Abstract：
:Sudden death accounts for up to 43% of all deaths in patients with familial dysautonomia (FD). The classic features of FD, namely, autonomic dysfunction, high blood pressure, and blood pressure labiality, are all risk factors for cardiac remodeling and hypertrophy. Myocardial remodeling and hypertrophy are independent risk factors for arrhythmias, cardiovascular events, and sudden death. An extensive review of the medical literature found no documentation of structural heart defects or myocardial remodeling in patients with FD. Sixteen patients with FD underwent physical examination, in-clinic blood pressure measurements, and echocardiographic study. On the basis of the findings, the patients were categorized by left ventricular geometric pattern. Twenty-four-hour ambulatory blood pressure monitoring was recommended to all participants. The majority of FD patients were found to have very high blood pressure values both during in-clinic measurements and during ambulatory blood pressure monitoring. Echocardiographic abnormalities were found in 43.75% of the study group; 18.75% of the study group had concentric hypertrophy, among which severe hypertrophy was found in 2 patients. Unknown previously, cardiac remodeling or hypertrophy is common in FD. We recommend that routine cardiac echocardiography be performed in this population, and attempts to treat high blood pressure should begin earlier in life.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Nussinovitch U,Katz U,Nussinovitch M,Blieden L,Nussinovitch Ndoi
10.1007/s00246-009-9497-zsubject
Has Abstractpub_date
2009-11-01 00:00:00pages
1068-74issue
8eissn
0172-0643issn
1432-1971journal_volume
30pub_type
杂志文章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：:M-mode and 2-dimensional (2D) echocardiographic imaging are routinely used to quantify left-ventricular (LV) size and function in pediatric patients with dilated cardiomyopathy (DCM). The reproducibility of and correlation between these techniques are unknown. This analysis sought to compare interreader, intrareader, ...
journal_title：Pediatric cardiology
pub_type： 临床试验,杂志文章,多中心研究
doi：10.1007/s00246-013-0835-9
更新日期：2014-04-01 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：:Unilateral absence of a pulmonary artery is a rare anomaly. Patients may present with hemoptysis, recurrent respiratory infections, pulmonary hypertension, or congestive heart failure. This report describes the case of a missing right pulmonary artery associated with anomalous left pulmonary venous connection not prev...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-011-0110-x
更新日期：2012-01-01 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：OBJECTIVES:The aim of this article is to examine the frequency, timing of formation of aneurysm of the ventricular membranous septum (AVMS), and prognosis in patients with a perimembranous ventricular septal defect (VSD). PATIENTS:One hundred forty-eight patients were diagnosed with a perimembranous VSD within 90 days...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-003-0572-6
更新日期：2004-07-01 00:00:00
abstract：:The hybrid pathway for the management of patients with hypoplastic left heart syndrome was pioneered at our institution and is the preferred approach compared with the traditional Norwood pathway. Patients undergoing this surgery are generally <6 months of age, and pain management in this age group after surgery for c...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0233-8
更新日期：2012-10-01 00:00:00
abstract：:A vascular approach for balloon aortic dilatation in infants is of major concern due to the high risk of femoral artery injury. To overcome this problem, a surgical right carotid artery (RCA) approach has been advocated. No reports are available on RCA after neonatal cutdown. Since 1990, RCA cutdown has been performed...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002460010202
更新日期：2001-05-01 00:00:00
abstract：:The prevalence of obesity in long-term survivors with complex congenital heart disease may be increasing, and little is known about the timing and onset of weight gain and growth patterns in these high-risk patients. Prevalence rates of overweight/obesity and longitudinal changes in body mass index (BMI) with age were...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-015-1265-7
更新日期：2016-01-01 00:00:00
abstract：:Embryonic development is a tightly regulated process, and many families of genes functions to provide a regulatory genetic network to achieve such a program. The homeobox genes are an extensive family that encodes transcription factors with a characteristic 60-amino acid homeodomain. Mutations in these genes or in the...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0179-x
更新日期：2012-08-01 00:00:00
abstract：:Ventricular dysfunction in patients after Fontan-like operations (FLOs) is a serious complication that might contribute to poor long-term results. Ischemic heart disease will have debilitating consequences on a Fontan heart. Ten patients (15.8 +/- 5.01 years) after FLO had transesophageal echocardiography and cardiac ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-002-0355-5
更新日期：2003-07-01 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
abstract：:Percutaneous balloon aortic valvuloplasty was attempted in an eight-month-old infant with severe aortic valve stenosis. The procedure resulted in a fall in the resting transvalvular systolic pressure gradient from 106 mmHg to 40 mmHg and no aortic regurgitation. Clinical and Doppler echocardiography findings suggest p...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02282747
