Abstract:
:A bicuspid aortic valve is not only a common congenital heart defect but also an enigmatic condition that can cause a large spectrum of diseases, such as aortic valve stenosis and severe heart failure in newborns whereas aortic dissection in adults. On the contrary, a bicuspid aortic valve can also occur with normal function throughout life and never need treatment. Numerous genetic mechanisms are involved in the abnormal cellular functions that may cause abnormal development of the aortic valve during early foetal life. As several chromosomal disorders are also associated with a bicuspid valve, there does not appear to be an apparent common trigger to the abnormal development of the aortic valve. The clinical care of the bicuspid aortic valve patient has been changed by a significant body of evidence that has improved the understanding of the natural history of the disease, including when to best intervene with valve replacement and when to provide prophylactic aortic root surgery. Moreover, as bicuspid valve disease is also part of various syndromes, we can identify high-risk patients in whom a bicuspid valve is much more unfavourable than in the normal population. This review provides an overview of all aspects of the bicuspid aortic valve condition and gives an updated perspective on issues from pathophysiology to clinical care of bicuspid aortic valve disease and associated aortic disease in asymptomatic, symptomatic, and pregnant patients, as well as our viewpoint on population screening.
journal_name
Cardiol Youngjournal_title
Cardiology in the youngauthors
Pedersen MW,Groth KA,Mortensen KH,Brodersen J,Gravholt CH,Andersen NHdoi
10.1017/S1047951118001658subject
Has Abstractpub_date
2019-01-01 00:00:00pages
1-10issue
1eissn
1047-9511issn
1467-1107pii
S1047951118001658journal_volume
29pub_type
杂志文章,评审abstract::Correction of tetralogy of Fallot during infancy usually eliminates the risks associated with general anaesthesia. In rare cases of uncorrected defects persisting into adulthood, anaesthetic management during non-cardiac surgery may therefore be challenging. We describe the use of continuous spinal anaesthesia to succ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951119000994
更新日期:2019-06-01 00:00:00
abstract::In utero diagnosis of complex progressive cardiac disease such as hypoplastic left heart syndrome presents a novel opportunity for antepartum, intrapartum, and neonatal management. The clinical possibilities and potential for differing outcomes challenge the mother-foetus dyad with regard to informed consent. Previous...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951111001715
更新日期:2011-12-01 00:00:00
abstract::In this study, we summarise a case of a myofibroma causing mid-aortic syndrome due to obstruction of the distal thoracic and abdominal aorta leading to severe left ventricular dysfunction. The patient was managed with percutaneous intervention via balloon dilation and stent placement. On follow-up, the patient has nor...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951115001936
更新日期:2016-04-01 00:00:00
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
更新日期:2018-12-26 00:00:00
abstract:BACKGROUND:This study evaluated the effectiveness of long-term intensive lipid-lowering therapy in children and adolescents with familial hypercholesterolaemia. METHODS:The charts of 89 children and adolescents with heterozygous familial hypercholesterolaemia among ∼1000 patients treated from 1974 to 2008 were reviewe...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951113000528
更新日期:2014-06-01 00:00:00
abstract:UNLABELLED:So as to determine the value of brain natriuretic peptide in the plasma as a biochemical marker for myocarditis of Kawasaki disease, we studied 69 patients. The blood samples, electrocardiograms and cross-sectional echocardiograms were obtained before the commencement of treatment and in the convalescent pha...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/s1047951102000318
更新日期:2002-03-01 00:00:00
abstract::In Respiratory Syncytial Virus infection, the early identification of infants at risk for severe disease in order to potentially decrease morbidity could be considered a major goal. Current guidelines recommend only clinical observation for this purpose in infants without known comorbidities. However, recent evidence ...
journal_title:Cardiology in the young
pub_type: 信件
doi:10.1017/S1047951119002348
更新日期:2019-11-01 00:00:00
abstract::The levoatriocardinal vein is the embryological remnant of the connection between the pulmonary and systemic venous systems. It is a rare lesion that usually occurs in the presence of left-sided obstruction, developing as a pathway for decompression of the pulmonary veins. We report the first case of a levoatriocardin...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951116000652
更新日期:2016-08-01 00:00:00
abstract::To date, no reference values have been provided for right and left atrial filling in normal children. The aim of our study, therefore, was to characterize measurements of superior caval, hepatic, and pulmonary venous flow using Doppler echocardiography in a large group of normal children to reflect the effects of age,...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/s1047951103000283
更新日期:2003-04-01 00:00:00
abstract::This brief report describes a case of flail anterior tricuspid valve leaflet in a neonate associated with maternal antiphospholipid syndrome. Fetal echocardiography at 27 weeks of gestation showed competent atrioventricular valves with biventricular echogenic chordae. Fetal distress was noted at delivery, and echocard...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951117000798
更新日期:2017-10-01 00:00:00
abstract::We describe our experience with 2 patients having Costello syndrome, aged 11 and 36 months, who suffered systolic anterior motion of the aortic leaflet of the mitral valve and obstructive cardiac hypertrophy requiring surgery, comparing their cardiac characteristics to those described previously. We conclude that the ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951109991181
更新日期:2009-12-01 00:00:00
abstract::A hyperinflammatory response to COVID-19 is being described in children. While this presents, and responds to management, similar to that of Kawasaki Disease it is being coined a new entity. But is it really? We explore how this phenomenon may be Kawasaki Disease with a new trigger. ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120001432
更新日期:2020-07-01 00:00:00
abstract:OBJECTIVES:The purpose of this study was to evaluate the safety and efficacy of the ASDOS-technique (Sulzer-Osypka GmbH, Germany) for transcatheter closure of atrial septal defects within the oval fossa. BACKGROUND:Although several attempts have been made to occlude defects within the oval fossa by transcatheter techn...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/s1047951100007125
