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A rare type of ‘coronary arterial — left ventricular fistula’ via thebesian veins in a Fragile X syndrome carrier

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IMAGES IN CARDIOLOGY

Cardiology Journal 2011, Vol. 18, No. 3, pp. 318–319 Copyright © 2011 Via Medica ISSN 1897–5593

318 www.cardiologyjournal.org

Address for correspondence: Dr. Sudheer Koganti, MRCP, University Hospitals of Coventry and Warwickshire,

Clifford Bridge Road, Coventry CV2 2DX, United Kingdom, tel: 00447966 332 456, e-mail: sudheerkoganti@hotmail.com Received: 08.08.2010 Accepted: 26.08.2010

A rare type of ‘coronary arterial

— left ventricular fistula’ via thebesian veins in a Fragile X syndrome carrier

Sudheer Koganti, Ashan Gunarathne, Purushotham Desai, Pritwish Banerjee

University Hospitals of Coventry and Warwickshire, Coventry, United Kingdom

A 62 year-old woman, who was initially inves- tigated for syncopal attacks, was referred to the car- diology department for further assessment of an incidentally found heart murmur. Clinical examina- tion revealed a continuous murmur best heard in the pulmonary area. She was known to be a carrier for Fragile X syndrome. All the previous investiga- tions performed to evaluate her syncopal episodes had been normal. A transthoracic echocardiogram showed multiple colour flow jets in the left ventri- cular (LV) cavity arising from lateral and septal walls.

Transesophageal imaging with colour Doppler revealed blood flow from the epicardial surface into

the LV cavity through the lateral and septal walls of the myocardium in both systole and diastole.

However, there was no evidence of any valvular or patent ductus arteriosus malformations. Coronary angiogram demonstrated a coronary arterial — LV fistula. Communication was seen between the left anterior descending artery and LV, as well as the obtuse marginal artery and LV via a leash of thin vessels, classically described as ‘thebesian veins’

[1], that drained from these arteries directly into the LV cavity (Figs. 1, 2).

Structural cardiac abnormalities such as mitral valve prolapse and dilatation of aorta have been re-

Figure 2. Multiple thebesian veins seen feeding the fi- stula from left anterior descending artery and obtuse marginal artery. Arteries — arrows.

Figure 1. Left ventricular angiogram showing the ab- normality.

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319 Sudheer Koganti et al., Rare type of ‘coronary arterial — left ventricular fistula’

www.cardiologyjournal.org

ported in patients suffering from Fragile X syn- drome [2]. To the best of our knowledge, this is the first reported case of coronary arterial — LV fistula in a patient with a Fragile X syndrome carrier state.

Acknowledgements

The authors do not report any conflict of inte- rest regarding this work.

References

1. Cha SE, Singer E, Maranhao V, Boldberg H. Coronary artery — left ventricular fistula: A disorder of the Thebesian system. An- giology, 1978; 29: 169.

2. Sreeram N, Wren C, Bhate M, Robertson P, Hunter S. Cardiac abnormalities in the Fragile X syndrome. Br Heart J, 1989; 61:

289–291.

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