Outpatient Follow Up
In all cases control of the blood pressure is important!
All Marfans patients should be followed up for life. Standard follow-up includes a 6 to 8 week post operative visit then once at 6 months , a year and then yearly. When to scan remains controversial, but yearly with MRI to reduce radiation dose seems reasonable in a stable patient. Any suggestion of aneurysmal dilation may need more frequent scanning intervals.
If old but well, with no aneurysmal dilation of their aortic arch or descending aorta then they can be discharged from outpatients back to the cardiologists at 6 to 8 weeks. If young, then they probably have a connective tissue disorder ??Bicuspid aortic valve disease. Follow up here should probably be yearly and lifelong, plain CXR both PA and lateral are actually quite cheap but good to monitor distal ascending aorta and arch dimensions to check for subsequent aneurysmal dilation.
Patients who have undergone valve preserving operations should probably have annual echocardiography to document valvular function.
If old but well, with no aneurysmal dilation of their aortic arch or descending aorta then they can be discharged from outpatients back to the cardiologists at 6 to 8 weeks. If young, then they probably have a connective tissue disorder ??Bicuspid aortic valve disease. Follow up here should probably be yearly and lifelong, plain CXR both PA and lateral are actually quite cheap but good to monitor distal ascending aorta and arch dimensions to check for subsequent aneurysmal dilation.
Patients who have undergone valve preserving operations should probably have annual echocardiography to document valvular function.
Isolated arch replacements are unusual, concomitant ascending +/- root replacement being the norm. The descending aorta remains a common site for later aneurysmal expansion and these patients need life long follow-up. These patients should be followed up every 3 months for 6 months, then 6 monthly for a year, and then yearly if static. Secondary interventions include descending or thoracoabdominal aneurysm repair or stenting.
Patients who have undergone valve preserving operations should probably have annual echocardiography to document valvular function.
Post thoracoabdominal replacement patients tend to have aneurysmal dilation of the "rest" of their aorta and peripheral branches and significant central and peripheral vascular disease. These should all be treated on their own merits. Young patients should probably be followed up lifelong with annual scanning. Aortic branch aneurysms and false aneurysms at suture sites are not uncommon.
A number of patients have aneurysms but the size of the aneurysm does not by itself necessitate surgery. The aneurysms often enlarge but at variable rates. These patients should be followed up every 3 months for 6 months, then 6 monthly for a year, and then yearly if static. Any indication of aneurysm expansion should prompt more frequent scanning with surgical or stent intervention at appropriate aneurysm trigger sizes depending on site and pathology.
If medically unfit for any surgical intervention then it should be decided are they ever a candidate for a stent. If they are not they can be discharged back to the referring physician with instructions on strict blood pressure control. If they are a candidate for a stent, if they need it now do it, or follow them up at 3 months, then 6 monthly intervals with scanning as appropriate to evaluate the aneurysm size, then stent them when the aneurysm reaches a size that indicates stenting is necessary.