Abstract
Coronary-cameral fistula (CCF) is an anomalous connection between a coronary artery and a cardiac chamber or major vessel, seen in about 0.8% of the cases undergoing coronary angiography. Most patients are asymptomatic and diagnosis is made incidentally during coronary angiography. We present an image case of CCF which was found incidentally during pre-liver transplantation work up.
Keywords: coronary cameral fistula, coronary angiography, Management
A 57-year-old Caucasian female with a past medical history of diabetes mellitus, chronic obstructive pulmonary disease, and hepatitis C cirrhosis planned for orthotopic liver transplantation was admitted to the hospital. An elective cardiac catheterization was done as a part of pre-transplant workup. Cardiac catheterizations revealed normal coronaries, with multiple coronary-cameral fistulas (CCF) terminating in the left ventricle arising from both coronary arteries (Figs. 1 and 2). No interventions were done because of the asymptomatic nature of these fistulas.
Fig. 1.
Multiple coronary-cameral fistulas to the left ventricle arising from the left anterior descending and left circumflex arteries with opacification of the left ventricle.
Fig. 2.
Multiple coronary-cameral fistulas to the left ventricle arising from the right coronary artery with opacification of the left ventricle.
CCF is an anomalous connection between a coronary artery and a cardiac chamber or major vessel, seen in about 0.8% of the cases undergoing coronary angiography (1). Although the exact etiology is unknown, liver disease may be contributory in our patient, as it is known to cause a variety of systemic arteriovenous malformations. Most patients are asymptomatic, and diagnosis is made incidentally during coronary angiography. The usual sites of origin are right coronary artery (55%), left coronary artery (35%), and both (5%). Depending on the site of communication, they are classified as arterioluminal (direct communication with the cardiac chambers) or arteriosinusoidal (communication via sinusoidal network rather than direct communication) (2). Common sites of termination are right ventricle (40%), right atrium (26%), or pulmonary artery (17%) (1). Termination in the left ventricle is seen in about 1% of all cases of coronary artery fistula. Bi-arterial fistulization to the left ventricle is even rare with only a few cases reported in the literature (1).
Most cases of CCF are asymptomatic, detected accidentally, and conservatively managed with serial follow up (2, 3). The indication of treatment in CCF includes hemodynamically significant fistulas with worsening right to left shunts, left or right ventricular overload, myocardial ischemia, and congestive heart failure. Although there is no consensus on the optimal strategy, a variety of interventions including surgical repair, catheter closure, and medical management have been successfully utilized. Arterio-luminal subtype can be successfully closed by surgery, whereas arterisinusoidal type is less amenable to surgery and use of beta-blockers has been described (2).
Conflict of interest and funding
The authors have not received any funding or benefits from industry or elsewhere to conduct this study.
References
- 1.Hoffman JI. Congenital anomalies of the coronary vessels and the aortic root. In: Emmanoulides GC, Riemenschneider TA, Allen HD, Gutgesell HP, editors. Heart disease in infants, children and adolescents . 5th ed. Baltimore, MD: Williams and Wilkins; 1995. p. 780. [Google Scholar]
- 2.Nagpal P, Khandelwal A, Saboo SS, Garg G, Steigner ML. Symptomatic coronary cameral fistula. Heart Views. 2015;16(2):65–7. doi: 10.4103/1995-705X.159225. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 3.Liberthson RR, Sagar KA, Berkoben JP, Weintraub RM, Levine FH. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management. Circulation. 1979;59:849–54. doi: 10.1161/01.cir.59.5.849. [DOI] [PubMed] [Google Scholar]