Atrial Switch Procedure For Transposition Research Articles

Contemporary Management Strategies of Baffle Leaks in Adults with a Failing Systemic Right Ventricle Late after Atrial Switch: A Case Series and Literature Overview

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.

Pregnancy outcomes in women with a systemic right ventricle and transposition of the great arteries results from the ESC-EORP Registry of Pregnancy and Cardiac disease (ROPAC)

ObjectiveCardiac disease is a major cause of maternal mortality. Data regarding pregnancy outcomes in women with a systemic right ventricle (sRV) are scarce. We studied pregnancy outcomes in women with...

Pregnancy outcomes in women with a systemic right ventricle and transposition of the great arteries. Results from the ESC-EORP Registry of Pregnancy and Cardiac disease (ROPAC)

Abstract Background Cardiac disease is one of the major causes of maternal mortality. We studied pregnancy outcomes in women with a systemic right ventricle (sRV) after the atrial switch procedure for transposition of the great arteries (TGA) or congenitally corrected TGA (CCTGA). Methods The ESC-EORP Registry of Pregnancy and Cardiac Disease is an international prospective registry of pregnant women with cardiac disease. Pregnancy outcomes (maternal and fetal) in women with a sRV are described. The primary endpoint was a major cardiovascular event (MACE) defined as maternal death, supraventricular or ventricular arrhythmias requiring treatment, heart failure, aortic dissection, endocarditis, ischemic coronary event and other thromboembolic events. Results Altogether, 163 women with a sRV (TGA n=121, CCTGA n=42, mean age 28.8±4.6 years) were included. Maternal mortality did not occur. At least one MACE occurred in 26 women (heart failure in 16 (9.8%), arrhythmias (atrial 5, ventricular 6) in 11 (6.7%), and others in 4 (2.5%)). Predictors of MACE were pre-pregnancy signs of heart failure [Odds ratio (OR) 6.05, 95% CI: 1.41–25.97, p=0.02] as well as a sRV ejection fraction below 40% [OR 2.81, 95% CI: 1.18–6.69, p=0.02]. One woman experienced fetal loss, while no neonatal mortality was observed. No significant differences were found between women with CCTGA and TGA. In the subset of women who had an echocardiogram before and after pregnancy, no clear deterioration in sRV was observed. Conclusion The majority of women with a sRV tolerated pregnancy well with a favorable maternal and fetal outcome. Heart failure and arrhythmias were the most common MACE. Funding Acknowledgement Type of funding source: None

Extract‐stent‐replace for treatment of upper baffle stenosis with pacing leads after atrial switch procedures for transposition of the great arteries: An approach to avoid “jailing” the lead

Venous stenosis is a late complication of the atrial switch (Mustard/Senning) procedure seen in patients with transposition of the great arteries ( d-TGA). Many atrial switch patients require cardiac implantable electronic devices (CIEDs) which further increases the incidence of venous stenosis. Stenosis of the superior limb of the systemic venous pathway (SLSVP) in the presence of CIED leads presents a management challenge. We propose a method for navigating SLSVP stenosis in atrial switch patients with CIEDs. The pulse generator and leads were removed using standard extraction techniques. Axillary access was retained via existing leads or new access was obtained. The interventional cardiology team, via groin access, performed stent-angioplasty of the stenotic SLSVP. After stent deployment, the axillary access wire was snared from below, guided through the stent, and pulled into a long groin sheath. A sheath was then advanced over the axillary wire and into the groin sheath creating a path for passage of leads through the stent. New leads were advanced through the axillary sheath into the heart. Leads were secured using standard techniques. All patients had a history of d-TGA and prior atrial switch procedures. In each case, there was stenosis of the SLSVP in the setting of a CIED lead. There were no immediate complications and there was no restenosis on follow-up. Post-atrial switch patients with CIEDs can develop stenosis of the SLSVP. A collaboration between electrophysiology and interventional cardiology can allow for device extraction, stent-angioplasty, and lead reimplantation to avoid "jailing" the leads.

