Overview
Coronary arterial fistula (also known as coronary artery fistula, coronary arteriovenous fistula, or coronary cameral fistula) is a rare congenital or acquired cardiac anomaly in which an abnormal communication exists between one or more coronary arteries and a cardiac chamber, great vessel, or other vascular structure, bypassing the normal myocardial capillary bed. This abnormal connection creates a shunt that diverts blood away from the myocardial circulation. The condition primarily affects the cardiovascular system. Most coronary arterial fistulas originate from the right coronary artery and drain into the right side of the heart, particularly the right ventricle, right atrium, or pulmonary artery. Many patients with small fistulas remain asymptomatic and the condition may be discovered incidentally during cardiac imaging or auscultation of a continuous heart murmur. However, larger fistulas can lead to significant hemodynamic consequences including congestive heart failure, myocardial ischemia (due to coronary steal phenomenon), angina pectoris, arrhythmias, infective endocarditis, pulmonary hypertension, and rarely, fistula rupture. Symptoms may develop gradually and can present at any age, though larger fistulas tend to become symptomatic earlier in life. In neonates and infants with large fistulas, heart failure may be the presenting feature. Treatment depends on the size and hemodynamic significance of the fistula. Small, asymptomatic fistulas may be managed conservatively with regular monitoring. Symptomatic or hemodynamically significant fistulas typically require closure, which can be achieved through transcatheter embolization (using coils, vascular plugs, or other occlusion devices) or surgical ligation. Transcatheter closure has become the preferred approach for many patients due to its less invasive nature. Surgical repair is generally reserved for complex fistulas, very large fistulas, or cases where transcatheter approaches are not feasible. Long-term outcomes after successful closure are generally favorable, though follow-up is recommended to monitor for recurrence or residual shunting.
Clinical phenotype terms— hover any for plain English:
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
1 eventUniversity of Sao Paulo General Hospital — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableXARELTO
to reduce the risk of major cardiovascular events in patients with coronary artery disease (CAD)
Clinical Trials
View all trials with filters →No actively recruiting trials found for Coronary arterial fistula at this time.
New trials open frequently. Follow this disease to get notified.
Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Coronary arterial fistula.
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Disease timeline:
New trial: Appropriateness of Angioplasty in Patients With Chronic Coronary Syndromes
Phase NA trial recruiting. Percutaneous Coronary Intervention (PCI)
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Common questions about Coronary arterial fistula
What is Coronary arterial fistula?
Coronary arterial fistula (also known as coronary artery fistula, coronary arteriovenous fistula, or coronary cameral fistula) is a rare congenital or acquired cardiac anomaly in which an abnormal communication exists between one or more coronary arteries and a cardiac chamber, great vessel, or other vascular structure, bypassing the normal myocardial capillary bed. This abnormal connection creates a shunt that diverts blood away from the myocardial circulation. The condition primarily affects the cardiovascular system. Most coronary arterial fistulas originate from the right coronary artery a
How is Coronary arterial fistula inherited?
Coronary arterial fistula follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Coronary arterial fistula?
2 specialists and care centers treating Coronary arterial fistula are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.