Dario , Calogera , Davide , Bernardo , Gregory , Paola Rosa , Carmela Rita , Giuseppe , Vincenzo , and Giovanni: Coronary artery fistulas: symptoms may not correlate to size. An emblematic case and literature review.

Introduction

Fistulous communication of coronary with pulmonary arteries in the adults is a common type of coronary artery fistula (CAF)1–3. In most reported cases, the fistula usually arises from the proximal left and/or right coronary arteries via the anterior conal branches that connect to the anterior wall of the main pulmonary artery. These anteriorly located abnormal communications are usually asymptomatic and are incidentally found during an angiography of the coronary arteries in 0.2–0.3% of the exams4.

Case report

We are here presenting a case of a 73-year-old man, history of effort angina from youth, poor controlled blood pressure and family history of coronary artery disease. No history of previous significant chest wall trauma nor any invasive cardiac procedures were reported. The patient did not receive previous investigations for his angina. His medication consisted of acetilsalicilate, Ca-antagonist, angiotensin-converting-enzyme inhibitor, and digoxin for permanent atrial fibrillation.

The patient was admitted to Cardiac Surgery Unit for ascending aorta aneurysm, diagnosed accidentally. At admission the patient was asymptomatic and in good physical condition, with a blood pressure of 140/80 mmHg and a heart rate of 70 beats/min. A diastolic murmur related to aortic regurgitation was heard on cardiac auscultation. The electrocardiogram (ECG) showed atrial fibrillation at 70 bpm. Trans Thoracic Echocardiography (TTE) showed a good left ventricular global and segmental function with left ventricular ejection fraction (EF) of 60%, severe aortic regurgitation, and dilation of the aortic root (55 cm) and ascending aorta (56 cm).

Coronary angiography showed no significant coronary atherosclerotic lesions, left coronary artery ectasia (Figure 1A) and reveled a coronary artery fistula arising from proximal right coronary artery (RCA) and draining to main pulmonary artery (Figure 1 B).

According to the symptoms, despite not significant shunt entity at rest, we decided to close the fistula during ascending aorta replacement through Bentall-De Bono operation in theatre. During the isolation of the right sinus a vessel enriched from the right coronary artery to the main pulmonary artery was ligated (Figure 1C, D, E). The intraoperative course was uneventful and transesoephageal echocardiography showed no changes to the preoperative status. The patient was discharged after ten days and was asymptomatic for angina or dispnea without complications.

At one-year clinical follow-up the patient is still asymptomatic for effort angina with improved quality of life.

Discussion and literature review

Although coronary artery fistulas are infrequent, they are becoming increasingly important, as their management and treatment could prevent serious complications5. They represent the most common hemodynamically significant congenital defect of the coronary arteries, including 13-14% of angiographically found coronary artery anomalies3,4,6.

We can distinguish types of coronary artery fistulas associated with other congenital cardiac malformations (eg. pulmonary atresia) or isolated forms7,8. The acquired forms may be secondary to infectious processes, dissecting thoracic aorta or traumatic or iatrogenic mechanism9,10. The majority of these fistulas arise from the right coronary artery (52-55% of cases) or the left anterior descending coronary artery (30-35% of cases), while the circumflex coronary artery is less frequently involved (5-18% of cases)2,3,10,11. Over 90% of the fistulas drain to low pressure structures, such as right-sided chambers (right ventricle 41%, right atrium 26%), pulmonary artery (17%), superior vena cava, and coronary sinus (7%)6,11.

The clinical presentation depends on the severity of the left-to-right shunt and the majority of adult patients are usually asymptomatic10, so that their real incidence is difficult to discern but coronary steal could induce ischemia in patients without atherosclerotic coronary artery disease5.

Clinical presentations include dyspnea, angina, endocarditis, arrhythmias, stroke, myocardial ischemia or myocardial infarction. Myocardial ischemia/infarction can occur from decreased coronary blood flow distal to the fistula and has been documented in patients with coronary fistulas with no evidence of coronary atherosclerosis. They often assure a blood shunt of small magnitude which doesn’t compromise myocardial blood flow. The majority of adult patients are usually asymptomatic. Unlike adults, a smaller percentage of pediatric patients tend to be asymptomatic12,13. Nevertheless, pediatric patients are usually identified due to electrocardiographic or chest X-ray abnormalities or for evaluation of a loud, superficial continuous cardiac murmur.

A continuous cardiac murmur may be the only clinical finding6,10,14. A presumptive diagnosis can occasionally be made upon hearing an atypical systolic, diastolic, or continuous murmur. In CAF the continuous murmur tends to be crescendo-decrescendo in both systole and diastole, but louder in diastole, in contrast, most of the other continuous murmurs reach their peak intensity at the time of the second sound. The place where the continuous murmur is loudest depends on where fistula enters in the heart. With entry into the right atrium the murmur is loudest along the sternal border, with entry into pulmonary artery it is loudest near the second intercostal space to the left of sternum, and with entry into the left ventricle the murmur is loudest near the apex.