Late-Onset Pulmonary Hypertension After the Atrial Switch Procedure for Transposition of the Great Arteries

Background: Pulmonary hypertension (PH) is one of the complications that can occur after the atrial switch procedure for transposition of the great arteries (TGA). This study aimed to assess the characteristics and prognosis of late-onset PH after the atrial switch procedure using catheterization data. Methods and Results: We retrospectively identified 40 patients with TGA after the atrial switch procedure that underwent catheterization between April 2007 and March 2020. Eligible patients were divided into two groups based on PH presence (PH group, n = 13 [33%]; non-PH group, n = 27 [67%]). Adverse events were defined as cardiac death and heart failure. Of the included patients, 63% were male, the mean (± standard deviation [SD]) age was 34.3 ± 8.8 years, and 43% had Mustard operations. During the follow-up period (mean ± SD, 4.3 ± 3.5 years), adverse events were significantly more common in the PH group than in the non-PH group (hazard ratio 4.5, 95% confidence interval 0.99-23.0, logrank p = 0.032). There were five patients who underwent catheterization twice during the follow-up period. Two of the five patients who had post capillary PH had improved PH and New York Heart Association (NYHA) class due to appropriate early treatment initiation for heart failure, which included diuretics, angiotensinconverting-enzyme inhibitor/angiotensin II receptor blocker, and β-blockers. Conclusions: PH is very prevalent in adults with TGA who underwent the atrial switch procedure and is associated with an adverse prognosis. Early appropriate therapy might improve PH and NYHA class after the atrial switch procedure.

Collagen turnover biomarkers and systemic right ventricle remodeling in adults with previous atrial switch procedure for transposition of the great arteries.

BackgroundMyocardial fibrosis is a potential pathophysiological mechanism leading to systemic right ventricular (SRV) deterioration. We hypothesize that circulating levels of collagen deposition markers are elevated in patients with SRV remodeling and this elevation may have a predictive value.MethodsWe prospectively evaluated 56 patients with D-TGA after the atrial switch procedure (mean age 25.6 ± 4.8, range 18–37 years; 67% males). Serum levels of procollagen type III amino-terminal propeptide (PIIINP), collagen type I carboxy-terminal telopeptide (CITP), procollagen type I N-terminal propeptide (PINP), matrix metalloproteinase (MMP 1, MMP 9) and a tissue inhibitor of matrix metalloproteinase (TIMP 1) and N-terminal pro-brain natriuretic peptide (NT-pro-BNP) were measured and compared with healthy controls. The relationship between these serum markers, echocardiographic and cardiac magnetic resonance parameters and the outcome at a follow-up of 61 months (range, 24–85 months) was determined.ResultsCompared with the healthy control group, the study group had significantly higher levels of TIMP1, PIIINP, CITP, PINP and NT-pro-BNP (p<0.05, each). The levels of PIIINP and CITP were significantly higher among patients with an SRV mass index above the mean value. The level of PIIINP was significantly higher among patients with an SRV EDV index above the mean value. CITP was significantly elevated in SRV late gadolinium enhanced (LGE) positive patients, compared to patients without SRV LGE. MMP9 and TIMP1 predicted an adverse clinical outcome on univariate Cox proportional hazard survival analysis in addition to well proven predictors of outcome (SRV EF and NYHA).ConclusionsWe demonstrated a pattern of altered collagen turnover adversely related with the indices of SRV remodeling and an adverse clinical outcome in patients with SRV.

Transbaffle catheter ablation of atrial re-entrant tachycardia within the pulmonary venous atrium in adult patients with congenital heart disease.

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. 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. 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.

