Title:Medical Treatment of Aortic Aneurysms in Marfan Syndrome and other Heritable Conditions
Volume: 10
Issue: 2
Author(s): Christine H. Attenhofer Jost, Matthias Greutmann, Heidi M. Connolly, Roland Weber, Marianne Rohrbach, Angela Oxenius, Oliver Kretschmar, Thomas F. Luscher and Gabor Matyas
Affiliation:
Keywords:
Angiotensin II receptor antagonists, aortic aneurysm, beta-blockers, genetics, Marfan syndrome, medical management,
risk factors, statins.
Abstract: Thoracic aortic aneurysms can be triggered by genetic disorders such as Marfan syndrome (MFS) and related
aortic diseases as well as by inflammatory disorders such as giant cell arteritis or atherosclerosis. In all these conditions,
cardiovascular risk factors, such as systemic arterial hypertension, may contribute to faster rate of aneurysm progression.
Optimal medical management to prevent progressive aortic dilatation and aortic dissection is unknown. β-blockers have
been the mainstay of medical treatment for many years despite limited evidence of beneficial effects. Recently, losartan,
an angiotensin II type I receptor antagonist (ARB), has shown promising results in a mouse model of MFS and subsequently
in humans with MFS and hence is increasingly used. Several ongoing trials comparing losartan to β -blockers
and/or placebo will better define the role of ARBs in the near future. In addition, other medications, such as statins and
tetracyclines have demonstrated potential benefit in experimental aortic aneurysm studies. Given the advances in our understanding
of molecular mechanisms triggering aortic dilatation and dissection, individualized management tailored to
the underlying genetic defect may be on the horizon of individualized medicine. We anticipate that ongoing research will
address the question whether such genotype/pathogenesis-driven treatments can replace current phenotype/syndromedriven
strategies and whether other forms of aortopathies should be treated similarly. In this work, we review currently
used and promising medical treatment options for patients with heritable aortic aneurysmal disorders.