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Cardiovascular system
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
8.23. Which of the following are associated with finger clubbing?Aortic coarctation.Tricuspid atresia.Tetralogy of Fallot.Asthma.Partial atrioventricular septal defect.
Medical Care of the Child with Down Syndrome
Published in Merlin G. Butler, F. John Meaney, Genetics of Developmental Disabilities, 2019
Approximately 50% of children with DS have a congenital heart defect. The most commonly occurring condition is atrioventricular septal defect (AVSD) which is also known as endocardial cushion defect (see Table 5). In most of these conditions, the diagnosis of a serious disorder will be made by the presence of signs and symptoms, such as a murmur, cyanosis, or congestive heart failure. Children with DS are prone to develop high pulmonary vascular resistance. In children with AVSD, this would prevent the left to right intracardiac shunt, minimize the heart murmur, and prevent heart failure from occurring. This, in turn, has the potential to lead to significant pulmonary hypertension. The only way to detect this problem with a high degree of accuracy is to perform a cardiac evaluation, including an echocardiogram on every infant with DS. (Note that in some centers, asymptomatic children with DS may be able to get a screening echocardiogram).
Congenital Heart Disease A Clinician's Perspective
Published in P. Chopra, R. Ray, A. Saxena, Illustrated Textbook of Cardiovascular Pathology, 2013
This defect, also called endocardial cushion defect, is relatively uncommon form of CHD. Association of atrioventricular septal defect (AVSD) with Down's syndrome is well-established (40% of Down's syndrome with CHD have this defect).
Management for atrial arrhythmias in adults with complex congenital heart disease
Published in Expert Review of Cardiovascular Therapy, 2023
Victor Waldmann, Christophe Vô, Stefano Bartoletti, Francis Bessière, Marie Wilkin, Nabil Dib, Nicolas Combes, Paul Khairy
In patients with an atrioventricular septal defect (AVSD), IART dependent on the right-sided cavo-annular isthmus is the most common arrhythmia. Other described circuits have predominantly been limited to case reports. Prior to ablating the right-sided cavo-annular isthmus, the His bundle potential associated with the posteroinferior displaced AV node (resulting from the ostium primum atrial septal defect) must be carefully identified. The cavo-annular ablation line should be performed more laterally in patients with AVSD in order to minimize the risks of AV block. Since patients with AVSD can also have a single AV valve, biatrial IART circuits around the common AV valve have been described [27,28]. In such cases, the arrhythmia can usually be terminated by a single ablation line between the inferior vena cava and the annulus. It is also common for patients with AVSD to undergo surgical reintervention of the left AV valve, which is associated with additional atrial incisions and sutures that predispose to arrhythmias. The terms right and left AV valves are preferred since the valves are derived from five leaflets and are not true mitral or tricuspid valves. The left AV valve is often surgically accessed using a transeptal approach, which results in septal scarring. In fact, post-surgical repair of the left AV valve, different biatrial circuits have been described depending on conduction pathways across the atrial septum (typically posteroinferior septum, fossa ovalis, Bachmann’s bundle, or ostium of the coronary sinus) [29].
Characteristics of prenatally detected right aortic arch cases in a single institution
Published in Journal of Obstetrics and Gynaecology, 2018
Melih Velipasaoglu, Metin Sentürk, Reyhan Ayaz, Barbaros Atesli, H. Mete Tanir
The total number of patients who were scanned during the study period (18 months) was 6632. A total of 228 foetuses with major anomalies were diagnosed in this low-risk group of patients with an incidence of 3.4%. Of these foetuses, 75 had major cardiac anomalies (32.8%). There were 12 cases of RAA detected prenatally. All cases were in singleton pregnancies. The prevalence in the study population was 1.8 in 1000 and constituted 16% of all major cardiac anomalies within the study cohort. Patient characteristics are presented in Table 1. The mean age of the patients was 30.4 (±3.9) years. The mean gestational age at diagnosis was 20.9 (±1.9) weeks. The only associated cardiac anomaly was atrioventricular septal defect (AVSD) in one foetus (8.3%). The only associated major extra-cardiac anomaly was oesophageal atresia in the same foetus with AVSD (8.3%).
Preventing disease progression in Eisenmenger syndrome
Published in Expert Review of Cardiovascular Therapy, 2021
Ana Barradas-Pires, Andrew Constantine, Konstantinos Dimopoulos
The ECG is fundamental to the assessment and follow-up of ES patients. Indeed, ventricular or atrial arrhythmias can lead to rapid clinical decompensation, and are a predictor of outcome in PAH-CHD [24]. Common features include signs of right ventricular hypertrophy (dominant R wave in lead V1 and right QRS axis deviation) and right atrial enlargement (so-called ‘P pulmonale’; Figure 1). The typical feature observed in patients with ES associated with an atrioventricular septal defect is a left axis deviation (or an extreme right superior axis deviation), caused by displacement of the conduction system.