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Background and purpose:  Our case report deals with the importance of detailed echocardiographic examination for differential diagnosis of coronary sinus dilation and development of abnormalities of great thoracic veins.    Case presentation:  A 49-year-old man underwent transthoracic echocardiography for atypical chest pain. A dilated coronary sinus was found and venous contrast echocardiography raised the suspicion of absent right and persistent left superior vena cava. Transesophageal echocardiography showed absence of right superior vena cava. The echocardiographic findings were confirmed by upper venous digital subtraction cavography.    Conclusion:  combination of agenesia of right SVC and isolated persistent left SVC in adult patients is a very rare abnormality. Both clinicians and sonographers should be alerted to the possible presence of this combined venous anomaly. Transthoracic echocardiograpy – including agitated saline infusion to the antecubital vein – is an important diagnostic tool for accurate diagnosis of this congenital thoracic venous malformation.

A case of absent right and persistent left superior vena cava