Annual meeting of the BWGACHD 2016

What to do?

Ao sinus 45 mm Ao ascendens 34 mm

I ‘m referred by my gynecologist, I’m 20 weeks pregnant

Latest check up 1,5 year ago with echo and CT : Ao sinus 41 mmNo betablocker due to subjective intoleranceFather aortic dissection at 42 years, sudden death at 47 yearsUncle elective Bentall procedure at 46 years

What to do?

Ao sinus 41 mm Ao ascendens 33 mm

I ‘m referred by my gynecologist, I’m 15 weeks pregnant

Latest check up 0,5 year ago with echo and MRI : Ao sinus 41 mmBetablocker since diagnosis at 15 years of ageKnown de novo mutation

We are being warned…..

Aortic dissection in women: a rare disease

0,4/100 000 person years in female population < 45 years

Associated with an underlyingcondition > 50 % of cases

Thalmann et al. Eur J Cardiothorac Surg 2011; 39: 159-63

Januzzi et al. Results from IRAD JACC 2004; 43:663-9

Aortic dissection in pregnant women

Immer et al. Ann Thorac Surg 2003

Retrospective literature review 1983-2002; n = 50

Maternal mortality > 15% !

Aortic dissection in pregnancy

Rajagopalan et al. Int J Obst Anesth 2014Literature review 2003-2013; n= 75

Aortic dissection in pregnancy

Rajagopalan et al. Int J Obst Anesth 2014

Sawlani et al. JACC 2015

NIS database

Aortic dissection during pregnancy: rare but deathly disease

CEMACH, London 2008

Overall death rates per million maternities UK, 2003–05.

Roos-Hesselink J W et al. Heart

.

Maternal mortality: cardiac causes

Changes in aortic root duringnormal pregnancy

Hyperplasia of smooth muscle cells in the tunica mediaFragmentation of reticulin fibers => elastic fibers less organisedAortic root growth with 1 mm

Manalo-Estrella Arch Pathol 1967

Aortopathy in Marfan syndrome

Meijboom et al Am J Cardiol 2005

Average aortic root growth was 0.42 mm/year (SE 0.05) in men and 0.38 mm/year (SE 0.04) in women

Aortic root growth in pregnant Marfan patients

Donnelly et al JACC 2012

+ 3 mm during pregnancy course

Retrospective registry n = 98

Aortic root growth in pregnant Marfan patients

Meijboom et al Eur Heart J 2005Prospective cohort study n =127; 61 pregnancies

Aortic dissection during pregnancy

1% risk if aortic root < 40 mm

10 % risk if aortic root > 40 mm

Low risk up to 45 mm

Time of diagnosis, use of betablockers; preventive surgery

Risk of dissection > 45 mm?

Immer et al. Ann Thorac Surg 2003

Risk of dissection > 45 mm?

Jondeau et al Circulation 2012

N = 1097 pts, 1996-2010; 82 % on betablocker

Safety zone ?

Acute type A dissectionIRAD dataN=591

Linda A. Pape et al. Circulation 2007

There is no absolute safe diameter !

Long term outcome after pregnancy

Donnelly et al JACC 2012

Retrospective review N= 98 pts, 199 pregnancies

Turner syndrome: cardiac risks

Aortic dissection in 33% of patients with ASI > 25 mm/m² within 3 years of follow up ( N = 166 TS, prospective study)

Risk factors:

Bicuspid aortic valveAortic coarctationArterial hypertensionAortic dilatationTurner syndrome

Matura et al Circulation 2007

Bondy Curr Opin Cardiol 2008

Incidence risk of aortic event 1,4%

Turner syndrome: pregnancy

Risk of maternal mortality during pregnancy 2%

Chevalier JCEM 2011Multicenter retrospective study French Study Group for Oocyte Donation

Aortic dissection in pregnancy

High maternal mortality of aortic dissection in TS patients!

Turner syndrome: recommendations for pregnancy

Contra-indications for pregnancy:

- Untreated / uncontrolled hypertension- Previous aortic coarctation or dissection- Aortic size index > 25 mm/m²- Aortic size > 35 mm- Previous aortic surgery- BAV = risk factor

French Study Group for Oocyte Donation

Turner syndrome: recommendations for pregnancy

Contra-indications for pregnancy:

- Any significant abnormality on cardiac MRI- Aortic size index > 20 mm/m²- Turner syndrome as relative contra-indication

Practice Committee of the American Society forReproductive Medicine (ASRM) 2012

Turner syndrome: true risks?

Hagmann et al Human Reproduction 2013

Population based retrospective studyN = 124 TS pts

Turner syndrome: true risks?

Bernard et al Human Reproduction 2016

Bicuspid aortic valve aortopathy

Mc Kellar et al Am J Cardiol 2011

Retrospective study Mayo ClinicN= 88 female BAV pts

Managing the risks

Preconception Pregnancy Delivery Postpartum

Counseling!Imaging with MRIOptimisation status pre pregnancy

Echocardiographic monitoringMRI without gadolinium if neededBetablockers /replace ARB’sFoetal monitoring

Long term effect on mother and childTTE / MRI postpartum

Elective surgery pre-pregnancySyndrome Indication elective surgery pre

pregnancy

Marfan ≥ 45 mm

Ehlers-Danlos ≥ 45 mm (except type IV)

Turner ≥ 27 mm/m²

Loeys-Dietz ≥ 45 mm (40 mm)

FTAAD ≥ 45 mm

Bicuspid aortic valve ≥ 50 mm

Others ≥ 50 mm

Prophylactic surgery preconception never reduces the risks of acute aortic dissection to the level of the normal population !

Pharmacological treatment

Donnelly et al JACC 2012Shores J et al. N Engl J Med 1994

Delivery

Expert opinion≤ 40 mm vaginal delivery with low threshold forepidural anesthesia40-45 mm: individual basis; assisted vaginal delivery> 45 mm: caesarean section

Sudden death in Marfan: notalways aortic dissection

N = 70 Marfan patientsMedian follow up 6 years4% sudden death

Yetman et al. JACC 2003; 41:329-32.

Sudden death in Marfan: notalways aortic dissection

Yetman et al. JACC 2003; 41:329-32.

Conclusion

team plan

Joint care, multidisciplinaryCounsellingExtensive preconception evaluationFrequent maternal and foetal follow upProspective studies on betablocker useand outcome in high risk situations