This patient with a bicuspid aortic valve should have annual transthoracic echocardiography. Bicuspid aortic valve occurs with other cardiovascular and systemic abnormalities. Specifically, ascending aortic dilation may occur in persons with a bicuspid aortic valve, in combination with aortic valve disease or as an independent condition. Previously considered a secondary event caused by abnormal aortic valve function, the aortopathy associated with a bicuspid aortic valve is now recognized to result from intrinsically abnormal connective tissue. As a result, serial evaluation of ascending aortic diameter should be performed by transthoracic echocardiography (or by CT angiography or magnetic resonance angiography if not adequately visualized by echocardiography). The frequency of surveillance depends upon aortic root and ascending aorta size. Expert consensus guidelines recommend reassessment of the aorta if the aortic root or ascending aorta dimension is greater than or equal to 4.0 cm, with the evaluation interval determined by degree and rate of aortic dilation and by family history. Annual evaluation should occur if the aortic diameter is greater than 4.5 cm. New or changing symptoms and pregnancy are indications for earlier imaging of the aorta. For this patient, in view of the similar findings on transthoracic echocardiogram and chest CT scan, serial transthoracic echocardiography is reasonable. Moreover, transthoracic echocardiography is more cost effective than both CT and cardiac magnetic resonance (CMR) imaging, and serial CT scans can result in significant cumulative doses of radiation in this young patient.

CMR imaging and multidetector CT are appropriate for further assessment of aortic pathology when transthoracic or transesophageal echocardiography is not conclusive. In this patient, CMR imaging and multidetector CT scanning are not indicated because the aortic root and ascending aorta are adequately visualized by transthoracic echocardiography. In addition, annual multidetector CT scanning would needlessly expose the patient to excessive radiation.

Transesophageal echocardiography is an invasive procedure and would be considered if transthoracic imaging was inadequate to measure aortic root size and evaluate the ascending aorta.

Because of the risk of aortic dissection and rupture, reassurance and clinical observation are inadequate follow-up for bicuspid valve–related aortopathy.