Abstract:
:Many, if not most, of the controversies regarding the description of the congenitally malformed heart have been resolved over the turn of the 20th century. A group of lesions that remains contentious is the situation in which both arterial trunks, in their greater part, are supported by the morphologically right ventricle. It was considered, for many years, that presence of bilateral infundibulums, or conuses, was a necessity for such a diagnosis. It has now been appreciated that this suggestion founders on many counts. In the first instance, such bilateral infundibulums are to be found in patients with other ventriculo-arterial connections, including the otherwise normal heart. In the second instance, it is clear that such an approach abrogates the important principle now known as the morphological method. This states that entities should be defined in terms of their intrinsic morphology and not on the basis of other variable features. It is now also clear that, when assessed simply on the basis of the ventricular origin of the arterial trunks, a significant number of patients fulfil the criteria for so-called "200%" origin of the trunks from the right ventricle when there is fibrous continuity between the leaflets of the atrioventricular and arterial valves. In this review, we show how attention to the morphology of the channel between the ventricles now provides the key to accurately diagnose the ventriculo-arterial connection in patients with suspected double-outlet right ventricle. This is because, when both arterial trunks arise exclusively or predominantly from the morphologically right ventricle, the outlet septum, of necessity, is itself a right ventricular structure. The channel between the ventricles, therefore, is roofed by the inner heart curvature, whether that structure is fibrous or muscular. Our observations then confirm that it is the attachment of the outlet septum, which itself can be muscular or fibrous, which determines the commitment of the interventricular communication to the subarterial outlets. The interventricular communication itself, when directly committed to the ventricular outlets, opens between the limbs of the septomarginal trabeculation or septal band. The defect is subaortic when the outlet septum is attached to the cranial limb of the trabeculation, subpulmonary when attached to the caudal limb, and doubly committed when attached to the inner heart curvature in the roof of the defect. Non-committed defects are no longer positioned within the limbs of the septomarginal trabeculation. Although readily demonstrable by a skilled echocardiographer, we show how these anatomical features are more easily demonstrated with added accuracy when using CT data sets.
journal_name
Cardiol Youngjournal_title
Cardiology in the youngauthors
Bharucha T,Hlavacek AM,Spicer DE,Theocharis P,Anderson RHdoi
10.1017/S1047951116001190subject
Has Abstractpub_date
2017-01-01 00:00:00pages
1-15issue
1eissn
1047-9511issn
1467-1107pii
S1047951116001190journal_volume
27pub_type
杂志文章,评审abstract::Plastic bronchitis is a severe complication after a Fontan procedure, with an estimated incidence around 1-2% and poor prognosis. We present the cases of four patients with plastic bronchitis after a total cavopulmonary connection with a stenosis of the left pulmonary artery that was stented successfully. In three of ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951113002163
更新日期:2015-01-01 00:00:00
abstract:BACKGROUND:This study aimed to examine the differences between arterial and inferior caval vein oxygen saturation, fractional oxygen extraction, and the shunt index, which were calculated in the diagnosis of patent ductus arteriosus. METHODS:Twenty-seven preterm infants were included in this study and were divided int...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951113000772
更新日期:2014-08-01 00:00:00
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
更新日期:2010-06-01 00:00:00
abstract::Untreated congenital long QT syndrome may result in potentially lethal ventricular tachycardia. In the most common type, risk of such an event has been linked to exercise. This originally resulted in very restrictive guidelines for sports participation in affected individuals. Although the complex interactions of a sp...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951116002225
更新日期:2017-01-01 00:00:00
abstract:BACKGROUND:In very low birth weight infants, persistence of a patent ductus arteriosus results in morbidity and mortality. Therapies to close the ductus are effective, but clinical outcomes may depend on the accuracy of diagnosis and the timing of administration. The objective of the present study was to characterise t...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951117001081
更新日期:2017-11-01 00:00:00
abstract:BACKGROUND:To the best of our knowledge, normal limits of heart rate with respect to gender, and as established using 24-hour ambulatory Holter electrocardiography, have yet to be published for the entire age range of children and adolescents. OBJECTIVES:To establish the normal limits for heart rate in newborns, infan...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951108002539
更新日期:2008-10-01 00:00:00
