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Heart Failure in Adult Congenital Heart Disease
Published in Andreas P. Kalogeropoulos, Hal A. Skopicki, Javed Butler, Heart Failure, 2023
Andrew Constantine, Ana Barradas-Pires, Isma Rafiq, Justyna Rybicka, Michael A. Gatzoulis, Konstantinos Dimopoulos
The atrial switch procedure was a pioneering operation that allowed the survival of those born with TGA beyond infancy and early childhood. This was the surgery of choice for TGA until it was superseded, in the 1980s and early 1990s, by the arterial switch operation. The atrial switch operation was aimed at redirecting flow through the heart (“physiologic correction”) but maintained the RV as the systemic ventricle (Figure 28.2). Medium-term survival following a Mustard or Senning repair has been excellent, with most patients remaining asymptomatic or minimally symptomatic until adulthood.30 However, the systemic RV is a substrate for late sequelae, including RV dysfunction,31 pulmonary hypertension, impaired exercise tolerance,32 arrhythmias, and premature death.33 Subendocardial ischemia related to RV hypertrophy and inadequate myocardial perfusion is thought to account for the long-term decline in RV function.34 In systemic RVs, the systemic AV valve is a tricuspid valve and is often regurgitant.
Adult Congenital Heart Disease
Published in Takahiro Shiota, 3D Echocardiography, 2020
Pastora Gallego, Silvia Montserrat
Complete transposition of the great arteries (also known as D-TGA) is one of the most common cyanotic defects seen in newborns. Many patients with D-TGA have survived into adulthood with an atrial switch procedure (Mustard or Senning operation) performed in infancy, which leaves the morphological RV supporting the systemic circulation. Unfortunately, the atrial switch surgeries are associated with several complications later in life including baffle leaks, obstructions, and TV insufficiency. Now, the standard of care includes surgery to switch the arterial structures.
Congenital Heart Disease in Pregnancy
Published in Afshan B. Hameed, Diana S. Wolfe, Cardio-Obstetrics, 2020
The first definitive surgical repairs for D-TGA were the Senning [18] and Mustard [19] atrial switch procedures. The atrial switch procedure is a physiologic correction that uses surgically placed baffles to redirect systemic venous return across the atrial septum to the left atrium, thus allowing deoxygenated blood to flow to the pulmonary artery; the pulmonary venous baffle directs the pulmonary venous return rightward across the atrial septum to the right atrium, thus allowing oxygenated blood to travel to the aorta. In the atrial switch procedure, the right ventricle thus serves as the systemic ventricle, pumping oxygenated blood to the aorta, while the left ventricle is the subpulmonary ventricle, pumping deoxygenated blood to the pulmonary artery. Long-term sequelae of the atrial switch procedures include baffle stenoses which can increase pressures in the systemic or pulmonary veins, baffle leaks which would result in an atrial level shunt, systemic right ventricle systolic and diastolic dysfunction, tricuspid regurgitation, atrial tachyarrhythmias, and sinus node dysfunction.
Robotic magnetic-guided catheter ablation in patients with congenital heart disease: a systematic review and pooled analysis
Published in Expert Review of Cardiovascular Therapy, 2023
Christophe Vô, Stefano Bartoletti, Karim Benali, Cecilia M Gonzalez, Blandine Mondésert, Marie-Hélène Gagnon, Katia Dyrda, Anne Fourier, Paul Khairy
When reported, the majority of procedures were performed through femoral venous access to the IVC, although a catheter trajectory through hemi-azygos continuation of an interrupted IVC was also described [12]. The second most frequently reported catheter trajectory was a retrograde aortic approach through a femoral artery, with one case using a brachial artery instead [33]. Reported reasons to perform a retrograde aortic approach were: (i) to avoid a transbaffle puncture to access the pulmonary venous atrium in patients with atrial switch surgery; (ii) to avoid a transseptal puncture to access the left atrium after percutaneous closure of an atrial septal defect or congenital absence of the IVC, and (iii) to avoid a transconduit puncture to access the pulmonary venous atrium in patients with single ventricle physiology after a total cavopulmonary connection or Fontan procedure. A superior venous approach, i.e. through right or left jugular or subclavian veins, was performed in the context of congenital absence of an IVC or acquired obstruction of femoral veins. Less common approaches were via transhepatic access in one patient [14] and peripheral access through brachial veins in 2 patients [33]. Transseptal or transbaffle punctures were performed in 10 (5.0%) procedures.
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.
Percutaneous techniques for treatment of tricuspid valve dysfunction in congenital heart disease – an emerging therapy
Published in Expert Review of Cardiovascular Therapy, 2021
Dysfunction of systemic TV in ccTGA or after Senning or Mustard operation is a more complex situation. After the initial atrial switch operation, most patients do well for some years; however, in some RV dysfunction with dilation and consecutive TVR occurs. Surgical tricuspid valve replacement, if indicated, is a high-risk procedure also bioprosthesis in this position has a limited durability [7,8]. Percutaneous tricuspid valve-in-valve implantation is a relatively new treatment option for these patients. For transcutaneous valve delivery in this anatomy, the atrial baffle has to be perforated. If a baffle has been formed by synthetic material as it is done in the Mustard procedure, the perforation could have been technically more demanding. Nevertheless, even in a Senning anatomy, a balloon dilatation of the septum can be appropriate before advancing a big sheath across the baffle. If in the future inevitable valve dysfunction reoccurs, surgical valve replacement with a mechanical prosthesis needs to be discussed [9].