Arterial Switch Operation Long-term outcomes - all good? Daniel Tobler Adult congenital heart disease University Hospital Basel

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1 Arterial Switch Operation Long-term outcomes - all good? Daniel Tobler Adult congenital heart disease University Hospital Basel

2 Arterial Switch Operation Long-term outcomes - all good? No conflict of interest for this presentation

3 Transposition of the Great Arteries all good? Atrial Switch All good?? historical worrisome lethal disease Birth 1 month 5 years 10 years 20 years 40 years

4 Reported cardiac outcomes in adults after ASO n 20y survival adult deaths median FU as adults Tobler et al JACC % years Kempny et al Int J Cardio N/A 3 2( cardiac) 9years Khairy et al Circ age > 14 % 97 1 (cardiac) NR Puras et al R Esp Card % 96 0 NR

5 Reported residual lesions in adults after ASO Intervention for PS ARD AR AVR Tobler et al JACC /65 34mm 48-25mm mild 52% 0moderate 0 Kempny et al Int J Cardio /146 54%dilated 3pts > 45 mm mild 22% 5% moderate 1 age 17 Khairy et al Circ 2013 NR 62%PS/RVOTO 31±8mm 18pts > 40 mm mild 47% 3% moderate 2 age unknown Puras et al R Esp Card /37 36> 16% mm mild 22% 3% moderate 0

6 Reported cardiac status in adults after ASO NYHA I LVEF MVO2 Tobler et al JACC 2010 % 100 % 60 55% < 14%.% 73±14 Kempny et al Int J Cardio 2012 % 92 3% III % 59 55% < 10% NR Khairy et al Circ 2013 % 97 0III % 60 55% < 4% % 86±14 Puras et al R Esp Card 2014 % 93 0III % 62 45% < 2.5% NR

7 All good? Source Internet

8 Paradigma shift in care Long-term outcome Lifelong management

9 4 challenges in lifelong management Villafañe et al, JACC The absence of current consensus regarding the appropriate interval and modality for surveillance imaging

10 Follow-up recommendations at least annually at least every 2 years FU* Echo BW CXR CPET CMR/CT Holter ASO BL BL BL NR consensus recommendation of WATCH

11 Surveillance imaging Echo LV-Function, aortic regurgitation and aortic root dilation, PA-stenosis

12 Surveillance imaging CMR LV/RV - Function/Volumes; PA s, Coronaries, Aorta

13 Surveillance imaging Ischemia? Tobler et al. J Cardiovasc Mag Res 2014

14 Surveillance imaging Ischemia? Tobler et al. J Cardiovasc Mag Res 2014

15 Surveillance imaging Care track for ischemia Surgical history: mode of reimplantation Cardiovascular risk factors (BW 5y) BL Clinics ECG: Signs of Ischemia Echo: regional wall motion, EF CPET: MVO2, O2-Pulse CMR baseline: branch PA, perfusion )?( cct baseline: coronary anatomy BL WATCH, unpublished

16 4 Challenges in lifelong management Villafañe et al, JACC Lack of defined management strategy when subclinical anatomic or physiologic abnormalities are identified

17 Challenges in lifelong management Intima fibrosis Aspirin? Statines? Interventions? Pedra et al. JACC 2005

18 Is ASO an additional risk factor for atherosclerosis? Modifiers of calculated total cardiovascular risk

19 Challenges in lifelong management Persistent neo-aortic growth during adulthood van der Bom et al. Heart 2014

20 Challenges in lifelong management 9pts with aortic root reoperation (2.3% of ASO patients) Surgery? 5.5cm? 6.0cm? Koolbergen D et al. Eur J Card-Thorac Surg 2014

21 Challenges in lifelong management Aortic root aneurysm Surgery? 5.5cm? 6.0cm? Depending on AR? Scheewe et al. Ann Thorac Surg 2013

22 Challenges in lifelong management Koolbergen D et al. Eur J Card-Thorac Surg 2014

23 Challenges in lifelong management Scheewe et al. Ann Thorac Surg 2013

24 4 Challenges in lifelong management Villafañe et al, JACC The rarity of symptoms attributable to potential complications (especially coronary obstruction)

25 Challenges in lifelong management Sympathetic denervation of the coronary arteries with ASO

26 4 Challenges in lifelong management Villafañe et al, JACC The unknown effects of acquired coronary artery disease superimposed in manipulated coronary arteries

27 Challenges in lifelong management Reduced coronary blood flow reserve Impaired myocardial blood flow under stress Hauser et al. Circ 2001

28 Challenges in lifelong management Impaired coronary reactivity w/wo Nitroglycerin Gagliardi et al. JACC 2005

29 Anticipatory care Akesson et al, JACC 2014

30 Anticipatory care

31 All good? almost 100% survival into adulthood after ASO very good long-term outcomes» but what about lifelong outcomes? Paradigma shift the aortic root seems to be a real and progressive threat the coronary fate is still speculative anticipatory care (lifestyle choices, cholesterol, hypertension and exercise) is key in lifelong follow-up care

32 Arterial Switch Operation Long-term outcomes - all good? Daniel Tobler Adult congenital heart disease University Hospital Basel

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