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/syndrome-driven 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.
Highlights
Aortic aneurysm, with its first description as the cause of death of King George II in 1760 [1] is defined as aortic dilatation of greater than 50% of the normal diameter for age and body surface area and occurs most commonly due to medial degeneration of a localized portion of the aorta [2]
These findings have recently been supported by a smaller prospective study in 28 young patients with Marfan syndrome (MFS), showing that the addition of losartan to a β -blocker in 15 patients versus β -blocker only in 13 patients reduced the rate of aortic dilation significantly [38]
Further data from randomized trials will become available in the near future and may elucidate whether ARB or angiotensin converting enzyme inhibitors (ACEIs) will reduce aortic dilatation but may affect the risk of aortic dissection and mortality in MFS patients [27]
Summary
With its first description as the cause of death of King George II in 1760 [1] is defined as aortic dilatation of greater than 50% of the normal diameter for age and body surface area and occurs most commonly due to medial degeneration of a localized portion of the aorta [2]. Efficacy of the β-blocker propranolol in decreasing the rate of aortic dilatation stems from a small randomized study of patients with MFS. These findings have recently been supported by a smaller prospective study in 28 young patients with MFS (mean age 13.1 years, NCT00651235 in Table 1), showing that the addition of losartan to a β -blocker (atenolol or propranolol) in 15 patients versus β -blocker only in 13 patients reduced the rate of aortic dilation significantly [38].
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