Coronary artery fistula

Rare. It is by definition an abnormal communication between a coronary artery (left or right, rather than the circumflex) and the cardiac cavity (90% right: ventricular or right atrium) or a large vessel (pulmonary artery, superior vena cava, coronary sinus). The malformation is generally isolated and does not cause symptoms or complications before 20 years of age if it is small. However a large fistula can lead to an overload of the pulmonary circulation and heart failure or angina in the neonatal period or in early childhood.

Later, the following complications can be observed:

-         localized myocardial hypoperfusion

-         thrombosis or embolism (with risk of myocardial infarction)

-         disorders of cardiac rhythm, including atrial fibrillation

-         endocarditis

-         spontaneous rupture with hemopericardium.


Clinical signs: continuous heart murmur (more intense in diastole)  on the  left laterosternal area.

One can also observe coronary fistulas in association with cardiac malformation: pulmonary atresia  with an intact septum is associated with coronary fistula in 30 to 60 % of cases. In 10 % of these cases, part of the coronary circulation depends directly from the right ventricle, which exposes the child to myocardial ischemia in case of decrease of volemia and/or decrease of the RV contractility (see case report by T.D. Brown).

Treatment: coronary surgery or embolization by coils during angiographic diagnosis


Anesthetic implications: 

in the absence of other cardiac anomality, and except if the fistula drains within a left cavity, left to right shunt the importance of which depends on the size of fistula and the pressure difference between the artery and the structure into which the fistula is draining.


References : 

-   Qureshi SA. 
Coronary arterial fistulas. 
Orphanet J Rare Diseases 2006; 1 ; 51, 6p

-        Brown TD, Mossad E, Motta P. 
Cardiac arrest at induction of anesthesia in a child with undiagnosed right-ventricular dependent coronary circulation: a case report. 
Pediatr Anesth 2006; 16: 1179-83.

-         Jerbi S, Tarmiz A, Fradi S, Brahem A et al. 
Fistule coronaire : rapport d’un cas opéré et revue de la littérature. 
Ann Cardiol Angéiologie 2009 ; 58 : 236-9.


Updated: September 2019