abstract::Improvement in operative survival of patients with hypoplastic left heart syndrome has led to increasing emphasis on prevention of interstage mortality. Many centres have improved interstage results through programmes of home monitoring following discharge after the Norwood (Stage 1) operation. Experience with heighte...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951111001600
更新日期:2011-12-01 00:00:00
abstract::Aim We aimed to study the dimensions, systolic and diastolic functions of the left ventricle; dimensions and elasticity of the aorta; and carotid intima-media thickness and flow-mediated dilatation of the brachial artery in mitral valve prolapse. METHODS:The study group consisted of 43 patients (mean age=13.3±3.9) an...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951117001950
更新日期:2018-02-01 00:00:00
abstract:BACKGROUND:Care of children with functionally univentricular hearts is resource-intensive. OBJECTIVES:To analyse pregnancy and early post-natal outcomes of fetuses with functionally univentricular hearts in the setting of a low-middle-income country. METHODS:A retrospective study was conducted during the period of Ja...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120002929
更新日期:2020-12-01 00:00:00
abstract::Communications between the pulmonary artery and left atrium cause cyanosis. The images document serial haemodynamic changes in such a fistula from fetal life to the postnatal period with a successful transcatheter intervention. ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951118000616
更新日期:2018-10-01 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::Milrinone is a drug frequently used for hemodynamic support in children during critical illness. Although the hemodynamic changes induced by milrinone in children may appear similar to those of adults, the physiologic contributors of these changes remain vastly unknown. A systematic review was conducted to identify st...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951119002865
更新日期:2020-01-01 00:00:00
abstract::Right atrial isomerism is associated with complex cardiac malformations, particularly single-ventricle lesions; right atrial isomerism is rarely associated with aorto-pulmonary collateral arteries. We report a foetal diagnosis of right atrial isomerism, with an unbalanced atrioventricular septal defect, pulmonary sten...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120003649
更新日期:2020-11-26 00:00:00
abstract:BACKGROUND:Although some prior studies have provided evidence to question the historical belief that pulmonary vascular resistance index ⩾6 Wood Units×m2 should be a contraindication to heart transplantation in children, no national analyses specific to the modern area have addressed this question. METHODS:Data were a...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951114001796
更新日期:2015-08-01 00:00:00
abstract::Congenitally corrected transposition is a rare congenital anomaly, with only a few cases diagnosed and reported prenatally even in the largest fetal series. To determine the morphologic features and outcome for the lesion as recognized during fetal life, we reviewed the fetal and postnatal echocardiograms and medical ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S104795110400304X
更新日期:2004-06-01 00:00:00
abstract::Undiagnosed coronary sinus obstruction can be a source of paediatric exertional chest pain and death. We present a case series of three interesting children who developed only exertional angina from various degrees of coronary sinus stenosis or atresia. We discuss the diagnosis and management of each case and the pote...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120000918
更新日期:2020-06-01 00:00:00
abstract:BACKGROUND:We have used the Amplatzer ductal occluder for transcatheter closure of large persistently patent arterial ducts, and used our experience to assess the safety and efficacy of the device in young children and infants. METHODS AND PATIENTS:We used the Amplatzer ductal occluder prospectively in 43 patients wit...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951105000570
更新日期:2005-06-01 00:00:00
abstract::Primary care cardiology, also known as ambulatory or outpatient cardiology, for the longitudinal management of patients with common arterial trunk or with transposition of the great arteries is both poorly described and has limited evidence to justify its basis. This article discusses some of the various complications...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951112001588
更新日期:2012-12-01 00:00:00
abstract:BACKGROUND:Previous studies have demonstrated structural changes in the heart and cardiac dysfunction in foetuses with intrauterine growth restriction. There are no available data that evaluated left ventricular dimensions and mass in neonates with symmetric and asymmetric intrauterine growth restriction. Therefore, we...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951113002199
更新日期:2015-02-01 00:00:00
abstract:BACKGROUND:Disagreement currently exists regarding the definition of aortic dextroposition. It is suggested that the term be used interchangeably with aortic overriding, along with suggestions that the aortic valve overrides in the normal heart. The dextroposed aorta, however, does not always override the crest of the ...
