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? I ‘m refer erre red by by my my gyneco ecolo logist st, , I’m 20 weeks ks pregn egnant ant Ao sinus 45 mm Ao ascendens 34 mm 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? I ‘m refer erre red by by my my gyneco ecolo logist st, , I’m 15 weeks eks preg egna nant nt Ao sinus 41 mm Ao ascendens 33 mm 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 Maternal mortality > 15% ! Retrospective literature review 1983-2002; n = 50 Immer et al. Ann Thorac Surg 2003

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

Aortic dissection in pregnancy NIS database Rajagopalan et al. Int J Obst Anesth 2014 Sawlani et al. JACC 2015

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 Average aortic root growth was 0.42 mm/year (SE 0.05) in men and 0.38 mm/year (SE 0.04) in women Meijboom et al Am J Cardiol 2005

Aortic root growth in pregnant Marfan patients + 3 mm during pregnancy course Retrospective registry n = 98 Donnelly et al JACC 2012

Aortic root growth in pregnant Marfan patients Prospective cohort study n =127; 61 pregnancies Meijboom et al Eur Heart J 2005

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? N = 1097 pts, 1996-2010; 82 % on betablocker Jondeau et al Circulation 2012

Safety zone ? Acute type A dissection IRAD data N=591 There is no absolute safe diameter ! Linda A. Pape et al. Circulation 2007

Long term outcome after pregnancy Retrospective review N= 98 pts, 199 pregnancies Donnelly et al JACC 2012

Turner syndrome: cardiac risks Incidence risk of aortic event 1,4% Risk factors: Bicuspid aortic valve Aortic coarctation Arterial hypertension Aortic dilatation Turner syndrome Aortic dissection in 33% of patients with ASI > 25 mm/m² within 3 years of follow up ( N = 166 TS, prospective study) Matura et al Circulation 2007 Bondy Curr Opin Cardiol 2008

Turner syndrome: pregnancy Risk of maternal mortality during pregnancy 2% Multicenter retrospective study French Study Group for Oocyte Donation Chevalier JCEM 2011

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? Population based retrospective study N = 124 TS pts Hagmann et al Human Reproduction 2013

Turner syndrome: true risks? Bernard et al Human Reproduction 2016

Bicuspid aortic valve aortopathy Retrospective study Mayo Clinic N= 88 female BAV pts Mc Kellar et al Am J Cardiol 2011

Managing the risks Preconception Pregnancy Delivery Postpartum Counseling! Long term effect on Echocardiographic monitoring Imaging with MRI MRI without gadolinium if needed mother and child Optimisation status TTE / MRI Betablockers /replace ARB’s pre pregnancy Foetal monitoring 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 Shores J et al. N Engl J Med 1994 Donnelly et al JACC 2012

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