Search

Despite their anatomical differences, congenitally corrected (ccTGA) and complete transposition of the great arteries (d-TGA) post-atrial switch are frequently studied together and managed similarly from a medical standpoint due to the shared systemic right ventricle (sRV). The aim was to assess differences in their underlying hemodynamics. The study is a retrospective review of 138 adults with ccTGA or d-TGA post-atrial switch undergoing cardiac catheterization at Mayo Clinic, MN between 2000 and 2021. ccTGA was categorized into isolated or complex ccTGA depending on concomitant ventricular septal defect and/or left ventricular outflow obstruction. There were 53 patients with d-TGA (91% post-Mustard procedure), 51 with complex and 34 with isolated ccTGA. Isolated ccTGA patients were older (51.8 ± 13.1 years) than those with d-TGA (37.5 ± 8.3 years) or complex ccTGA (40.8 ± 13.4 years). There were no differences in sRV or left ventricular size and function across groups. The ccTGA group more commonly had ≥ moderate tricuspid regurgitation than those with d-TGA; ≥ moderate mitral and ≥ moderate pulmonary regurgitation were most prevalent in complex ccTGA. There were no differences in sRV end-diastolic pressure (sRVEDP) or PAWP between groups. However, the ratio of PAWP:sRVEDP was higher in those with d-TGA compared to those with ccTGA. Cardiac index was higher in the d-TGA group than both groups of ccTGA patients with the latter showing higher indices of ventricular afterload. In conclusion, despite sharing a sRV, adults with d-TGA and ccTGA have substantial differences in hemodynamics and structural/valvular abnormalities. Further investigation regarding disease-specific responses to heart failure therapy in those with d-TGA and ccTGA is warranted. [ABSTRACT FROM AUTHOR]

Background Data on the incidence of arrhythmias, associated cardiac interventions, and outcome in patients with dextro‐transposition of the great arteries and atrial switch are scarce. Methods and Results In this multicenter analysis, we included adult patients with dextro‐transposition of the great arteries and atrial switch regularly followed up at 3 Swiss tertiary care hospitals. The primary outcome was a composite of left ventricular assist device, heart transplantation, and death. The secondary outcome was occurrence of ventricular tachycardia, ventricular fibrillation, or sudden cardiac death. We identified 207 patients (34% women; median age at last follow‐up, 35 years) with dextro‐transposition of the great arteries and atrial switch. Arrhythmias occurred in 97 patients (47%) at a median age of 22 years. A pacemaker or an implantable cardioverter‐defibrillator was implanted in 39 (19%) and 13 (6%) patients, respectively, and 33 (16%) patients underwent a total of 51 ablation procedures to target 60 intra‐atrial re‐entry tachycardias, 4 atrioventricular nodal re‐entry tachycardias, and 1 atrial fibrillation. The primary outcome occurred in 21 patients (10%), and the secondary outcome occurred in 18 patients (9%); both were more common in patients with concomitant ventricular septum defect than in those without (hazard ratio [HR], 3.06 [95% CI, 1.29–7.27], P=0.011; and HR, 3.62 [95% CI, 1.43–9.18], P=0.007, respectively). Conclusions In patients with dextro‐transposition of the great arteries and atrial switch reaching adulthood, arrhythmias occur in almost half of patients, and associated rhythm interventions are frequent. One‐tenth of those patients do not survive until the age of 35 years free from left ventricular assist device or heart transplantation, and the outcome is worse in patients with concomitant ventricular septum defect.

