Transcatheter treatment of IVC channel obstruction and baffle leak after Mustard procedure for d-transposition of the great arteries using Amplatzer ASD device and multiple stents

A patient with d-transposition of the great arteries who underwent the Mustard operation at one year of age developed intermittent symptomatic cyanosis as a young adult. Evaluation demonstrated a large baffle leak with bidirectional flow and stenosis of the intra-atrial IVC baffle channel. Initially, a single stent was placed to relieve the obstruction, followed by placement of an Amplatzer septal occluder device which assumed suboptimal position after release. Placement of additional stents securely repositioned the ASD device into excellent position, resulting in complete occlusion of the baffle leak and no residual obstruction in the IVC channel.     

Catheter therapy for Fontan baffle obstruction and leak,using an endovascular covered stent

A 6½-year-old (weight 20 kg) patient was found to have significant cavo-atrial obstruction and significant right-to-left shunt via multiple large fenestrations in the baffle, with oxygen saturation of 81% 3½ yr after a fenestrated Fontan operation. In one session, this patient had undergone placement of a Palmaz stent at the cavo-atrial narrowing in order to increase anterograde venous return into the pulmonary artery. This was unsuccessful in improving the oxygen saturation because of the large leak in the baffle. Therefore, at a second catheterization session, two telescoping dacron-covered experimental vascular self-expanding stents were placed from the right atrial origin (distal to the hepatic veins) into the pulmonary artery connection delineated by the Palmaz stent, with complete reconstruction of the baffle and improvement in oxygen saturation to 95%. Cathet. Cardiovasc. Diagn. 45:158–161, 1998. © 1998 Wiley-Liss, Inc.     

Simultaneous transcatheter occlusion of two atrial baffle leaks and stent implantation for SVC obstruction in a patient after Mustard repair.

A 19.5-year-old patient after Mustard operation was found to have baffle leaks and obstruction. This patient underwent successful device closure of the leaks using the Amplatzer device and stent implantation with complete resolution of the symptoms. Cathet Cardiovasc Intervent 2001;54:72-76.     

Effect of transcatheter closure of baffle leaks following senning or mustard atrial redirection surgery on oxygen saturations and polycythaemia

The aim of this study was to describe the clinical importance and methods of transcatheter closure of systemic venous baffle leaks after atrial redirection procedures for transposed great vessels. Until the late 1970s, atrial redirection surgery was the principal surgical palliative approach to manage transposed great vessels. Baffle leaks are among the many long-term complications of this type of surgery, and their prevalence increases over time. The clinical consequences of baffle leaks in this population are poorly understood, and the indications for closure are incompletely defined. During outpatient follow-up of 126 patients after atrial redirection surgery, 15 baffle leaks were detected in 11 patients. All underwent transcatheter closure using either an occluding device or a covered stent if there was concomitant baffle obstruction. The average age at the time of the procedure was 26 years (range 6 to 42). Ten of 11 patients were cyanosed at rest or on a simple walk test (median oxygen saturation level 80%, range 65% to 96%). Six of 11 patients were polycythemic before leak closure (median hemoglobin concentration 19 g/dl, range 13.8 to 23). After closure, there was a significant improvement in saturation (median 97%, p <0.0001) and a significant reduction in hemoglobin concentration at 6 months after the procedure (median 14.8 g/dl, p <0.05). There were no procedural adverse events. One patient experienced late device embolization necessitating surgical removal. In conclusion, transcatheter closure of baffle leaks is a technically feasible although frequently complex and lengthy procedure. Closure is associated with an improvement in oxygen saturations and a reduction in polycythaemia.     

Self expandable stents for relief of venous baffle obstruction after the Mustard operation