更新日期：1985-01-01 00:00:00
abstract：:We report a case of isolated ventricular inversion in a 42-year-old woman. This rare congenital cardiac anomaly was corrected by an intraatrial baffle procedure. She also underwent left-sided double-chamber endocardial pacemaker implantation for postoperative tachycardia bradycardia syndrome. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-003-0583-3
更新日期：2004-09-01 00:00:00
abstract：:The epidemiologic data for pediatric hypertrophic cardiomyopathy (HCM) needs to be periodically updated as diagnostic techniques and management strategies improve. Herein, the incidence, prevalence, and mortality rates of pediatric HCM in a population-based treatment system are described. Patients aged ≤ 17 years and ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-020-02472-2
更新日期：2020-10-03 00:00:00
abstract：:Cardiac intervention remains controversial in patients with trisomy 13 and 18 and little is known about factors that may affect outcomes. The goal of this study was to evaluate preoperative factors and surgical approach with respect to outcomes in these patients. Patients with congenital heart disease and trisomy 13 o...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-017-1738-y
更新日期：2018-01-01 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：:Subtotal cor triatriatum sinister (partially divided left atrium) is characterized by a membrane dividing the left atrium into two portions: a dorsal accessory proximal chamber that receives part of the pulmonary venous return and a small restrictive defect in the membrane that obstructs blood flow from the accessory ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-008-9205-4
更新日期：2008-07-01 00:00:00
abstract：:Increasingly the importance of how and why we make decisions in the medical arena has been questioned. Traditionally the aeronautical and business worlds have shed a light on this complex area of human decision-making. In this review we reflect on what we already know about the complexity of decision-making in additio...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-020-02295-1
更新日期：2020-03-01 00:00:00
abstract：:Three patients (ages 3, 5, and 8 years) with various forms of functionally univentricular heart lesions received a total cavopulmonary connection with an extracardiac conduit as a final reconstructive procedure. Failure of the Fontan circulation occurred immediately after surgery because of spontaneous closure of surg...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-004-0693-6
更新日期：2005-01-01 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
abstract：:Two-dimensional echocardiography was performed on a patient with criss-cross atrioventricular (AV) relationship and ventriculo-arterial discordance. A systematic approach from the subcostal area revealed the complex cardiac pathology including the criss-cross AV relationship of right and left heart, indicating that th...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02427034
更新日期：1982-01-01 00:00:00
abstract：:Interventional occluder implantation has become the preferred mode of treatment in children with clinically significant atrial septal defect (ASD). The continuous increase in the number of patients treated calls for long-term data on device integrity and biocompatibility. We report a child who underwent successful int...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-010-9757-y
更新日期：2010-10-01 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：:Double outlet right ventricle (DORV) was produced experimentally in the chick embryo by preventing the incorporation of the posteromedial conus into the left ventricle. This was accomplished by placing a ligature around the caudal end of the conus at a stage in which it is exclusively continuous with the right ventric...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02240456
更新日期：1982-01-01 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：:Patients with anomalies of the heart frequently suffer from arrhythmias that either are associated with a congenital heart defect or result from the course of the disease. For most of the bradyarrhythmias, appropriate timing of the initiation of treatment is more challenging than its eventual execution. In the case of...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s002460010134
更新日期：2000-11-01 00:00:00
abstract：:Despite improved outcomes following modifications to the Fontan technique, significant morbidity and mortality persist. We sought to determine if abnormal pre-Fontan catheterization hemodynamic data will predict postoperative prolonged hospital stay (PHLOS) and adverse post-discharge outcomes. This is a retrospective ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-020-02430-y
更新日期：2020-12-01 00:00:00
abstract：:In adult heart failure (HF) patients, a higher ventricular arterial (VA) coupling ratio measured non-invasively is associated with worse HF prognosis and response to treatment. There are no data regarding the relationship of VA coupling to outcome in pediatric dilated cardiomyopathy (DCM) patients. We investigated the...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-018-2021-6
更新日期：2019-02-01 00:00:00
abstract：:Assessment of ventricular dysfunction and asynchrony is very important in predicting the outcome for children with a single right ventricle. However, the assessment is inaccurate and subjective because of the unusual ventricular shape. This study aimed to evaluate the feasibility and clinical value of velocity vector ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-014-0908-4
更新日期：2014-10-01 00:00:00
