The clinical management for persons with MFS requires multidisciplinary care. Evaluations by multiple specialists are recommended once a diagnosis is made, including detailed evaluations by an ophthalmologist, cardiologist, orthopedist, and medical geneticist, all of whom should have expertise in MFS. Specific testing that is recommended at that time includes slit-lamp ophthalmologic examination, intraocular pressure assessment, echocardiography, and selected radiographic tests such as evaluation of the entire aorta by magnetic resonance angiography or computed tomography and radiography of the spine.

There is a considerable literature on the medical and surgical management of the commonly occurring findings in MFS, as well as recommendations for surveillance of each at-risk organ system. Discussion of treatment of the common complications is beyond the scope of this review, but several clinical management issues merit comment.

The cardiovascular complications of untreated MFS can be severe and can lead to premature death. Normalization or near-normalization of lifespan and a marked reduction of cardiopulmonary-associated morbidity can be achieved if there is early attention to the medical and, if needed, surgical management of cardiovascular manifestations. Pharmacologic treatment with β-adrenergic blockade can prevent or decrease the progression of aortic dilatation by reducing the force of ventricular ejection, and there are specific recommendations regarding appropriate dosing. Specific criteria for when surgical repair of the aorta is indicated are also described. Surgical repair or replacement of the mitral valve is needed when there is refractory congestive heart failure due to mitral valvular disease.

There can be a significant acceleration of cardiovascular pathology in pregnant women with MFS, including a risk for rapid aortic root dilatation and dissection or rupture if the aortic root diameter exceeds 4 cm. Pregnancy should be considered only after appropriate counseling. Women with MFS who become pregnant must be carefully followed by a high-risk obstetrician from the onset of pregnancy through delivery and the postpartum period. Beta-adrenergic blockers can be continued during a pregnancy, but certain other medications such as angiotensin-converting enzyme inhibitors or angiotensin receptor blockers should be discontinued before pregnancy because of potential teratogenic effects.

There are still other important issues in the lifelong clinical care for persons with MFS. Orthopedic surveillance is important; many individuals have chronic joint pain, and a subset of persons with MFS have significant scoliosis that requires surgical stabilization of the spine. Individuals with cardiac valvular pathology require antibiotic prophylaxis for bacterial endocarditis. There are also various situations that should be avoided, such as contact and competitive sports and activities that will cause undue trauma to joints. Exposure to stimulants of the cardiovascular system and LASIK eye surgery should also be avoided.

Because MFS is an autosomal dominant condition, there may be others in the kindred who may be at risk to have this condition. Genetic counseling should accompany the diagnostic process. Medical genetics professionals can provide information not only about the diagnosis, natural history, and reproductive issues relating to MFS but also about relevant registries, clinical trials, and support groups. The latter, in turn, can provide important information, support, and, sometimes, access to resources for those affected with this condition and their families.