In vivo myofibre architecture in the systemic right ventricle

Heart failure in the systemic right ventricle (RV) is a common pathway in end-stage disease in patients affected with congenital heart disease. Using state-of-the-art magnetic resonance (MR) diffusion acquisition schemes, we present the first in vivo diffusion tensor imaging (DTI) data of the beating heart acquired in an adult with a systemic RV following an atrial switch procedure for transposition of the great arteries. Magnetic resonance-based DTI …

Aerobic training in adults after atrial switch procedure for transposition of the great arteries improves exercise capacity without impairing systemic right ventricular function

BackgroundExercise training safely and efficiently improves symptoms in patients with heart failure due to left ventricular dysfunction. However, studies in congenital heart disease with systemic right ventricle are scarce and results are controversial. In a randomised controlled study we investigated the effect of aerobic exercise training on exercise capacity and systemic right ventricular function in adults with d-transposition of the great arteries after atrial redirection surgery (28.2±3.0years after Mustard procedure). Methods48 patients (31 male, age 29.3±3.4years) were randomly allocated to 24weeks of structured exercise training or usual care. Primary endpoint was the change in maximum oxygen uptake (peak VO2). Secondary endpoints were systemic right ventricular diameters determined by cardiac magnetic resonance imaging (CMR). Data were analysed per intention to treat analysis. ResultsAt baseline peak VO2 was 25.5±4.7ml/kg/min in control and 24.0±5ml/kg/min in the training group (p=0.3). Training significantly improved exercise capacity (treatment effect for peak VO2 3.8ml/kg/min, 95% CI: 1.8 to 5.7; p=0.001), work load (p=0.002), maximum exercise time (p=0.002), and NYHA class (p=0.046). Systemic ventricular function and volumes determined by CMR remained unchanged. None of the patients developed signs of cardiac decompensation or arrhythmias while on exercise training. ConclusionsAerobic exercise training did not detrimentally affect systemic right ventricular function, but significantly improved exercise capacity and heart failure symptoms. Aerobic exercise training can be recommended for patients following atrial redirection surgery to improve exercise capacity and to lessen or prevent heart failure symptoms. (Clinical Trial Registration: ClinicalTrials.gov #NCT00837603)

Left-sided Pulmonary Venous Pathway Obstruction after Mustard Operation

Pulmonary venous pathway obstruction (PVPO) is a serious complication of the atrial switch procedure for transposition of the great arteries (TGA). Redistribution of pulmonary arterial blood flow may make conventional echocardiographic measures of unilateral PVPO difficult to interpret. Previous studies have demonstrated altered arterial flow patterns using phase-contrast magnetic resonance imaging (PC-MRI). The purpose of this study was to investigate arterial flow patterns in the setting of unilateral PVPO following the Mustard operation. We retrospectively reviewed the arterial flow patterns of 12 patients undergoing magnetic resonance imaging between 2006 and 2011 who had had the Mustard operation. Three patients had left PVPO and all showed >65% flow distributed to the right pulmonary artery (RPA). One had severe obstruction and was the only one to show significant continuous diastolic forward flow in the RPA or diastolic flow reversal in the left pulmonary artery. RPA/left pulmonary artery flow ratio appears to be a sensitive marker for PVPO. In the absence of pulmonary regurgitation, reversed diastolic flow in the ipsilateral and continuous in the contralateral pulmonary artery appears specific for severe obstruction. Branch pulmonary artery phase-contrast magnetic resonance imaging (PC-MRI) should be performed routinely for patients who have had an atrial switch for transposition of the great arteries.

