Marfan syndrome with aortic dilation

This code identifies Marfan syndrome, a genetic connective tissue disorder, specifically when it manifests with an enlargement of the aorta. Aortic dilation in Marfan syndrome is a critical cardiovascular complication that can lead to dissection or rupture.

Use this code for patients diagnosed with Marfan syndrome who also have documented evidence of aortic dilation, regardless of whether it's in the ascending, descending, or arch segments. Documentation should clearly state both the Marfan syndrome diagnosis and the presence of aortic dilation, often confirmed by imaging studies like echocardiograms or CT scans.

• Ensure documentation specifies "Marfan syndrome" and "aortic dilation" to support this specific code.
• Do not use this code for isolated aortic dilation without a confirmed Marfan syndrome diagnosis.
• Consider additional codes for specific complications of aortic dilation, such as dissection or rupture, if present.