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 and require early surgical intervention. However, with improved surgical techniques these patients are surviving later into adulthood which comes with a new set of medical complications providers need to be aware of.
Case Presentation We present a case of a patient with a complex cardiac history including an incomplete Shone’s complex diagnosed in childhood with multiple surgical interventions who presents with persistent symptomatic atrial flutter and a type A aortic dissection in the setting of severe patient prosthesis mismatch. The patient was being followed outpatient for an enlarging chronic aortic aneurysm and was non-compliant with his medications when he presented with sharp chest pain radiating to his back. He was found to have an acute type A aortic dissection and 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 in adulthood, such as 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.