Insufficient Evidence?

1. Insufficient Evidence? Jimmy Klemis, MD Cardiology/CT Surgery Conference

2. Case Presentation • 49 WM sent for “transplant” evaluation from local cardiologist • HPI – DOE x 6mos-1year, insidious onset, also with L sided Chest tightness when tired/stressed and occasionally awakens him at night, last 1-2hrs and relieved with anxiolytics. Occasional lightheadedness after taking Coreg. Denies PND, Orthopnea, cough, pre/syncope. Former maintenance worker, now on medical leave. Pt states trying to remain active (walking/ swimming) but limited by dyspnea

3. Case Presentation • PMHx: • Chronic LBP • Obesity • OSA/ CPAP • Depression/Anxiety • Basal Cell Carcinoma • Social: • no alcohol, cocaine, tobacco or drugs; married with 1 teenage son • Meds (at presentation) • Carvedilol 50 bid, Celebrex, Lasix, ASA, anxiolytic, hydrocodone, Combivent MDI

4. Case Presentation • PE: HR 65 BP 170/67 • HNT: jvp est 10cm, • CV: nl S1/2, + S3, no S4; PMI displaced laterally to ant ax; 3/6 diastolic decrescendo m USB • Resp: basilar rales • Abd: obese, no ascites/masses • Ext: tr edema, distal pulses brisk

5. Case Presentation • Lab: • Chem 142 \ 110 / 14 H/H 16.6/ 46 4.4 / 27 \ 1.4 TSH, LFT, FLP, WBC/PPC, Coags nl ECG: NSR, PRWP, nl axis, no ischemia

6. Previous workup • April 2001 • ETT-Sesta: 9.2 METS, (-) ischemia, EF 24% • ECHO: dilated LV, conc LVH, EF 40% “sclerotic” AV, AVA 1.7, minimal AI • June 2001 • R/L Cath: nl coronaries CI 1.9 L/min RA 21/15 RV 76/27 PA 67/25 PCWP 32 PVR 1.0 Wood Units SVR 2812 Nipride: PA systolic 674740 • July 2001 – VA consult NP clinic • Carvedilol refilled, sent back to PCP/Cardiologist.

7. Clinical Course • July 2001 • Cardiologist recommends transplant for “idiopathic dilated CMP” • September 2001 • 2nd opinion referral to VAMC • NYHA Class IV, exam with AI – admitted • carvedilol, cox-2 d/c’ed; ACEI/diuresis initiated • November 2001 • NYHA Class II, symptomatic improvement • January 2001 • NYHA Class III despite maximal med Rx • Cath : nl coronaries, Ao Root 3+ AI

8. Serial ECHO/clinical findings

9. Chronic Aortic Insufficiency • Etiology • Pathophysiology • History / Physical Findings • Natural History • Diagnosis • Management

10. Etiology • Aortic Root • Age related dilatation • Medial degeneration/ Marfans • Dissection • HTN • Other ( osteogenesis imperfecta, Reiters, syphilitic aortitis, Bechet, psoriatic arthritis, relapsing polychondritis, UC arthritis, AS, giant cell arteritis • Aortic Valve • Rheumatic • Calcific degeneration • Congenital (Bicuspid, VSD) • Myxomatous degeneration • Endocarditis • Structural degeneration of Bioprosthetic valve • Other (SLE, AS, Takayasu, Whipple, Crohns)

11. Anatomy / Pathology Braunwauld 6th ed

12. Chronic AI - Pathophysiology • increased LV EDV • addition of new sarcomeres in series/ elongation of myocytes and myocardial fibers (Eccentric Hypertrophy) • enlarged chamber/ increased wall stress is stimulus for concentric hypertrophy • dilatation and hypertrophy with resultant recruitment of preload reserve allow compensation and maintenance of LV systolic function • may be asymptomatic for decades until decompensated state develops, wall thickening unable to keep pace with hemodynamic load, increased interstitial fibrosis and decreased compliance  symptoms of CHF ensue

13. Pressure Volume Relationships in Chronic AI CO at rest may approach 25 L/min in severe AI with little increase in EDP very large EDV (Cor Bovinum) Braunwald 6th ed

14. Hemodynamics Braunwauld 6th ed

15. Hemodynamic/ Auscultory Braunwauld 6th ed

16. History • DOE, Orthopnea, PND • usually after 4th / 5th decade and significant cardiomegaly and LV dysfxn • Angina pectoris • develops later, nocturnal sxs prominent; often with diaphoresis due to HR slowing with arterial DBP falling to low levels • Palpitations / Head pounding • especially in supine position, pounding of heart against chest wall • tachycardia from stress/exertion may precipitate and cause extreme discomfort for pt From Braunwauld. Cardiovascular Dz, 6th ed.

