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Adult Congenital Heart Disease
Published in Takahiro Shiota, 3D Echocardiography, 2020
Pastora Gallego, Silvia Montserrat
Ebstein anomaly is a rare congenital cause of isolated TR that has an extremely variable natural history.23 It may present at any age, with a variety of hemodynamic and electrophysiologic sequelae. Patients with isolated minor or moderate TV displacement may remain asymptomatic until late adult life. It is characterized by the tethering of TV leaflets (most commonly the septal and posterior leaflets) to the walls of the RV and ventricular septum. The tethering process of the septal leaflet to the ventricular septum results in an apparent displacement of the leaflet attachment toward the apex of the RV, which is a characteristic feature seen on 2D TTE. Atrial septal defects, mainly secundum type, are present in over 80% of patients with Ebstein abnormality.
Congenital Heart Disease in Pregnancy
Published in Afshan B. Hameed, Diana S. Wolfe, Cardio-Obstetrics, 2020
Ebstein anomaly is a congenital malformation of the tricuspid valve defined by failure of delamination of the septal leaflet from the septum, apical and posterior displacement of the functional tricuspid annulus, and elongation of the anterior leaflet. Part of the anatomic right ventricle is “atrialized,” and the right atrioventricular junction (true tricuspid annulus) is dilated. The right ventricular wall is typically thinned and dilated, and Wolf-Parkinson-White is often seen due to disruption of the normal electrical separation between the right atrium and the right ventricle. Patients with more mild forms of Ebstein anomaly may not ever require intervention, whereas those with very severe forms may require transplant consideration as infants due to severe ventricular dysfunction. Adults with a diagnosis of Ebstein anomaly may be unrepaired with chronic tricuspid regurgitation, may have had prior tricuspid valve repair with variable degrees of residual tricuspid regurgitation, or may have had prior tricuspid valve replacement.
Cardiology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Ebstein anomaly describes a malformed tricuspid valve where the posterior and septal leaflets are dis-placed inferiorly, effectively atrialising part of the right ventricle. The remaining right ventricle is small and unable to function efficiently, reducing pulmonary blood flow and resulting in varying degrees of cyanosis. Development of this lesion is associated with maternal lithium use during pregnancy.
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 Ebstein anomaly, right atrial dilation predisposes to various types of atrial arrhythmias [35]. The most common arrhythmia is cavotricuspid isthmus-dependent IART, although circuits may also propagate around atrial incisions and septal scars in patients with prior surgical repair [36,37]. Arguments can also be made in support of routine electrophysiological studies in patients without documented arrhythmias to unmask and address potential arrhythmic substrates by catheter ablation before surgery or to inform concomitant surgical ablation [38]. Replacing a tricuspid valve or inserting an annuloplasty ring can compromise future attempts to ablate accessory pathways along the right atrioventricular groove or to electrically block the cavotricuspid isthmus. Acute success rates for catheter ablation of substrates around the tricuspid annulus were reported to be 94%, 92%, and 73% in patients with no surgery, tricuspid valve repair, and tricuspid valve replacement or ring, respectively [39]. In patients with a prosthetic tricuspid valve, ablation along the ventricular side of the annulus is sometimes required to achieve bidirectional block for cavotricuspid isthmus-dependent IART [40]. Atrial fibrillation and other left atrial arrhythmias can also occur in patients with Ebstein anomaly, especially in those with left atrial enlargement due to acquired comorbidities or right-to-left-shunting.
Peripartum anesthetic management in patients with Ebstein anomaly: a case series
Published in Baylor University Medical Center Proceedings, 2023
Carmelina Gurrieri, Emily E. Sharpe, Heidi M. Connolly, Carl H. Rose, Katherine W. Arendt
Ebstein anomaly (EA) is a rare congenital cardiac malformation that affects 1 in 10,000 to 1 in 50,000 live births1 and is characterized by abnormalities of both the tricuspid valve leaflets and the right ventricle (RV)1(Figure 1). Typically, the valve is displaced and presents various degrees of regurgitation. The RV is divided into two portions: the more proximal, referred to as the “atrialized” RV, and the more distal, functional RV, which is typically small, and it can be affected by an inherent myopathy with declining function over time.2 Patients with EA may have other associated cardiac abnormalities, including atrial septal defect or patent foramen ovale, with a right-to-left shunt and an RV outflow tract obstruction. Arrhythmias are also common, including atrial fibrillation, atrial flutter, and Wolff-Parkinson-White syndrome.2 Pregnant women affected by EA are at increased risk of adverse outcomes, such as fetal loss, premature birth, cesarean delivery (CD), postpartum hemorrhage, congenital heart disease in offspring, and major adverse cardiac events, including congestive heart failure and arrhythmias requiring intervention.3–5 We retrospectively assessed the peripartum anesthetic management and outcomes of patients with EA who delivered at our institution.
Updates in the management of congenital heart disease in adult patients
Published in Expert Review of Cardiovascular Therapy, 2022
Danielle Massarella, Rafael Alonso-Gonzalez
It is hypothesized that avoidance of acute and chronic insults to the electromechanical milieu over time might decrease individuals’ likelihood of developing arrhythmia; however, the vast heterogeneity of the patient population makes substrate management and risk factor modification challenging. One example in which anatomical substrate modification has shown to reduce the risk of arrhythmia is in the surgical approach to d-transposition of the great arteries (d-TGA). Twenty five years following atrial switch repair only 36% of patients will be free of arrhythmia [12], whereas only 3–5% of patients will develop arrhythmias after arterial switch [12]. On the contrary, the new onset of atrial arrhythmias in patients with Ebstein anomaly is often considered one of the indications for surgery in those with severe tricuspid regurgitation.