Successful endovascular repair of an unusual right-to-left shunt presenting with cerebral ischemia

Ischemic stroke due to congenital cardiopulmonary vasculature anomalies is rare in adults. We report a 54-year-old man with a stroke due to a unique right-to-left shunt who underwent successful endovascular treatment. This patient developed acute onset of right arm weakness with facial droop and aphasia which improved after intravenous thrombolysis. An MRI showed acute cerebral ischemia in the left middle cerebral artery and left posterior cerebral artery distribution. The patient developed recurrent stroke symptoms during agitated saline injection while undergoing a transthoracic echocardiogram which showed right-to-left shunting. Chest CT scan and conventional angiography revealed near occlusion of the superior vena cava. Head and upper limb venous return drained via a large left vertical vein into an anomalous left pulmonary vein into the left atrium. He underwent endovascular surgery to relieve the superior vena cava obstruction and to occlude the source of right-to-left shunt. While rare, congenital cardiopulmonary vascular anomalies may result in ischemic stroke in adults. CT angiography may be necessary to evaluate cardiopulmonary vasculature when right-to-left shunting is discovered on echocardiography in the setting of ischemic stroke. With large right-to-left shunts, agitated saline should be avoided.