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congenital systemic arteriovenous fistula - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

congenital systemic arteriovenous fistula

MONDO:0016078

Overview

A rare, potentially life-threatening, vascular disease characterized by a direct communication between an artery and a vein, without the interposition of the capillary bed, ocurring in the systemic circulation (mainly the cranium, liver, lungs, extremities, and vessels in or near the thoracic wall). Manifestations are variable depending on size and extent of the fistula, the involved blood vessels and the precise location of the collaterals and may include systolic or continuous murmur over the affected organ, tachycardia, increased stroke volume, cardiomegaly and increased pulmonary vascular markings.

TreatmentsNo approved treatments · 1 in pipeline

ResearchLimited published research (4 articles)

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

No approved treatments are currently available for congenital systemic arteriovenous fistula. An additional 1 compound holds orphan drug designation.

Orphan Drug Designations

While no drugs are FDA-approved specifically for congenital systemic arteriovenous fistula, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.

The following drugs have received orphan drug designation from the FDA for congenital systemic arteriovenous fistula. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name	Sponsor

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for congenital systemic arteriovenous fistula.

4 publications have been identified in PubMed for congenital systemic arteriovenous fistula. Research spans Case Report / Case Series (75%) and Review / Meta-Analysis (25%).

Recent Publications

Callegari A (2026). [PMID: 40097841](https://pubmed.ncbi.nlm.nih.gov/40097841/). *Pediatric cardiology*. [Case Report / Case Series]
Peluso N (2025). [PMID: 40054609](https://pubmed.ncbi.nlm.nih.gov/40054609/). *Annals of vascular surgery*. [Review / Meta-Analysis]
Takada S (2025). [PMID: 40607261](https://pubmed.ncbi.nlm.nih.gov/40607261/). *Case reports in gastroenterology*. [Case Report / Case Series]
Kiel MD (2024). [PMID: 38770406](https://pubmed.ncbi.nlm.nih.gov/38770406/). *European heart journal. Case reports*. [Case Report / Case Series]

Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 11:13 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:2039

GARD

Genetic and Rare Diseases Info Center

GARD:18758

Questions for Your Care Team

→What is the current development status of vonapanitase, and when might it become available?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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Kisho

congenital systemic arteriovenous fistula

Overview

Treatment

Orphan Drug Designations

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

Latest News & Developments

Endovascular Repair of Huge Twin Aneurysms of Renal Artery and Vein Connected by Arteriovenous Fistula.

Endovascular Repair of an Ilio-Iliac Arteriovenous Fistula Presenting as Acute Lower Limb Edema.

congenital systemic arteriovenous fistula

Overview

Treatment

Orphan Drug Designations

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

Latest News & Developments

Endovascular Repair of Huge Twin Aneurysms of Renal Artery and Vein Connected by Arteriovenous Fistula.

Endovascular Repair of an Ilio-Iliac Arteriovenous Fistula Presenting as Acute Lower Limb Edema.