© 2011 Published by European Association for Cardio-Thoracic Surgery
1FM, GS and PK took part in the care of the patient and contributed equally
to the medical literature search. AM had the supervision report. All authors
approved the final manuscript.
*Corresponding author. Onassis Cardiac Surgery Center, 356 Syggrou Av.,
17674 Athens, Greece. Tel.: +30-210-9493000.
E-mail address: firstname.lastname@example.org (G. Samanidis).
Interactive CardioVascular and Thoracic Surgery 13 (2011) 672-675
Case report - Congenital
Tortuous right coronary artery to coronary sinus fistula
Fotios Mitropoulosa,1, George Samanidisb,1,*, Panagiotis Kalogrisb,1, Alkiviadis Michalisb
aDepartment of Pediatric Cardiac Surgery and Congenital Heart Surgery, Onassis Cardiac Center, Athens, Greece
b2nd Department of Adult Cardiac Surgery, Onassis Cardiac Surgery Center, Athens, Greece
Received 14 July 2011; received in revised form 18 August 2011; accepted 19 August 2011
We are reporting the successful surgical treatment of a 23-year-old female with a giant right coronary artery to coronary sinus fistula. This
woman had complaints of chest pain and dyspnea on exertion for few months. Transthoracic echocardiography (TTE) showed a large tortu-
ous right coronary artery and a dilated coronary sinus. Preoperative multi-detector computed tomography (MDCT) coronary angiography and
cardiac catheterization confirmed the diagnosis of a right coronary artery to coronary sinus fistula. The patient underwent surgical closure
of the fistula and division of the communication between the right coronary artery and the coronary sinus with the use of cardiopulmonary
bypass. The patient was discharged home on postoperative day 5 and at one-year follow-up is symptom-free.
© 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.
Keywords: Coronary artery fistula; Coronary sinus; Congestive heart failure; Transthoracic echocardiography
Coronary artery fistula (CAF) is the most common coro-
nary arterial malformation but is generally a rare cardiac
anomaly. Congenital fistulous connection of the coronary
artery into a cardiac chamber or major vessel often causes
a marked dilation of the donor coronary artery leading to
Conventional angiography is the golden standard of
diagnosing this abnormality. The recent development of
electrocardiographically gated multi-detector computed
tomography (MDCT) coronary angiography has allowed
accurate and non-invasive depiction of coronary artery
diseases and malformation.
The most frequent symptoms and fistula-related complica-
tions are dyspnea on exertion, palpitations, congestive heart
failure, myocardial infarction, infective endocarditis and
death . Surgical closure of the CAF is indicated to prevent
progressive congestive heart failure, endocarditis, coronary
aneurysm formation with rupture or embolization, and when
left to right shunt (Qp/Qs) is >1:1.5 in asymptomatic patients.
We describe the case of a female who underwent success-
ful surgical treatment of a severely enlarged tortuous right
coronary artery (RCA) with a fistula draining into the coro-
nary sinus (CS).
2. Case report
A 23-year-old female patient was admitted to our hospital
with chest pain and dyspnea on exertion for the past 12
months. Her vital signs were unremarkable. Transthoracic
echocardiography (TTE) showed dilation of the proximal
and middle portion of the RCA as well as an anomalous ves-
sel-like structure around the CS. The left ventricular ejec-
tion fraction (LVEF) was 60% with no valvular abnormalities.
Cardiac catheterization showed a dilated tortuous RCA with
communication to the CS (Fig. 1a). It was unclear if there
were one or more points of communication. Further imag-
ing with MDCT coronary angiography, showed a dilated RCA
connecting to a giant CS (Fig. 1b,c).
In this case, indications for surgery included a symptom-
atic patient, large CAF characterized by the hemodynami-
cally significant shunt, significant aneurysmal formation
from RCA and a giant CS.
The surgical approach to the heart was achieved via a
bilateral submammary skin incision and median sternotomy.
