Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Home Print this page Email this page
Users Online:12

 Table of Contents  
Year : 2017  |  Volume : 5  |  Issue : 3  |  Page : 130-132

A rare cause of acute coronary syndrome in a middle-aged woman: Coronary artery fistula

1 Department of Cardiology, Sree Uthram Thirunal Royal Hospital, Trivandrum, Kerala, India
2 Department of Cardiology, Amiri Hospital, Kuwait City, Kuwait
3 Department of Cardiology, Kerala Institute of Medical Sciences, Trivandrum, Kerala, India

Date of Web Publication12-Sep-2017

Correspondence Address:
Biji Soman
Meditrina Hospital, Pallikkal, Kottarakara Kollam - 691 566, Kerala
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/heartindia.heartindia_9_17

Rights and Permissions

Coronary artery fistula is a rare anomaly of the coronary artery. Majority are congenital, rarely, acquired. Large proportions of these are detected incidentally during evaluation of the coronary artery disease. Most of them remain asymptomatic and do not need any active management, for those who are symptomatic both surgical closure and transcatheter closure devices are available. We present a case of middle-aged woman with a history of hypothyroidism and pulmonary hypertension, who presented with acute coronary syndrome and her coronary angiogram revealed a fistula connecting the left anterior descending artery to pulmonary artery.

Keywords: Acute coronary syndrome, coronary angiogram, coronary artery fistula, pulmonary hypertension

How to cite this article:
Soman B, Rajesh R, Rahaman MA. A rare cause of acute coronary syndrome in a middle-aged woman: Coronary artery fistula. Heart India 2017;5:130-2

How to cite this URL:
Soman B, Rajesh R, Rahaman MA. A rare cause of acute coronary syndrome in a middle-aged woman: Coronary artery fistula. Heart India [serial online] 2017 [cited 2021 Dec 2];5:130-2. Available from: https://www.heartindia.net/text.asp?2017/5/3/130/214427

  Introduction Top

Coronary artery fistulae (CAF) are abnormal connections between the coronary arteries and either the cardiac chambers (coronary cameral fistulae) or vascular structures close to the heart (coronary artery or arteriovenous fistulae).[1] CAF are present in 0.002% of the general population and in nearly 0.25% of the patients undergoing cardiac catheterization.[2] Patients usually remain asymptomatic, when symptomatic, they present with features suggestive of myocardial ischemia, myocardial infarction, congestive heart failure, or sudden death.[3]

We present a case of fistulae between the left anterior descending (LAD) coronary artery and the pulmonary artery, found incidentally at coronary angiography.

  Case Report Top

A 57-year-old female with a history of hypothyroidism and pulmonary hypertension secondary to pulmonary embolism on regular medication, presented with complaints of sudden onset breathlessness and chest pain, associated with sweating. She was hemodynamically stable and her physical examination was unremarkable. Electrocardiogram showed sinus rhythm with T inversion in leads I, aVL, V2–V6. Serial cardiac troponin I was elevated, other investigations were unremarkable. Coronary angiogram was done, which showed the right dominant system with no flow limiting atherosclerotic lesions, but revealed a small fistula communicating from the LAD to the pulmonary artery [Figure 1], [Figure 2], [Figure 3].
Figure 1: Selective left coronary angiogram in anteroposterior caudal view showing a small fistula from the left anterior descending artery draining into pulmonary artery. Labeled with broad white arrow

Click here to view
Figure 2: Selective left coronary angiogram in left anterior oblique caudal view showing a small fistula from the left anterior descending artery draining into pulmonary artery. Labeled with broad white arrow

Click here to view
Figure 3: Selective left coronary angiogram in right anterior oblique caudal view showing a small fistula from the left anterior descending artery draining into pulmonary artery. Labeled with broad white arrow

Click here to view

In the absence of atherosclerotic flow limiting lesions in the coronaries, it was decided not to do any immediate active intervention for the fistula and to further evaluate the functional significance of the CAF. The patient was not willing for the same, hence, discharged with advice to continue her present medications including dual antiplatelets and long-term oral anticoagulants and regular outpatient follow-up. Patient and the relatives were briefed accordingly.

  Discussion Top

CAF are primarily congenital, rarely acquired, following cardiac surgery, such as valve replacement, coronary artery bypass grafting, and after myocardial biopsies in cardiac transplantation.[4],[5]

The feeding artery arises from the main coronary artery or its branches. It is more dilated when originating from the proximal segment and more tortuous when arising from the distal segment of the coronaries.[6]

Patients with CAF usually remain asymptomatic or may occasionally present with symptoms of dyspnea, fatigue and angina, depending on the site and the size of the fistula, amount of shunting, and resistance of recipient vessel.[7],[8] Coronary “steal phenomenon” is believed to be the primary mechanism for the symptoms related to myocardial ischemia and is caused by the runoff from the high-pressure coronary circulation to a low-resistance pulmonary circulation due to a diastolic pressure gradient.[6]

Complications of CAF are coronary artery dilatation, aneurysm formation, intimal ulceration, medial degeneration, intimal rupture, calcification, mural thrombosis and rupture, bacterial endocarditis, premature atherosclerosis, pulmonary hypertension, and myocardial ischemia or infarction.[6],[9]

