Insufficient Evidence?Insufficient Evidence?

Jimmy Klemis, MDJimmy Klemis, MD

Cardiology/CT Surgery Cardiology/CT Surgery ConferenceConference

Case PresentationCase Presentation

49 WM sent for “transplant” evaluation from local 49 WM sent for “transplant” evaluation from local cardiologistcardiologist

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

Case PresentationCase Presentation PMHx: PMHx:

Chronic LBPChronic LBP ObesityObesity OSA/ CPAPOSA/ CPAP Depression/AnxietyDepression/Anxiety Basal Cell CarcinomaBasal Cell Carcinoma

Social:Social: no alcohol, cocaine, tobacco or drugs; married with 1 no alcohol, cocaine, tobacco or drugs; married with 1

teenage son teenage son Meds (at presentation)Meds (at presentation)

Carvedilol 50 bid, Celebrex, Lasix, ASA, anxiolytic, Carvedilol 50 bid, Celebrex, Lasix, ASA, anxiolytic, hydrocodone, Combivent MDIhydrocodone, Combivent MDI

Case PresentationCase Presentation

PE: HR 65 BP 170/67PE: HR 65 BP 170/67 HNT: jvp est 10cm, HNT: jvp est 10cm, CV: nl S1/2, + S3, no S4; PMI displaced CV: nl S1/2, + S3, no S4; PMI displaced

laterally to ant ax; 3/6 diastolic laterally to ant ax; 3/6 diastolic decrescendo m USBdecrescendo m USB

Resp: basilar ralesResp: basilar rales Abd: obese, no ascites/massesAbd: obese, no ascites/masses Ext: tr edema, distal pulses briskExt: tr edema, distal pulses brisk

Case PresentationCase Presentation

Lab: Lab: Chem 142 \ 110 / 14 H/H 16.6/ 46 Chem 142 \ 110 / 14 H/H 16.6/ 46

4.4 / 27 \ 1.4 4.4 / 27 \ 1.4

TSH, LFT, FLP, WBC/PPC, Coags nlTSH, LFT, FLP, WBC/PPC, Coags nl

ECG: NSR, PRWP, nl axis, no ischemiaECG: NSR, PRWP, nl axis, no ischemia

Previous workupPrevious workup

April 2001April 2001 ETT-Sesta: 9.2 METS, (-) ischemia, EF 24%ETT-Sesta: 9.2 METS, (-) ischemia, EF 24% ECHO: dilated LV, conc LVH, EF 40%ECHO: dilated LV, conc LVH, EF 40%

“ “sclerotic” AV, AVA 1.7, minimal AIsclerotic” AV, AVA 1.7, minimal AI June 2001June 2001

R/L Cath: nl coronariesR/L Cath: nl coronariesCI 1.9 L/min CI 1.9 L/min

RA 21/15 RV 76/27 PA 67/25 PCWP 32RA 21/15 RV 76/27 PA 67/25 PCWP 32 PVR 1.0 Wood Units SVR 2812PVR 1.0 Wood Units SVR 2812 Nipride: PA systolic 67Nipride: PA systolic 6747474040

July 2001 – VA consult NP clinicJuly 2001 – VA consult NP clinic Carvedilol refilled, sent back to PCP/Cardiologist.Carvedilol refilled, sent back to PCP/Cardiologist.

Clinical CourseClinical Course

July 2001July 2001 Cardiologist recommends transplant for “idiopathic dilated CMP”Cardiologist recommends transplant for “idiopathic dilated CMP”

September 2001September 2001 22ndnd opinion referral to VAMC opinion referral to VAMC NYHA Class IV, exam with AI – admittedNYHA Class IV, exam with AI – admitted carvedilol, cox-2 d/c’ed; ACEI/diuresis initiated carvedilol, cox-2 d/c’ed; ACEI/diuresis initiated

November 2001November 2001 NYHA Class II, symptomatic improvementNYHA Class II, symptomatic improvement

January 2001January 2001 NYHA Class III despite maximal med RxNYHA Class III despite maximal med Rx Cath : nl coronaries, Ao Root 3+ AICath : nl coronaries, Ao Root 3+ AI

Serial ECHO/clinical findingsSerial ECHO/clinical findings

DateDate LVESDLVESD EFEF AIAI ClinicalClinical

19951995 35 mm35 mm normalnormal moderatemoderate No sxsNo sxs

19971997 46 mm46 mm normalnormal moderatemoderate No sxsNo sxs

9/019/01 6565 40%40% severesevere NYHA IVNYHA IV

11/0111/01 5656 55%55% moderatemoderate NYHA IINYHA II

1/021/02 5757 43%43% moderatemoderate NYHA IIINYHA III

Chronic Aortic InsufficiencyChronic Aortic Insufficiency

EtiologyEtiology PathophysiologyPathophysiology History / Physical FindingsHistory / Physical Findings Natural HistoryNatural History DiagnosisDiagnosis ManagementManagement

