Multimodality Imaging in Diagnosis and Management of Aorto-Atrial Fistula

Multimodality Imaging in Diagnosis and Management of Aorto-Atrial Fistula | Authorea try { document.documentElement.classList.add('js'); } catch (e) { } var _gaq = _gaq || []; _gaq.push(['_setAccount', 'G-8VDV14Y67G']); _gaq.push(['_trackPageview']); (function() { var ga = document.createElement('script'); ga.type = 'text/javascript'; ga.async = true; ga.src = ('https:' == document.location.protocol ? 'https://ssl' : 'http://www') + '.google-analytics.com/ga.js'; var s = document.getElementsByTagName('script')[0]; s.parentNode.insertBefore(ga, s); })(); Skip to main content Preprints Collections Wiley Open Research IET Open Research Ecological Society of Japan All Collections About About Authorea FAQs Contact Us Quick Search anywhere Search for preprint articles, keywords, etc. Search Search ADVANCED SEARCH SCROLL This is a preprint and has not been peer reviewed. Data may be preliminary. 2 May 2025 V1 Latest version Share on Multimodality Imaging in Diagnosis and Management of Aorto-Atrial Fistula Authors : Thomas M. Rieth 0009-0007-7958-5126 [email protected] , Hyehyun Reynolds , Byron Garn , Vasu Gooty , Jordan Awerbach , and Neda Mulla Authors Info & Affiliations https://doi.org/10.22541/au.174619650.04930302/v1 209 views 107 downloads Contents Abstract Information & Authors Metrics & Citations View Options References Figures Tables Media Share Abstract A 15-year-old female with VACTERL association and a history of truncus arteriosus repair who presented with progressive exertional dyspnea was found to have an aorto-atrial fistula. Due to difficult acoustic windows secondary to severe scoliosis, cardiac magnetic resonance imaging and transesophageal echocardiography were used to confirm a fistulous connection between the aortic root and the left atrium which was successfully closed percutaneously. This case highlights the value of multimodality imaging in diagnosing and managing complex postoperative structural complications in congenital heart disease. Multimodality Imaging in Diagnosis and Management of Aorto-Atrial Fistula Authors: Thomas M. Rieth MD a , Hyehyun Reynolds RDCS a , Byron Garn MD a,b , Vasu Gooty MD a,b , Jordan Awerbach MD a,b , Neda Mulla MD a,b Author Affiliations: Phoenix Children’s Center for Heart Care, Phoenix, Arizona University of Arizona, Phoenix, Arizona Conflict of Interest: All the authors have no conflicts of interest to disclose. No funding was secured for this case report. Contact Information: Thomas Rieth MD 1919 E Thomas Rd, Phoenix Az 85016 (651)-707-5399 [email protected] Abstract: A 15-year-old female with VACTERL association and a history of truncus arteriosus repair who presented with progressive exertional dyspnea was found to have an aorto-atrial fistula. Due to difficult acoustic windows secondary to severe scoliosis, cardiac magnetic resonance imaging and transesophageal echocardiography were used to confirm a fistulous connection between the aortic root and the left atrium which was successfully closed percutaneously. This case highlights the value of multimodality imaging in diagnosing and managing complex postoperative structural complications in congenital heart disease. Keywords: Aorto-Atrial Fistula, Truncus Arteriosus, Echocardiography, Multimodal Cardiac Imaging Introduction: An aorto-atrial fistula is a rare condition characterized by an abnormal communication between the aorta and an atrial chamber. Clinical presentation can include symptoms such as dyspnea, peripheral edema, and palpitations 3,7 . We present a rare case of a patient with a history of repaired truncus arteriosus who developed an aorto-atrial fistula, presenting with progressive shortness of breath. The fistula was successfully managed with percutaneous closure, highlighting the role of multimodality imaging and catheter-based intervention in the management of this rare complication. Case Presentation: A 15-year-old female with VACTERL, severe scoliosis, and truncus arteriosus who was repaired at one month of life with a 12 mm Contegra conduit, ventricular septal defect closure and partial closure of atrial septum presented to clinic with worsening shortness of breath. Two years prior to her presentation, she was referred for surgical replacement of her truncal valve due to severe truncal valve insufficiency. She subsequently underwent placement of a 25mm Inspiris valve in the aortic position, as well as an ascending aorta replacement and an upsizing of her right ventricle to pulmonary artery conduit to 24 mm homograft. Since her repair, she has complained of