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Cardiac conditions
Published in Judy Bothamley, Maureen Boyle, Medical Conditions Affecting Pregnancy and Childbirth, 2020
The tetralogy of Fallot is the most common cyanotic congenital heart defect and consists of a ventricular septal defect, overriding aorta, pulmonary stenosis and right ventricular hypertrophy with consequent cyanosis (see Figure 2.6). Women with an uncorrected condition are rarely seen in pregnancy and those with a previous repair without residual defects and normal functional status should do well, although arrhythmias may be present. The risks in pregnancy after repair depend on the status of the repair, and surgery may lead to pulmonary regurgitation, which could lead to right ventricular dysfunction2, which may in turn result in ventricular tachycardia and sudden death44.
Cardiac surgery
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
A right-to-left shunt resulting in decreased pulmonary blood flow. Many of these lesions consist of a septal defect in conjunction with a right-sided obstructive lesion, producing an obligatory right-to-left shunt. The most common cause of this is tetralogy of Fallot.
Congenital cardiac anomalies
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
Tetralogy of Fallot is characterised by the development of right ventricular outflow tract obstruction, with a subaortic ventricular septal defect that shunts blood from the right ventricle to the left ventricle and aorta. Patients are cyanotic and have a harsh systolic ejection murmur (from right ventricular obstruction). Repair is accomplished by division of obstructing right ventricular muscle bundles, closure of the ventricular septal defect, dilatation of the pulmonary valve and relief of pulmonary stenosis with placement of a patch across the right ventricular outflow tract in most cases (either in the infundibular location or across the pulmonary valve annulus).
Clinical Presentations and Diagnostic Imaging of VACTERL Association
Published in Fetal and Pediatric Pathology, 2023
Gabriele Tonni, Çağla Koçak, Gianpaolo Grisolia, Giuseppe Rizzo, Edward Araujo Júnior, Heron Werner, Rodrigo Ruano, Waldo Sepulveda, Maria Paola Bonasoni, Mario Lituania
In VACTERL, anatomical anomalies must occur between the 23rd and 56th day post-conception, as this embryological window is critical for the development of the vertebrae (23-32 days), heart, tracheoesophageal structures, forearm bones (29-41 days), and anorectal region (45-56 days). Malsegmentation of the vertebrae is the result of timing abnormalities in the segmentation clock. Anorectal and tracheoesophageal defects are due to disturbed mesodermal proliferation and migration, epithelial-mesenchymal interactions, and apoptosis. Radial aplasia can be caused by impairment in laying down, condensing, or chondrifying the angle of the radius. Renal and urinary tract anomalies may be the result of failed growth of the ureteric bud, metanephric mesenchyme, and mesonephros/mesonephric duct. Cardiac defects involve altered cardiac septal development, as atrioventricular septal defects and tetralogy of Fallot defects are common [13].
The challenges of an aging tetralogy of Fallot population
Published in Expert Review of Cardiovascular Therapy, 2021
Jennifer P. Woo, Doff B. McElhinney, George K. Lui
Key issues related to an aging tetralogy of the Fallot population include pulmonary regurgitation, heart failure, arrhythmias, acquired cardiovascular disease and pregnancy.Current recommendations for pulmonary valve replacement rely on late disease findings of right ventricular dysfunction by cardiac magnetic resonance. Advances in imaging have the potential to detect subtle early right heart dysfunction using myocardial deformation, 4D flow magnetic resonance, and cardiopulmonary stress testing.The management of arrhythmias, heart failure, and aortic abnormalities can be challenging due to a paucity of data to guide surveillance, prevention, and treatment.Fragmented medical care is common in adults with repaired tetralogy of Fallot. Regular follow-up with adult congenital cardiology is essential to prevent and mitigate heart failure, arrhythmias, and acquired atherosclerotic diseases.
Results of Pulmonary Valve Replacement with a Newly Introduced Bioprosthesis in Children and Young Adults with Congenital Heart Disease
Published in Structural Heart, 2020
Víctor Bautista-Hernández, María García-Vieites, José M. Arribas-Leal, Antonio Jiménez-Aceituna, Sergio Cánovas, José J. Cuenca-Castillo
Results: Between June 2017 and October 2019, 21 patients (14 males) underwent implantation of an INSPIRIS bioprosthesis in the pulmonary position. The most common diagnosis was repaired tetralogy of Fallot (n=14). Mean age was 31±16.6 years. 7 patients were children (the youngest having the replacement at the age of 4). 18 patients had previous surgeries. Demographics and in-hospital features are shown in table 1. One patient with tetralogy of Fallot died after surgery (1.6%) from multi-organ failure after developing severe right ventricular failure requiring ECMO. No patient had a permanent pacemaker after surgery or other major complications. Early echocardiogram depicted a mean peak gradient across the valve of 19.49±7.4 mm Hg. No paravalvular leaks were observed. For a median follow-up of 20 months (range 1-29 months), all patients are alive with no clinical complications. Most recent echocardiogram shows good hemodynamics (peak gradient 17±5.4 mm Hg) with no paravalvular leaks.