EtiologyEtiology Aortic RootAortic Root

Age related dilatationAge related dilatation Medial degeneration/ MarfansMedial degeneration/ Marfans DissectionDissection HTNHTN Other ( osteogenesis imperfecta, Reiters, syphilitic aortitis, Bechet, Other ( osteogenesis imperfecta, Reiters, syphilitic aortitis, Bechet,

psoriatic arthritis, relapsing polychondritis, UC arthritis, AS, giant cell psoriatic arthritis, relapsing polychondritis, UC arthritis, AS, giant cell arteritisarteritis

Aortic ValveAortic Valve RheumaticRheumatic Calcific degenerationCalcific degeneration Congenital (Congenital (BicuspidBicuspid, VSD), VSD) Myxomatous degenerationMyxomatous degeneration EndocarditisEndocarditis Structural degeneration of Bioprosthetic valveStructural degeneration of Bioprosthetic valve Other (SLE, AS, Takayasu, Whipple, Crohns) Other (SLE, AS, Takayasu, Whipple, Crohns)

Anatomy / Pathology

Braunwauld 6th ed

Chronic AI - PathophysiologyChronic AI - Pathophysiology increased LV EDVincreased LV EDV addition of new sarcomeres in series/ elongation of addition of new sarcomeres in series/ elongation of

myocytes and myocardial fibers (Eccentric Hypertrophy)myocytes and myocardial fibers (Eccentric Hypertrophy) enlarged chamber/ increased wall stress is stimulus for enlarged chamber/ increased wall stress is stimulus for

concentric hypertrophyconcentric hypertrophy dilatation and hypertrophy with resultant recruitment of dilatation and hypertrophy with resultant recruitment of

preload reserve allow compensation and maintenance of preload reserve allow compensation and maintenance of LV systolic functionLV systolic function

may be asymptomatic for decades until may be asymptomatic for decades until decompensated state develops, wall thickening unable to decompensated state develops, wall thickening unable to keep pace with hemodynamic load, increased interstitial keep pace with hemodynamic load, increased interstitial fibrosis and decreased compliance fibrosis and decreased compliance symptoms of CHF symptoms of CHF ensueensue

Pressure Volume Relationships in Chronic AIPressure Volume Relationships in Chronic AI

Braunwald 6th ed

CO at rest may approach 25 L/min in severe AI with little increase in EDP very large EDV (Cor Bovinum)

HemodynamicsHemodynamics

Braunwauld 6th ed

Hemodynamic/ AuscultoryHemodynamic/ Auscultory

Braunwauld 6th ed

HistoryHistory DOE, Orthopnea, PNDDOE, Orthopnea, PND

usually after 4usually after 4thth / 5 / 5thth decade and significant decade and significant cardiomegaly and LV dysfxncardiomegaly and LV dysfxn

Angina pectorisAngina pectoris develops later, nocturnal sxs prominent; often with develops later, nocturnal sxs prominent; often with

diaphoresis due to HR slowing with arterial DBP diaphoresis due to HR slowing with arterial DBP falling to low levelsfalling to low levels

Palpitations / Head poundingPalpitations / Head pounding especially in supine position, pounding of heart especially in supine position, pounding of heart

against chest wallagainst chest wall tachycardia from stress/exertion may precipitate and tachycardia from stress/exertion may precipitate and

cause extreme discomfort for ptcause extreme discomfort for pt

From Braunwauld. Cardiovascular Dz, 6th ed.

Physical FindingsPhysical Findings

de Musset signde Musset sign – – head bobbing with heartbeathead bobbing with heartbeat

Corrigan pulseCorrigan pulse – – “water hammer” pulse“water hammer” pulse

Bisferiens pulseBisferiens pulse – – brach/ fem arteriesbrach/ fem arteries Hill signHill sign – – popliteal > brachial by 60mmHgpopliteal > brachial by 60mmHg

Traube signTraube sign – – “pistol shot sounds” over fem artery“pistol shot sounds” over fem artery

Duroziez signDuroziez sign – – sys m when femoral artery compressed proximally sys m when femoral artery compressed proximally and diastolic m when compressed distallyand diastolic m when compressed distally

Quincke sign – Quincke sign – capillary pulsationscapillary pulsations

Apical impulse - Apical impulse - diffuse, hyperdynamic and displaced inf/latdiffuse, hyperdynamic and displaced inf/lat

systolic thrillsystolic thrill – – base/suprasternal notch / carotid arteriesbase/suprasternal notch / carotid arteries

