Many patients with ventriculoarterial discordance have survived to adulthood. Those with complete transposition of the great arteries have often had an atrial switch procedure (Mustard or Senning operation) performed, which leaves the morphological right ventricle (RV) supporting the systemic circulation. RV failure and tricuspid regurgitation are common. Some patients may ultimately require cardiac transplantation. Sinus node dysfunction is increasingly common with longer follow-up, and some patients need pacemaker implantation. Atrial arrhythmias are frequent, and atrial flutter may be a marker for sudden death. Patients with an arterial switch procedure are also surviving to adulthood. Long-term problems include coronary stenoses, distortion of the pulmonary arteries, dilatation of the neoaortic root, and aortic regurgitation. Patients with congenitally corrected transposition have both atrioventricular and ventriculoarterial discordance and therefore also have a morphological RV and delicate tricuspid valve in the systemic circulation. Associated defects, such as abnormalities of the tricuspid valve, ventricular septal defect, and pulmonary stenosis, occur in the majority of patients. Heart block occurs with increasing age. Atrial arrhythmias occur frequently, and their occurrence should prompt a search for a hemodynamic problem. Progressive tricuspid regurgitation occurs with age and is associated with deterioration of RV function. Surgical treatment should be considered at the earliest sign of RV dilatation or dysfunction. All patients should be seen periodically in a center where expertise in the clinical evaluation, imaging, and hemodynamic assessment of adult congenital heart disease is available.
The population of patients with adult congenital heart disease is approximately 800,000 in the U.S. Those with prior cardiac surgery often consider themselves “cured,” although the majority faces a lifetime of problems including arrhythmias, ventricular dysfunction, and one or more re-operations. Even patients with repaired “simple” lesions such as an atrial septal defect may not have normal survival if they are repaired in adulthood. Patients with repaired coarctation may have premature cardiov...
Objectives: To evaluate late mortality and morbidity after an atrial switch procedure for correction of transposition of the great arteries (TGA) and to assess predictive factors for adverse outcome. Setting: Tertiary referral centre. Design and patients: Retrospective follow up study of 137 patients surviving hospitalisation for TGA atrial switch procedure (Mustard or Senning) in a single institution and divided into two groups (simple and complex) depending on presurgical anatomy. Several surg...
Objectives The purpose of the study was to examine long-term outcome after traditional surgical treatment of corrected transposition of the great arteries to provide a basis for comparison with new procedures, such as the double-switch or Senning-Rastelli procedures. Methods Patient- and procedure-related variables in 123 patients with corrected transposition and 2 functional ventricles operated on between 1963 and 1996 were analyzed. Patients with intracardiac procedures underwent either a trad...
Background— Assessment of systemic right ventricular (RV) function is a key point in the follow-up of patients with transposition of the great arteries (TGA). Current echocardiographic assessment of RV function is at best an estimate, and cardiac magnetic resonance (CMR) is considered the gold standard. However, this technique is expensive, has limited availability, and requires significant expertise to acquire and interpret the images. The myocardial performance index (MPI) has recently been st...
Last. Seshadri Balaji(OHSU: Oregon Health & Science University)H-Index: 27
view all 10 authors...
Objectives The goal of this research was to identify predictors for sudden death (SD) in patients with transposition of the great arteries (TGA) who have undergone atrial inflow repair. Background Sudden death is the most common cause of late death after atrial inflow repair of TGA. Little is known about the predictors of SD. Methods This was a retrospective, multicenter, case-controlled study. We identified 47 patients after Mustard's or Senning's operation who experienced an SD event (34 SD, 1...
#1Antoine Guédès(MHI: Montreal Heart Institute)H-Index: 4
#1Antoine Guedes(MHI: Montreal Heart Institute)H-Index: 8
Last. Annie Dore(MHI: Montreal Heart Institute)H-Index: 27
view all 6 authors...
Objectives We sought to determine the impact of pregnancy on the systemic right ventricle (RV) after a Mustard operation for transposition of the great arteries. Background Dysfunction of the RV remains a continuing problem after a Mustard operation. Concerns exist about the potentially deleterious effects of pregnancy on this ventricle. Methods The records of 16 women who completed 28 pregnancies were reviewed for clinical status, echocardiographic evaluation of RV dimensions, RV function, and ...