Although diagnosis of coronary artery fistulas is based on angiography, non-invasive cardiac imaging techniques, such as 2D and color Doppler echocardiography, cardio-MRI and cardiac CT may be helpful10,15,16.

Several angiographic classifications have been proposed. Coronary fistulas can be categorized into two different anatomical types: fistulas with one large channel or one or more small channels; fistulas composed of a plexiform network of vessels5,17.

Congenital coronary to pulmonary fistula are rare. Their incidence ranges from 0.3 to 0.8% of patients who undergo a coronarography and the vast majority is asymptomatic. Their natural course is to develop with time, however a few case of spontaneous closure have been described. They usually become symptomatic on the 5th or 6th decade with sudden death, myocardial ischemia, pulmonary artery systolic hypertension, heart failure, arrhythmia, rupture or endocarditis18,19.

Concerning the treatment, surgical or catheter-based closure is strongly recommended in symptomatic patients and in high flow-shunting asymptomatic patients, especially in pediatric; while there are no clear indications on treatment of asymptomatic adult patients with non-significant shunting10. When they become symptomatic, treatment is mandatory to prevent complications. The appearance of these symptoms depends on the size of the communication, the amount of blood that is drained and the resistance of the chamber into which the fistula drains. Potential complications in the presence of large left-to-right shunt are pulmonary hypertension and congestive heart failure; others include rupture or thrombosis of the fistula or associated arterial aneurysm or coronary steal phenomena19.

Figure 1:

A: Coronary angiography shows ectatic left coronary artery.

B: Coronary angiography shows coronary artery fistula (arrows) arises from RCA draining in pulmonary artery.

C, D, E: images during surgery confirm that a little coronary artery fistula (arrow) arising from RCA and draining to pulmonary trunk.

icfj.2015.4.79-g001.jpg

The guidelines of the ‘”American College of Cardiology/American Heart Association” (ACC/AHA) indicate as Class I recommendation the percutaneous or surgical closure for large fistulas regardless of symptoms, while for small or medium size only in cases of myocardial ischemia, arrhythmia, ventricular dysfunction, ventricular dilatation or endarteritis20. Until a few years ago, the surgical treatment of coronary artery fistulas was the only option21,22. Since the 90s a possible alternative to surgical closure of the fistula is percutaneous management with the use of coils that promote thrombosis (coil embolization) or stents, but only in cases with favorable anatomy (e.g. origin vessel, tortuosity, caliber of the lumen, uniqueness of fistula, drainage site, etc.)23, 24. In the last years ithas been proposed the use of the “Amplatzer Duct Occluder” and “Amplatzer Septal Occluder” (AGA Medical Corporation, Golden Valley, MN), a braided-nitinol device for transcatheter patent ductus arteriosus (PDA) closure25. Surgery is still the first choice in case of cardiac malformations but coronary angiography is needed to determine the best therapeutic choice24. Medical therapy with anti-anginal drugs may improve the clinical and symptoms. Strongly recommended are antiplatelet agents and prophylaxis for bacterial endocarditis10, 26, 27.

Conclusion

Coronary artery fistula should be considered and investigated in patients in which there are not clear causes of effort angina, especially in young patients. According to current opinion a symptomatic fistula should be closed, regardless of the size, with percutaneous coronary intervention or by surgery considering also if patient needs cardiac surgery for aortic valve replacement, as this case, or other congenital heart disease.

Statement of ethical publishing

The authors state that they abide by the statement of ethical publishing of the International Cardiovascular Forum Journal28.

Acknowledgements

The authors have nothing to declare.

Conflict of interest:

The authors declare that there is no conflict of interest.

References

1. 

Kim M.S., Jung J.I., andChun H.J Coronary to pulmonary artery fistula: morphologic features at multidetector CT. Int J Cardiovasc Imaging 2010; 26: 273–280 10.1007/s10554-010-9711-3

2. 

Zhang L.J., Zhou C.S., Wang Y et al Prevalence and types of coronary to pulmonary artery fistula in a Chinese population at dual-source CT coronary angiography. Acta Radiol Nov262013; 10.1177/0284185113512299

3. 

Matsunaga N., Hayashi K., Sakamoto I., Ogawa Y., Matsuoka Y., Imamura T et al Coronary-to-pulmonary artery shunts via the bronchial artery: analysis of cineangiographic studies. Radiology 1993; 186: 877–882

4. 

Roberts WC Major anomalies of coronary arterial origin seen in adulthood. Am Heart J 1986; 111: 941–3

5. 

Said SAM, Thiadens AAHJ, Fieren MJHC, Meijboom EJ, van der Werf T, Bennink GBWE Coronary Artery fistulas. Neth Heart J 2002; 10265–78

6. 

Levin DC, Fellows KE, Abrams HL Hemodinamically significant primary anomalies of the coronary arteries. Circulation 1978; 58: 25–34

7. 

Baltaxe HA, Wixson D The incidence of congenital anomalies of the coronary arteries in the adult population. Radiology 1977; 122: 47–52

8. 

