Intracardiac Echocardiography to Guide Percutaneous Closure of Atrial Baffle Defects

Abstract: Background. Patients with complex congenital heart disease may require surgical construction of interatrial baffles to shunt blood between atria. Long-term complications of these procedures may include stenosis or leak of the baffle, typically along the suture line. There are limited data on transcatheter management and intraprocedural imaging of these anatomically complex lesions. Methods. We describe three cases of adults who each presented with baffle leaks more than 20 years after surgical construction of an atrial baffle. In each case, intracardiac echocardiography was essential for intraprocedural guidance, sizing of the defect, and successful percutaneous deployment of an Amplatzer septal occluder device to close the baffle leak. Results and Conclusions. One patient had a baffle leak along the inferior surface of the baffle suture line; the second patient had a baffle leak along the superior border with the left atrium; the third patient had a leak along the sutures of surgical shunt for an anomalous pulmonary vein. Percutaneous closure was successful in all cases, with deployment of an Amplatzer occluder device in each case. Intracardiac echocardiography may be may be useful for procedural guidance during percutaneous closure of atrial baffle defects.

J INVASIVE CARDIOL 2012;24(9):473-476

Key words: atrial baffle defects, Amplatzer occluder device

_____________________________________________________

Prior to development of the arterial switch (Jatene) procedure, atrial switch procedures were frequently performed in patients with D-transposition of the great arteries. These procedures create baffles that partition the atria and direct the pulmonary venous blood through the sub-systemic right-sided ventricle, and systemic venous blood through the mitral valve into the sub-pulmonic left-sided ventricle (Figure 1). The two most common variations of this procedure are the Mustard operation (which uses Dacron or pericardial grafts at the atrial level) and the Senning operation (which uses atrial flaps to achieve physiologic correction).^1,2 Long-term complications of these procedures may include stenosis or leak of the baffle, typically along the suture line.^3,4

Atrial baffles may also be used to correct anomalous return of the pulmonary veins, which can occur as an isolated defect or part of a spectrum of congenital heart disease.⁵ Although patients with a single anomalous pulmonary vein may initially be asymptomatic, they can eventually develop right heart failure from chronic volume overload. Since re-implantation of the anomalous pulmonary vein to the left atrium can be technically challenging, a baffle that directs the pulmonary vein blood flow across an atrial septal defect and into the left atrium can be used.

We describe three cases of adults who each presented with baffle leaks more than 20 years after surgical construction of an atrial baffle. In each case, intracardiac echocardiography (ICE) was essential for intraprocedural guidance, sizing of the defect, and successful percutaneous deployment of an Amplatzer septal occluder device to close the baffle leak.

Methods

For each patient, an 8 Fr Acuson intracardiac ultrasound catheter (Siemens) was advanced via the right femoral vein to the right atrium under fluoroscopic and intracardiac echocardiographic guidance. The intracardiac ultrasound catheter was used to guide real-time imaging of the atrial baffle defect. Doppler echocardiography was used to guide direction of shunt flow and identify any residual shunt. In each case, a sizing balloon was used to estimate the circularized diameter of the defect. Closure was then performed under direct intracardiac echocardiography guidance using an appropriately-sized Amplatzer septal occluder device.

Results

Case 1. A 22-year-old man with congenital D-transposition of the great arteries, Senning repair as a child, and atrial pacemaker implantation at 11 years of age presented with abrupt onset of substernal chest pain. An initial electrocardiogram was notable for inferior T-wave inversions, and a transthoracic echocardiogram (TTE) showed a new wall motion abnormality of the inferior left ventricle. Coronary angiography was notable for thrombus in the right posterior descending artery. The apparent embolus was treated with aspiration thrombectomy, and a follow-up transesophageal echocardiogram (TEE) revealed a baffle leak as the likely source for the paradoxical embolism, possibly from a small thrombus on the atrial lead.

