LAUSR

Marfan syndrome is an autosomal dominant disease of connective tissue affecting approximately one in 5 000 people. However, the majority of patients (60-80%) with Marfan syndrome have some degree of aortic root dilation or thoracic aortic aneurysm that can lead to aortic dissection and rupture, which has long been recognised as the leading cause of death in Marfan syndrome. The funnel breast (pectus excavatum), often seen in patients with Marfan syndrome, radically complicates the access during aortic root or arch interventions, forcing cardiosurgery practitioners to seek alternative approaches. We present a series of clinical observations of two patients with Marfan syndrome and aneurysm of the aortic root and ascending aorta. A left-sided thoracotomy provides the possibility of successful implementation of extensive surgical interventions, decreasing the risks of intra-operative damage to the cardiac structures adjacent to the sternum in this complex cohort of patients.