journal_title:Cardiology in the young
pub_type: 杂志文章,评审
doi:10.1017/S1047951114001139
更新日期:2015-04-01 00:00:00
abstract:OBJECTIVE:We sought to use techniques of decision analysis to compare values or preferences for balloon angioplasty versus surgery for treatment of aortic coarctation in children. BACKGROUND:Balloon angioplasty and surgery for aortic coarctation have a differing spectrum and prevalence of outcomes and complications, m...
journal_title:Cardiology in the young
pub_type: 杂志文章,meta分析,评审
doi:10.1017/S1047951107001795
更新日期:2008-02-01 00:00:00
abstract::Oxidative stress may contribute to the pathogenesis of congenital heart defects, but the role of dynamic thiol/disulphide homeostasis has not been evaluated. The objective of this study was to assess whether there are changes in thiol/disulphide homeostasis and nitric oxide levels in children with tetralogy of Fallot ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S104795111900012X
更新日期:2019-04-01 00:00:00
abstract:OBJECTIVE:To evaluate the family psycho-social outcomes of children with Down syndrome and atrioventricular septal defect, and examine the impact of these variables on the child's neurodevelopmental outcome. METHODS:This was a cross-sectional study that consisted of 57 children with Down syndrome - 20 cases and 37 con...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951115000062
更新日期:2016-02-01 00:00:00
abstract::Torsades de Pointes is an extremely rare arrhythmia in children associated to LQT syndrome. Pheochromocytomas are also extremely rare tumours in the paediatric age. We present a case of a young patient with an acquired long QT syndrome complicating with Torsades de Pointes as first clinical manifestation of a pheochro...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S104795111400033X
更新日期:2015-02-01 00:00:00
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
更新日期:2021-01-18 00:00:00
abstract::We report an extremely rare case of a 14-month-old girl who was diagnosed with a single right coronary artery with coronary artery fistula communicating with the right ventricle and congenital absence of left coronary artery. Angiography showed a dilated and tortuous single right coronary artery draining into the righ...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951119002105
更新日期:2019-11-01 00:00:00
abstract::Persistent fifth - double-lumen - aortic arch is a rarely seen entity, which is usually associated with other cardiac anomalies; it has been previously reported only in children. We report a new case of double-lumen aortic arch with a systemic-to-systemic connection diagnosed incidentally during cardiac multi-detector...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951111001855
更新日期:2012-06-01 00:00:00
abstract::Hypereosinophilic syndrome is defined as persistent eosinophilia in the blood for more than 6 months, without any identifiable cause and with end-organ involvement evidence. Cardiac manifestations of HES include heart failure due to restrictive cardiomyopathy, arrhythmia, intraventricular thrombosis, and coronary arte...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951120004862
更新日期:2021-01-25 00:00:00
abstract::Atrioventricular valve regurgitation is widely known as a risk factor for Fontan completion in patients with univentricular physiology. To date, indications and timing for atrioventricular valve repair remain unclear and different surgical techniques have been advocated. Since 2013, 50 consecutive patients underwent e...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951114002352
更新日期:2015-10-01 00:00:00
abstract:OBJECTIVE:Efficient diagnosis of an underlying genetic aetiology in a patient with congenital heart disease is essential to optimising clinical care. Copy number variants are one aetiology of congenital heart disease; the majority are identifiable by targeted fluorescence in situ hybridisation or array comparative geno...
journal_title:Cardiology in the young
pub_type: 杂志文章
doi:10.1017/S1047951113001868
更新日期:2015-01-01 00:00:00