Abstract Objectives Systemic right ventricular failure after atrial switch procedures for transposition of the great arteries has been addressed at Melbourne's Royal Children's Hospital (1981-1993) and the Cleveland Clinic Foundation (1993-2001) with reconditioning of the morphologically left ventricle by means of pulmonary artery banding followed by an arterial switch operation and an atrial reseptation. Methods Thirty-nine patients (Royal Children's Hospital, 19; Cleveland Clinic Foundation, 2...
Abstract Objectives The aim of this study was to assess the utility of tissue Doppler echocardiography in the setting of repaired transposition of the great arteries when the right ventricle (RV) functions as the systemic ventricle. Background Myocardial acceleration during isovolumic contraction, “isovolumic myocardial acceleration” (IVA), has been validated as a sensitive non-invasive method of assessing RV contractility. Although traditional indexes may be less valid for the abnormal RV, the ...
#1Michael Hauser(TUM: Technische Universität München)H-Index: 15
#2Frank M. Bengel(TUM: Technische Universität München)H-Index: 70
Last. John Hess(TUM: Technische Universität München)H-Index: 69
view all 8 authors...
Objectives: To investigate myocardial blood flow of the morphological right systemic ventricle in unoperated patients with congenitally corrected transposition of the great arteries (CCTGA) by positron emission tomography (PET). Design: Prospective cross sectional clinical study. Setting: Tertiary referral centre for paediatric cardiology. Patients: 15 patients with CCTGA were investigated by PET with nitrogen-13 ammonia at rest and during adenosine vasodilatation. A subgroup of seven patients h...
BACKGROUND Management of the congenitally corrected transposition of the great arteries (ccTGA) with intact ventricular septum (IVS) is controversial and includes either anatomic repair with systemic left ventricle (morphological left ventricle) or maintenance of a systemic right ventricle (morphological right ventricle). The purpose of this study was to describe outcomes of ccTGA-IVS with either strategy. METHODS A single-institution, retrospective review of all patients with a diagnosis of ccT...
Although arterial switch operations (ASOs) have been performed globally to repair d-transposition of the great arteries (d-TGA) in neonates and infants, few studies have been reported regarding the influence of the hemodynamics of patients with d-TGA who have undergone ASO on the development of neo-aortic valve regurgitation (AR). We aimed to investigate the relationship between the hemodynamics and development of AR after ASO in patients with d-TGA by catheter evaluation. This observational stu...
#1Anthony Azakie(UMN: University of Minnesota)H-Index: 2
#1Anthony Azakie(UMN: University of Minnesota)H-Index: 3
Last. Cindy M. Martin(UMN: University of Minnesota)H-Index: 16
view all 7 authors...
There are no reports on the performance of the arterial switch operation (ASO) in a normal heart with normally related great vessels. The objective of this study was to determine whether the ASO could be performed in a healthy animal model. Cardiopulmonary bypass (CPB) and coronary translocation techniques were used to perform ASO in neonatal piglets or a staged ASO with prior main pulmonary artery (PA) banding. Primary ASO was performed in four neonatal piglets. Coronary translocation was effec...
Objective To assess the prevalence of elevated systemic right ventricular (sRV) end-diastolic pressure and pulmonary arterial hypertension in adults with transposition of the great arteries (TGA) who have undergone atrial switch operation. Methods Forty-two adults (aged ≥18 years) with complete TGA and atrial switch palliation undergoing cardiac catheterisation between 2004 and 2018 at Mayo Clinic, MN, were identified. Clinical, echocardiographic and invasive haemodynamic data were abstracted fr...
A term baby boy was delivered overnight by emergency caesarean section for pathological cardiotocography. An uncle died in childhood due to ‘a problem with the aorta’. After initial cry, he required resuscitation including chest compressions. He was stabilised on nasal continuous positive airway pressure therapy (nCPAP). Oxygen saturations remained labile with a notable preductal and postductal gradient of 10% with brief preductal saturations of greater than 90%. Cardiovascular examination was n...
PURPOSE Dilated aortic root and ascending aorta (AAO) with progressive aortic regurgitation is a well-known sequela after arterial switch operation (ASO) in adults with transposition of the great arteries (TGA). We aimed to quantitatively assess aortic flow profiles in adults with TGA after ASO (Jatene procedure with LeCompte maneuver) using echo planar imaging (EPI) 4D flow MRI. METHODS Prospectively, 9 consecutive adults (30.2 ± 6.6 years) after ASO (Jatene operation with LeCompte technique), ...