Pregnancy in Marfan Syndrome and Bicuspid Aortic Valve Related Aortopathy

Transcription

1 Pregnancy in Marfan Syndrome and Bicuspid Aortic Valve Related Aortopathy Heidi M. Connolly, MD Cardiac Problems in Pregnancy Venice Italy, February 2014 No Disclosures 2012 MFMER slide-1

2 Regitz-Zagrosek V, Lundqvist C, Borghi C, et al. European Heart Journal MFMER slide-2

3 Marfan Syndrome Preexisting medial disease Physiologic, hormonal changes Unpredictable maternal risk Aortic dissection, rupture Fetal - 50% inheritance (AD) 2012 MFMER slide-3

4 Congenitally Bicuspid Valve 2012 MFMER slide-4

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6 20-Year-Old Female with MFS Pre-pregnancy counseling FH of Marfan and dissection Metoprolol Asymptomatic Ao root 41 mm Echo and CT 2012 MFMER slide-6

7 What would you recommend? Counsel patient against pregnancy 2012 MFMER slide-7

8 Preconceptual Counseling In addition to routine obstetric screening Detailed CV history, FH, meds, exam, genetic eval Echo aorta and valves CT/MR to look at entire aorta Aortic dimension MFS BAV/aortopathy No pregnancy >45 mm >50 mm OK if low risk <40 mm <45 mm Individualize mm mm ESC Pregnancy Guidelines: EHJ MFMER slide-8

9 Preconceptual Counseling Postop MFS Patient Elective operation Composite graft Anticoagulation challenges Bioprosthesis function Valve sparing limited data Concern AR, adjacent aorta 2012 MFMER slide-9

10 Preconceptual Counseling Postop BAV Patient Elective operation BAV function Aorta adjacent to graft 2012 MFMER slide-10

11 Advise Against Pregnancy? 2012 MFMER slide-11

12 Preconceptual Counseling in MFS Advise against pregnancy Prior aortic dissection Aortic dimension >45 mm Aortic dimension 45 mm FH of ao dissection High risk features ESC Pregnancy Guidelines: EHJ MFMER slide-12

13 Preconceptual Counseling in BAV Advise against pregnancy Prior aortic dissection Aortic dimension >50 mm* Aortic dimension 50 mm FH of ao dissection High risk features *ESC Pregnancy Guidelines: EHJ MFMER slide-13

14 Key Point 2012 MFMER slide-14

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16 22-Year-Old Female with MFS 26 Weeks Pregnant 30 Weeks Pregnant Sinus = 47 mm Confirmed by MRI Sinus = 53 mm 2012 MFMER slide-16

17 What would you recommend? Additional imaging 2012 MFMER slide-17

18 22-Year-Old Female with MFS Additional Imaging MRI Aortic Root = 47 mm 2012 MFMER slide-18

19 Key Point 2012 MFMER slide-19

20 Prenatal Multidisciplinary Care Frequent OB and CV visits (individualize) Echo every 4 8 weeks 10 mm ao consider elective surgery Beta-blocker metoprolol Anesthesiology consult 2012 MFMER slide-20

21 Delivery Challenges in MFS Lumbosacral Dural Ectasia Epidural Concerns >70% MFS pt lumbosacral dural ectasia Increase in CSF volume Risk of CSF leak with dural puncture Buser et al: Int J Obstet Anesth MFMER slide-21

22 Risks of Pregnancy OB complication rate up to 40% Preterm delivery (24-36 weeks) in 15% Neonatal mortality 7% Singh et al: J Obstet Gynecol 2008 Meijboom et al: Int J Cardiology 2006 Goland et al: Circulation MFMER slide-22

23 Delivery Management in MFS and BAV Vaginal delivery for pt with ao 40 mm Cesarean OB reasons, ao root >45 mm, ao during preg, prior dissection repair Delivery at Center with CV surgery Antibiotic prophylaxis ESC Pregnancy Guidelines: EHJ MFMER slide-23

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25 Aortic Dissection in Pregnancy 1-5% with normal aortic root in MFS 10% in high-risk patients Maternal mortality rate up to 50% Repair during pregnancy <28 wk dissection repair alone 28 wk Cesarean and repair Goal - save 2 lives Lachandani et al: Eur J Obstet Gyn 2003 Lipscomb et al: Br. J OB Gyn MFMER slide-25

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27 Postpartum Aortic dissection risk persists postpartum Oxytocin possibly implicated in dissection* Postpartum hemorrhage risk Gelpi et al: J Cardiovasc Surg 2008 Goland et al: Circulation 2009 *Dietz et al - unpublished 2012 MFMER slide-27

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30 Long-term Cardiovascular Outcomes Variable Pregnant (n = 69) Nulliparous (n = 29) P= value Elective surgery Adverse outcome 13% (9) 6.5% (2) P = % (16) 0% (0) P=0.002 Donnelly et al: JACC MFMER slide-30

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32 Pregnancy in MFS and BAV Aortopathy Take Home Points Make the diagnosis Risk stratify prior to pregnancy Multimodality imaging Regular FU, medical therapy Facilitated delivery at experienced center Dissection risk persists postpartum (MFS)* 2012 MFMER slide-32

33 Questions & Discussion 2012 MFMER slide-33