The intraoperative findings were a dilated tortuous RCA
with communication to the CS. Therefore, the heart was
arrested and the distal RCA was dissected and opened lon-
gitudinally at its bifurcation and the communication points
were identified from inside (Fig. 2a,b). This was double
ligated and divided with 4/0 prolene proximal to the RCA
and distally to the CS. Cross-clamp time and cardiopulmo-
nary bypass time were 60 min and 70 min, respectively. The
postoperative course was uneventful and the patient was
discharged home in an excellent condition on postoperative
day 5. At one-year follow-up she is asymptomatic, and the
673F. Mitropoulos et al. / Interactive CardioVascular and Thoracic Surgery 13 (2011) 672-675
0.2% in the adult population referred for cardiac catheter-
ization . CAF is classified as an abnormality in the termi-
nation of a coronary artery. It can communicate with any
cardiac chamber, the RCA being the most frequent (55% of
the cases) . Fistulous drainage to the left heart occurs
in 11% and to the CS only in a minority of cases (approx.
7%) . Associated congenital cardiovascular abnormalities
have been reported in the literature in 5–30% of cases .
CAF is defined as a direct precapillary connection between
a branch of a coronary artery and the lumen of a cardiac
chamber, the CS or superior vena cava, or a pulmonary
artery or pulmonary vein close to the heart .
Clinical symptoms and age at manifestation of a congeni-
tal CAF depend on the underlying anatomy and on the size
of the fistulous connection to the left or right side of the
heart. Most patients with CAF are asymptomatic, however,
some studies have emphasized that the incidence of symp-
toms and complications increases with age, particularly
after the age of 20 [1, 7]. The longstanding left to right
shunt can produce significant volume overload, with pro-
gressive dilatation of both tricuspid and mitral valve annuli,
with consequent severe mitral valve regurgitation (MR) and
tricuspid valve regurgitation (TR). Rarely sudden death has
been attributed to these anomalies especially in the young
Fig. 2. Intraoperative view: (a) tortuous right coronary artery (black arrow).
(b) Open right coronary artery to coronary sinus fistula.
Fig. 1. Imaging findings: (a) coronary angiography (tortuous right coronary
artery). (b,c) Image of the multi-detector computed tomography coronary
repeat echo showed a patent and normal size CS, with LVEF
60%. The aesthetic result was very gratifying.
CAF was first described in 1865 by Krause . It is a rare
congenital anomaly with a reported incidence of 0.1% to
674 674F. Mitropoulos et al. / Interactive CardioVascular and Thoracic Surgery 13 (2011) 672-675
. Rupture of coronary artery aneurysms in the pericar-
dium with tamponade is a very rare but life-threatening
complication of CAF. Early surgical correction is indicated
because of the high prevalence of late symptoms and com-
plications, especially when the shunt is significant (Qp/Qs)
A markedly enlarged coronary artery can usually be
detected with TTE and transesophageal echocardiography
(TOE). Magnetic resonance imaging (MRI) and CT cardiac
coronary angiography are useful, non-invasive, and accu-
rate imaging techniques for the detection of major coro-
nary artery anomalies. Conventional coronary angiography
has been the diagnostic modality to detect CAFs, but MDCT
is now being widely applied for diagnosing cardiovascular
anomalies and the number of incidentally detected CAFs on
MDCT has been increasing.
Spontaneous closure of the fistula secondary to sponta-
neous thrombosis has been reported, although it is very
uncommon (1–2% of cases) . Current treatment options
for CAF include surgical ligation alone (either with or
without cardiopulmonary bypass), surgical ligation accom-
panied by coronary artery bypass surgery, and transcath-
eter closure. Techniques for transcatheter closure of CAF
include; the use of various types of occlusion coils, vascu-
lar plugs, umbrella devices, covered stents and histoac-
ryl resin. When surgery is contemplated it is of paramount
importance to know the exact angiographic visualization
of the anatomy of a CAF and the regular coronary ves-
sels branching off proximally and distally of the fistula. In
asymptomatic patients with high-flow shunting, closure is
indicated to prevent occurrence of symptoms or complica-
tions, especially in the pediatric population. In most cases
a median sternotomy and cardiopulmonary bypass are nec-
essary to open the cardiac chamber in order to find and
ligate the points of entry and to avoid postoperative haz-
ards like myocardial ischemia and arrhythmias. Long-term
follow-up is essential due to the possibility of postopera-
tive recanalization, persistent dilatation of the coronary
artery and ostium, thrombus formation, calcification,
arrhythmias, and myocardial infarction. Surgery for CAF is
a safe and effective procedure with a very low morbidity
and mortality rate (0–4%) .
