Coronary arterial fistulae are the most common malformations
of the coronary arterial circulation (1-9).
The
incidence of isolated fistulae is 1 : 50 000
live births.
Congenital
heart defects are associated with coronary artery fistulae in 0.25-0.4% of
cases (8)
Coronary vascular development is initiated by proliferating
blood islands, which coalesce into a network of myocardial sinusoids,
that does not communicate with the ventricle. Ventriculo-coronary
fistulae are thought to be initiated at this early stage of development (2).
- 50%
originate from the right coronary artery, 45% from the left coronary
artery and 5% originate from both coronary arteries (1).
·
The left, right, or both coronary arteries may be involved. The
fistulae predominantly drain into the right side of the heart (92%):
1.
into the right ventricle in 41% of cases
2.
into the right atrium in 26% of cases
3.
into the coronary sinus in 7% of cases
4.
into the pulmonary artery in 17% of cases
5.
into the superior vena cava in 1% of cases
6.
into the left ventricle in only 3% of cases
7.
into the left atrium in 5% of cases
8.
drainage into both ventricles is rare (9).
- These
connections have been diagnosed as early as the first trimester (3).
- Abnormal
connections between the coronary vasculature and ventricular chambers are
more in obstructive lesions of the outflow tracts with intact ventricular
septum. Ventricular hypertension and hemodynamic
disturbances are believed to result in abnormal connections between
myocardial sinusoids and cardiac chambers.
- Cardiac
anomalies associated with ventriculo-coronary
fistula (3-6):
- There
have been numerous publications of pulmonary atresia with an intact interventricular septum.
- Hypoplastic
left heart with transposition of the great vessels and pulmonary atresia
with intact interventricular septum.
- The
abnormal connections appear to coalesce into a single vessel connecting
the right ventricular apex to the left coronary system (7).
- A
large unobstructed fistula can cause a “steal” phenomenon
because aortic blood flows freely into the right ventricle during diastole
(2).
- There
may be concurrent abnormalities of coronary venous drainage and intracardiac hemodynamics.
- The
presence of this anomaly may have a significant impact on the approach of
corrective postnatal surgery.
- Color Doppler
echocardiography and pulsed Doppler can detect high velocity and turbulent
flow, as well as the site of drainage.
Serious
complications due to congenital coronary artery fistulae have been reported:
·
congestive heart failure
·
pulmonary hypertension
·
bacterial endocarditis
·
premature arteriosclerotic changes
within the fistulae
·
aneurysm
·
thromboembolic events
·
arrhythmia, symptoms such as angina pectoris and myocardial infarction
resulting from coronary steal (9).
Spontaneous closure of
fistulae may occur (10). However, most authors recommend early intervention at the
time of diagnosis to prevent complications.
Mortality resulting from
surgical closure ranges from 0% to 4% (9).
Successful transcatheter occlusion of coronary artery fistulae by coil
embolization have been reported (11-13).
- Levin
DC, Fellows KE, Abrams HL. Hemodynamically
significant primary anomalies of the coronary arteries. Angiographic
aspects. Circulation 1978;58:25-34.
- Baschat AA, Love JC, Stewart PA et.al.
Prenatal diagnosis of ventriculocoronary fostula. Ultrasound Obstet Gynecol 2001;18:39-43.
- Chaoui R, Machlitt A, Tennstedt C. Prenatal diagnosis of ventriculo-coronary fistula in a late first-trimester
fetus presenting with increased nuchal translucency. Ultrasound Obstet Gynecol 2000;15:160-162.
- Chaoui R, Tennstedt C, Goldner B et.al. Prenatal
diagnosis of ventriculocoronary communication
in a second trimester fetus using transvaginal
and transabdominal color doppler sonography.
Ultrasound Obstet Gynecol
1997;9:194-197.
- Arabin B, Aardenburg R, Schasfoort-Van Leeuven M et.al. Prenatal diagnosis of ventriculocoronary
arterial communications combined with pulmonary atresia. Ultrasound Obstet Gynecol 1996;7:461-462.
- Patel
CR, Shah DM, Dahms BB. Prenatal diagnosis of a
coronary artery fistula in a fetus with pulmonary atresia with intact
ventricular septum and trisomy 18. J Ultrasound
Med 1999;18:429-431.
- Yokose T, Doi M, Kimura Y,
Ogata T. Ventriculo-coronary micro
communications in pulmonary atresia and sequential changes of coronary
arteries. Acta Pathol
Jpn 1987;37:1033-1040.
- Apitz J. Angeborene
Anomalien der Koronararterien. In Apitz
J, ed. Pädiatrische Kardiologie. Darmstadt: Steinkopff-Verlag,
1998: 419-29
- 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
Surg 1997; 45: 287-94
- Wong K, Menahem
S. Coronary arterial fistulas in childhood. Cardiol
Young 2000; 10: 15-20
- Ragnarsson A, Emanuelsson
H. Treatment of a large congenital coronary fistula with coil embolization. Scand Cardiovasc
J 1999; 33: 57-9
- McElhinney DB, Burch GH, Kung GC,
Villegas MD, Silverman
NH, Moore P. Echocardiographic guidance for transcatheter
coil embolization of congenital coronary
arterial fistulas in children. Pediatr
Cardiol 2000; 21: 253-8
- Pedra CA, Pihkala
J, Nykanen DG, Benson LN. Antegrade
transcatheter closure of coronary artery
fistulae using vascular occlusion devices. Heart 2000; 83: 94-6