Coronary angiography remains the gold standard for diagnosis of coronary fistulae. Noninvasive methods, such as transthoracic echocardiography with Doppler and color flow imaging, magnetic resonance imaging and contrast enhancing computed tomography, are used when the proper and complete evaluation of the anatomy, flow, and function of CAF is difficult by coronary angiography alone.[10]

The natural history of CAF is variable and there are some anecdotal reports of spontaneous closure of fistula. Symptomatic patients are treated with either surgical ligation or percutaneously, using transcatheter closure devices depending on the fistula characteristics, whereas asymptomatic patients with small CAF are managed with regular medical follow-up.[6]

Surgical closure is recommended for fistulae which are aneurysmal and tortuous. Surgical ligation and closure of fistulae was first described by Bjork and Crafoord in 1947. Its excellent long-term efficacy and safety made it the procedure of choice,[11] till percutaneous closure by vascular stents and coils was started in 1983. Percutaneous closure was associated with lower procedural risk and therefore became the preferred method of treatment.[12] The choice of the occlusion device is based on the anatomical characteristics of the fistula. Coils are favored for the small fistulae, whereas umbrella devices are preferred for larger fistulae. Distal embolisation of the occlusion device is the most common complication associated with transcatheter closure.[13]

Our patient presented with acute coronary syndromes. The presence of CAF was an incidental finding, during coronary angiography. As there was no significant narrowing of the coronaries, patient was managed medically including dual antiplatelets and long-term oral anticoagulants and advised to undergo further evaluation to assess the functional severity and significance of the fistula.

  Conclusion Top

CAF are a rare cardiac anomaly and it should always be included in the differential diagnosis, especially in symptomatic patients with low risk for developing atherosclerotic coronary artery disease. Correction is indicated for large CAF and in symptomatic patients. Small CAF should be assessed for their functional significance before a management strategy is planned.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Ata Y, Turk T, Bicer M, Yalcin M, Ata F, Yavuz S. Coronary arteriovenous fistulas in the adults: Natural history and management strategies. J Cardiothorac Surg 2009;4:62.  Back to cited text no. 1
Dodge-Khatami A, Mavroudis C, Backer CL. Congenital heart surgery nomenclature and database project: Anomalies of the coronary arteries. Ann Thorac Surg 2000;69 4 Suppl: S270-97.  Back to cited text no. 2
Kugelmass AD, Manning WJ, Piana RN, Weintraub RM, Baim DS, Grossman W. Coronary arteriovenous fistula presenting as congestive heart failure. Cathet Cardiovasc Diagn 1992;26:19-25.  Back to cited text no. 3
Early SA, Meany TB, Fenlon HM, Hurley J. Coronary artery fistula; coronary computed topography – The diagnostic modality of choice. J Cardiothorac Surg 2008;3:41.  Back to cited text no. 4
Qureshi SA. Coronary arterial fistulas. Orphanet J Rare Dis 2006;1:51.  Back to cited text no. 5
Challoumas D, Pericleous A, Dimitrakaki IA, Danelatos C, Dimitrakakis G. Coronary arteriovenous fistulae: A review. Int J Angiol 2014;23:1-10.  Back to cited text no. 6
Tirilomis T, Aleksic I, Busch T, Zenker D, Ruschewski W, Dalichau H. Congenital coronary artery fistulas in adults: Surgical treatment and outcome. Int J Cardiol 2005;98:57-9.  Back to cited text no. 7
Ibrahim MF, Sayed S, Elasfar A, Sallam A, Fadl M, Al Baradai A. Coronary fistula between the left anterior descending coronary artery and the pulmonary artery: Two case reports. J Saudi Heart Assoc 2012;24:253-6.  Back to cited text no. 8
Dimitrakakis G, Von Oppell U, Luckraz H, Groves P. Surgical repair of triple coronary-pulmonary artery fistulae with associated atrial septal defect and aortic valve regurgitation. Interact Cardiovasc Thorac Surg 2008;7:933-4.  Back to cited text no. 9
Armsby LR, Keane JF, Sherwood MC, Forbess JM, Perry SB, Lock JE. Management of coronary artery fistulae. Patient selection and results of transcatheter closure. J Am Coll Cardiol 2002;39:1026-32.  Back to cited text no. 10
Kamiya H, Yasuda T, Nagamine H, Sakakibara N, Nishida S, Kawasuji M, et al. Surgical treatment of congenital coronary artery fistulas: 27 years' experience and a review of the literature. J Card Surg 2002;17:173-7.  Back to cited text no. 11
Spektor G, Gehi AK, Love B, Sharma SK, Fuster V. A case of symptomatic coronary artery fistula. Nat Clin Pract Cardiovasc Med 2006;3:689-92.  Back to cited text no. 12
Raju MG, Goyal SK, Punnam SR, Shah DO, Smith GF, Abela GS. Coronary artery fistula: A case series with review of the literature. J Cardiol 2009;53:467-72.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded157    
    Comments [Add]    

Recommend this journal