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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 incidence of HF varies greatly depending on the underlying CHD. Patients with single ventricles or systemic RVs are at increased risk of HF from early life. By adulthood, HF is present in up to 40% of patients with a Fontan circulation and 20% of patients following an atrial switch (Mustard or Senning) procedure for transposition of the great arteries (TGA).12 In the Dutch national registry, chronic HF accounted for over one-third of deaths in those with congenitally corrected transposition of the great arteries (ccTGA), univentricular hearts, and tetralogy of Fallot (ToF).13
pH Management During Hypothermic Cardiopulmonary Bypass with Circulatory Arrest
Published in Richard A. Jonas, Jane W. Newburger, Joseph J. Volpe, John W. Kirklin, Brain Injury and Pediatric Cardiac Surgery, 2019
There are several limitations to this study. Data were collected retrospectively and were subject to incomplete and inconsistent recording. In addition many other aspects of surgical and support procedures, other than acid-base management, changed over the same period. For example, the oxygenator was changed from bubble to membrane in this time frame. This was associated with a change in the tubing circuit design in that a recirculation line was added to allow continuous recirculation during the period of circulatory arrest. Recirculation was also used prebypass. Even with minimal gas flow there was often a very alkaline pH of the perfusate at the onset of bypass. The patient population may have also changed importantly over this time frame of 1983 to 1988, which was the period of introduction of the arterial switch for transposition. Early in the time frame, the study patients were likely to have been representative of the spectrum of transposition, but this is not likely to have been the case late in the series when children having the Senning procedure had to have been rejected from the arterial switch protocol, perhaps because of complex coronary artery anatomy. In addition the surgeons’ recent familiarity with the Senning procedure decreased later in the series.
Congenital cardiac anomalies
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
Congenital heart surgeryNorwood procedureRoss procedureDor procedureBlalock–Taussig shuntGlenn procedureFontan procedureMustard procedureSenning procedureRastelli procedureJatene procedure
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
Accessory pathways are commonly found along the Ebsteinoid tricuspid valve of the systemic right ventricle in patients with ccTGA and are most often posteroseptal in our experience. Moreover, IART circuits have been described that depend on the right-sided cavo-mitral isthmus or that propagate around atriotomy incisions [41]. Patients with ccTGA are more likely to undergo surgical intervention for tricuspid regurgitation than those with D-TGA and Mustard or Senning baffles. Tricuspid regurgitation is believed to be an important driver of systemic right ventricular dysfunction in this patient population and can result in increasing left atrial dilatation and left atrial arrhythmias [42–44]. The L-transposed great arteries are typically associated with a posterior and rightward main pulmonary artery. Akin to ablating an atrial arrhythmia in an aortic cusp in the context of a structurally normal heart, successful catheter ablation of atrial tachycardia has been reported in the pulmonary sinus of a patient with ccTGA [45]. One pulmonary sinus is in proximity to the right atrium, while the second is adjacent to the left atrium. Finally, some patients with ccTGA will have undergone a double switch procedure (so-called anatomic repair) that involves a Mustard or Senning procedure in combination with an arterial switch or Rastelli-type ventriculo-arterial redirection. While this surgery restores the left ventricle to the systemic position, patients are subject to the atrial arrhythmias associated with Mustard and Senning baffles.
Management of congenitally corrected transposition from fetal diagnosis to adulthood
Published in Expert Review of Cardiovascular Therapy, 2023
All the surgical options mentioned above leave the morphologically RV in the systemic position. A completely new approach to surgical management of ccTGA was proposed at the turn of the 1980s and 1990s [34–36]. The aim of so-called anatomical correction or double-switch procedure is to restore the morphologically left ventricle into the subaortic position. It can be achieved by atrial switch (Mustard/Senning procedure) in combination with arterial switch operation. In patients with pulmonary valve stenosis, the arterial switch procedure cannot be performed. In such cases, the Rastelli procedure, the Nikaidoh procedure and the REV procedure are the options [37]. The long-term survival of anatomic repair is acceptable (20-year survival of 83%) [38], and the long-term complication of the arterial switch is the neoaortic regurgitation [39]. Of importance, this surgical option is available only for infants and young children in whom the morphologically left ventricle is still capable to sustain systemic pressure. In patients without subpulmonary ventricular flow obstruction, the pulmonary artery banding has been proposed as a way to adapt the left ventricle to work as a systemic ventricle. The results of pulmonary artery banding as a bridge to double switch procedure are inconsistent [40–42]. An intention-to-treat analysis of pulmonary artery banding showed that patients above 16 years of age are unlikely to achieve anatomic repair [43]. Some authors noted that individuals with palliative pulmonary artery band had better survival than the anatomic repair group [44], whereas others observed the lowest transplant-free survival at 10 years in this group of patients [45].
Effect of medical treatment in patients with systemic right ventricle
Published in Scandinavian Cardiovascular Journal, 2020
Kristofer Skoglund, Joel Heimdahl, Zacharias Mandalenakis, Ulf Thilén, Bengt Johansson, Christina Christersson, Peder Sörensson, Mikael Dellborg
The study was designed as a nationwide, multicenter, registry-based, retrospective cohort study. The study was observational in design and included all patients who were older than 18 years (the minimum age of inclusion in SWEDCON-GUCH) with S-RV (both ccTGA and TGA after the Mustard or Senning procedure). Patients were identified from International Classification of Disease codes and a registry-specific identification number for S-RV. The time for data extraction was set to December 2016. The start of the retrospective observational period or baseline was set to the patient-specific start date for follow-up in SWEDCON-GUCH (e.g. the first visit to adult cardiology with data input to SWEDCON-GUCH).