Cardioembolic Stroke in Adults With a History of Congenital Heart Disease

A 47-year-old right-handed white woman with a history of congenital heart disease (CHD) had witnessed onset of aphasia. She was taken to a nearby emergency department where laboratory values, ECG, and a computed tomography of the head were normal. She had a right hemiparesis and aphasia with an National Institutes of Health stroke score (NIHSS) of 20; however, her examination improved and she was not given recombinant tissue-type plasminogen activator. Aspirin was administered for suspected ischemic stroke, and she was transferred to a comprehensive stroke center. On arrival, her NIHSS worsened to 16 and intravenous recombinant tissue-type plasminogen activator was administered 3 hours and 44 minutes after onset. Emergent magnetic resonance imaging showed diffusion restriction within the left basal ganglia and occlusion of the left middle cerebral artery. Because of a large perfusion/diffusion mismatch, embolectomy was performed using a retrievable stent with complete revascularization (thrombolysis in cerebral infarction revascularization score: 2b). After the procedure, her NIHSS reduced to 8. She had no other stroke risk factors other than her CHD.At birth, she was diagnosed with a double-inlet left ventricle with dextro-transposition of the great arteries. A hybrid procedure with bilateral pulmonary artery banding and bilateral arterial duct stenting was performed at 6 weeks of age. She underwent a Glenn operation at the age of 12 years, which was complicated by transient postoperative left hemiparesis. She had a Fontan operation at age 25 years after which she recovered to New York Heart Association functional class I. No antithrombotic medications were prescribed.Work up for her current stroke included transthoracic echocardiogram and cardiac magnetic resonance imaging, which showed a double-inlet left ventricle with D-transposition of the great arteries. There was a hypoplastic right ventricular chamber. A right to …