Multimodality Imaging and Percutaneous Closure of a Large Left Main Coronary Artery to Superior Vena Cava Fistula

J INVASIVE CARDIOL 2018;30(2):E16-E17.

Key words: wall-motion abnormality, cardiac imaging

Congenital coronary artery fistulas have been reported in 0.1%-0.25% of patients undergoing coronary angiography.^1,2 Fistulas arising from the left main coronary artery (LMCA) are rare. The majority drain into the right side of the heart and a few drain into the left side of the heart. Draining into the superior vena cava (SVC) is extremely rare, at 1%.² Most of the patients are asymptomatic, but can present with shortness of breath, angina, and myocardial infarction due to coronary steal phenomenon. Endocarditis, spontaneous dissection, or rupture can also occur. Fistulas can be managed medically if they are smaller in size without a significant shunt. Percutaneous closure (using coils or vascular plugs) or surgical ligation are the definitive treatment options in patients with large shunts where pulmonary to systemic flow ratio is >1.5 or who have documented ischemia or congestive heart failure.^2,3

A 59-year-female was admitted with nausea, vomiting, shortness of breath, and chest discomfort. She was diagnosed with diverticulitis and partial small-bowel obstruction. Echocardiography showed multiple wall-motion abnormalities, with left ventricular ejection fraction of 15%-20%. Coronary angiography showed the patient had patent coronary arteries and a large fistula from LMCA to SVC (Figure 1C, Video 1). By oximetry, the shunt ratio was 1.0. Thereafter, three-dimensional cardiac computed tomographic images were performed to evaluate the fistula and its course (Figures 1A, 1B). Once her symptoms resolved, repeat echo showed ejection fraction had improved to 50%. However, she continued to have inferolateral and apical wall-motion abnormalities by echo. The patient also continued to have chest discomfort with mild to moderate exertion. She was brought back electively and her fistula was closed percutaneously (Figure 1D, Video 2) using two 7 mm Amplatzer Vascular Plugs (St. Jude Medical). Follow-up echo revealed 60% ejection fraction and no wall-motion abnormality, with resolution of symptoms.

Acknowledgment. The authors would like to thank Roma Mehta for helping us prepare the images for this article.

View Video Series here.

References

1.    Vavuranakis M, Bush CA, Boudoulas H. Coronary artery fistulas in adults: incidence, angiographic characteristics, natural history. Catheter Cardiovasc Diagn. 1995;35:116-120.

 2.    Said SAM, van der Werf T. Dutch survey of coronary artery fistulas in adults: congenital solitary fistulas. Int J Cardiol. 2006;106:323-332.

 3.    Latson LA. Coronary artery fistulas: how to manage them. Catheter Cardiovasc Interv. 2007;70:111-118.

From ¹the Carle Foundation Hospital, University of Illinois at Urbana-Champaign, Urbana, Illinois; and ²The University of Chicago Medicine, Chicago, Illinois.

Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. Dr Shah reports consultant/proctor income from St. Jude Medical/Abbott Vascular. Dr Mehta reports no conflicts of interest regarding the content herein.

Manuscript accepted May 22, 2017. 

Address for correspondence: Sanjay S. Mehta, MD, FACC, FSCAI, Carle Foundation Hospital, 611 W. Park, Urbana, IL 61801. Email: Sanjay.Mehta@Carle.com