The patient returned to the catheterization laboratory for percutaneous baffle repair. Intracardiac imaging localized the defect to the inferior surface of the right atrium near the coronary sinus, at the point where the baffle separated the inferior from the superior right atrial flow (Figure 2A). A 6 Fr Judkins Right 4 diagnostic catheter was then advanced across the baffle defect over a J-tipped wire. The baffle leak was in the same plane as the tricuspid valve, and a jet of tricuspid regurgitation was directed toward the baffle leak (Figure 2B). An 8 Fr, 80 cm, TorqVue delivery catheter was advanced across the baffle defect, followed by successful deployment of the left atrial limb of a 12-mm diameter Amplatzer septal occluder device. The left atrial wing was retracted to the baffle leak, and ICE imaging confirmed apposition of the midsection before deployment of the occluder (Figure 2C). Left coronary artery cineangiography with levo phase imaging confirmed adequate drainage of coronary sinus blood around the septal occluder (Figure 2D). Color Doppler and bubble contrast study showed no further shunting across the baffle defect. At 2-year follow-up, TTE with agitated saline contrast did not show any peri-device leak, and the patient continues to feel well.

Case 2. A 25-year-old man with congenital D-transposition of the great arteries and history of Mustard procedure at 2 months of age presented with junctional bradycardia, shortness of breath, and presyncope. TTE demonstrated a baffle leak with left to right shunting. A pacemaker was required to treat the bradycardia, but with the shunt present, the patient was at increased risk for systemic paradoxical embolism from any potential thrombus on a pacemaker lead. Therefore, it was decided to close the baffle leak percutaneously prior to pacemaker insertion.

An 8 Fr AccuNav intracardiac ultrasound catheter was advanced into the inferior vena cava under fluoroscopic guidance into the venous baffle (Figure 3A). Directing the intracardiac echocardiography probe upward identified a 9 mm left-to-right shunt located at the superior border of the baffle within the left atrium, near the origin of the left upper pulmonary vein (Figure 3B). The baffle hole was crossed using a 7 Fr multipurpose A2 diagnostic catheter, through which a 0.035˝ Terumo wire was passed into the left upper pulmonary vein. The catheter was advanced and the wire was exchanged for a 1.5 mm J-tipped Amplatzer wire. After removal of the diagnostic catheter, a 25 mm NMT sizing balloon was advanced and inflated (Figure 3C). The waist diameter measured approximately 9 mm, consistent with the sizing estimates on intracardiac echocardiography. The balloon was withdrawn and an 8 Fr guiding sheath was advanced through the baffle hole into the left pulmonary vein. A 10 mm Amplatzer septal occluder device was advanced and the left atrial disc was unfurled and brought against the baffle followed by unsheathing of the proximal disc. Echocardiographic and fluoroscopic views showed excellent device position (Figure 3D). Agitated saline contrast study at the conclusion of the procedure revealed no right-to-left shunting, and color Doppler showed minimal residual left-to-right shunting. The patient subsequently underwent uncomplicated implantation of a dual-chamber transvenous pacemaker.

Case 3. A 66-year-old woman with a history of chronic atrial fibrillation and polyarteritis nodosa presented with shortness of breath and symptoms of NYHA class III-IV heart failure. She had a history of an atrial septal defect that had been repaired surgically in Peru 40 years prior. At the age of 40, she had a reoperation in the United States, where it was discovered that she had an anomalous right upper pulmonary vein and an ostium secundum atrial septal defect. This was repaired with construction of a baffle to shunt the pulmonary venous blood across the atrial defect. At the time of consultation, TEE demonstrated a markedly enlarged right atrium and right ventricle, with an inferiorly located baffle defect. Because of difficulty localizing the defect, a cardiac CT was also performed. This demonstrated a peri-caval septal defect superior to the coronary sinus with an associated anomalous right upper pulmonary vein. A previously constructed baffle redirecting blood flow from this anomalous pulmonary vein across the interatrial septum had developed a large defect along the inferior border of the suture line (Figure 4A).

