Aortic dilatation in patients with Turner's syndrome without structural cardiac anomaly.

Abstract:

INTRODUCTION:Dilatation of the ascending aorta is described in Turner's syndrome with variable prevalence (6.8-32%). Reported series typically include patients with associated cardiac anomalies. OBJECTIVE:To characterise the prevalence, age of onset, and the progress of dilatation of the ascending aorta in Turner's syndrome patients free of structural cardiac anomalies. Potential risk factors such as karyotype and growth hormone therapy were analysed for correlation with aortic dilatation. METHODS:We carried out a retrospective study with data collected from medical records and echocardiography studies. Patients with Tuner's syndrome followed-up between 1992 and 2010 with at least two echocardiography studies were eligible. Patients with previous cardiac surgery or under anti-hypertensive medication were excluded. Ascending aorta diameter measurements were adjusted for body surface area, and dilatation was defined as Z-score>2. RESULTS:The study population consisted of 44 patients, aged 11.9±7.4 years at the first echocardiogram and 17.9±7.3 years at the last follow-up, with a follow-up duration of 6.0±3.7 years. A total of 13 (29.5%) patients exhibited aortic dilatation during follow-up, suggesting an actuarial estimate of the freedom from aortic dilatation dropping from 86 to 70% and then to 37% at 10, 20, and 30 years of age, respectively. There was no statistically significant impact of karyotype or growth hormone therapy on aortic Z-score progression. CONCLUSION:The prevalence of dilatation of the ascending aorta in Turner's syndrome patients free of structural aortic anomalies is comparable with published data with associated lesions. Growth hormone therapy and karyotype had no significant impact; however, longitudinal follow-up is warranted.

journal_name

Cardiol Young

journal_title

Cardiology in the young

authors

Alami Laroussi N,Dahdah N,Dallaire F,Thérien J,Fournier A

doi

10.1017/S1047951115000682

subject

Has Abstract

pub_date

2016-03-01 00:00:00

pages

539-46

issue

3

eissn

1047-9511

issn

1467-1107

pii

S1047951115000682

journal_volume

26

pub_type

杂志文章
  • Semi-automated speckle-tracking for quantitative right ventricular assessment in children and adolescents.

    abstract:BACKGROUND:Assessment of right ventricular size and function is an important part of the clinical cardiac evaluation; however, these quantitative measures are challenging by echocardiography. Automated software could be useful in place of manual measurements and qualitative assessment. This study evaluates a semi-autom...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951119001641

    authors: McCloud AK,Lowisz J,Roberson DA,Lefaiver CA,Penk JS

    更新日期:2019-09-01 00:00:00

  • Tetralogy of Fallot with unilateral absent pulmonary artery.

    abstract:BACKGROUND:Tetralogy of Fallot is a common congenital cardiac malformation. A rare subgroup includes unilateral absence of the pulmonary artery, either the left or the right main branch. The literature lacks an established treatment for these cases, and surgical options carry certain mortality and morbidity. PATIENTS ...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951112000911

    authors: Ugurlucan M,Arslan AH,Yildiz Y,Ay S,Besikci RT,Cicek S

    更新日期:2013-06-01 00:00:00

  • Cardiac rehabilitation in the paediatric Fontan population: development of a home-based high-intensity interval training programme.

    abstract:INTRODUCTION:We evaluated the safety and feasibility of high-intensity interval training via a novel telemedicine ergometer (MedBIKE™) in children with Fontan physiology. METHODS:The MedBIKE™ is a custom telemedicine ergometer, incorporating a video game platform and live feed of patient video/audio, electrocardiograp...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951120002097

    authors: Khoury M,Phillips DB,Wood PW,Mott WR,Stickland MK,Boulanger P,Rempel GR,Conway J,Mackie AS,Khoo NS

    更新日期:2020-10-01 00:00:00

  • Treatment strategies for protein-losing enteropathy in Fontan-palliated patients.

    abstract:OBJECTIVE:Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on cl...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951120000864

    authors: Schleiger A,Ovroutski S,Peters B,Schubert S,Photiadis J,Berger F,Kramer P

    更新日期:2020-05-01 00:00:00

  • Epidemiological investigation of Kawasaki disease in Jilin province of China from 2000 to 2008.

    abstract:OBJECTIVE:To investigate the epidemiological characteristics of Kawasaki disease in Jilin province of China and explore its clinical features. METHODS:The medical records of children with Kawasaki disease hospitalised in the First Affiliated Hospital of Jilin University and Yanbian University between January, 2000 and...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951110000375

    authors: Piao JH,Jin LH,Lv J,Zhou Y,Jin CJ,Jin ZY

    更新日期:2010-08-01 00:00:00

  • The World Congress of Paediatric Cardiology and Cardiac Surgery: "The Olympics of our profession".

