title: Loeys-Dietz syndrome and pregnancy: The first ten years.
abstract: The physiological and haemodynamic changes that occur in pregnancy and the postpartum period increase the risk of aortic dissection. Loeys-Dietz syndrome results from mutations in the genes encoding components of the TGF-β signalling pathway; aortic pathology is of particular concern in this condition but other vascular abnormalities can also be present. Significant maternal morbidity and mortality has been described in patients with Loeys-Dietz syndrome, but successful and uncomplicated pregnancies are still possible. Nevertheless, all patients with this condition should, at present, be treated as very high risk in pregnancy and the postpartum period, until reliable risk prediction tools become available. This review summarises the recent advances in the understanding of the pathophysiology of this condition, and the management strategies currently advocated.
authors: Consultant Obstetric Physician, Oxford University Hospitals NHS Foundation Trust, UK. Electronic address: charlotte.frise@ouh.nhs.uk.
authors: Charlotte J
authors: CJ
authors: Frise
authors: Clinical Lecturer, Division of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, UK.
authors: Alex
authors: A
authors: Pitcher
authors: Consultant Obstetric Physician, Oxford University Hospitals NHS Foundation Trust; Honorary Senior Clinical Lecturer, University of Oxford, UK.
authors: Lucy
authors: L
authors: Mackillop
mesh_terms: D000784:Aortic Dissection / Q000453:epidemiology / Q000503:physiopathology* / Q000517:prevention & control; D001014:Aortic Aneurysm / Q000453:epidemiology / Q000503:physiopathology* / Q000517:prevention & control; D005260:Female; D006801:Humans; D055947:Loeys-Dietz Syndrome / Q000453:epidemiology / Q000503:physiopathology* / Q000628:therapy*; D011247:Pregnancy; D018566:Pregnancy, High-Risk / Q000502:physiology*; D011295:Prenatal Care / Q000379:methods / Q000639:trends*; D013997:Time Factors
keywords: Aortic dissection; High-risk pregnancy; Loeys-Dietz syndrome