Abstract 18126: Calcium Channel Blockers Accelerate Aortic Aneurysm and Cause Premature Lethality in Marfan Syndrome and Related Conditions

Calcium channel blockers (CCBs) are prescribed to patients with Marfan syndrome and related inherited thoracic aortic aneurysm (TAA) syndromes for prophylaxis against TAA progression, despite limited evidence for their efficacy in these disorders. Unexpectedly, Marfan mice treated with CCBs showed accelerated TAA expansion, rupture and premature lethality. In collaboration with other members of the GenTAC consortium, we conducted a case-control study to assess the effect of CCBs in humans with Marfan syndrome and related TAA conditions. These primarily included Loeys-Dietz, Turner and Ehlers-Danlos syndromes, familial thoracic aortic aneurysm, and bicuspid aortic valve with aneurysm. Marfan patients with native aortic roots at the time of enrollment who received CCBs (compared to other antihypertensive agents) had an increased risk of aortic dissection (odds ratio (OR) 12.5, p<0.05). Strong trends were maintained after correction for blood pressure (OR 12.7, p=0.06) or aortic root size (OR 11.2, p=0.08) a...