Introduction: Atrial tachycardias (AT) in patients with congenital heart disease (CHD) are significantly contributing to morbidity and mortality. Aim of this study was to evaluate the long‐term course of CHD patients requiring repeat ablation procedures (RAP) of AT. Patients and Methods: All 144 patients with CHD who had undergone ablation of AT at our center between January 2003 and October 2018 were enrolled. Patients were classified according to the complexity of CHD: complex CHD (cCHD), moderate CHD (mCHD), and simple CHD (sCHD). Results: A total of 101 RAP were performed in 64 patients. One RAP was performed in n = 40, two in n = 13, three in n = 10, and five in n = 1. Acute success rate was 82% (83/101) and was not associated with the complexity of CHD (p = 1.0). Number of procedures was lower in patients with sCHD than in patients with mCHD and cCHD (sCHD 1.3 ± 0.6, mCHD 1.8 ± 1.0, and cCHD 1.8 ± 1.1, p =.04). RAP were most frequent in patients after Fontan palliation or Atrial switch procedure (2.0 ± 1.1 [n = 41] vs. 1.6 ± 0.9 all others, p =.016) and in patients with multiple unstable AT's (2.5 ± 1.1 [n = 11] vs. 1.7 ± 1.0, p =.008). Major complications occurred in 4/101 procedures. Complete follow‐up was available in 125 patients. Since last RAP 73% of the patients were in sinus/atrial rhythm and 34/125 patients (27%) with AT recurrence did not require re‐ablation with mean follow‐up of 52 ± 40 months. Conclusions: Recurrences after ablation of AT in CHD patients were frequent. After RAP promising long‐term results could be achieved. Data encourage repetitive ablation procedures in this patient population. [ABSTRACT FROM AUTHOR]

Baffle leaks are a frequently encountered and often overlooked complication after the atrial switch procedure for transposition of the great arteries. Baffle leaks are present in up to 50% of non-selected patients, and while they initially may not cause clear symptoms, they can complicate the hemodynamic course and influence the prognosis in this complex patient group. A shunt from the pulmonary venous atrium (PVA) to the systemic venous atrium (SVA) can lead to pulmonary overflow and subpulmonary left ventricular (LV) volume overload, while a shunt from the SVA to the PVA can result in (exercise-associated) cyanosis and paradoxical embolism. We report three cases of baffle leaks in patients with systemic right ventricular (sRV) failure late after the atrial switch procedure. Two symptomatic patients who presented with exercise-associated cyanosis due to SVA to PVA shunting over the baffle leak underwent successful percutaneous baffle leak closure with a septal occluder device. One patient with overt sRV failure and signs of subpulmonary LV volume overload due to PVA to SVA shunting was managed conservatively, as baffle leak closure was expected to lead to an increase in sRV end-diastolic pressure and aggravation of sRV dysfunction. These three cases illustrate the considerations made, challenges faced, and necessity of a patient-tailored approach when addressing baffle leaks.

Despite right ventricular (RV) dysfunction being a major concern in Senning patients, long-term follow-up data is lacking. This study aimed (1) at evaluating regional (base-mid-apex) RV and left ventricular (LV) function using Colour-Doppler myocardial imaging over a 15-year follow-up period and (2) at comparing results with matched controls. For the longitudinal analysis (2004–2019), we compared systolic and diastolic function in 10 Senning patients. For the cross-sectional analysis, we compared the subaortic RV (sRV) of Senning patients with the RV and LV of matched controls and the subpulmonary LV (spLV) of Senning patients with the LV of matched controls. The longitudinal analysis of sRV function showed a significant decrease in apical peak systolic strain (−17 ± 7% vs −12 ± 4%; p = 0.025) and apical peak systolic strain rate (−1.1 ± 0.3s−1 vs −0.8 ± 0.4s−1; p = 0.012). spLV function showed a significant decrease in peak systolic velocity (mid; p = 0.013 and apex; p = 0.011) and peak systolic strain rate (mid; p = 0.048). The cross-sectional analysis revealed significant lower values for basal, mid and apical peak systolic velocity, peak systolic strain rate, peak systolic strain of the sRV of Senning patients when compared to both LV and RV of matched controls (all p < 0.05). Our study showed that systolic and diastolic sRV function did not change over a 15-year follow-up period, except in the apical region. There was a decline in spLV systolic function, which may be of clinical value. On the other hand, when compared to age- and gender-matched controls, the sRV of Senning patients exhibits significantly decreased measurements of longitudinal systolic function. [ABSTRACT FROM AUTHOR]