 Liberthson RR, Sagar K, Berkoben JP, Weintraub RM, Levine FH.
Congenital coronary arteriovenous fistula. Report of 13 patients,
review of the literature and delineation of management. Circulation
 Krause W. Uber den ursprung einer accessorischen a. coronaria aus der
a. pulmonalis. Z Ratl Med 1865;24:225–227.
 Vavuranakis M, Bush CA, Boudoulas H. Coronary artery fistulas in
adults; incidence, angiographic characteristics, natural history. Cathet
Cardiovasc Diagn 1995;35:116–120.
 Wauthy P, Demanet H, Deuvaert E. Surgical treatment of coronary
artery fistula with aneurysm. Acta Chir Belg 2003;103:532–533.
 Said SA, Lam J, van der Werf T. Solitary coronary artery fistulas: a con-
genital anomaly in children and adults. Congenit Heart Dis 2006;1:63–76.
 Schumacher G, Roithmaier A, Lorenz HP, Meisner H, Sauer U, Müller KD,
Sebening F, Bühlmeyer K. Congenital coronary artery fistula in infancy
and childhood: diagnostic and therapeutic aspects. Thorac Cardiovasc
 Hong GJ, Lin CY, Lee CY, Loh SH, Yang HS, Liu KY, Tsai YT, Tsai CS.
Congenital coronary artery fistulas: clinical considerations and surgical
treatment. ANZ J Surg 2004;74:350–355.
 Eckart RE, Scoville SL, Campbell CL, Shry EA, Staiduhar KC, Potter RN,
Pearse LA, Virmani R. Sudden death in young adults: a 25-year review of
autopsies in military recruits. Ann Intern Med 2004;141:829–834.
 Zenooz NA, Habibi R, Mammen L, Finn JP, Gilkeson RC. Coronary artery
fistulas: CT findings. Radiographics 2009;29:781–789.
eComment: Surgical treatment of coronary arteriovenous fistulas
Authors: Georgios Dimitrakakis, Department of Cardiothoracic Surgery,
University Hospital of Wales, Cardiff CF144XW, UK; Ulrich Otto von Oppell
In their well written paper, Mitropoulos and associates  report on the
successful surgical management of a coronary arteriovenous fistula (CAVF).
We would like to share our experience related to two patients and to add a
The first patient had a combination of double CAVF associated with mitral
and tricuspid regurgitation, PFO and chronic AF . The patient success-
fully underwent suture ligation of CAVF, combined mitral and tricuspid
valve repair plus closure of PFO and modified Maze procedure with radio-
The second patient had multiple coronary-pulmonary fistulas with asso-
ciated aortic valve regurgitation and ASD and successfully underwent AVR,
repair of the ASD with autologous pericardial patch and obliteration of CAVF
and feeding vessels with surgical clips . We found the additional manoeu-
vre of opening the pulmonary artery to be a helpful technique: this enabled
direct identification of the entry points of all the fistulas into the pulmo-
nary artery, and their direct closure with a patch of autologous pericardium
sealed with Bioglue to avoid further recurrence of malformation .
CAVF is an uncommon entity and a major coronary anomaly according to
Ogden's classification, being present in 0.002% of the general population and
representing 0.4% of all cardiac malformations [3, 4]. CAVF of congenital
origin can arise due to persistence of sinusoidal connections between the
lumens of the primitive tubular heart that supply myocardial blood flow in
the early embryologic period .
Nomenclature is based on a descriptive analysis of the vessel origin and
the chamber of termination. According to the angiographic classification by
Sakakibara et al., there are types A and B: type A – proximal type, proximal
coronary segment dilated to the origin of the fistula, distal end normal; type
B – distal type, coronary artery dilated over entire length, terminating as a
fistula into the right side of the heart (end-artery type), proximal to fistula,
regular branching of coronary [3, 4]. The surgical implications of this classifi-
cation are: type A fistula may be treated by epicardial ligation of the fistula,
maintaining normal branch flow or, alternatively, ligation of the coronary
artery proximal and distal to the origin of fistula might be necessary. Type
B fistula requires ligation by intracameral purse-string sutures at the site of
termination with CPB .
