Annual meeting of the BWGACHD 2016 What to do? I m refer erre red - - PowerPoint PPT Presentation

Annual meeting of the BWGACHD 2016

What to do?

Ao sinus 45 mm Ao ascendens 34 mm

I ‘m refer erre red by by my my gyneco ecolo logist st, , I’m 20 weeks ks pregn egnant ant

Latest check up 1,5 year ago with echo and CT : Ao sinus 41 mm No betablocker due to subjective intolerance Father aortic dissection at 42 years, sudden death at 47 years Uncle elective Bentall procedure at 46 years

What to do?

Ao sinus 41 mm Ao ascendens 33 mm

I ‘m refer erre red by by my my gyneco ecolo logist st, , I’m 15 weeks eks preg egna nant nt

Latest check up 0,5 year ago with echo and MRI : Ao sinus 41 mm Betablocker since diagnosis at 15 years of age Known 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 underlying condition > 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 2014

Literature 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 during normal pregnancy

Hyperplasia of smooth muscle cells in the tunica media Fragmentation of reticulin fibers => elastic fibers less organised Aortic 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 2005

Prospective 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 dissection IRAD data N=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 valve Aortic coarctation Arterial hypertension Aortic dilatation Turner 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 2011

Multicenter 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 for Reproductive Medicine (ASRM) 2012

Turner syndrome: true risks?

Hagmann et al Human Reproduction 2013

Population based retrospective study N = 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 Clinic N= 88 female BAV pts

Managing the risks

Preconception Pregnancy Delivery Postpartum

Counseling! Imaging with MRI Optimisation status pre pregnancy Echocardiographic monitoring MRI without gadolinium if needed Betablockers /replace ARB’s Foetal monitoring Long term effect on mother and child TTE / MRI postpartum

Elective surgery pre-pregnancy

Syndrome 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 2012 Shores J et al. N Engl J Med 1994

Delivery

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

Sudden death in Marfan: not always aortic dissection

N = 70 Marfan patients Median follow up 6 years 4% sudden death

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

Sudden death in Marfan: not always aortic dissection

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

Conclusion

team plan

Joint care, multidisciplinary Counselling Extensive preconception evaluation Frequent maternal and foetal follow up Prospective studies on betablocker use and outcome in high risk situations