Aortic Regurgitation in Connective Tissue Disorders Special precautions? Carlos A. Mestres MD PhD FETCS

Transcription

1 Aortic Regurgitation in Connective Tissue Disorders Special precautions? Carlos A. Mestres MD PhD FETCS Senior Consultant Department of Cardiovascular Surgery University Hospital Zürich (Switzerland) Extraordinary Professor University of the Free State Bloemfontein (South Africa)

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4 Conflict of Interest: NONE

5 Outline 1. The problem and definitions 2. The information 3. The facts 4. My precautions

6 CTD CTD - any disease that affects the parts of the body that connect the structures of the body * Collagen * Elastin Collagen - tendons, ligaments, skin, cornea, cartilage, bone and blood vessels Elastin - major component of ligaments and skin

7 The term "mixed connective tissue disease" (MCTD) * Systemic autoimmune disease typified by overlapping features between two or more systemic autoimmune diseases * Antibodies against the U1 small nuclear ribonucleoprotein autoantigen (U1snRNP) * Central pathogenetic role of anti-u1rnp autoantibodies Tani C et al. Diagnosis and classification of MCTD. J Autoimmun 2014; 48:46-49

8 * Cutis Laxa * Loeys-Dietz Syndrome * Marfan Syndrome * Mixed CTD * Stickler Syndrome * Synovial Chondromatosis * Undifferentiated CTD

9 Niccoló Paganini Genoa Nice EMSN, IFMSO, NMFUS, EuroDIS

10 Loeys BL, Chen J, Neptune ER, Judge DP, Podowski M, Holm T, Meyers J, Leitch CC, Katsanis N, Sharifi N, Xu FL, Myers LA, Spevak PJ, Cameron DE, De Backer J, Hellemans J, Chen Y, Davis EC, Webb CL, Kress W, Coucke P, Rifkin DB, De Paepe AM, Dietz HC. A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet 2005; 37:

11 Definitions

12 precaution noun UK /prɪˈkɔː.ʃən/ US /prɪˈkɑː.ʃən/ an action that is done to prevent something unpleasant or dangerous happening something that is done to try and protect a person or thing from something dangerous or harmful

13 Surgical anatomy Aortic root: Aortic annulus Cusps Sinuses of Valsalva Sinotubular junction

14 Hospital Clínico late HC UB 1991

15 Outline 1. The problem and definitions 2. The information 3. The facts 4. My precautions

16 Aneurysmal dilatation of the ascending aorta is often associated with ectasia of the aortic valve ring and presents clinically as aortic incompetence.

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18 Aortic Valve Pathology Aortic Valve Stenosis Aortic Valve Regurgitation Rheumatic 26% Endocarditis 21 % Aortic root dilatation 23% Bicuspid aortic valve 16% Inflammatory 8%

19 A Problem prob lem / pr bl m; NAmE pr b/ noun, adj. noun 1 a thing that is difficult to deal with or to understand: big / major / serious problems health / family, etc. problems financial / practical / technical problems to address / tackle / solve a problem (especially NAmE) to fix a problem the problem of drug abuse If he chooses Mary it s bound to cause problems. Let me know if you have any problems

20 The Summary CTD present in cardiac surgery * Young patients * Aneurysmal disease * Aortic regurgitation The valve is endangered CTD and AR closely associated

21 Why we do something? * Drugs * Operations The life expectancy of patients with the Marfan syndrome is reduced by complications caused by dilatation of the ascending aorta (Root Aortic Regurgitation)

22 The History and the Data

23 Gott VL, Pyeritz RE, Magovern GJ Jr, Cameron DE, McKusick VA Surgical treatment of aneurysms of the ascending aorta in the Marfan syndrome. Results of composite-graft repair in 50 patients N Engl J Med 1986; 314:

24 N 50 consecutive Composite valved graft Mean age 32.2 Mean aortic diameter 7.1 (5.3-10) Dissection 14 (28%) HM 2% LM 5y 10.2% Actuarial survival 87.5 at 5y Do it prophylactically at 60 mm

25 Ann Thorac Surg 1991;52:3845 N = 400 screened

26 HM = 1%

27 78.5% Late mortality 5.7%

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33 The new confounder

34 J Thorac Cardiovasc Surg 2014;147:

35 AVS % AVR 77 24% FUc 99% Imaging 94% HM 0.6% 1y survival 97% AVR 98% AVS AI>2+ 7% AVS

36 JTCVS Conclusions * No differences in 30-d mortality * No differences in survival, VR morbidity, MAVRE * 7% of AVS >2+ aortic regurgitation * Follow-up mandatory