Coronary steal phenomenon is the primary patho-physiological problem
seen in CAVF without outflow obstruction. There is general agreement that
symptomatic patients should be treated. The surgical obliteration of the fis-
tula is the cornerstone of surgical treatment, first described by Bjorck in
1947, and remains until now the most effective treatment .
Anatomy of CAVF and associated anomalies/pathologies determines which
technique should be chosen. CABG may be performed if the coronary circula-
tion has been compromised during the procedure and the entire procedure
can be done on or off-CPB [3, 5].
In addition, intraoperative TOE greatly contributes to the facilitation of
surgical treatment by identifying the origin, the draining points for the fis-
tula, the quantification of valve regurgitation and finally the efficacy of CAVF
 Mitropoulos F, Samanidis G, Kalogris P, Michalis A. Tortuous right coro-
nary artery to coronary sinus fistula. Interact CardioVasc Thorac Surg
 Dimitrakakis G, Wheeler R, Von Oppell U, Zilidis G, Masani N, Wood
A, Grooves P. Surgical management of a double coronary cameral fis-
tula associated with mitral and tricuspid regurgitation, patent fora-
men ovale, and chronic atrial fibrillation. Heart Surg Forum 2008;11:
 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
675F. Mitropoulos et al. / Interactive CardioVascular and Thoracic Surgery 13 (2011) 672-675
fistula to the coronary sinus is the direct run-off of cardioplegic solution
to the right atrium; consequently the myocardium is not protected cor-
rectly. It is advisable to administer antegrade cardioplegia with digital
compression of the fistula at the level of the coronary sinus [2, 3]. Ret-
rograde cardioplegia represents a valuable alternative in the setting of a
coronary artery fistula to the coronary sinus. Our technique would have
been to administer antegrade cardioplegia with external compression of
the coronary sinus then, after heart arrest, to perform a 2 cm longitudinal
incision on the external aspect of the coronary sinus immediately prior to
its distal extremity. This opening, which is obligatory to identify the entry
point of the fistula, also allows us to introduce a retrograde cardioplegia
 Mitropoulos F, Samanidis G, Kalogris P, Michalis A. Tortuous right coro-
nary artery to coronary sinus fistula. Interact CardioVasc Thorac Surg
 Hajj-Chahine J, Haddad F, El-Rassi I, Jebara V. Surgical manage-
ment of a circumflex aneurysm with fistula to the coronary sinus. Eur J
Cardiothorac Surg 2009;35:1086–1088.
 Chamberlain MH, Henry R, Brann S, Angelini GD. Surgical management
of a gigantic circumflex coronary artery aneurysm with fistulous connec-
tion to the coronary sinus. Eur J Cardiothorac Surg 2001;20:1255–1257.
 Dodge-Khatami A, Mavroudis C, Backer CL. Congenital heart surgery
nomenclature and database project: anomalies of the coronary arter-
ies. Ann Thorac Surg 2000;69:S270–S297.
 Mahesh B, Navaratnarajah M, Mensah K, Amrani M. Treatment of
high-output coronary artery fistula by off-pump coronary artery
bypass grafting and ligation of fistula. Interact CardioVasc Thorac Surg
eComment: Cardioplegia in coronary artery fistula to coronary sinus
Authors: Jamil Hajj-Chahine, CHU de Poitiers, 2 rue de la Milétrie, 86000
Poitiers, France; Romain Belmonte, Pierre-Yves Lefant, Jacques Tomasi
We read with great interest the article by Mitropoulos and colleagues 
concerning the successful surgical treatment of a giant right coronary artery
fistula to coronary sinus. Fistulas between coronary arteries and coronary
sinus are unusual. Volume overload due to the fistulous connection may
cause a marked dilation of the coronary artery, which ultimately leads to
However, we would like to comment on an important point regarding
the surgical management in the abovementioned case report. The authors
did not describe the technique used for myocardial protection. One of
the main drawbacks of antegrade cardioplegia in the presence of a large