    abstract::The first World Congress of Paediatric Cardiology was held in London, United Kingdom, in 1980, organised by Dr. Jane Somerville and Prof. Fergus Macartney. The idea was that of Jane Somerville, who worked with enormous energy and enthusiasm to bring together paediatric cardiologists and surgeons from around the world....

    journal_title:Cardiology in the young

    pub_type: 社论

    doi:10.1017/S1047951112002107

    authors: Hugo-Hamman C,Jacobs JP

    更新日期:2012-12-01 00:00:00

  • Impact of the location of the fenestration on Fontan circulation haemodynamics: a three-dimensional, computational model study.

    abstract:OBJECTIVES:There is no consensus or theoretical explanation regarding the optimal location for the fenestration during the Fontan operation. We investigated the impact of the location of the fenestration on Fontan haemodynamics using a three-dimensional Fontan model in various physiological conditions. METHODS:A three...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951117000099

    authors: Sughimoto K,Asakura Y,Brizard CP,Liang F,Fujiwara T,Miyaji K,Liu H

    更新日期:2017-09-01 00:00:00

  • Current subjective state of health, and longitudinal psychological well-being over a period of 10 years, in a cohort of adults with congenital cardiac disease.

    abstract:OBJECTIVE:To examine the current subjective state of health, and the longitudinal course of psychological well-being, in adult patients with congenital cardiac malformations. METHODS:Our study concerns the second follow-up of a cohort of patients with congenital cardiac malformations. We examined 362 consecutive patie...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951105000351

    authors: van Rijen EH,Utens EM,Roos-Hesselink JW,Meijboom FJ,van Domburg RT,Roelandt JR,Bogers AJ,Verhulst FC

    更新日期:2005-04-01 00:00:00

  • Tetralogy of Fallot in men: quality of life, family, education, and employment.

    abstract:INTRODUCTION:Little is known about the quality of life, health, family, education, and employment status among adult men with repaired tetralogy of Fallot. MATERIAL AND METHODS:A total of 68 men who underwent repair of tetralogy of Fallot between 1971 and 1991 were studied. Fifty-three patients answered the SF-36 heal...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951111001934

    authors: Bygstad E,Pedersen LC,Pedersen TA,Hjortdal VE

    更新日期:2012-08-01 00:00:00

  • Despite reductions in muscle mass and muscle strength in adults with CHD, the muscle strength per muscle mass relationship does not differ from controls.

    abstract:BACKGROUND:Patients with CHD exhibit reduced isometric muscle strength and muscle mass; however, little is known how these parameters relate. Therefore, the aim was to investigate the relation between isometric limb muscle strength and muscle mass for patients in comparison to age- and sex-matched control subjects. ME...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951120004709

    authors: Sandberg C,Crenshaw AG,Christersson C,Hlebowicz J,Thilén U,Johansson B

    更新日期:2021-01-18 00:00:00

  • Functional health status in children following surgery for congenital heart disease: a population-based cohort study.

    abstract:BACKGROUND:Functional health is becoming an important part of outcome assessment following congenital heart surgery. METHODS:The Child Health Questionnaire was used to evaluate self-reported functional health in a cohort of children operated on for congenital heart disease between 1996 and 2002, now aged 10-20 years. ...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951110000843

    authors: Larsen SH,McCrindle BW,Jacobsen EB,Johnsen SP,Emmertsen K,Hjortdal VE

    更新日期:2010-12-01 00:00:00

  • A model for a nurse-led programme of bedside placement of peripherally inserted central catheters in neonates and infants with congenital cardiac disease.

    abstract:BACKGROUND:Neonates and infants with congenital and acquired cardiac disease often require placement of central venous lines for extended intravenous therapy. It may be advantageous to avoid the larger venous vessels of the head and neck and lower extremities in order to preserve these for future interventions and ther...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951110000090

    authors: King DS,da Cruz E,Kaufman J

    更新日期:2010-06-01 00:00:00

  • Endocarditis of a congenital coronary fistula in a child.

    abstract::Endocarditis of congenital coronary fistulas in the cardiac chambers is rare, especially in the paediatric age group. We describe the case of a 9-year-old boy with a fistula from the dilated right coronary artery to the junction of the superior caval vein to the right atrium, complicated by endocarditis. Treatment con...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951117001846

    authors: Krasemann T,van Beynum IM,Frohn-Mulder IME,Dalinghaus M

    更新日期:2018-02-01 00:00:00

  • The bronchial cast syndrome after the fontan procedure: further evidence of its etiology.

