Catheterization and Cardiovascular Diagnosis 44:431–433 (1998)

Coronary Artery-Descending Aorta Fistula as an Unusual

Collateral in a Patient With Postductal Coarctation
Attila Kardos,1,2* MD, PhD, Piotr Musialek,2 MD, and Miklós Csanády,1 MD, DSc
We report on the case of a 45-year-old man with recurrent syncope and angina with
shortness of breath on exertion. Invasive and noninvasive diagnostic methods revealed
severely stenosed bicuspid aortic valve, postductal coarctation of the aorta, and a
coronary artery-descending aorta fistula. After surgical correction of the coarctation,
ligation of the fistula, and aortic valve replacement, the patient’s symptoms resolved.
Cathet. Cardiovasc. Diagn. 44:431–433, 1998. r 1998 Wiley-Liss, Inc.

Key words: congenital heart disease; coronary anomaly; syncope; angina; coronary steal

CASE REPORT The patient underwent two-step cardiothoracic surgery.

A 45-year-old farmer with a 6-mo history of exertional
angina and dyspnea and two syncopal attacks was
referred to the Cardiology Division at Albert Szent-
Györgyi Medical University (Szeged, Hungary). He had
long-standing hypertension treated with captopril. Both
parents had suffered from hypertension and had died of
stroke.
Examination of the patient revealed lateral displace-
ment of the apex, a grade 5/6 ejection systolic murmur
radiating to the carotid arteries, and blood pressure of
170/90 mm Hg in both arms but 120/90 mm Hg in the
legs. There was no pulmonary or peripheral edema.
The ECG was consistent with left ventricular hypertro-
phy with strain. The chest X-ray showed a calcified aortic
valve with poststenotic dilatation of the aorta and moder-
ate enlargement of the left ventricle.
Transthoracic echocardiography showed a hypertro-
phied nondilated left ventricle. The aortic valve was
bicuspid and calcified, with a calculated Doppler gradient
of 100 mm Hg. The suprasternal view revealed a postste-
notic dilatation of the ascending aorta and the proximal
part of the aortic arch, whereas the distal part of the arch
could not be visualized.
The aortic arch angiogram showed a severe narrowing
of the descending aorta just below the origin of the left
subclavian artery. The pressure gradient across the coarc-
tation was 55 mm Hg. Internal mammary arteries and
some intercostal arteries were enlarged (rib notching,
First, the coarctation was explored, revealing a membrane
with an internal diameter of 1.5–2 mm. Numerous
collaterals to the descending aorta were visible. The
coronary artery fistula was identified by its course into the
pericardium and ligated. The coarctation was repaired
with an end-to-end Cooley graft. Two months later, when
the aortic valve was replaced, the remnant of the fistula
was seen lying within the pericardium. The postoperative
course was uneventful, and the patient became asymptom-
atic and returned to full normal activities.
DISCUSSION
It is estimated that congenital heart anomalies affect 8
per 1,000 infants. Although severe congenital heart
defects are evident at birth, milder defects may not be
detected until adulthood. Aortic stenosis accounts for 6%
of congenital cardiac abnormalities. Coarctation of the
aorta occurs approximately 3 times in every 10,000 births
[1], and in 27–46% it is associated with a bicuspid aortic
valve [2]. Both aortic stenosis and coarctation of the aorta
may present as angina, heart failure, syncope, or endocar-
ditis.
Coronary artery anomalies, i.e., variations in the ori-
gin, course, or distribution of the coronary arteries, are
1Division of Cardiology, Second Department of Medicine, Albert
Szent-Györgyi Medical University, Szeged, Hungary
2Department of Cardiovascular Medicine, John Radcliffe Hospi-

however, was not apparent on the chest X-ray in this
patient). Coronary angiography revealed no stenoses of
the coronary arteries but rather an elongated, tortuous
artery originating from the proximal part of the circum-
flex artery and draining anteriorly into the thoracic aorta,
just below the coarctation membrane (Figs. 1–3).
tal, Oxford, UK
*Correspondence to: Attila Kardos, M.D., Ph.D., Department of
Cardiovascular Medicine, John Radcliffe Hospital, Oxford OX3 9UD,
UK. E-mail: attila.kardos@cardiovascular-medicine.oxford.ac.uk
Received 2 December 1997; Revision accepted 28 February 1998

r 1998 Wiley-Liss, Inc.