Objective—Obstruction of the venous pathways after Mustard repair for transposition of the great arteries is associated with an increased risk of arrhythmia and sudden death. The purpose of this study was to assess the effectiveness of the largest (tracheal 22 × 40 mm) Wallstents in treating baffle obstructions.
Design—Retrospective analysis of patients with stented venous pathways.
Subjects—Eleven patients with baffle obstruction after Mustard repair for transposition of the great arteries.
Interventions—Stenoses were dilated with an 18 or 20 mm balloon. However, recoil was noticed in 11 patients: immediately (n = 7) or on repeat angiography (n = 4). Eighteen stents were implanted (mean (SD)) 18 (3.3) years postoperatively. After dilatation a tracheal Wallstent (11.5 F) was deployed.
Main outcome measures—Relief of obstruction, haemodynamic improvement.
Results—In the inferior vena cava, 10 stents were deployed in seven baffle obstructions with an increase in diameter from 9.8 (2.4) mm to 16.5 (1.4) mm (p < 0.01) and a mean (SD) pressure gradient decrease from 5.1 (3.6) mm Hg to 1.4 (2.0) mm Hg; in the superior vena cava, eight stents were implanted increasing the diameter from 9.1 (3.7) mm to 15.6 (3.8) mm (p < 0.001) with a decrease in mean pressure gradient from 5.1 (2.7) mm Hg to 1.9 (1.5) mm Hg. No complications were experienced during implantation. No anticoagulation was prescribed. During follow up (1.7 (0.6) years; range, 0.9-2.6) no problems were noted; five patients were re-catheterised without change in measurements. There was no evidence of peal formation in any of the stents.
Conclusion—It is concluded that Wallstents are safe, easy to use, and effective in relieving baffle obstruction. Anticoagulation does not seem neccessary.

Keywords: Mustard procedure;  venous baffle obstruction;  stent     

Echocardiographic visualisation of the interatrial baffle after Mustard's operation.

Thirty children aged from 7 weeks to 14 years were examined by echocardiography after Mustard's operation for transposition of the great arteries. Discrete and persitent echoes were noted within the original left atrial cavity and contrast echocardiography was used to establish that these originated from the interatrial baffle. In the presence of caval channel obstruction, caused by malposition or shrinkage of the baffle, significant differences were seen in the echocardiographic appearances of the baffle, namely limitation of baffle motion, thickening, and multiplicity of the baffle echoes. These findings suggest that the technique may be of value in the postoperative assessment of patients with transposition of the great arteries.     

Transcatheter repair of Fontan baffle stenosis and fenestration in two patients using Cook Zenith aortic endografts

Patients with Fontan baffles for single ventricle may have cyanosis from right-to-left shunt through leaks in the baffle or due to intentionally created fenestrations. Typically this right-to-left shunt may be addressed with catheter-based occlusion devices. However, in narrowing of the Fontan baffle, placement of occluders within the Fontan baffle may additionally narrow the pathway and is therefore undesirable. We describe 2 patients with the combination of Fontan baffle stenosis and patent fenestration treated with a Zenith abdominal aortic aneurysm endograft (Cook Medical). The covered stent graft both occluded the right-to-left shunt and eliminated the baffle stenosis. Both patients have had symptomatic improvement.     

Fate of the Fontan connection: Mechanisms of stenosis and management

Background: Stenosis of the venous connections and conduits is a well‐known late complication of the Fontan procedure. Currently, data on the outcomes of percuta‐ neous intervention for the treatment of extra‐ or intracardiac conduits and lateral tunnel baffles obstruction are limited. In an attempt to better define the nature and severity of the stenosis and the results of catheter interventional management, we reviewed Fontan patients with obstructed extra‐ or intracardiac conduits and lateral tunnel baffles.
Methods: Retrospective review of all Fontan patients who had cardiac catheteriza‐ tion from January 2002 to October 2018 was performed. Hemodynamic and angio‐ graphic data that assessed extra‐ or intracardiac conduit, or lateral tunnel baffle obstruction/stenosis were evaluated.
Results: Twenty patients underwent catheter intervention because of conduit steno‐ sis, including calcified homografts, stenotic Gore‐Tex conduits and obstructed lateral tunnels. Six other patients had Fontan obstruction but were referred for surgical revi‐ sion. After stenting, there was a significant reduction in the connection gradient [2.0 mm Hg (IQR 2; 3) vs 0 mm Hg (IQR 0; 1), P < .0001]. Fontan conduit/connection di‐ ameter increased [10.5 mm (IQR 9; 12) vs 18 mm (IQR 14.9; 18); P < .0001] and New York Heart Association class [III (IQR II; III) vs I (IQR II; III); P = .03) with stent placement.
Conclusions: We demonstrated the hemodynamics and angiographic subtypes of conduit stenosis in patients after Fontan, We showed that calcified homografts, sten‐ otic Gore‐Tex conduits and lateral tunnels pathways can be safely and effectively stented to eliminate obstruction. Percutaneous stenting is associated with a decrease in connection gradients and improvement in functional capacity.     

