Download Left Circumflex to Coronary Sinus Fistula

MARCH 2011
ISSUE 35
Left Circumflex to Coronary Sinus Fistula
Leila Khorashadi, MD; Brett W. Carter, MD; Felipe Martinez, MD; Vikram Venketash, MD; Wilfred Mamuya, MD;
Mannudeep K. Kalra, M.D; Suhny Abbara, MD
Clinical History
A 68-year-old man with a history of hypertension, hyperlipidemia,
diabetes and prior tobacco use presented with recurrent
complaints of intermittent dizziness, lightheadedness, and
syncope. A myocardial perfusion study and transthoracic cardiac
ultrasound were unremarkable, and his left ventricular ejection
fraction (LVEF) was noted to be 65%. A subsequent Holter
revealed symptomatic non-sustained ventricular tachycardia.
Cardiac catheterization demonstrated multivessel coronary
artery disease. His left circumflex artery was aneurysmal and
ectatic, and a fistulous tract extending to the coronary sinus was
identified. Coronary CTA was requested prior to bypass surgery
(CABG) for better evaluation of the coronary AV-fistula (CAF), and
its relationship with surrounding structures.
Figure 1 (A,B,C)
Findings
Cardiac CTA confirmed the presence of a CAF between a markedly
dilated and tortuous left circumflex coronary artery and the coronary
sinus. Furthermore, no additional fistulas, thrombus formation, or
other associated anatomical abnormalities were noted.
Discussion
CAF is a rare congenital anomaly accounting for less than 0.5%
of all cardiac anomalies. A CAF is defined as a direct connection
between a coronary artery with right heart chambers including
the right atrium, right ventricle, coronary sinus, superior vena
cava, or pulmonary arteries. Although a single communication is
most common, there are reports of multiple fistulas. Older patients
usually present with symptoms including congestive heart failure,
endocarditis, myocardial infarction, pulmonary hypertension, and
sudden cardiac death.
The development of symptoms attributable to a CAF, significant
aneurysm formation, multiple fistulous connections, and high flow
fistulas are among the indications for intervention. Management
of CAFs includes transcatheter embolization or surgical ligation.
Long-term follow up is recommended due to possible postoperative recanalization, persistent dilatation of the involved
artery, thrombus formation, and myocardial infarction.
Figure 2 (A,B)
Figure 1 (A,B,C): (A) MPR image along the long axis of the left circumflex
coronary artery (LCx) in the atrioventricular (AV) groove showing a fistula
between the LCx (long arrow), which is dilated and markedly tortuous and a
small distal aneurysm (*) just before it drains into the distal coronary sinus
(arrow head). (B) MPR image along the long axis of the coronary sinus
(arrowheads) a few millimeters lateral to figure 1a shows the small LCx
aneurysm (*) and its connection with the coronary sinus (arrow). (C) MPR
image orthogonal to Figures 1a and 1b showing the relationship between the
great cardiac vein (short arrow) and tortuous dilated LCx (long arrow).
Figure 2 (A,B): Left anterior oblique and inferior views of 3D VRT images
showing a tortous dilated lcx artery (long arrow) paralleling the great cardiac
vein (short arrow) in the atrioventricular groove and a small aneurysm at the
distal Left circumflex – coronary sinus fistula (*).
REFERENCES
1. Liberthson, RR et al. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of
management. Circulation 1979;59:849-854.
2. Jung, SH et al. Multiple coronary arteriovenous fistulas to the coronary sinus with an unruptured coronary sinus aneurysm and restrictive
coronary sinus opening into the right atrium. Circulation 2009;120:1138-1140.
3. Zenooz, NA et al. Coronary Artery Fistulas: CT Findings. RadioGraphics 2009; 29: 781-789.
Editors:
Suhny Abbara, MD, MGH Department of Radiology
Wilfred Mamuya, MD, PhD, MGH Division of Cardiology