Abstract:
:We sought to document morbidities and growth for patients with hypoplastic left heart syndrome (HLHS) to inform the initial surgical decision and understand healthcare needs. Data were obtained on 137 patients with HLHS, born between 1989 and 1994, who survived staged surgery ( n = 62) or transplantation ( n = 75) and had follow-up information available from four pediatric cardiac surgical centers. In patients with HLHS older than 1 year of age at follow-up, 93% experienced at least one major postsurgical morbidity. Morbidities depended on the surgery received. Hypertension, renal compromise, and abnormal infections were more common in transplanted patients than staged surgery patients. Staged surgery patients used more anticongestive medications and experienced more morbidities requiring interventional catheterization than did transplanted patients. Rejection was common for transplanted patients. On average these children spent 23 days per year in the hospital. Patients with HLHS were small for their age; 43% of staged surgery patients weighed below the third percentile at last information, compared to 19% of transplanted patients ( p = 0.003). The median height percentile was the 10th in both groups. Normal activity level was reported in more transplanted patients (90%) than staged surgery patients (49%; p < 0.001). Trade-offs between mortality and morbidity outcomes can help inform the initial surgical decision.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Jenkins PC,Flanagan MF,Jenkins KJ,Sargent JD,Canter CE,Chinnock RE,Vincent RN,O'Connor GTdoi
10.1007/s00246-003-0471-xsubject
Has Abstractpub_date
2004-01-01 00:00:00pages
3-10issue
1eissn
0172-0643issn
1432-1971journal_volume
25pub_type
杂志文章,多中心研究abstract::Restrictive cardiomyopathy (RCM) is characterized by irreversible diastolic dysfunction with preserved systolic function. The aim of this study was to investigate the presence of impaired ventricular contractility even in the presence of normal ejection fraction (EF) in children with RCM. Longitudinal Doppler tissue v...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-011-9948-1
更新日期:2011-06-01 00:00:00
abstract::We report the case of a fetus diagnosed at 24 weeks' gestation with complete heart block associated with maternal collagen vascular disease and Sjögren's antibody. Serial fetal echocardiograms noted increased echogenicity along the tricuspid and mitral valves without insufficiency. Postnatally, severe tricuspid insuff...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF00800677
更新日期:1994-07-01 00:00:00
abstract::Antenatal diagnosis of right heart enlargement has a wide spectrum of differential diagnosis from maternal, placental and fetal causes, and outcomes of all are not known. Coarctation of the aorta is in the differential diagnosis of right heart enlargement. In our study, we focused to measure multiple cardiac dimension...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-015-1168-7
更新日期:2015-10-01 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::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::The Norwood procedure with a modified Blalock-Taussig shunt (MBTS) is the first of the three-stage surgical palliation for infants with hypoplastic left heart syndrome. We report a patient with schistocytic hemolytic anemia that developed following a right MBTS with a Gore-Tex graft. Hemolysis associated with a MBTS h...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-006-1410-4
更新日期:2007-05-01 00:00:00
abstract::DiGeorge syndrome (DGS) is commonly associated with both congenital heart disease (CHD) and immunologic abnormalities. While CHD may prompt consideration for heart transplantation (HTx), little is known about HTx management or outcomes in this group. The aim of this study was to describe the spectrum of patients with ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-019-02063-w
更新日期:2019-04-01 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::For premature infants with congenital heart disease (CHD), it may be unclear when the burdens of treatment outweigh potential benefits. Parents may thus have to choose between comfort care at birth and medical stabilization until surgical repair is feasible. Better defined outcome data, including risk factors for mort...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-016-1374-y
更新日期:2016-06-01 00:00:00
abstract::Dextro-transposition of the great arteries (D-TGA) is one of the most common cyanotic heart lesions. The arterial switch operation (ASO) is the preferred surgical palliation for D-TGA. One of the primary concerns following the ASO is complications arising from the coronary artery transfer. There is a need for myocardi...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-018-1890-z
更新日期:2018-08-01 00:00:00
abstract::In contrast to the percentile-based definitions of elevated blood pressure (BP) and hypertension for children and adolescents of all ages in the 2004 fourth report, the 2017 American Academy of Pediatrics (AAP) BP guideline recommends a change to single BP cut-offs for clinical diagnosis (120/< 80-129/< 80 mmHg for el...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-019-02058-7
更新日期:2019-04-01 00:00:00
abstract::Adult studies suggest a better functional outcome after aortic valve replacement with a pulmonary autograft compared with mechanical or homograft valves. Little is known about functional results after Ross surgery in growing children. This study reports formal exercise stress echocardiographic data from 26 pediatric R...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-012-0218-7
更新日期:2012-06-01 00:00:00
abstract::The hypothesis that mild recurrent aortic obstruction produces subtle changes in ambulatory blood pressure was investigated by performing 24-hour monitoring on 11 postoperative coarctation patients. Patients (age 16.1 +/- 2.7 years) were compared with normal controls (age 15.7 +/- 2.5 years, n = 15). Surgery (end-to-e...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF00794187
更新日期:1995-07-01 00:00:00
abstract::Information and communication technology has been widely applied to various fields, including clinical medicine. We report here a telediagnosis system using ultrasound image transmission. The effect of telediagnosis, using a medical link between local maternity hospitals and our children's medical center, was verified...
