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Coronary arterial fistula

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Disease definition

Coronary arterial fistulas are a connection between one or more of the coronary arteries and a cardiac chamber or great vessel.

ORPHA:2041

Classification level: Disorder

Prevalence: Unknown

Inheritance: Not applicable

Age of onset: All ages

ICD-10: Q24.5

ICD-11: LA8C.2 BA83

UMLS: C0265898

GARD: 1533

MedDRA: 10069441

Summary
Epidemiology

Their exact incidence is unknown but the defect is rare and usually occurs in isolation.

Clinical description

The majority of these fistulas are congenital in origin although they may occasionally be detected after cardiac surgery. They do not usually cause symptoms or complications in the first two decades of life, especially when small. After this age, the frequency of both symptoms and complications increases. Complications include 'steal' from the adjacent myocardium, thrombosis and embolism, cardiac failure, atrial fibrillation, rupture, endocarditis/endarteritis and arrhythmias. Thrombosis within the fistula is rare but may cause acute myocardial infarction, paroxysmal atrial fibrillation and ventricular arrhythmias. Spontaneous rupture of the aneurysmal fistula causing haemopericardium has also been reported.

Diagnostic methods

Whilst two-dimensional echocardiography helps to differentiate between the different shunts, coronary angiography is the main diagnostic tool for the delineation of the anatomy.

Differential diagnosis

The main differential diagnosis is patent arterial duct, although other congenital arteriovenous shunts need to be excluded.

Management and treatment

Surgery was the traditional method of treatment but nowadays catheter closure is recommended using a variety of closure devices, such as coils. With the catheter technique, the results are excellent with infrequent complications.

Last update: December 2006 - Expert reviewer(s): Dr Shakeel QURESHI
A summary on this disease is available in Français (2006) Español (2006) Deutsch (2006) Italiano (2006) Nederlands (2006)
Detailed information
Disease review articles
Review article
English (2006) - Orphanet J Rare Dis

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