    abstract::We describe a three-year-old boy who presented with recurrent expectoration of bronchial casts six months following creation of a fenestrated lateral tunnel Fontan circulation for pulmonary atresia with intact ventricular septum. Cardiac catheterization demonstrated elevated central venous pressure with two areas of s...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/s1047951101000385

    authors: McMahon CJ,Nihill MR,Reber A

    更新日期:2001-05-01 00:00:00

  • A new approach to hypoplastic left heart syndrome with an intact atrial septum.

    abstract::An intact atrial septum places infants with hypoplasia of the left heart into a group with an extremely high rate of mortality. We report a neonate, diagnosed antenatally, who was delivered by Caesarian section in the cardiac theatre, urgently placed onto cardiopulmonary bypass, and who then underwent an atrial septec...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951107000819

    authors: Barker C,Moscuzza F,Anderson D

    更新日期:2007-08-01 00:00:00

  • Emerging trends in the prenatal diagnosis of complex CHD and its influence on infant mortality in this cohort.

    abstract:BACKGROUND:Fetal echocardiography is the main modality of prenatal diagnosis of CHD. This study was done to describe the trends and benefits associated with prenatal diagnosis of complex CHD at a tertiary care centre. METHODS:Retrospective chart review of patients with complex CHD over an 18-year period was performed....

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951118002147

    authors: Gorla SR,Chakraborty A,Garg A,Gugol RA,Kardon RE,Swaminathan S

    更新日期:2018-12-26 00:00:00

  • Objectively measured physical activity levels of young children with congenital heart disease.

    abstract:UNLABELLED:Physical activity tends to be lower in school-age children with congenital heart disease than in healthy controls. To the best of our knowledge, objectively measured physical activity levels of preschool-age children with congenital heart disease have not been studied. METHODS:A total of 10 children with ei...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951114000298

    authors: Stone N,Obeid J,Dillenburg R,Milenkovic J,MacDonald MJ,Timmons BW

    更新日期:2015-03-01 00:00:00

  • Clinical and molecular characterisation of Holt-Oram syndrome focusing on cardiac manifestations.

    abstract:BACKGROUND:Holt-Oram syndrome is characterised by CHD and limb anomalies. Mutations in TBX5 gene, encoding the T-box transcription factor, are responsible for the development of Holt-Oram syndrome, but such mutations are variably detected in 30-75% of patients. METHODS:Clinically diagnosed eight Holt-Oram syndrome pat...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951114001656

    authors: Jhang WK,Lee BH,Kim GH,Lee JO,Yoo HW

    更新日期:2015-08-01 00:00:00

  • Ultrasound assessment of mesenteric blood flow in neonates with hypoplastic left heart before and after hybrid palliation.

    abstract:BACKGROUND:Altered mesenteric perfusion may be a contributor to the development of necrotising enterocolitis in patients with hypoplastic left heart syndrome. The goal of this study was to document mesenteric flow patterns in patients with hypoplastic left heart syndrome pre- and post-hybrid procedure. METHODS:A prosp...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951114001607

    authors: Cozzi CT,Galantowicz M,Cheatham JP,Nicholson L,Fernandez R,Backes CH,McCaw C,Cua CL

    更新日期:2015-08-01 00:00:00

  • An international survey of the nutrition management of chylothorax: a time for change.

    abstract:INTRODUCTION:Although chylothorax is an uncommon complication following paediatric cardiothoracic surgery, it has significant associated morbidities and increased in-hospital mortality, as well as results in higher costs. A lack of prospective evidence or consensus guidelines for management of chylothorax further hinde...

    journal_title:Cardiology in the young

    pub_type: 杂志文章,多中心研究

    doi:10.1017/S1047951119001525

    authors: Marino LV,Bell KL,Woodgate J,British Dietetic Association Paediatric Cardiology Interest Group.,Doolan A

    更新日期:2019-09-01 00:00:00

  • Array comparative genomic hybridisation testing in CHD.

    abstract:BACKGROUND:CHD is the leading cause of mortality due to birth defects. Array comparative genomic hybridisation (aCGH) detects submicroscopic copy number changes and may improve identification of the genetic basis of CHD. METHODS:This is a retrospective analysis of 1252 patients from a regional referral centre who had ...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951114001838

    authors: Hightower HB,Robin NH,Mikhail FM,Ambalavanan N

    更新日期:2015-08-01 00:00:00

  • Role of toxicological determinations of amphetamines and cannabinoids in hair of adolescent patients in cardiologic diagnostic management.

    abstract:BACKGROUND:Parental suspicions that the child may possibly take narcotics may be substantiated by hair analysis, which may not only identify the narcotic abuse profile, but also define the minimum period of narcotic usage. OBJECTIVE:A toxicological hair analysis aiming at detecting the presence of amphetamines and can...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951111000667

    authors: Kłys M,Szydłowski L,Rojek S

    更新日期:2012-02-01 00:00:00

  • Intra-operative arrhythmia predicts post-operative arrhythmia and the need for temporary pacing wires.

