# Case report: a rare case of left-sided sinus of Valsalva aneurysm with concomitant coronary artery fistula and carotid artery aneurysm

**Authors:** Marsioleda Kemberi, Gentjan Jakaj, Richard Stratton, Daryll Baker, Olaf Wendler

PMC · DOI: 10.1093/ehjcr/ytaf508 · European Heart Journal. Case Reports · 2025-10-03

## TL;DR

A rare case of a left-sided heart aneurysm with a coronary artery fistula and carotid aneurysm was successfully treated surgically in a 56-year-old woman.

## Contribution

This case highlights the successful surgical management of a rare combination of cardiac and vascular anomalies.

## Key findings

- A 56-year-old woman had a left-sided sinus of Valsalva aneurysm, coronary artery fistula, and carotid aneurysm.
- Staged surgical repair with a modified Bentall procedure and carotid aneurysm excision was successful.
- Genetic testing confirmed Marfan's syndrome, emphasizing the importance of genetic evaluation in atypical aneurysmal cases.

## Abstract

Sinus of Valsalva aneurysms (SVAs) are rare cardiac anomalies often occurring with co-existing cardiac lesions, such as ventricular septal defects and aortic regurgitation. While often asymptomatic, SVAs can lead to severe complications, including rupture, aortic regurgitation, and dissection. Carotid aneurysms are also uncommon and pose a significant stroke risk if left untreated.

We report the case of a 56-year-old woman who initially presented with a pulsatile right neck mass and was diagnosed with a right internal carotid artery aneurysm. Subsequent imaging revealed a 45 mm left-sided sinus of Valsalva aneurysm (SVA) and a concomitant coronary artery fistula involving the right coronary artery. She underwent staged surgical management with a modified Bentall procedure and later excision of the carotid aneurysm. Post-operative recovery was uneventful. Genetic testing later confirmed a diagnosis of Marfan's syndrome.

SVAs originating from the left coronary sinus are exceedingly rare, but their potential for fatal complications demands early intervention. This case demonstrates that surgical repair of the left SVA, associated coronary fistula, and carotid aneurysm is both safe and effective, and it reinforces the role of genetic testing in patients with atypical presentations of aneurysmal disease. Multimodal imaging and a multidisciplinary approach are essential in guiding treatment decisions in such unusual presentations.

## Linked entities

- **Diseases:** Marfan's syndrome (MONDO:0007947)

## Full-text entities

- **Diseases:** stroke (MESH:D020521), Carotid aneurysms (MESH:D020212), coronary artery fistula (MESH:D003324), Marfan's syndrome (MESH:D008382), ventricular septal defects (MESH:D006345), dissection (MESH:D000784), carotid artery aneurysm (MESH:D002340), cardiac anomalies (MESH:D006331), coronary fistula (MESH:D005402), rupture (MESH:D012421), aortic regurgitation (MESH:D001022), SVA (MESH:D000783), neck (MESH:D006258)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12559787/full.md

## References

9 references — full list in the complete paper: https://tomesphere.com/paper/PMC12559787/full.md

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Source: https://tomesphere.com/paper/PMC12559787