更新日期:1998-10-01 00:00:00
abstract::Senning operation is a surgical treatment for transposition of great arteries that can be complicated by post-procedural atrial or caval baffle leaks. We present a 6-year-old boy with a history of Senning repair for transposition of great arteries, who developed a pulmonary venous baffle leak. Percutaneous baffle leak...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120002255
更新日期:2020-10-01 00:00:00
abstract::Infants with hypoplastic left heart syndrome often experience difficulty with oral feeding, which contributes to growth failure, morbidity, and mortality. In response to feeding difficulty, clinicians often change the bottle nipple, and thus milk flow rate. Slow-flow nipples have been found to reduce the stress of fee...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951116000251
更新日期:2017-01-01 00:00:00
abstract:AIM:In present study, we aimed to evaluate the changes in valvular regurgitations in mid-term follow-up of children with first attack acute rheumatic fever diagnosed after updated Jones criteria. MATERIALS AND METHODS:The medical records of the children diagnosed with acute rheumatic fever between June 2015 and Novemb...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951119003317
更新日期:2020-03-01 00:00:00
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
更新日期:2015-08-01 00:00:00
abstract::An unusual combination of three types of interatrial communications - coronary sinus defect, primum defect, and secundum defect - occurring together in a 3-year-old child is presented. ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S104795112000400X
更新日期:2020-12-01 00:00:00
abstract::We report three-dimensional imaging of a rare finding of Ebstein's anomaly of the tricuspid valve in a patient with hypoplastic left heart syndrome, which has been previously reported only by two-dimensional echocardiography. A fetal echocardiogram was performed at 19 weeks that showed a moderately hypoplastic left ve...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951118000367
更新日期:2018-06-01 00:00:00
abstract::Body mass index, race/ethnicity, and payer status are associated with operative mortality in congenital heart disease (CHD). Interactions between these predictors and impacts on longer term outcomes are less well understood. We studied the effect of body mass index, race/ethnicity, and payer on 1-year outcomes followi...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120003911
更新日期:2020-11-19 00:00:00
abstract:BACKGROUND:Fungal endocarditis classically involves dense heterogenous vegetations. However, several patients with fungal infections were noted to have myocardial changes ranging from focal brightening to nodular thickening of chordae or papillary muscles. This study evaluates whether these findings are associated with...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120003674
更新日期:2020-11-13 00:00:00
abstract::Although cardiac tumours are rare, such tumours are increasingly being diagnosed with increasing frequency and great accuracy by antenatal ultrasound. Cardiac haemangiomas account for less than one-twentieth of all primary cardiac tumours, with most being diagnosed in the neonatal period. We report 3 instances of succ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951109004168
更新日期:2009-08-01 00:00:00
abstract:BACKGROUND:We previously reported four heterozygous missense mutations of MYH7, KCNQ1, MYLK2, and TMEM70 in a single three-generation Chinese family with dual Long QT and hypertrophic cardiomyopathy phenotypes for the first time. However, the clinical course among the family members was various, and the potential myoca...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951118000860
更新日期:2018-09-01 00:00:00
abstract::Chest pain in adolescents is rarely associated with cardiac disease. Adolescents with medically unexplained chest pain usually have high levels of anxiety and depression. Psychological stress may trigger non-cardiac chest pain. This study evaluated risk factors that particularly characterise adolescence, such as major...
journal_title:Cardiology in the young
pub_type: 杂志文章,多中心研究
doi:10.1017/S1047951116002109
更新日期:2017-08-01 00:00:00
abstract:BACKGROUND:The initial classic Fontan utilising a direct right atrial appendage to pulmonary artery anastomosis led to numerous complications. Adults with such complications may benefit from conversion to a total cavo-pulmonary connection, the current standard palliation for children with univentricular hearts. METHOD...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S104795111900146X
更新日期:2019-08-01 00:00:00
abstract:BACKGROUND:Syncope is common in children and adolescents and most commonly represents neurocardiogenic syncope. No information has been reported regarding the effect of syncope on health-related quality of life in children. METHODS:This was a retrospective cohort study of patients seen in the Heart Institute Syncope C...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951112000133
更新日期:2012-10-01 00:00:00
abstract::Herein, we present the first case of dilated cardiomyopathy due to premature constriction of the ductus arteriosus. A fetal echocardiography showed narrowing in the ductus arteriosus, and colour Doppler flow mapping could not identify blood flow through the ductus. Neonatal echocardiography revealed a left ventricular...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951118001002
更新日期:2018-09-01 00:00:00
abstract::In many parts of the world, mostly low- and middle-income countries, timely diagnosis and repair of congenital heart diseases (CHDs) is not feasible for a variety of reasons. In these regions, economic growth has enabled the development of cardiac units that manage patients with CHD presenting later than would be idea...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951117002591
更新日期:2017-12-01 00:00:00
abstract::We present a rare case of incidentally diagnosed Twiddler's syndrome in a child 7 years after implantation of a dual-chamber pacemaker system with epicardial leads. During revision, an insulation defect of the ventricular lead was evident, despite unremarkable prior pacemaker lead testing. The lead was repaired and a ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120001031
更新日期:2020-06-01 00:00:00
abstract::A 5-week-old child presented with a cardiac arrest secondary to myocardial ischaemia. Echocardiography demonstrated a single coronary artery arising from the right sinus of Valsalva. The coronary artery branched into left and right arteries, with the left artery then coursing anomalously in the tissue plane between th...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951104006092
更新日期:2004-12-01 00:00:00