Comparative values of the precordial and transesophageal approaches in the echocardiographic evaluation of atrial baffle function after an atrial correction procedure

Previous methods used to assess atrial baffle function after correction of transposition of the great arteries have included precordial echocardiography and cardiac catheterization. To evaluate whether single plane transesophageal echocardiography might provide additional information, its findings were correlated with information derived from both precordial echocardiography and cardiac catheterization in 15 patients (14 Mustard procedures, 1 Senning procedure) aged 4.2 to 33 years (mean 16.3). Precordial ultrasound with combined imaging, color flow mapping and pulsed Doppler ultrasound visualized the supramitral portion of the common systemic venous atrium in every case but could identify only superior limb obstruction in three of six patients, mid-baffle obstruction in zero of two and inferior limb obstruction in zero of two patients. Transesophageal studies with use of the same range of ultrasound methods demonstrated superior limb obstruction (severe in four, mild in two) in six of six patients, mid-baffle obstruction in two of two and inferior limb obstruction in two of two patients. The entire pulmonary venous atrium was equally well interrogated by either ultrasound approach, with both identifying three cases (two mild, one moderate) of mid-pulmonary venous atrium obstruction. However, individual pulmonary vein velocity profiles could only be recorded by transesophageal pulsed Doppler ultrasound. Precordial studies identified baffle leaks (1 large, 2 small) in only three patients, whereas transesophageal studies identified 11 such baffle leaks (1 large, 10 small), which were multiple in two patients. It is concluded that transesophageal echocardiography provides a more detailed and accurate assessment of atrial baffle morphology and function than is provided by either precordial ultrasound or cardiac catheterization.(ABSTRACT TRUNCATED AT 250 WORDS)     

Three-dimensional transesophageal echocardiographic demonstration of intraatrial baffle obstruction

We report an adult patient with transposition of the great arteries status post-Mustard procedure in whom three-dimensional transesophageal echocardiography demonstrated intraatrial baffle obstruction. The baffle could be visualized in both long-axis and "en face" short-axis views.     

Three-dimensional transesophageal echocardiographic demonstration of intraatrial baffle obstruction

We report an adult patient with transposition of the great arteries status post-Mustard procedure in whom three-dimensional transesophageal echocardiography demonstrated intraatrial baffle obstruction. The baffle could be visualized in both long-axis and "en face" short-axis views.     

Resolution of inferior venous baffle leak and total venous occlusion using a bare stent and the gore® excluder ® aortic extension in transposition of the great arteries after mustard procedure

We present a patient with a history of Mustard repair for transposition of the great arteries. The patient presented with complete inferior venous baffle obstruction and a large baffle leak after several years of cyanosis. Complete relief of the obstruction and exclusion of the baffle leak were accomplished with the use of a combination of bare metal stenting and the Gore^® Excluder^® aortic extender. To our knowledge, this represents the first reported use of the Gore^® Excluder^® aortic extender in the setting of inferior venous baffle leak and associated total inferior vena cava obstruction. © 2015 Wiley Periodicals, Inc.     

Protein-losing enteropathy caused by baffle obstruction after Mustard's operation

Three patients developed protein-losing enteropathy caused by intra-atrial obstruction of the systemic venous return after Mustard's operation. The enteropathy resolved in one case after reoperation and in the others after balloon dilatation of the stenosed caval pathways. Protein-losing enteropathy may occur as a complication of Mustard's operation. Balloon dilatation of the obstructed baffle is an effective alternative to reoperation.     

Protein-losing enteropathy caused by baffle obstruction after Mustard''s operation.

Three patients developed protein-losing enteropathy caused by intra-atrial obstruction of the systemic venous return after Mustard's operation. The enteropathy resolved in one case after reoperation and in the others after balloon dilatation of the stenosed caval pathways. Protein-losing enteropathy may occur as a complication of Mustard's operation. Balloon dilatation of the obstructed baffle is an effective alternative to reoperation.     