17. Physical Findings • de Musset sign – head bobbing with heartbeat • Corrigan pulse – “water hammer” pulse • Bisferiens pulse – brach/ fem arteries • Hill sign – popliteal > brachial by 60mmHg • Traube sign – “pistol shot sounds” over fem artery • Duroziez sign – sys m when femoral artery compressed proximally and diastolic m when compressed distally • Quincke sign – capillary pulsations • Apical impulse - diffuse, hyperdynamic and displaced inf/lat • systolic thrill – base/suprasternal notch / carotid arteries

18. Physical Findings • Diastolic murmur • high frequency, sitting up, leaning forward • duration > intensity correlates with severity • mild AR – early diastole, hi pitched blowing • severe AR – holodiastolic, rough • musical (“cooing dove”) – eversion/perforation of Ao cusp • Primary valve dz – heard best LSB 3-4 intercostal • Ao Root dz – heard best RSB • Austin Flint murmur • mid-late diastolic apical rumble – severe AR • Wide Pulse Pressure • Systolic flow murmur (/thrill)

19. Natural History Mortality rate for severe AI+CHF sxs > 20-50%/yr2 2Aronow , et a. Am J Cardiol 1994; 74: 286. l Bonow, et al. JACC Nov 1988

20. CXR

21. ECHO • 2D/ M-Mode • AV/ Ao Root anatomic abnormalities • LV dimension / sphericity • AMVL – fluttering, reverse doming • increased EPSS • Doppler • Color Flow Mapping • Continuous Wave • Flow reversal in desc Ao (100% sens 97% spec for severe AI) • Limitations – What is severe AI?

22. AMVL fluttering Color Flow – top mild, bottom moderate

23. Continuous Wave Doppler Chronic AI Acute AI

24. Cardiac Catheterization

25. Medical Management • Vasodilators • goal is to reduce SBP, improve forward SV, reduce regurgitant volume • Uses • severe AR + sxs/ LV dysfxn • short term hemodynamic improvement in pt with symptomatic AR before AVR • prolong compensated phase of asymptomatic patients • No indication for asymptomatic pt with mild AI and normal LV fxn • Studied in AI • Nifedipine, Hydralizine, ACEI, Nipride, Prazosin • Children/ severe AR – ACEI reversed LV dilatation/wall stress • avoid (-) inotrope in LV dysfxn

26. Effect of Nifedipine in pt with severe asymptomatic AR and nl LV fxn Scognamiglio, et al. NEJM 1994;331:689-694

27. Medical Management • Rx CHF – diuretics, aldactone, dig • avoid vigorous exertion if symptomatic AI • control diastolic BP (increases regurg) • avoid BB - prolong diastole, increase AR

28. Paradigm Shifts…

29. Timing of Surgery • Goal is to intervene before irreversible LV systolic dysfxn ensues • initially reversible, mainly due to afterload excess – full recovery in LV size/fxn possible • with progressive chamber dilatation, decreased myocardial contractility >> afterload excess as cause of LV dysfxn. • associated with worse recovery of LV fxn and increased mortality

30. Surgical Therapy • Indications for AVR (Severe AR)1 • Sxs (NYHA III-IV) regardless of LV fxn • Sxs (NYHA II) with evidence of progressing LV dysfxn ( LV ESD ~ 55, LV EF <50-55%) • Angina (CHA Class II or higher) w or w/o CAD • mild-mod LV dysfxn (EF 25-49%) regardless of sxs • mod-sev AR and undergoing CABG or other valvular surgery • Predictors of Postoperative Prognosis • LV systolic function • LV End Systolic Size ( LV ESD) 1 Bonow, et al. Circulation 1998;98:1949-84

31. Bonow, et al. JACC Nov 1998

32. Postoperative Mortality - Operative Mortality Rate 3-8% - Late Mortality 5-10%/yr in survivors with preop marked cardiomegaly and /or prolonged preop LV dysfxn Braunwauld 6th ed

33. Summary of Surgical Timing • Asymptomatic, nl LV size/fxn • Asymptomatic, ESD >55 EF < 50-55% • serial exam/ measurements q 2-4 mos • Symptomatic, mild-mod LV dysfxn • Symptomatic, severe LV dysfxn • Hi surgical risk, but worse with med Rx (mortality 20-50%) • individualize

34. Post Operative Considerations • Preload kept high immediate postop period to fill dilated LV • temporary IABP use may be necessary until LV fxn improves early post op

35. Surgical Options • Ao Root disease • annuloplasty or other valve sparing surgery possible if pure Ao Root dz • Primary AV disease • valve replacement

36. AV sparing conduit Figure 46-42 Repair of the aortic valve in patient with severe AR. Conduit tailoring in the supravalvular position. The conduit is cut to replace three (left), two (middle), or one (right) individual sinuses. The aortic aneurysm is replaced and the valve is spared. (From David TE, Feindel CM, Bos J: Repair of the aortic valve in patients with aortic insufficiency and aortic root aneurysm. J Thorac Cardiovasc Surg 109:345, 1995.) Braunwauld 6th ed

37. Figure 29-16 A. Carpentier-Edwards Supra- annular porcine bioprosthesis. B. Hancock II porcine bioprosthesis. C. Hancock modified orifice porcine bioprosthesis. D. St. Jude Medical Bioimplant porcine bioprosthesis. Figure 29-15 A. Björk-Shiley Monostrut mechanical prosthesis. B. Sorin Allcarbon monoleaflet mechanical prosthesis. C. Medtronic-Hall mechanical prosthesis. D. Omnicarbon mechanical prosthesis. Edmunds. Cardiac Surgery in the Adult. Ch 29

38. Key Points • Severe AR is clinical dx • murmur, pulse pressure, etc • ECHO – flow reversal desc Ao • Serial clinical/ noninvasive followup • Med Rx: ACEI/Vasodilator; avoid exertion/BB • Predictors of worse prognosis in Severe AR “55” rule • LV ESD >55mm • LV EF < 50-55% • Mortality HF/Severe AR 20-50% 1yr • Individualize therapy and tailor to pt presentation

39. 55 Saves Lives