Obstetric Management of Loeys-Dietz Syndrome

Abstract

Women with Loeys-Dietz syndrome warrant special consideration in obstetric management secondary to the risk for aortic dissection. It is recommended that a multidisciplinary team with knowledge about connective tissue disorders and expertise in aortic surgery coordinate maternal obstetric and cardiovascular care.

Clinical features and complications of Loeys-Dietz syndrome: A systematic review

“…Previous reviews have been limited to small sample of patients with LDS in pregnancy, limiting their generalisability (80). In case reports of LDS in pregnancy we identified 21 women with LDS who underwent 27 pregnancies (58,(81)(82)(83)(84)(85)(86)(87)(88)(89)(90)(91)(92)(93)(94)(95)(96)(97). In cohort studies, we identified a further 201 women with LDS who underwent 495 pregnancies (1,12,16,27,46,48,(98)(99)(100)(101).…”

Maternal health and pregnancy outcome in diagnosed and undiagnosed Marfan syndrome: A registry‐based study

“…Moreover, we cannot completely exclude that there may be women with Loeys‐Dietz syndrome or other rare connective tissue diseases among women who clinically were considered as having MFS. The handling of these patients is very much like the clinical care taking of women with MFS (Russo et al, 2018).…”

A Postpartum Type A Dissection

“…These guidelines oversimplify this rather complex disease process, failing to take into account the specific underlying aortic pathology and the family history. Aortic dissections have been described in pregnant patients with normal-sized aortas in Marfan and Loeys Dietz syndromes ( 9 , 10 ). Irrespective of pregnancy, one study demonstrated that patients with Marfan syndrome who did not have aortic complications were found to have a mean aortic dimension of 3.3 cm, which is well below what one would consider normal dimensions assuming an average height and body surface area in these patients ( 11 ).…”