BACKGROUND: Shone\'s complex is a rare congenital heart disease consisting of a variety of left ventricular inflow and outflow tract lesions. Patients typically present in childhood requiring early surgical intervention; however, with improved surgical techniques, these patients are surviving later into adulthood. This increased survival comes with a new set of medical complications that providers need to be aware of.
METHODS: A 27 year old man with a complex cardiac history including an incomplete Shone\'s complex and persistent symptomatic atrial flutter presented with sharp chest pain radiating to his back. He was found to have type A aortic dissection on imaging in the setting of severe patient-prosthesis mismatch. He had multiple valvular surgeries in childhood. The patient was being followed-up as an outpatient for an enlarging chronic aortic aneurysm and was non-compliant with his medications. He was taken emergently to the operating room for a skirted Bentall procedure, aortic valve replacement, and right sided MAZE.
CONCLUSIONS: Shone\'s complex is a rare congenital heart disease associated with significant morbidities including atrial flutter, patient-prosthesis mismatch, and aortic dissection. As patients continue to live longer into adulthood with this disease, it is important to raise awareness of this rare syndrome for providers and highlight its potential complications. Further research is needed to determine appropriate guidelines for when to intervene on aortopathy-associated CHD.

A 67-year-old woman developed weakness of the entire left side of the body and disturbance of consciousness, and was admitted to our hospital. She had atrial fibrillation (AF) on arrival at the hospital. Diffusion weighted magnetic resonance imaging showed high intensity area in the right basal ganglia, and magnetic resonance angiography showed occlusion of the right internal carotid artery (ICA). Thrombolytic therapy with intravenous tissue plasminogen activator (IV tPA) was administered 225 minutes after onset, and endovascular procedure also performed. After endovascular therapy, the patient had successful recanalization of the right ICA. Transesophageal echocardiography (TEE) showed a mass in the left atrium. Cardiac surgery for the excision of a left atrial mass and the maze procedure for atrial fibrillation were performed on the 29th hospital day. The mass was pathologically confirmed as thrombus. Follow up TEE after cardiac surgery revealed recurrence of thrombus at the both origin of pulmonary vein in the left atrium. Finally, the thrombus was disappeared at 6-month after onset with taking warfarin. She had no stroke events during the clinical course.