Since dissection and rupture of aortic aneurysms tend to occur at a younger age and at smaller diameters in LDS3 patients compared to Marfan syndrome patients, early and aggressive intervention is recommended. Angiotensin receptor blockers, beta-adrenergic receptor blockers or other medications are used to reduce hemodynamic stress. All individuals with LDS require echocardiography at frequent intervals to monitor the status of the ascending aorta. Using computed tomography angiography (CTA) or magnetic resonance angiography (MRA), 3D reconstruction of images from the head to pelvis is needed to identify arterial tortuosity and aneurysms in the rest of the arterial tree. This is essential because aneurysms distant from the aortic root can be easily overlooked using echocardiography. The frequency of CTA or MRA depends on clinical findings. Malformations, dysfunction or pain ensuing from the extra-cardiovascular anomalies are dealt with in a case-by-case manner, based on the input of a multidisciplinary team of medical specialists.
SMAD3