worsening shortness of breath that occurs mostly with activity. At her appointment, she was noted to have a continuous murmur heard at the left upper sternal border and her transthoracic echocardiogram (TTE) showed a competent aortic valve with no stenosis or regurgitation, no stenosis through her pulmonary homograft, and prominent continuous color jet entering the left atrium concerning for a possible fistula. Due to her history of VACTERL and severe scoliosis, her acoustic windows on TTE were poor making it difficult to precisely determine the exact origin the flow entering the left atrium. To further delineate her anatomy, a cardiac magnetic resonance imaging (cMRI) was obtained that confirmed a communication between the inferior aspect of the aortic root in the region of the non-coronary cusp to the superior wall of the left atrium. There is no evidence of leaflet or cusp distortion. She was referred to the cardiac interventional team for potential percutaneous occlusion of the defect. Figure 1: CMR, (A) Steady-state free precession (SSFP) cine single shot image and (B) phase contrast image in the sagittal plane showing a flow from the inferior aspect of the aortic root to the superior wall of the left atrium Figure 2: CMR, Phase contrast single shot image in the axial plane demonstrating that the fistula originates from the region of the non-coronary cusp of the aortic root. In the catheterization lab, a transesophageal echocardiogram (TEE) was obtained demonstrating that the fistula was in fact arising from a posteriorly rotated left coronary cusp. Furthermore, the left anterior descending coronary artery and circumflex arteries were arising from separate origins within 5 mm from the fistula. Figure 3: Two-dimensional TEE, mid esophageal long axis view (138 o ) view with color-flow Doppler showing the aorto-atrial fistula with flow from aortic root to the left atrium Figure 4: Two-dimensional TEE, mid esophageal short axis view (39 o ) without (A) and with (B) color-flow Doppler demonstrating the relationship of aorto-atrial fistula to the origins of the coronary artery. There were separate origins of the left anterior descending (LAD) artery and the circumflex (Cx) artery. Through TEE guidance, closure of the fistula was achieved successfully with a 4-6 Amplatzer Ductal Occluder II. There was no residual flow seen after placement of the device and no compression of the coronary arteries. The patient was monitored overnight and discharged the next day without any complications. Figure 5: Three-dimensional TEE, mid esophageal long axis view (115 o ) showing the 4-6 Amplatzer Ductal Occluder II device from the left atrial perspective occluding the aorto-atrial fistula. The relationship of the device to the left anterior descending (LAD) artery and the circumflex (Cx) artery is demonstrated. Discussion: Aorto-atrial fistulas are abnormal communications between the aorta and one of the atrial chambers that are most often acquired rather than congenital 5,7,8 . Etiologies include complications from cardiac surgery, infective endocarditis, trauma, aortic dissection, or erosion from prosthetic material such as an atrial septal defect closure device 8 . In patients with complex congenital heart disease, particularly those who undergo multiple surgical interventions involving the aortic root or valve, iatrogenic fistula formation is an important consideration 1,8 . Aorto-atrial fistulas more commonly involve the right atrium than the left atrium⁵,⁷. This is thought to be due to the anatomical relationship between the ascending aorta and the right atrium, which lies adjacent to the right lateral and posterior aspects of the ascending aorta, beginning at the level of the sinus of Valsalva⁵. Fistulas involving the left atrium are less common due to its more posterior position. Clinically, aorto-atrial fistulas can present with a spectrum of symptoms. Small fistulas may be asymptomatic and discovered incidentally, whereas larger defects can cause symptoms of heart failure such as dyspnea on exertion, fatigue, and peripheral edema 7 . In our patient, progressive exertional dyspnea was the primary presenting symptom, raising concern for residual or new hemodynamic abnormalities following her most recent surgical intervention. Diagnosis can be challenging particularly in patients with complex anatomy or limited imaging windows. In this case, although TTE raised suspicion for abnormal flow into the left atrium, the imaging was limited. Cardiac magnetic resonance imaging played a crucial role in defining the anatomy and confirming the presence and location of the fistula. In