Coronary arterial fistula

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1FDA treatments2Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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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:

Abnormal left ventricular functionHP:0005162Systolic heart murmurHP:0031664Continuous heart murmurHP:0031670Patent foramen ovaleHP:0001655Vascular dilatationHP:0002617
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

FDA & Trial Timeline

1 event
Dec 2017Appropriateness of Angioplasty in Patients With Chronic Coronary Syndromes

University of Sao Paulo General Hospital — NA

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

1 available

XARELTO

RIVAROXABAN· Janssen Pharmaceuticals, Inc.■ Boxed Warning

to reduce the risk of major cardiovascular events in patients with coronary artery disease (CAD)

No actively recruiting trials found for Coronary arterial fistula at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Coronary arterial fistula community →

Specialists

2 foundView all specialists →
ZC
Zuheir Kabbani, Cardiology
Specialist
PI on 1 active trial
LP
Luis Henrique W Gowdak, MD, PhD
São Paulo, São Paulo
Specialist

Rare Disease Specialist

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Coronary arterial fistula.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Coronary arterial fistula

Disease timeline:

New trial: Appropriateness of Angioplasty in Patients With Chronic Coronary Syndromes

Phase NA trial recruiting. Percutaneous Coronary Intervention (PCI)

Caregiver Resources

NORD Caregiver Resources

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Mental Health Support

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Family & Caregiver Grants

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Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

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.