Exercise Testing and Training in Children With Congenital Heart Disease

The primary function of the cardiopulmonary system is to provide blood flow (and oxygen) in quantities sufficient to support the metabolic needs of the body. The capacity of the cardiopulmonary system to fulfill this function is maximally stressed when an individual's metabolic rate is increased, a condition that occurs most commonly during physical activity/exercise. A number of physiological changes accompany and facilitate the accommodation of the circulatory system to the hemodynamic demands of exercise (Figure 1). In normal individuals, these changes (which during upright exercise include a tripling of the resting heart rate, a >60% reduction in systemic and pulmonary vascular resistance, and a >50% increase in stroke volume) can ultimately produce a >5-fold increase in cardiac output. The increase in cardiac output is accompanied by enhanced ventricular preload (as the ventricles move up their Starling curves to accommodate the increased workload), a doubling of systolic and mean pulmonary artery pressures (most of the increase in pulmonary artery pressures is due to the concomitant rise in left-sided filling pressures; the increase in transpulmonary pressure gradient is relatively small), and a more modest increase in systemic arterial pressures.1,–,4 Figure 1. Some of the physiological changes that accompany and facilitate the accommodation of the circulatory system to the hemodynamic demands of exercise. RAp indicates right atrial pressure; LAp, left atrial pressure; PVR, pulmonary vascular resistance; SVR, systemic vascular resistance; PAp, pulmonary artery pressure; and AOp, aortic pressure. Congenital heart disease (CHD) may, in a variety of ways and to a variable extent, adversely affect these hemodynamic adaptations. For instance, patients with a Fontan procedure lack a pulmonary ventricle. They therefore cannot increase their pulmonary blood flow and pressures normally (and consequently cannot maintain their ventricular preload and systemic blood flow) during exercise.5 Patients with tetralogy of Fallot and …

Incidence and results of reoperations following the Senning operation: 27 years of follow-up in 314 patients at a single center☆

Atrial switch procedures for transposition of the great arteries are associated with reoperations mainly for systemic ventricular dysfunction and baffle complications. This study aims at identifying the results of reoperations following the Senning operation. Records of 314 hospital survivors who had undergone the Senning operation were reviewed for details concerning cardiac reoperations. Reoperations were required in 32 patients (systemic ventricular failure n=12, baffle complications n=11, left ventricular outflow tract obstruction n=7, aortic coarctation n=2) during a mean follow-up time of 18.2+/-5.7 years. Freedom from reoperation and survival at 25 years was 88.0+/-2.1% and 90.9+/-2.3%, respectively. Among patients who underwent reoperation for systemic ventricular failure (arterial switch and Senning take-down without prior pulmonary artery banding n=2, with prior banding n=3, banding without conversion n=4, tricuspid valve repair n=3), two patients died at the time of arterial switch and Senning take-down, and two patients died 4 and 16 months after tricuspid valve repair, respectively. Mean follow-up time after the first reoperation was 7.7+/-5.9 years. Survival after reoperation for systemic ventricular failure at 30 days, 1 year, and 10 years, was 91.7+/-8.0%, 83.3+/-10.8%, and 64.8+/-14.3%, respectively. Survival after reoperation for baffle complications and left ventricular outflow tract obstruction at 10 years was 85.7+/-13.2% and 83.3+/-15.2%, respectively. Reoperations following the Senning operation are rare. Reoperations for baffle complications or left ventricular outflow tract obstruction can be performed with good results in the mid-term. However, reoperations for systemic ventricular failure are demanding, and are associated with a high operative and mid-term mortality.

The right ventricular response to high afterload: Comparison between atrial switch procedure, congenitally corrected transposition of the great arteries, and idiopathic pulmonary arterial hypertension

We aimed to define differences between systemic right ventricle (RV) in patients with atrial switch procedure for transposition of the great arteries, and congenitally corrected transposition of the great arteries (ccTGA), which remodels early on in life and the subpulmonary RV in patients with idiopathic pulmonary arterial hypertension (iPAH) which remodels later in adulthood to the effects of progressive pulmonary hypertension. Prospective echocardiographic assessment of consecutive patients with atrial switch procedure, ccTGA, and iPAH attending adult congenital heart program. Right ventricular long axis function by M-mode and tissue Doppler imaging; myocardial performance index; and total isovolumic time (t-IVT), ventricular filling time, and ejection time (ET) were studied and compared with normal left ventricle and RV. Seventy-eight patients (20 atrial switch, 18 ccTGA, 20 iPAH, and 20 normal) were studied. Right ventricular long axis function was most reduced after atrial switch procedure. Diastolic filling and dysfunction varied across the groups, with atrial switch patients having the lowest myocardial early diastolic (Em) and atrial diastolic (Am) velocities and iPAH patients with the longest t-IVT, shortest filling time and ET, and lowest Em/Am, reflecting predominantly late diastolic filling. Patients with ccTGA had better preserved global systemic RV systolic and diastolic indices. The RV develops adaptative mechanisms when faced with increased afterload, behaving more like normal left ventricle. This adaptation is closer when present from birth (ccTGA) without facing subsequent surgical insults. In iPAH, the RV adapts poorly, showing prolonged t-IVT and shortened filling and effective ETs, eventually resulting in lower stroke volume and overall poorer prognosis.