432 Kardos et al.
Fig. 1. Left lateral projection of the left coronary artery. Arrow
indicates tortuous branch originating from the circumflex artery,
coursing backwards and upwards and draining into the descend-
ing aorta just below the coarctation membrane (during left
coronarography, a subtle runoff of contrast was visible from the
fistula into the descending aorta on the motion picture cineangi-
ography). Note the large size of the fistula.
Fig. 2. Postero-anterior projection of the left coronary artery
and the fistula.
present in 0.6–1.3% of the population [3,4]. These
anomalies may make angiographic visualization of the
coronary circulation more difficult and may increase the
risk of coronary artery trauma during cardiac surgery [5].
Certain types of coronary anomalies may cause myocar-
dial ischemia [6].
Fig. 3. Right anterior oblique projection of the left coronary
artery and the fistula.
The mechanism of this patient’s angina, in the absence
of coronary stenosis, could be explained by valvular
aortic stenosis and postductal coarctation. These abnor-
malities are known to increase the myocardial oxygen
demand (by increasing workload and left ventricle mass)
and to decrease the myocardial oxygen supply (since
elevated left ventricular diastolic pressure reduces coro-
nary perfusion) [7,8]. It is interesting that this patient
remained symptom-free for over 40 yr. Over this period,
in the absence of atherosclerotic coronary artery disease,
the myocardial blood flow supply would have been, in
spite of the fistula, sufficient to match the increased
demand of the hypertrophied myocardium. The symp-
toms developed in a relatively short time, and this could
be due to the acceleration of aortic stenosis and/or an
increase in the gradient across the coarctation membrane.
Moreover, in this particular case, coronary steal through
the coronary artery fistula could have impaired myocar-
dial perfusion, especially during exercise, when the
vasodilatation of the peripheral vascular bed increases the
diastolic gradient between the high-pressure and lower-
pressure region of the aorta (in this case, between the left
coronary artery and descending aorta).
The angiographic incidence of congenital coronary
artery fistula is 0.05–0.12% [4,9]. The frequency distribu-
tion among the congenital coronary anomalies of the
fistulas is 3.7–9.7% [4,9]. Interestingly, previous reports
refer to fistulas communicating the coronary artery with
the pulmonary artery, right or left ventricular cavity, or
left or right atrium [4,9,10]. Our patient demonstrated a
very unusual type of congenital coronary artery fistula
(between the circumflex coronary artery and descending
aorta), which to our knowledge has not been previously
 Coronary Artery-Descending Aorta Fistula in Coarctation
reported. This malformation, occurring in the presence of
aortic coarctation, could provide an atypical part of the
collateral circulation usually seen with postductal coarcta-
tion. Such a bizarre fistulous communication could con-
tribute to the coronary steal phenomenon and anginal
presentation.
REFERENCES
1. Lilly SL (ed): ‘‘Pathophysiology of Heart Disease.’’ Philadelphia
and London: Lea & Febiger, 1993. 268–269.
2. Perloff JK (ed): ‘‘The Clinical Recognition of Congenital Heart
Disease,’’ 4th ed. Philadelphia: W.B. Saunders, 1994.
3. Liberthson RR, Dinsmore R, Bharati S, Rubenstein JJ, Caulfield J,
Wheeler EO, Harthorne JW, Lev M: Aberrant coronary artery
origin from the aorta: Diagnosis and clinical significance. Circula-
tion 50:774–779, 1974.
4. Kardos A, Babai L, Rudas L, Gaál T, Horváth T, Tálosi L, Tóth K,
Sárváry L, Szász K: Epidemiology of congenital coronary artery
433
anomalies: A coronary arteriography study on a Central European
population. Cathet Cardiovasc Diagn 42:270–275, 1997.
5. Neufeld HN, Blieden LC: Coronary artery disease in children.
Prog Cardiol 4:119–124, 1975.
6. Levin DC, Fellows KE, Abrams HL: Hemodynamically significant
primary anomalies of coronary arteries. Angiographic aspects.
Circulation 58:25–31, 1978.
7. Bertrand ME, LaBlanche JM, Tilmant PY, Thieuleux FP, Delforge
MR, Carre AG: Coronary sinus blood flow at rest and during
isometric exercise in patients with aortic valve disease. Mecha-
nism of angina pectoris in presence of normal coronary arteries.
Am J Cardiol 47:199–205, 1981.
8. Marcus ML, Dot DB, Hiratzka LF, Wright CB, Eastham CL:
Decreased coronary reserve. A mechanism for angina pectoris in
patients with aortic stenosis and normal coronary arteries. N Engl J
Med 307:1362–1366, 1982.
9. Yamanaka O, Hobbs R: Coronary artery anomalies in 126,595
patients undergoing coronary arteriography. Cathet Cardiovasc
Diagn 21:28–40, 1990.
10. Lin FC, Chang HJ, Chern MS, Wen MS, Yen SJ, Wu D: Multiplane
transesophageal echocardiography in the diagnosis of congenital
coronary artery fistula. Am Heart J 130:1236–1244, 1995.