Repair of transposition of the great arteries usually involves an atrial switch or arterial switch operation, which can complicate physiological adaptation to the demands of pregnancy and adversely affect the fetus. We retrospectively compared outcomes of 48 completed pregnancies in 23 women with surgically corrected transposition of the great arteries (38 atrial switch/10 arterial switch operation) under joint cardiac-obstetric care in our tertiary referral clinic between 1997 and 2017. Most women delivered vaginally (85%). The pre-term delivery rate was high (atrial switch 39%; arterial switch operation 40%). Small for gestational age occurred in 56% of babies, significantly more in the atrial switch group (66%) than arterial switch operation (20%), p = 0.013. Women with surgically corrected transposition of the great arteries wishing to become pregnant are at high risk of obstetric complications, primarily pre-term delivery and small for gestational age baby. They require more careful ultrasound surveillance beyond 36 weeks' gestation and/or may benefit from early induction of labour. Trial registration : Text/Not applicable. [ABSTRACT FROM AUTHOR]

The natural history of a systemic right ventricle after an atrial switch procedure has yet to be fully characterised. We describe the case of the longest surviving patient at our institution who underwent a Mustard Baffle correction for dextro‐transposition of great arteries in childhood. Over following decades he was reviewed regularly with deteriorating systemic right ventricle function. At around 50 years of age he developed worsening heart failure on maximal medical therapy. He was subsequently assessed for cardiac transplantation which he underwent successfully at the age of 55 years. [ABSTRACT FROM AUTHOR]

Aims: Catheter ablation of atrial re-entrant tachycardia in patients after atrial switch procedure for transposition of the great arteries or with a Fontan circulation is technically challenging if the critical part of the re-entry circuit is located within the pulmonary venous atrium (PVA). We report our experience in transbaffle access (TBA) to the PVA for ablation of atrial re-entrant tachycardia focusing on technical details.Methods and Results: In eight patients, six after Mustard procedure and two with a Fontan circulation, endocardial mapping of atrial re-entrant tachycardia revealed the critical part of the re-entry circuit within the PVA. A total of 10 ablation procedures were performed. Detailed angiographic assessment of the anatomy of the systemic and pulmonary venous atria was performed prior to baffle puncture. Transbaffle access was successfully established with a standard transseptal needle in 9 of 10 procedures. No major complications occurred. At the end of the procedure and the removal of the transseptal sheath, there was no residual shunt in any patient.Conclusion: Transbaffle access to the PVA for ablation of atrial re-entrant tachycardia is feasible, less invasive than alternative approaches and can be safely applied in patients after Mustard procedure or with a Fontan circulation. However, the rigidity of prosthetic material may preclude baffle puncture at least in a subset of those patients. [ABSTRACT FROM AUTHOR]

Aims Data about the acute and long-term outcome of catheter ablation in patients with D-transposition of the great arteries (d-TGA) post-Mustard/Senning operation are scarce. Methods and results This single-centre retrospective analysis includes 26 patients (mean age 28.7 ± 6.7 years, 8 females) after Mustard (n = 15) or Senning (n = 11) operation who underwent catheter ablation for intra-atrial re-entrant tachycardia (IART) or atrioventricular nodal re-entrant tachycardia (AVNRT) from January 2004 to May 2011. The electrophysiological studies were performed using a three-dimensional mapping system (CARTO®). Remote magnetic navigation (RMN) was available since 2008. Follow-up on an outpatient basis was conducted 3, 6, and 12 months after ablation and yearly thereafter. In the 26 patients, 34 procedures were performed (one procedure n = 19, two n = 6, and three n = 1). Overall, 34 tachycardia forms (IART n = 30; AVNRT n = 4) were ablated manually (n = 25) or by RMN (n = 9). Acute success reached in 29/34 forms (85.3%). Mean fluoroscopy time (FT) was 28.2 ± 20.7 min and mean procedure duration (PD) was 290.9 ± 107.6 min. Conclusion Catheter ablation of IART or AVNRT in patients post-Mustard/Senning operation for d-TGA has a high acute success rate. The recurrence rate for IART is about 30%; however, after a second ablation, long-term results are excellent. Remote magnetic navigation seems to improve single-procedure acute and long-term success and significantly reduces FT and PD. [ABSTRACT FROM PUBLISHER]