Catheter management of occluded superior baffle after atrial switch procedures for transposition of great vessels

We present successful catheter intervention in 4 patients with total occlusion of the superior baffle after an atrial switch procedure. A transseptal needle (in addition to the transhepatic route in 1 patient with known occluded femoral veins) was used to open the occluded segments and place stents. Additionally, we report the regression of multiple systemic-to-pulmonary venous fistulas that developed as a result of the occluded baffle.

MDCT of Postoperative Anatomy and Complications in Adults with Cyanotic Heart Disease

Siegel MJ, Bhalla S, Gutierrez FR, Billadello J [AQ 1] pproximately 800,000 adults in the United States have congenital heart disease, and their numbers are growing [1]. Echocardiography is the imaging examination of choice for the initial evaluation of these patients. However, this technique does not allow adequate definition of intraatrial baffles in patients who have undergone an atrial switch procedure for transposition of the great vessels or conduits in patients who have undergone a Fontan procedure for treatment of tricuspid atresia. MRI offers excellent anatomic and functional information, but it is time-consuming, is contraindicated in patients with pacemakers, and can be technically difficult in uncooperative and seriously ill patients. Electron beam and 4-MDCT have been described as examination techniques for a variety of congenital heart diseases [2, 3], but little information has been published on the use of MDCT in the evaluation of postoperative anatomy and complications. We present a pictorial review on the use of MDCT in the evaluation of adults with surgically treated cyanotic heart lesions. Recognizing the postoperative appearance of these complicated lesions is important because most adult patients with repaired cyanotic heart disease require lifelong cardiac care.

Long-term results of left ventricular reconditioning and anatomic correction for systemic right ventricular dysfunction after atrial switch procedures

ObjectivesSystemic 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. MethodsThirty-nine patients (Royal Children's Hospital, 19; Cleveland Clinic Foundation, 20) with a median age of 10.8 years (range, 13 months−24 years) entered this protocol a median of 10.3 years (range, 0.5-24 years) after an atrial switch procedure. ResultsThe median duration of pulmonary artery banding was 13 months (range, 0.5-5.4 years). Ten (28%) patients responded unfavorably to morphologically left ventricular reconditioning (5 mortalities: 4 transplantations and 1 PAB still in place). Twenty-four (83%) of the 29 patients who underwent an atrial switch operation and atrial reseptation survived. During a median follow-up period of 8.2 years (range, 1-16 years), 3 patients had cardiac-related deaths. All 18 long-term survivors are asymptomatic. At last echocardiographic evaluation, the morphologically left ventricular function was normal or mildly decreased in 16 (89%) patients, and all had normal or mildly decreased systemic right ventricular function with no or mild tricuspid regurgitation. Age greater than 12 years was associated with a greater probability of morphologically left ventricular failure and not completing the protocol (P = .02) and a higher operative mortality at anatomic correction (P = .02). ConclusionsMorphologically left ventricular reconditioning and an anatomic correction protocol should be integrated into a cardiac transplantation program when treating patients with morphologically right ventricular failure after Mustard and Senning procedures. It is an alternative to cardiac transplantation in selected patients, with good long-term results. The response to morphologically left ventricular reconditioning past adolescence is inconsistent.