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The Use of Transesophageal Echocardiography in the Management of Baffle Leaks in a Patient With Transposition of the Great Arteries

Joffe, Denise C. MD*; Krishnan, Sandeep K. MD; Eisses, Mike MD; Krieger, Eric V. MD; Jones, Thomas K. MD

doi: 10.1213/XAA.0000000000000805
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From the *Department of Anesthesiology and Pain Medicine

Cardiology Division, Department of Medicine, University of Washington, Seattle, Washington

Department of Anesthesiology and Pain Medicine, Seattle Children’s Hospital, Seattle, Washington.

Accepted for publication March 14, 2018.

Funding: None.

The authors declare no conflicts of interest.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s website.

Address correspondence to Denise C. Joffe, MD, Department of Anesthesiology and Pain Medicine, University of Washington, University of Washington, Box 356540, 1959 NE Pacific St, BB-1469, Seattle, WA 98195. Address e-mail to denise.joffe@seattlechildrens.org.

A 39-year-old woman with a history of transposition of the great arteries (TGA) presented to the adult congenital heart disease (CHD) clinic with worsening exercise tolerance and shortness of breath. Her history included a surgical balloon atrial septostomy as a newborn followed by a Mustard atrial switch procedure at the age of 6 weeks. Cardiac computed tomography showed a possible small baffle leak, so she was scheduled for catheterization for shunt and hemodynamic evaluation and possible baffle leak closure. We received consent from the patient for publication of the patient’s history, echocardiographic pictures and any relevant information.

Intraprocedural transesophageal echocardiography (TEE) assessment in the midesophageal 4-chamber view showed a dilated systemic right ventricle with mild to moderately depressed systolic function. The subpulmonary left ventricle (LV) was mildly dilated with normal systolic function. She had mild to moderate tricuspid regurgitation and trace mitral regurgitation. Using 2-dimensional imaging, 2 very large defects were visible in the baffle wall, one in the inferior part of the baffle close to the tricuspid valve in proximity to where the mid native interatrial septum would be, and the second defect was in the posterior aspect of the baffle as it traversed the roof of the left atrium (Supplemental Digital Contents 1 and 2, Videos 1 and 2, http://links.lww.com/AACR/A195 and http://links.lww.com/AACR/A196; Figure 1A). Color flow Doppler while sweeping the heart in a long-axis imaging plane (102°) showed bidirectional direction flow through the defects (Supplemental Digital Content 3, Video 3, http://links.lww.com/AACR/A197). Pulsed wave Doppler demonstrated flow predominantly from the pulmonary venous pathway to the systemic venous pathway (left-to-right shunt).

Figure 1.

Figure 1.

Figure 2.

Figure 2.

TEE was used to direct the placement of 2 septal Amplatzer occluder devices in the defects. (Note that this is an off-label use of a US Food and Drug Administration–approved device.) Postprocedure, the devices were stable and did not interfere with the function of either atrioventricular valve (Figure 2).

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DISCUSSION

TGA is one of the most common forms of cyanotic CHD. In TGA, there is a discordant connection between the ventricles and great vessels, that is, the great vessels originate from the incorrect ventricle. Systemic venous blood flows from the cavae to the right atrium, right ventricle, and aorta. Pulmonary venous blood enters the left atrium, LV, and exits the pulmonary artery. Definitive surgical therapy has evolved from an atrial switch to an arterial switch procedure performed in the first weeks of life. There are an estimated 9000 adult CHD patients in the United States with atrial switches.1–4

The Senning and Mustard atrial switch procedures were used to create a second discordant connection between the atria and ventricles to correct the physiology. A baffle is made from either pericardium (Mustard) or atrial wall (Senning) and is used to separate the atrium into 2 pathways enabling systemic and pulmonary venous blood to flow to opposite atrioventricular valves (Figure 1).

When imaging a patient with an atrial switch using TEE, the optimal views to image the baffle and pathways include the midesophageal 4-chamber view and an orthogonal (perpendicular) plane usually starting from a bicaval view and rotating leftward (counterclockwise).4 The transgastric view can be used to identify the intrahepatic inferior vena cava and systemic venous pathway.4 It is important to remember that all structures except for the great vessels are in their normal anatomic position.