Ventricular Pacing in Transposition of the Great Arteries. Introduction: Ventricular pacing may add additional risk for right ventricular (RV) dysfunction in patients with transposition of the great arteries (TGA) and atrial redirection. The aim of our study was to evaluate the effects of long-term nonsystemic ventricular pacing on cardiac function, dyssynchrony, and clinical performance in patients with systemic RV. Methods and Results: Forty-six adults with TGA and atrial redirection, of whom 11 were permanently paced at the nonsystemic ventricle, underwent assessment of clinical status and exercise stress testing, as well as echocardiography to assess parameters of RV function and dyssynchrony. In paced patients, median NYHA functional class was II, which was significantly higher than in nonpaced patients (median class I; P = 0.002). Maximum performance and peak oxygen consumption on exercise testing were significantly lower in paced patients when compared with nonpaced patients (100 ± 30 vs 120 ± 32 W and 22 ± 6 vs 27 ± 7 mLO2/kg/min, respectively; P < 0.05 for both). On echocardiography, RV shortening fraction (27 ± 11 vs 33 ± 10%), RV ejection fraction (39 ± 7 vs 44 ± 10%) and RV dP/dtmax (891 ± 470 vs 1,024 ± 318 mmHg/s) were significantly lower (P < 0.05 for all) in paced versus nonpaced patients. Inter- and intraventricular dyssynchrony was most pronounced in the paced group (99 ± 10 vs 25 ± 9 ms and 70 ± 29 vs 21 ± 15 ms, respectively; P < 0.001 for both). Conclusions: Long-term pacing of the nonsystemic ventricle in patients with atrial switch for TGA was associated with significantly impaired functional status, exercise capacity, and systemic ventricular function. (J Cardiovasc Electrophysiol, Vol. 23, pp. 766-770, July 2012) [ABSTRACT FROM AUTHOR]

Abstract: Background: Patients with atrioventricular concordance and ventriculoarterial discordance (DTGA) and a Mustard procedure may develop stenosis of the superior limb of the systemic venous baffle (SLSVB). The frequency of this complication in an adult cohort was evaluated. Methods: Patients >18years with DTGA and a Mustard procedure with and without a pacemaker (PM)/implantable cardioverter defibrillator (ICD) were identified through an institutional database. Subjects were included following a cardiac imaging study (computed tomography, magnetic resonance imaging, venography or cardiac catheterization) and follow-up in the PM/ICD or congenital cardiac clinics from 2001 to 2007. The primary end-point was narrowing of the SLSVB (<10mm) on cardiac imaging. Hemodynamically significant narrowing was defined by: azygous vein dilatation with retrograde flow or superior vena cava syndrome or the need for dilatation±stenting of the SLSVB. Results: Narrowing of the SLSVB was observed in 49/112 patients (70 males) age 31±6years (range 18–49) and was hemodynamically significant in 15/49. Of 29 patients with a PM (23) or ICD (6) and cardiac imaging, 17 had narrowing of the SLSVB which was hemodynamically significant in 8. Non-echocardiographic imaging had a sensitivity of 88% at detecting narrowing of the SLSVB in contrast to pulse-wave Doppler, which yielded a sensitivity of 16% (61% negative predictive value, 88% positive predictive value). Conclusions: In our adult cohort of Mustard patients, narrowing of the SLSVB had a prevalence of 44% and was more likely to be detected by non-echocardiographic imaging. Baffle patency should be evaluated before transvenous device implantation. [Copyright &y& Elsevier]

Aims: Catheter ablation of atrial re-entrant tachycardia in patients after atrial switch procedure for transposition of the great arteries or with a Fontan circulation is technically challenging if the critical part of the re-entry circuit is located within the pulmonary venous atrium (PVA). We report our experience in transbaffle access (TBA) to the PVA for ablation of atrial re-entrant tachycardia focusing on technical details., Methods and Results: In eight patients, six after Mustard procedure and two with a Fontan circulation, endocardial mapping of atrial re-entrant tachycardia revealed the critical part of the re-entry circuit within the PVA. A total of 10 ablation procedures were performed. Detailed angiographic assessment of the anatomy of the systemic and pulmonary venous atria was performed prior to baffle puncture. Transbaffle access was successfully established with a standard transseptal needle in 9 of 10 procedures. No major complications occurred. At the end of the procedure and the removal of the transseptal sheath, there was no residual shunt in any patient., Conclusion: Transbaffle access to the PVA for ablation of atrial re-entrant tachycardia is feasible, less invasive than alternative approaches and can be safely applied in patients after Mustard procedure or with a Fontan circulation. However, the rigidity of prosthetic material may preclude baffle puncture at least in a subset of those patients., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

