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Congenital cardiac anomalies
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
Pulmonary valve atresia may be associated with dual-ventricle lesions (such as tetralogy of Fallot with pulmonary atresia, also known as pulmonary atresia with ventricular septal defect), or single-ventricle lesions (such as tricuspid atresia with pulmonary atresia, or pulmonary atresia with intact ventricular septum).
Cardiac catheterization for the adult with complex congenital heart disease
Published in Debabrata Mukherjee, Eric R. Bates, Marco Roffi, Richard A. Lange, David J. Moliterno, Nadia M. Whitehead, Cardiovascular Catheterization and Intervention, 2017
Significantly decreased pulmonary blood flow is a stimulus for the development of collateral vessels from the systemic circulation. Major aortopulmonary collaterals are present in patients with pulmonary atresia and ventricular septal defect.
Cyanotic congenital heart diseases in adulthood
Published in Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček, Congenital Heart Disease in Adults, 2008
Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček
These conditions include shunt-related defects with restricted pulmonary blood flow in the presence of significant obstruction across the pulmonary outflow tract (i.e. subvalvular, valvular, and/or supravalvular pulmonary stenosis or atresia). These cases include tetralogy of Fallot, pulmonary atresia with ventricular septal defect (VSD), a functionally single ventricle with significant pulmonary stenosis or tricuspid atresia (see Chapter 15), Ebstein anomaly with atrial septal defect (ASD; see Chapter 14), significant subvalvular, valvular or supravalvular pulmonary stenosis with atrial and/or ventricular septal defect, etc. While most of these CHD have been managed by surgical repair in childhood, some children who have not had surgery may survive until adulthood. Some of these CHD can be managed by univentricular or biventricular repair even in adulthood.
Anisotropic Polytetrafluoroethylene Cardiovascular Conduits Spontaneously Expand in a Growing Lamb Model
Published in Journal of Investigative Surgery, 2021
Anthony Azakie, John P. Carney, Matthew T. Lahti, Yuriy Moklyak, Richard W. Bianco
There is tremendous potential for further engineering of this conduit, for example, valve cusps could be added to the conduit or small intestinal submucosal (SIS) patches, thin-walled (0.1 mm–0.4 mm) PTFE, femoral venous valves or other polymer can be intussuscepted to variable heights to create a bicuspid or tricuspid valve mechanism [21,22]. Valveless RVOT connections have been used for the repair of several lesions, such as truncus arteriosus, pulmonary atresia with ventricular septal defect, tetralogy of Fallot with absent pulmonary valve, and D-Transposition of the great arteries with ventricular septal defect and severe pulmonary stenosis. Valve-less repair for reconstruction of the RVOT in infants has been shown to be associated with lower risk of reoperation. However, many surgeons have not adopted this approach because of concern with pulmonary insufficiency, which may make the early post-operative course more challenging. Dr. Alsoufi et al expressed that given the current advances in perioperative care, valve-less repair strategies might need to be reconsidered, especially because many of the conduits in small neonates and infants show early incompetence [34]. The detrimental effects of chronic pulmonary regurgitation on the late RV function and size can be addressed with transcatheter implantation of a stented-valve, which for anisotropic conduits, would allow for radial expansion to “adult” diameters while establishing pulmonary valve competence [35].
The accuracy of prenatal diagnosis of major congenital heart disease is increasing
Published in Journal of Obstetrics and Gynaecology, 2020
Rebekka Lytzen, Niels Vejlstrup, Jesper Bjerre, Olav Bjørn Petersen, Stine Leenskjold, James Keith Dodd, Finn Stener Jørgensen, Lars Søndergaard
Patients, who at any time had been given an International Classification of Disease (ICD)-10 code corresponding to a CHD (DQ20–DQ25), were identified in the NPR. Major CHD was defined as morphologically complex malformations of the heart and great arteries that typically necessitate surgery or catheter-based intervention within the first year of life (Bull 1999; Ewer et al. 2011; Oster et al. 2014; Sainz et al. 2015). Included diagnoses were derived from Hoffman and Kaplan (2002) and consisted (in hierarchical order modified from Allan et al. 1985) of: (1) univentricular heart, (2) congenitally corrected transposition of the great arteries, (3) truncus arteriosus, (4) transposition of the great arteries, (5) interrupted aortic arch, (6) atrioventricular septal defects (AVSDs), (7) double outlet right ventricle, (8) coarctation of the aorta (CoA), (9) Ebstein’s anomaly, (10) pulmonary atresia with ventricular septal defect, (11) pulmonary atresia with intact ventricular septum and (12) tetralogy of fallot.
Current clinical management of dysfunctional bioprosthetic pulmonary valves
Published in Expert Review of Cardiovascular Therapy, 2020
Varun Aggarwal, Zachary A Spigel, Gurumurthy Hiremath, Ziyad Binsalamah, Athar M Qureshi
As the indications for valve replacement expanded and the patient population aged, sutureless bioprosthetic valves were developed to promote faster implantation, a technology originally developed in 1963 by Magovern and Cromie [41,42] and modified over time [42]. The development of sutureless bioprosthetic valves allowed for the implantation to be performed percutaneously, for the first time in the pulmonary position in 2000 in an ovine model [43] and subsequently in a 12-year-old boy [44] with pulmonary atresia and ventricular septal defect who had an 18-mm right ventricle to pulmonary artery (RV-PA) conduit placed at the age of 4 years. Since its revolutionary introduction in 2000, transcatheter pulmonary valve replacement has gained widespread acceptance and usage as a non-surgical alternative among patients who have dysfunctional RV-PA conduits [45] and dysfunctional bioprosthetic valves in the pulmonary position [46] [47,48].