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39 The populations More elective surgery in AVS Longer AoX time in AVS Longer CPB time in AVS More MV replacement in AVR p<.01 p<.01 p<.01 p<.01

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41 100 MFS 169 operations VSRR 29 (49%) FU 6.5±4 y MRR 30 (51%) FU 8.8±9 y (p=.03) Mean age 33 Reoperation higher in VSRR (Yacoub p=.01) No differences in reoperation MRR VSRR 0.8%/pt/y Neuro events MRR p= % pt/y

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44 J Thorac Cardiovasc Surg 2016;151:

45 N = 165 MFS Age >20 Bentall-De Bono 67 VSRR 98 (69 D 29 Y) Bentall-De Bono Older Larger Aneurysmal size More dissections More AR

46 JTCVS Conclusions After prophylactic root replacement in patients with Marfan syndrome, patients undergoing Bentall and valve-sparing procedures have similar late survival, freedom from root reoperation, and freedom from endocarditis. However, valve-sparing procedures result in significantly fewer thromboembolic and hemorrhagic events.

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50 Morbidity included 3 strokes (1%), 10 perioperative myocardial infarctions (5%), and 8 reoperations for bleeding (4%). Actuarial survival at 5, 10, 15, and 20 years was 93% (95% confidence interval [CI] 88% to 97%), 79% (95% CI 71% to 87%), 67% (95% CI 57% to 79%), and 52% (95% CI 36% to 69%), respectively. Freedom from reoperation was 72% (95% CI 54% to 86%) at 20 years. Complications with anticoagulation occurred in 29 patients; with valve thrombosis, in 2; and with hemorrhage, in 27 (4 life threatening and 23 minor). Freedom from thromboembolism was 91% (95% CI 77% to 98%) at 20 years. Freedom from endocarditis was 99% (95% CI 92% to 100%) at 20 years. Multivariate analysis revealed preoperative mitral valve regurgitation (3 to 4) and older age to be significant predictors of late death (P0.005), and Marfan syndrome, initial valve-preserving aortic root reconstruction, and need for a concomitant procedure at initial operation to be significant predictors of the need for reoperation (P0.01).

51 Conclusions Aortic root replacement for aortic root aneurysms can be done with low morbidity and mortality. Composite valve conduit reconstruction resulted in a durable result. There were few serious complications related to the need for long-term anticoagulation or a prosthetic valve. Reoperation was most commonly required because of failure of the aortic valve when a valve-preserving aortic root reconstruction was performed or for other cardiac or aortic disease elsewhere.

52 Ann Thorac Surg 2007; 83:S N = 35

53 Mean FU 19 months AR>2 11.4%

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57 Ann Thorac Surg 2009; 87:

58 Valve-sparing procedures, particularly us- ing the reimplantation technique with the Valsalva graft, show promise but have not yet proven as durable as the Bentall.

59 Outline 1. The problem and definitions 2. The information 3. The facts 4. My precautions

60 1. MRR and VSRR work very well 2. Low perioperative mortality 3. Low morbidity 4. The Achilles heel of MRR (BB) is TE/Hem 5. The Achilles heel of VSRR is AI 6. Patient population may not be the same for each procedure 7. Limited evidence (Retrospective, size, 1-man show )

61 Calculated annual failure about 1%/yr * Slightly higher for the risk of TE in MRR * Slightly less for the risk with VSRR Cameron DE. EJCTS 2-15; 48: ?

62 I think that we would not necessarily be inclined to perform a VSRR in: * Very severely enlarged annulus (TBD) * Large, thin, stretched cusps with multiple fenestrations and/or partial detachment of the commissures * Careful in very asymmetrical sinus * Cusp repair a problem * BAV if asymmetric and has some calcification, I think we would rather go for the Bentall procedure Schoenhoff F. EJCTS 2-15; 48:

63 Outline 1. The problem and definitions 2. The information 3. The facts 4. My precautions

64 precaution noun UK /prɪˈkɔː.ʃən/ US /prɪˈkɑː.ʃən/ an action that is done to prevent something unpleasant or dangerous happening something that is done to try and protect a person or thing from something dangerous or harmful

65 1. Appropriate selection of the candidate 2. Identify risk 3. Confirm the valve anatomy (fenestrations, thin leaflets ) 4. Confirm AR 5. Understand if concomitant procedures are required (MR, ) 6. Select the VSRR technique (Reimplantation better) 7. MRR and VSRR are good options