the catheterization lab, TEE provided detailed anatomical resolution and real-time procedural guidance. Multimodality imaging was essential to characterize the fistula, assess surrounding structures, and plan the interventional strategy. Management of aorto-atrial fistulas depends on symptom severity, fistula size, hemodynamic impact, and anatomic feasibility for closure. While surgical intervention is often the standard treatment, percutaneous approaches have emerged as a safe and effective alternative 1,8 . In this case, transcatheter closure with an Amplatzer Ductal Occluder II under TEE guidance was successful, with complete closure and no residual flow. Conclusion This case illustrates a rare aorto-left atrial fistula in a patient with complex congenital heart disease and prior aortic root surgery. The patient’s nonspecific symptoms and limited transthoracic imaging due to scoliosis highlight the diagnostic challenges associated with such structural complications. A multimodality imaging approach including transthoracic echocardiography, cardiac MRI, and transesophageal echocardiography was essential for accurate diagnosis and procedural guidance. References: 1. Abaya Ghazaleh J, Cacdac R, Lim A, Oudah B, Naderi Mohabadi A. Aorta to Right Ventricular Fistula Status Post-TAVR. J Investig Med High Impact Case Rep. 2023 Jan-Dec; 11:23247096231191875. doi: 10.1177/23247096231191875. PMID: 37560796; PMCID: PMC10413882. 2. Agrawal Y, Konda M, Kalavakunta JK. Aorto-left atrial fistula: Rare cause of acute cardiac failure in a previously healthy individual. J Saudi Heart Assoc. 2016 Oct;28(4):270-3. doi: 10.1016/j.jsha.2016.02.005. Epub 2016 Feb 26. PMID: 27688677; PMCID: PMC5034481. 3. Ananthasubramaniam K. Clinical and echocardiographic features of aorto-atrial fistulas. Cardiovasc Ultrasound. 2005 Jan 17;3:1. doi: 10.1186/1476-7120-3-1. PMID: 15655075; PMCID: PMC546197. 4. Archer TP, Mabee SW, Baker PB, Orsinelli DA, Leier CV. Aorto-left atrial fistula. A reversible cause of acute refractory heart failure. Chest. 1997 Mar;111(3):828-31. doi: 10.1378/chest.111.3.828. PMID: 9118732. 5. Fierro, Elizabeth A. OMS-II * ; Sikachi, Rutuja R. MBBS, DNB † ; Agrawal, Abhinav MD ‡ ; Verma, Isha MD § ; Ojrzanowski, Marcin MD ¶ ; Sahni, Sonu MD *‡ . Aorto-Atrial Fistulas: A Contemporary Review. Cardiology in Review 26(3):p 137-144, May/June 2018. | DOI: 10.1097/CRD.0000000000000182 6. Ghandour A, Rajiah P. Unusual fistulas and connections in the cardiovascular system: A pictorial review. World J Radiol. 2014 May 28;6(5):169-76. doi: 10.4329/wjr.v6.i5.169. PMID: 24876921; PMCID: PMC4037543. 7. Jainandunsing JS, Linnemann R, Bouma W, Natour N, Bidar E, Lorusso R, Gelsomino S, Johnson DM, Natour E. Aorto-atrial fistula formation and closure: a systematic review. J Thorac Dis. 2019 Mar;11(3):1031-1046. doi: 10.21037/jtd.2019.01.77. PMID: 31019793; PMCID: PMC6462689. 8. Jainandunsing JS, Linnemann R, Maessen J, Natour NE, Lorusso R, Gelsomino S, Johnson DM, Natour E. Aorto-atrial fistula formation and therapy. J Thorac Dis. 2019 Mar;11(3):1016-1021. doi: 10.21037/jtd.2019.02.63. PMID: 31019791; PMCID: PMC6462686. 9. Villablanca PA, Sukhal S, Maitas O, Onuegbu A, Muñoz-Peña JM, Joseph A, Requena C, Mohananey D. Aorto-right atrial fistula: Late complication of tricuspid valve infective endocarditis. World J Cardiol. 2014 Oct 26;6(10):1122-6. doi: 10.4330/wjc.v6.i10.1122. PMID: 25349657; PMCID: PMC4209439. Information & Authors Information Version history V1 Version 1 02 May 2025 Copyright This work is licensed under a Non Exclusive No Reuse License. Keywords aorto-atrial fistula echocardiography multimodal cardiac imaging truncus arteriosus Authors Affiliations Thomas M. Rieth 0009-0007-7958-5126 [email protected] Phoenix Children's Hospital View all articles by this author Hyehyun Reynolds Phoenix Children's Hospital View all articles by this author Byron Garn Phoenix Children's Hospital View all articles by this author Vasu Gooty Phoenix Children's Hospital View all articles by this author Jordan Awerbach Phoenix Children's Hospital View all articles by this author Neda Mulla Phoenix Children's Hospital View all articles by this author Metrics & Citations Metrics Article Usage 209 views 107 downloads .FvxKWukQNSOunydq8rnd { width: 100px; } Citations Download citation Thomas M. Rieth, Hyehyun Reynolds, Byron Garn, et al. Multimodality Imaging in Diagnosis and Management of Aorto-Atrial Fistula. Authorea . 02 May 2025. DOI: https://doi.org/10.22541/au.174619650.04930302/v1 If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. 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