Landmarks such as the liver, cavae, pulmonary veins, and atrioventricular valves are helpful to identify the pathway being evaluated (Table). Two-dimensional and Doppler techniques are used to assess the baffle for leaks and the pathways for obstruction.

Table.

Table.

In the midesophageal 4-chamber view, the pulmonary veins are detected allowing to the pulmonary venous pathway to be identified. The pathway is always posterior, closest to the probe, and as the probe is turned to the right, it can be followed to the tricuspid valve. Part of the systemic venous pathway in the left atrium, adjacent to the mitral valve is also seen in this view (Table; Supplemental Digital Contents 1 and 2, Videos 1 and 2, http://links.lww.com/AACR/A195 and http://links.lww.com/AACR/A196).

From a midesophageal bicaval view, the probe is rotated from right to left (counterclockwise) to image the entire heart (Supplemental Digital Content 3, Video 3, http://links.lww.com/AACR/A197). The anatomy appears more complex because the superior and inferior vena cava limbs of the systemic venous baffle join as they course leftward to the mitral valve. The systemic and pulmonary venous pathways will appear to intersect during rotation which gives a confusing appearance of multiple baffles and pathways. During rotation, first, the tricuspid and then the mitral valves appear and can provide landmarks to help confirm the identification of the pathway.

The structure visible in the atrium is the baffle and it should always appear intact, without defects. Flow in the pathways should be of low velocity and phasic unless obstruction is present.4

Baffle complications including stenosis and leaks require intervention in about 30% of patients.1–4 Echocardiography cannot differentiate the type of atrial switch performed but it is important to know the surgical history because systemic venous baffle obstruction is more common after the Mustard, whereas pulmonary venous baffle obstruction is more common after the Senning procedure.

Baffle leaks are less common than stenosis. It is crucial to distinguish a defect from echocardiographic dropout that can occur when the baffle orientation is parallel to the ultrasound beam. Ideally, the defect should be visible in multiple planes and seen with color flow Doppler. When inconclusive, agitated saline can be used to verify communication. The color flow and spectral Doppler pattern of a leak is similar to an atrial septal defect from a physiology and anatomic perspective. The leak usually results in a left-to-right shunt from the higher pressure pulmonary venous pathway to the lower pressure systemic venous pathway. Pulse wave Doppler will demonstrate low-velocity flow reflecting the small pressure difference between the 2 pathways. Other supportive findings include dilation of the systemic venous atrium (LA) and ventricle (LV).

Large defects result in pulmonary overcirculation and symptoms of congestive heart failure. Most small defects are well tolerated and only require closure to avoid systemic emboli if pacemaker or defibrillator leads are being inserted. Whenever possible transcatheter procedures are used to treat complications related to the baffle and have a high success rate. After device placement, the absence of a shunt, the stability of the devices, and lack of interference with atrioventricular valve function must be confirmed.

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DISCLOSURES

Name: Denise C. Joffe, MD.

Contribution: This author helped prepare the manuscript and echocardiographic images.

Name: Sandeep K. Krishnan, MD.

Contribution: This author helped prepare the manuscript and participated in the procedure.

Name: Mike Eisses, MD.

Contribution: This author helped prepare the manuscript and figures.

Name: Eric V. Krieger, MD.

Contribution: This author helped prepare the manuscript and participated in the patient’s care.

Name: Thomas K. Jones, MD.

Contribution: This author helped prepare the manuscript and participated in the procedure.

This manuscript was handled by: Nikolaos J. Skubas, MD, DSc, FACC, FASE.

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REFERENCES

1. Warnes CA. Transposition of the great arteries. Circulation. 2006;114:26992709.
2. De Pasquale G, Bonassin Tempesta F, Lopes BS, et al. High prevalence of baffle leaks in adults after atrial switch operations for transposition of the great arteries. Eur Heart J Cardiovasc Imaging. 2017;18:531535.
3. Cuypers JA, Eindhoven JA, Slager MA, et al. The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years. Eur Heart J. 2014;35:16661674.
4. Cohen MS, Eidem BW, Cetta F, et al. Multimodality imaging guidelines of patients with transposition of the great arteries: a report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr. 2016;29:571621.

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