Based on the patient’s worsening symptoms of dyspnea, desaturation to 80% on room air, and high risk for a third cardiac surgery, the decision was made to close the baffle leak percutaneously. A right heart catheterization was performed, and the baseline Qp:Qs was measured at 1.8:1. The defect could not be visualized well with TEE, so an intracardiac echocardiography probe was advanced via the right femoral vein to the right atrium. This demonstrated a large defect along the inferior border of the interatrial septum at the site of the baffle suture line (Figure 4B). A JR4 diagnostic catheter was then advanced over a wire across the defect. Based on sizing using intracardiac echocardiography and a 30 cc sizing balloon, the defect measured approximately 20 mm. A 22 mm Amplatzer septal occluder was then advanced via a delivery sheath and was deployed across the baffle leak. Intracardiac echocardiography demonstrated excellent apposition of the closure device across the baffle leak (Figure 4C), and postprocedure oxygen saturations confirmed that the patient no longer had evidence of shunting at the level of the atria. At follow-up, the patient reported dramatically improved exercise tolerance without limitation in her daily activities.

Discussion

Atrial baffles are an important tool in the management of congenital heart disease, but many baffles develop late complications, including right ventricular failure, arrhythmias, baffle obstructions, or leaks.^6,7 Traditionally, therapy for baffle complications required surgical correction with redo-sternotomy. Re-operation for baffle leaks or obstruction has necessitated re-operation in about 5% of survivors.⁸

With advancing technologies in percutaneous intervention, procedures to correct baffle leaks with less risk and potentially increased efficacy have become increasingly feasible.^9-12 Baffle leak closures can also preserve right-sided ventricular function; the ability to perform this procedure percutaneously rather than surgically may allow earlier intervention and facilitate improved long-term outcomes. Published case series have almost exclusively utilized Amplatzer septal occluder devices, demonstrating the versatility of this device in multiple orientations along the possible failure points of the baffle suture lines. In the largest series, Daehnert et al reported 6 patients where baffle leaks occurred at the confluence of the IVC, the SVC, the posterior wall of the systemic venous atrium, and the mid-part of the systemic venous atrium.⁴ In each case, an Amplatzer device was deployed successfully without complications. Covered stents have also been employed in a few cases where the baffle leak was located along the superior vena cava and in cases where the leak existed concomitantly with a neighboring baffle obstruction.^11,13

When positioning an occluder device, it is important to confirm that device deployment has not created a new functional stenosis or impinged on other structures. Our first case demonstrates the importance of considering coronary sinus outflow when positioning an occluder device along the inferior border of an atrial baffle. In this case, intracardiac echocardiography as well as levo-phase imaging of the left coronary artery confirmed adequate outflow from the coronary sinus after device deployment. In all three cases, intracardiac echocardiography provided real-time guidance to confirm that the device was adequately seated across the baffle defect without anatomic compromise to surrounding structures.

Because atrial baffle leaks can occur in unusual orientations and in more posterior locations, TEE is superior to TTE for detection of baffle leaks.¹⁴ The majority of prior reported cases have employed a combination of fluoroscopy and TEE for intraprocedural localization of baffle leaks. In cases of small leaks or those with unusual orientation, TEE imaging may not adequately localize a leak. Previous authors have reported the possible utility of using real-time three-dimensional TEE imaging,¹⁵ and there is one other reported case of using ICE to guide percutaneous baffle leak closure.¹⁶ In our first reported case, ICE imaging provided spatial resolution for accurate sizing of the occlude device; in our third reported case, the inferior location of the baffle defect made the leak difficult to visualize on TEE, but the defect was immediately apparent with ICE imaging. Although the preprocedure CT scan was helpful in procedural planning, the ICE imaging provided real-time guidance, demonstrating the importance of multimodality imaging in repair of anatomically complex lesions. In cases with complicated anatomic defects such as these, ICE helps localize the defect, verify placement, and confirm that there are not any other small defects that may not have been appreciated on TTE or TEE.

In summary, this case series demonstrates the feasibility and utility of using ICE to guide percutaneous closure of baffle leaks. Given that there are now more adults than children with congenital heart disease, interventional cardiologists will play an increasing role in the long-term management of initial surgical repairs for congenital heart disease.

