[Persistent left superior vena cava with right-left shunt into the left atrium].

UNLABELLED: Persistent left superior vena cava with right-left shunt into the left atrium.

HISTORY AND CLINICAL FINDINGS: A 72-year-old patient was admitted to the hospital following bleeding into the basal ganglia secondary to a hypertensive crisis.

INVESTIGATIONS: The patient was found to suffer from marked hypoxaemia (pO2 49 mmHg) and erythrocytosis (Hb 18,5 g/dl). Subsequent investigations raised suspicion of a right-left shunt. This was verified by a contrast echocardiogram which was performed transthoracically by injection of echo-contrast material from the left. To improve imaging of the shunt a transoesophageal contrast-echocardiogram was carried out. This showed that the persistent left superior vena cava did not, as previously expected, lead directly into the left atrium, but had a connection to the left superior pulmonary vein. This anatomical variant, which so far to our knowledge has not been reported in the literature, could be confirmed by spiral computed tomography. Apart from an atrial septal aneurysm no other cardiac anomaly could be identified.

TREATMENT AND COURSE: Ligation of the left superior vena cava could have been a therapeutic option, but the patient declined operative intervention.

CONCLUSION: In cases of profound hypoxemia and erythrocytosis the differential diagnosis must include a persistent left superior vena cava with anomalous connection to the left atrium. Trans-thoracic and transoesophageal contrast-echocardiography is a simple and reliable method to diagnose persistent left superior vena cava as well as concomitant cardiac anomalies.