Management of cardiovascular manifestations in Loeys – Dietz syndrome: a systematic review

Ieva Daniliauskaitė1, Olivija Gustienė2

1 Faculty of Medicine, Medical Academy, Lithuanian University of Health Sciences, Kaunas, Lithuania

2 Department of Cardiology, Lithuanian University of Health Sciences, Kaunas, Lithuania


Loeys – Dietz syndrome (LDS) is an autosomal dominantly inherited genetic disorder associated with significant and often life-threatening cardiovascular damage. The major cause of death in these individuals is aortic dissection. The primary choice for both prophylactic and emergency surgical interventions remains aortic valve-sparing root replacement due to the lower incidence of complications and the need for reoperation. Also, open surgery is more often recommended than endovascular stenting due to the possible accelerated development of aortic aneurysm in the native aortic stent fixation zones. To date, there are no approved guidelines for the treatment of aortic aneurysm and dissection in patients with LDS, but it is well known that multidisciplinary management of this syndrome must involve cardiovascular surgeons, cardiologists and geneticists. Nevertheless, the current literature provides a number of treatment options and recommendations for LDS. Therefore, the purpose of this systematic literature review is to analyze and discuss treatment options for aortic injury in patients with LDS, including both prophylactic and emergency interventions.

Keywords: Loeys – Dietz, aortic aneurysm, aortic dissection, management of aortic aneurysm.