 2006 was performed. Analysis was performed to evaluate the risk of graft loss for those with a transplantation age less tha...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-011-0077-7
更新日期:2012-01-01 00:00:00