Acknowledgment.  We thank Gary Raff, MD, for reviewing the manuscript and Matt Webster for assistance with drawing the figures.

References

1. Mustard WT. Successful two-stage correction of transposition of the great vesels. Surgery. 1964;55:469-472.
2. Senning A. Surgical correction of transposition of the great vessels. Surgery. 1959;45:966-980.
3. Stark J, Silove ED, Taylor JF, Graham GR. Obstruction to systemic venous return following the Mustard operation for transposition of the great arteries. J Thorac Cardiovasc Surg. 1974;68(5):742-749.
4. Daehnert I, Hennig B, Wiener M, Rotzsch C. Interventions in leaks and obstructions of the interatrial baffle late after Mustard and Senning correction for transposition of the great arteries. Catheter Cardiovasc Interv. 2005;66(3):400-407.
5. Majdalany DS, Phillips SD, Dearani JA, Connolly HM, Warnes CA. Isolated partial anomalous pulmonary venous connections in adults: twenty-year experience. Congenit Heart Dis. 2010;5(6):537-545.
6. Wilson NJ, Clarkson PM, Barratt-Boyes BG, et al. Long-term outcome after the Mustard repair for simple transposition of the great arteries. 28-year follow-up. J Am Coll Cardiol. 1998;32(3):758-765.
7. Vetter VL, Tanner CS, Horowitz LN. Electrophysiologic consequences of the Mustard repair of d-transposition of the great arteries. J Am Coll Cardiol. 1987;10(6):1265-1273.
8. Gelatt M, Hamilton RM, McCrindle BW, et al. Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience. J Am Coll Cardiol. 1997;29(1):194-201.
9. Apostolopoulou SC, Papagiannis J, Hausdorf G, Rammos S. Transcatheter occlusion of atrial baffle leak after mustard repair. Catheter Cardiovasc Interv. 2000;51(3):305-307.
10. Balzer DT, Johnson M, Sharkey AM, Kort H. Transcatheter occlusion of baffle leaks following atrial switch procedures for transposition of the great vessels (d-TGV). Catheter Cardiovasc Interv. 2004;61(2):259-263.
11. Sharaf E, Waight DJ, Hijazi ZM. Simultaneous transcatheter occlusion of two atrial baffle leaks and stent implantation for SVC obstruction in a patient after Mustard repair. Catheter Cardiovasc Interv. 2001;54(1):72-76.
12. Schneider DJ, Moore JW. 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. J Invasive Cardiol. 2001;13(4):306-309.
13. Dragulescu A, Sidibe N, Aubert F, Fraisse A. Successful use of covered stent to treat superior systemic baffle obstruction and leak after atrial switch procedure. Pediatr Cardiol. 2008;29(5):954-956.
14. Kaulitz R, Stumper OF, Geuskens R, et al. Comparative values of the precordial and transesophageal approaches in the echocardiographic evaluation of atrial baffle function after an atrial correction procedure. J Am Coll Cardiol. 1990;16(3):686-694.
15. Klein AJ, Kim MS, Salcedo E, Fagan T, Kay J. The missing leak: a case report of a baffle-leak closure using real-time 3D transoesophageal guidance. Eur J Echocardiogr. 2009;10(3):464-467.
16. Kuppahally SS, Litwin SE, Green LS, Ishihara SM, Freedman RA, Michaels AD. Utility of intracardiac echocardiography for atrial baffle leak closure in repaired transposition of the great arteries. Echocardiography. 2010;27(8):E90-E93

_____________________________________________________

From the ¹Division of Cardiovascular Medicine, University of California, Davis Medical Center Sacramento, California, ²Division of Pediatric Cardiology, University of California, Davis Medical Center Sacramento, California, ³Division of Cardiology, David Grant Medical Center, Travis Air Force Base, California.
Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein.
Manuscript submitted January 25, 2012, provisional acceptance given February 14, 2012, final version accepted February 27, 2012.
Address for correspondence: Jason H. Rogers, MD, 4860 Y Street, Suite 2820, Sacramento, CA 95817. Email: jason.rogers@ucdmc.ucdavis.edu