Objectives: We sought to review our current philosophy that all primary invasive electrophysiologic (EP) studies in patients with atrial switch procedures (ASPs) should undergo hemodynamic evaluation and have interventional expertise available., Background: Patients who have undergone an ASP for dextro-transposition of the great arteries have a high incidence of both hemodynamic and EP sequelae. We present our data to support the combined assessment approach for these patients., Methods: Hemodynamic evaluation and interventions performed concurrently during a primary invasive EP procedure in patients with ASP were reviewed., Results: A total of 18 patients underwent concurrent EP invasive procedure and cardiac catheterization. The median age was 31 (14-43 years) with the majority being male (67%). Patients underwent a total of 30 concurrent primary invasive EP procedure and cardiac catheterization; 14 (47%) of the catheterization procedure were interventional. Some of the catheterization procedures required more than one intervention with total of 19 separate interventions. There were nine (47%) unexpected interventions. The majority of patients (n = 14, 77.8%) had one or more abnormal hemodynamic finding including baffle obstruction (n = 13, 72%), elevated filling pressures (n = 3, 17%), and secondary pulmonary hypertension (n = 3, 17%). Non-EP-related interventional procedures included systemic or pulmonary venous baffle stenting for significant obstruction (n = 7). EP-related interventions included transbaffle puncture for ablation of left-sided reentry circuits (n = 5), closure of previously undiagnosed baffle leaks prior to pacemaker/implantable cardioverter defibrillator (ICD) placement to prevent paradoxical embolism (n = 3), superior baffle stenting to facilitate pacemaker/ICD lead placement (n = 2), and retrieval of retained transvenous pacemaker/ICD lead (n = 2)., Conclusion: Due to the frequency of abnormal hemodynamics or interventional needs, strong consideration for routine concurrent hemodynamic assessment and availability of interventional expertise is warranted during primary invasive EP procedures in patients post ASP., (© 2014 Wiley Periodicals, Inc.)

Aims: Data about the acute and long-term outcome of catheter ablation in patients with D-transposition of the great arteries (d-TGA) post-Mustard/Senning operation are scarce., Methods and Results: This single-centre retrospective analysis includes 26 patients (mean age 28.7 ± 6.7 years, 8 females) after Mustard (n = 15) or Senning (n = 11) operation who underwent catheter ablation for intra-atrial re-entrant tachycardia (IART) or atrioventricular nodal re-entrant tachycardia (AVNRT) from January 2004 to May 2011. The electrophysiological studies were performed using a three-dimensional mapping system (CARTO). Remote magnetic navigation (RMN) was available since 2008. Follow-up on an outpatient basis was conducted 3, 6, and 12 months after ablation and yearly thereafter. In the 26 patients, 34 procedures were performed (one procedure n = 19, two n = 6, and three n = 1). Overall, 34 tachycardia forms (IART n = 30; AVNRT n = 4) were ablated manually (n = 25) or by RMN (n = 9). Acute success reached in 29/34 forms (85.3%). Mean fluoroscopy time (FT) was 28.2 ± 20.7 min and mean procedure duration (PD) was 290.9 ± 107.6 min. After a mean follow-up of 34.1 ± 24.5 months, 25/26 (96.2%) patients were free from IART or AVNRT. In the nine RMN ablations (mean follow-up 14.2 ± 5.8 months) acute and long-term success was 100%. Fluoroscopy time and PD were significantly reduced using RMN compared with manual ablation (11.9 ± 6.2 vs. 34.6 ± 20.6 min, 225.7 ± 24.1 vs. 312 ± 118.2 min, P = 0.02)., Conclusion: Catheter ablation of IART or AVNRT in patients post-Mustard/Senning operation for d-TGA has a high acute success rate. The recurrence rate for IART is about 30%; however, after a second ablation, long-term results are excellent. Remote magnetic navigation seems to improve single-procedure acute and long-term success and significantly reduces FT and PD.