Cardiac monitoring in EDS

What are the recommended tests for monitoring hart and aorta in patients with EDS? I have either classical or hypermobile EDS, but my aunt dies of a ruptured aorta, so I want to have proper monitoring done to ensure that I don't also have an aorta problem. The cardiologist that I want to had me do a stress test and did an echo of my heart and aortic root, but didn't check out the rest of my aorta. I've read various articles that suggest various monitoring tests, but I don't know which are the latest recommended most agreed upon tests for this. My sister had an MRI done, and the last thing I read suggested a CT scan. Is it recommended to check out more of the aorta than what is seen in just a typical cardiac echo? My doctor also ordered a tilt table test for me too because I sometimes pass out, but I don't think that test is necessary because the occasional passing out isn't a problem for me and it is post exertional. I just want to have the exact best monitoring test for my heart and aorta and then be done with it or a few years. My stress test was perfectly fine until it was over, then a couple of minutes after getting the wires off of me, the lightheadedness started. Is a tilt table test really needed to contribute to the long term monitoring of my cardiac stuff? And is a CT or an MRI the best way to visualize and measure the aortic diameter?

[Jordanna Joaquina, MS, CGC]

We recommend that you meet with your cardiologist to discuss your symptoms, questions and concerns. We also recommend that you meet with a medical geneticist who can review your history and discuss any appropriate treatment options. You can find a medical geneticist at the American College of Medical Genetics.