Effectiveness of transvenous pacemaker leads placed through intravascular stents in patients with congenital heart disease

Eight patients with venous obstruction secondary to Mustard baffle obstruction or previous transvenous pacemaker leads underwent intravascular stent relief of their obstructions followed by the insertion of new leads. Patients were followed from 1.3 to 6.3 years (median 3) by clinical, hemodynamic, angiographic, and intravascular ultrasound methods and pacemaker evaluations. The median stent patency was 84%, with 1 patient developing complete stent occlusion. Pacing energy thresholds and impedances remained unchanged.     

Balloon dilatation of complete obstruction of the superior vena cava after Mustard operation for transposition of great arteries.

Balloon dilatation was successfully performed in two patients with complete obstruction of the superior vena cava baffle junction after a Mustard operation for transposition of the great arteries. Evidence for complete relief of obstruction in the first patient, aged 4 years, was obtained by angiography, which showed improved calibre at the site of obstruction and improved haemodynamic pressure measurement after the balloon dilatation. In the second patient, aged 14 years, the relief was incomplete; in this patient a 3 cm long 3 mm diameter Palmaz stent was successfully implanted.     

Subaortic obstruction after the use of an intracardiac baffle to tunnel the left ventricle to the aorta.

Postoperative hemodynamic studies in five patients document subaortic obstruction after surgical repair utilizing an intracardiac baffle to establish continuity between the left ventricle and the aorta. Four of the patients had a Rastelli procedure for D-transposition of the great arteries with a ventricular septal defect and pulmonary stenosis; one patient had repair of double outlet right ventricle with a ventricular septal defect and pulmonary stenosis. The left ventricular outflow was shown to be a long narrow tunnel by angiography in four of five patients and by echocardiography in one patient. Resting aortic peak systolic pressure gradient ranged from 10 to 42 mm Hg (mean 24). The obstruction was localized to the proximal end of the left ventricule to aorta tunnel (i.e., at the site of ventricular septal defect) in five patients. One patient with a gradient of 42 mm Hg has angina and decreased exercise tolerance. Subaortic obstruction is a newly described sequelae after the Rastelli procedure for transposition or repair of double outlet right ventricle. The obstruction may be hemodynamically significant and should be searched for at postoperative cardiac catheterization.     

Echocardiographic localization of interatrial baffle after mustard operation for dextrotransposition of the great arteries

The position of the interatrial baffle was determined by echocardiography in 11 patients with complete transposition of the great arteries who had undergone the Mustard operation. The location of the interatrial baffle and the newly created systemic venous atrium and the pulmonary venous atrium behind the pulmonary arterial root was established by echocardiographic contrast studies during cardiac catheterization. Angiographic analysis indicated that the segments of the systemic and pulmonary venous atria seen behind the pulmonary arterial root in the echocardiogram were, respectively, the superior limb of the systemic venous atrium and the posterior segment of the pulmonary venous atrium. The area of mitral-pulmonary valve continuity in the echocardiogram was used as a reference point for dimensional measurements of the systemic and pulmonary venous atria. It is suggested that longitudinal measurements of these structures by echocardiogram may help evaluate their growth in size. Fine to coarse fibrillatory movements of the atrioventricular valves and, more frequently, of the mitral valve were seen in the majority of patients.     

Simultaneous transcatheter implantation of systemic and pulmonary venous baffle stents after mustard operation for d‐transposition of the great arteries

We report a case of simultaneous transcatheter systemic and pulmonary venous baffle obstruction stenting in a post operative Mustard patient with d‐transposition of the great arteries. © 2015 Wiley Periodicals, Inc.     

Echocardiographic study following corrective surgery for transposition of the great arteries using a baffle

Sixteen patients undergoing corrective surgery of transposition of the great arteries following Arcas' technique were studied by echocardiography. Mean age was 8.7 years, ranging from 2 to 25 years. The echocardiographic studies were performed between 6 months and 10 years after surgery (X: 5.6 years). Paradoxical movement of the interventricular septum, right ventricle dilatation, diastolic fluttering of the mitral valve and tricuspid incompetence are commonly recorded. Tricuspid insufficiency does not necessarily mean right ventricular disfunction in these patients. Due to the particular baffle shape, bidimensional echocardiographic recognition of superior cava vein obstruction is easy. In addition, the waves of cava veins showed a characteristic pattern with predominant diastolic wave, which is an argument against its obstruction. In conclusion, echo and Doppler studies are reliable means for an adequate follow-up of these patients.