    abstract:OBJECTIVE:Protocols for the placement of temporary pacing wires vary among institutions. Our current protocol is to selectively place temporary pacing wires in those patients who develop haemodynamically significant intra-operative arrhythmia. We wished to identify how effective our current protocol is at identifying w...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951114000043

    authors: Piggott K,Nehgme R,Decampli W,Pourmoghadam K,Fakioglu H,Blanco C

    更新日期:2015-03-01 00:00:00

  • Proteomics of pediatric heart failure: from traditional biomarkers to new discovery strategies.

    abstract::Heart failure in children is a complex clinical syndrome with multiple aetiologies. The underlying disorders that lead to heart failure in children differ significantly from those in adults. Some clinical biomarkers for heart failure status and prognosis appear to be useful in both age groups. This review outlines the...

    journal_title:Cardiology in the young

    pub_type: 杂志文章,评审

    doi:10.1017/S1047951115000839

    authors: Xu M,Ramirez-Correa GA,Murphy AM

    更新日期:2015-08-01 00:00:00

  • Use of diagnostic information submitted to the United Kingdom Central Cardiac Audit Database: development of categorisation and allocation algorithms.

    abstract:OBJECTIVE:To categorise records according to primary cardiac diagnosis in the United Kingdom Central Cardiac Audit Database in order to add this information to a risk adjustment model for paediatric cardiac surgery. DESIGN:Codes from the International Paediatric Congenital Cardiac Code were mapped to recognisable prim...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951112001369

    authors: Brown KL,Crowe S,Pagel C,Bull C,Muthialu N,Gibbs J,Cunningham D,Utley M,Tsang VT,Franklin R

    更新日期:2013-08-01 00:00:00

  • Databases for assessing the outcomes of the treatment of patients with congenital and paediatric cardiac disease--the perspective of cardiac surgery.

    abstract::This review includes a brief discussion, from the perspective of cardiac surgeons, of the rationale for creation and maintenance of multi-institutional databases of outcomes of congenital heart surgery, together with a history of the evolution of such databases, a description of the current state of the art, and a dis...

    journal_title:Cardiology in the young

    pub_type: 杂志文章,评审

    doi:10.1017/S1047951108002813

    authors: Jacobs ML,Jacobs JP,Franklin RC,Mavroudis C,Lacour-Gayet F,Tchervenkov CI,Walters H,Bacha EA,Clarke DR,William Gaynor J,Spray TL,Stellin G,Ebels T,Maruszewski B,Tobota Z,Kurosawa H,Elliott M

    更新日期:2008-12-01 00:00:00

  • A 6-year follow-up study of adult patients with congenitally corrected transposition.

    abstract::The aims of this study were to assess the development of heart failure in patients with congenitally corrected transposition of the great arteries in a medium-term follow-up, to identify the impact of tricuspid regurgitation on the development of heart failure, and to determine the most reliable marker for its identif...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951114002479

    authors: Koželj M,Cvijić M,Berden P,Podnar T

    更新日期:2015-10-01 00:00:00

  • Incidental finding of complete interruption of the aorta in a septuagenarian.

    abstract::Adult survivors of aortic interruption is extremely rare. Diagnosis mostly depends on additional imaging with CT and MRI. We present a rare case of acquired complete interruption of the aorta that was found incidentally at the time of coronary angiography. This finding was confirmed by CT scan. ...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951114001693

    authors: Ozturk S,Gurbuz AS,Efe SC

    更新日期:2015-03-01 00:00:00

  • The relationship between scimitar syndrome, so-called scimitar variant, meandering right pulmonary vein, horseshoe lung and pulmonary arterial sling.

    abstract::We report a case in which a meandering right pulmonary vein connecting to the left atrium is associated with hypoplasia of the right lung, horseshoe lung, abnormal pulmonary lobation, and abnormal branching of the pulmonary arteries. We discuss its relationship to the so-called scimitar variant, and to the scimitar sy...

    journal_title:Cardiology in the young

    pub_type: 杂志文章

    doi:10.1017/S1047951106000461

    authors: Yoo SJ,Al-Otay A,Babyn P

    更新日期:2006-06-01 00:00:00

  • Ebstein's malformation in the setting of Down's syndrome.

    abstract::We report a patient with Down's syndrome referred to our Department of Echocardiography for evaluation after an episode of tachyarrhythmia. We diagnosed Ebstein's malformation. As far as we know, this association has been reported previously on only three occasions. We review these previous cases, describe our echocar...

    journal_title:Cardiology in the young

    pub_type: 杂志文章,评审

    doi:

    authors: Bauk L,Espinola-Zavaleta N,Muñoz-Castellanos L

    更新日期:2003-08-01 00:00:00