journal_title:Pediatric cardiology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00246-013-0834-x
更新日期:2014-04-01 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::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::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::Branch pulmonary artery stenosis (BPAS) in the setting of systemic-pulmonary artery shunts (SPS) may result in significant sequelae. Limited information exists regarding the safety and efficacy of pulmonary artery balloon angioplasty and stent implantation via SPS in neonates and infants. This study aimed to examine t...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-013-0657-9
更新日期:2013-08-01 00:00:00
abstract::At the University of California Davis Medical Center, a screening fetal ultrasound examination (level I or II) incorporates a comprehensive segmental evaluation of the fetal heart. This study evaluated the reliability of the fetal ultrasound exam in the detection of abnormal heart anatomy. Our retrospective study revi...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-001-0073-4
更新日期:2002-11-01 00:00:00
abstract::For neonates with right atrial isomerism (RAI), functional single ventricle (f-SV), and obstructive total anomalous pulmonary venous connection (TAPVC), primary TAPVC repair (TAPVCR) has a poor outcome. At our hospital, the survival rate at 1 year of such neonates undergoing primary TAPVCR between 1999 and 2010 (TAPVC...
journal_title:Pediatric cardiology
pub_type: 杂志文章,收录出版
doi:10.1007/s00246-018-1902-z
更新日期:2018-10-01 00:00:00
abstract::Congenital heart disease (CHD) is the most common congenital anomaly, affecting 1 % of live births. The field of pediatric cardiology has witnessed major advances over the past 25 years triggered by research initiatives focusing on CHD. However, large disparities exist in research capabilities between Arab developing ...
journal_title:Pediatric cardiology
pub_type: 杂志文章,评审
doi:10.1007/s00246-012-0466-6
更新日期:2013-02-01 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::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::Quantification of left ventricular (LV) mass by echocardiography has not been validated against the gold standard of cardiac magnetic resonance imaging (CMR) in the pediatric population. The purpose of this study was to compare LV mass by two-dimensional and conventional M-mode echocardiography versus CMR in children....
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-018-1991-8
更新日期:2019-02-01 00:00:00
abstract::An 11 day-old female infant underwent resection of a mass in the subaortic region secondary to critical aortic stenosis. At 3 months of age, recurrent severe left-ventricular outflow obstruction (LVOT) in the setting of heart failure prompted redo surgery, and the resected mass revealed an intracardiac foregut cyst, w...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-012-0623-y
更新日期:2013-01-01 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::The purpose of the study was to evaluate school-age developmental and educational outcomes for children with and without a cardiac procedure in the first year of life to improve understanding of longer-term neurodevelopmental outcomes in children who have had a cardiac procedure for congenital heart disease, the most ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-018-2029-y
更新日期:2019-03-01 00:00:00
abstract::Non-invasive evaluation of right ventricular (RV) systolic function in neonates with pulmonary hypertension (PH) with traditional metrics including RV fractional area change (FAC) and tricuspid annular systolic plane excursion (TAPSE) has improved outcomes. Apical three-chamber (3C) RV-FAC, a novel tripartite assessme...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-020-02376-1
更新日期:2020-08-01 00:00:00
abstract::Novel COstatus system (Transonic Systems, Inc., NY), based on ultrasound dilution (UD), works off in situ arterial and central venous catheters in pediatric patients to measure cardiac output (CO). The purpose of the present study was to validate CO measurement by UD (COUD) with pulmonary artery (PA) thermodilution (C...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-011-9915-x
更新日期:2011-06-01 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