Kirklin JW, Barratt-Boyes BG Congenital anomalies of the coronary arteries. in:. Kirklin JW, Barratt-Boyes EG (Eds.)Cardiac surgery Churchill LivingstoneNew York1993; 167–1193

9. 

Caruso A, Iarussi D, Materazzi C, Dialetto G, Covino F, Bossone E, Cotrufo M Aortic dissection with fistula to left atrium: diagnosis by transesophageal echocardiography with successful repair. J Am Soc Echocardiogr 2000; Jan13169–72

10. 

Latson LA Coronary artery fistulas: how to manage them. Catheter Cardiovasc Interv. 2007; 701110–6

11. 

Gowda RM, Vasavada BC, Khan IA Coronary artery fistulas: Clinical and therapeutic considerations. Int J Cardiol 2006; 107: 7–10

12. 

Wang NK, Hsieh LY, Shen CT, Lin YM Coronary arteriovenous fistula in pediatric patients: a 17-year institutional experience. J Formos Med Assoc 2002; 101: 177–82

13. 

Balanescu S, Sangiorgi G, Castelvecchio S, Medda M, Inglese L Coronary artery fistulas: clinical consequences and methods of closure: a literature review. Ital Heart J 2001; 2: 669–76

14. 

Kidawa M, Peruga JZ, Fory J, Krzemi ska-Pakuła M, Kasprzak JD Acute coronary syndrome or steal phenomenon - a case of right coronary to right ventricle fistula. Kardiol Pol 2009; 673287–90

15. 

Black IW, Loo CK, Allan RM Multiple coronary artery-left ventricular fistulae: clinical, angiographic, and pathologic findings. Cathet Cardiovasc Diagn 1991; 23: 133–5

16. 

Cademartiri F., Malagò R., La Grutta L., Alberghina F, Palumbo A, Maffei E, Brambilla V, Pugliese F, Runza G, Midiri M, Mollet N.R, Krestin G.P Coronary Variants and Anomalies: Methodology of Visualization with 64-Slice CT and Prevalence in 202 Consecutive Patients. Radiol Med 2007; 11281117–1131

17. 

Mangukia CV Coronary artery fistula. Ann Thorac Surg 2012 Jun; 9362084–92 10.1016/j.athoracsur.2012.01.114

18. 

Angelini P, Velasco J-A, Flamm S Coronary anomalies: incidence, pathophysiology, and clinical relevance. Circulation 2002; 105: 2449–54

19. 

Sunder KR, Balakrishnan KG, Tharakan JA, Titus T, Pillai VR, Francis B, Kumar A, Bhat A, Shankaran S Coronary artery fistula in children and adults: a review of 25 cases with long-term observations. Int J Cardiol 1997; Jan358147–53

20. 

Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, del Nido P, Fasules JW, Graham TP Jr, Hijazi ZM, Hunt SA, King ME, Landzberg MJ, Miner PD, Radford MJ, Walsh EP, Webb GD, Smith SC Jr, Jacobs AK, Adams CD, Anderson JL, Antman EM, Buller CE, Creager MA, Ettinger SM, Halperin JL, Hunt SA, Krumholz HM, Kushner FG, Lytle BW, Nishimura RA, Page RL, Riegel B, Tarkington LG, Yancy CW American College of Cardiology; ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol 2008; Dec25223e143–263 10.1016/j.jacc.2008.10.001

21. 

Lowe JE, Newland OH, Subiston DC Surgical management of congenital coronary fistula. Ann Surg 1981; 194: 373–80

22. 

Cheung D, Au W, Cheung H, Chiu C, Lee W Coronary artery fistulas: long-term results of surgical correction. Ann Thorac Surg 2001; 71: 190–5

23. 

Reidy JF, Jones OD, Tynan MJ, Baker EJ, Joseph MC Embolisation procedures in congenital heart disease. Br Heart J 1985; 54: 184–192

24. 

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: 1026–1032

25. 

Zhu XY, Zhang DZ, Han XM, Cui CS, Sheng XT, Wang QG, Cha YM, Abhiram P, Rihal CS Transcatheter closure of congenital coronary artery fistulae: immediate and long-term follow-up results. Clin Cardiol 2009; Sep329506–12 10.1002/clc.20650

26. 

Olgunturk R, Kula S, Tunaoglu FS Transcatheter closure of a rare form of coronary arteriovenous fistula (circumflex artery to coronary sinus). Int J Cardiol 2006; 1132261–263

27. 

Buccheri D, Dendramis G, Piraino D, Chirco PR, Carità P, Paleologo C, Andolina G, Assennato P, Novo S Coronary artery fistulas as a cause of angina: how to manage these patients? Cardiovasc. Revasc. Med May82015; 10.1016/j.carrev.2015.04.011

28. 

Shewan LG, Coats AJS, Henein M Requirements for ethical publishing in biomedical journals. International Cardiovascular Forum Journal 2015; 22 10.17987/icfj.v2i1.4



Copyright (c) 2015 International Cardiovascular Forum Journal

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.