Physical FindingsPhysical Findings

Diastolic murmurDiastolic murmur high frequency, sitting up, leaning forwardhigh frequency, sitting up, leaning forward duration > intensity correlates with severity duration > intensity correlates with severity mild AR – early diastole, hi pitched blowingmild AR – early diastole, hi pitched blowing severe AR – holodiastolic, roughsevere AR – holodiastolic, rough musical (“cooing dove”) – eversion/perforation of Ao cuspmusical (“cooing dove”) – eversion/perforation of Ao cusp Primary valve dz – heard best LSB 3-4 intercostalPrimary valve dz – heard best LSB 3-4 intercostal Ao Root dz – heard best RSBAo Root dz – heard best RSB

Austin Flint murmurAustin Flint murmur mid-late diastolic apical rumble – severe ARmid-late diastolic apical rumble – severe AR

Wide Pulse PressureWide Pulse Pressure Systolic flow murmur (/thrill)Systolic flow murmur (/thrill)

Natural HistoryNatural History

Mortality rate for severe AI+CHF sxs > 20-50%/yr2

Bonow, et al. JACC Nov 1988 2Aronow , et a. Am J Cardiol 1994; 74: 286. l

CXRCXR

ECHOECHO 2D/ M-Mode2D/ M-Mode

AV/ Ao Root anatomic abnormalitiesAV/ Ao Root anatomic abnormalities LV dimension / sphericityLV dimension / sphericity AMVL – fluttering, reverse domingAMVL – fluttering, reverse doming increased EPSSincreased EPSS

DopplerDoppler Color Flow MappingColor Flow Mapping Continuous WaveContinuous Wave Flow reversal in desc Ao (100% sens 97% spec for Flow reversal in desc Ao (100% sens 97% spec for

severe AI)severe AI) Limitations – What is severe AI?Limitations – What is severe AI?

AMVL fluttering

Color Flow – top mild, bottom moderate

Chronic AI

Acute AI

Continuous Wave Doppler

Cardiac CatheterizationCardiac Catheterization

Medical ManagementMedical Management VasodilatorsVasodilators

goal is to reduce SBP, improve forward SV, reduce regurgitant goal is to reduce SBP, improve forward SV, reduce regurgitant volumevolume

UsesUses severe AR + sxs/ LV dysfxnsevere AR + sxs/ LV dysfxn short term hemodynamic improvement in pt with symptomatic short term hemodynamic improvement in pt with symptomatic

AR before AVRAR before AVR prolong compensated phase of asymptomatic patientsprolong compensated phase of asymptomatic patients No indication for asymptomatic pt with mild AI and normal LV No indication for asymptomatic pt with mild AI and normal LV

fxnfxn Studied in AIStudied in AI

Nifedipine, Hydralizine, ACEI, Nipride, PrazosinNifedipine, Hydralizine, ACEI, Nipride, Prazosin Children/ severe AR – ACEI reversed LV dilatation/wall stressChildren/ severe AR – ACEI reversed LV dilatation/wall stress avoid (-) inotrope in LV dysfxnavoid (-) inotrope in LV dysfxn

Effect of Nifedipine in pt with severe Effect of Nifedipine in pt with severe asymptomatic AR and nl LV fxnasymptomatic AR and nl LV fxn

Scognamiglio, et al. NEJM 1994;331:689-694

Medical ManagementMedical Management

Rx CHF – diuretics, aldactone, digRx CHF – diuretics, aldactone, dig avoid vigorous exertion if symptomatic AIavoid vigorous exertion if symptomatic AI control diastolic BP (increases regurg)control diastolic BP (increases regurg) avoid BB - prolong diastole, increase ARavoid BB - prolong diastole, increase AR

Paradigm Shifts…

Timing of SurgeryTiming of Surgery

Goal is to intervene before irreversible LV Goal is to intervene before irreversible LV systolic dysfxn ensuessystolic dysfxn ensues initially reversible, mainly due to afterload initially reversible, mainly due to afterload

excess – full recovery in LV size/fxn possibleexcess – full recovery in LV size/fxn possible with progressive chamber dilatation, with progressive chamber dilatation,

decreased myocardial contractility >> decreased myocardial contractility >> afterload excess as cause of LV dysfxn.afterload excess as cause of LV dysfxn.

associated with worse recovery of LV fxn and associated with worse recovery of LV fxn and increased mortalityincreased mortality

Surgical TherapySurgical Therapy

Indications for AVR (Severe AR)Indications for AVR (Severe AR)11

Sxs (NYHA III-IV) regardless of LV fxnSxs (NYHA III-IV) regardless of LV fxn Sxs (NYHA II) with evidence of progressing LV Sxs (NYHA II) with evidence of progressing LV

dysfxn ( LV ESD ~ 55, LV EF <50-55%)dysfxn ( LV ESD ~ 55, LV EF <50-55%) Angina (CHA Class II or higher) w or w/o CADAngina (CHA Class II or higher) w or w/o CAD mild-mod LV dysfxn (EF 25-49%) regardless of sxsmild-mod LV dysfxn (EF 25-49%) regardless of sxs mod-sev AR and undergoing CABG or other valvular mod-sev AR and undergoing CABG or other valvular

surgerysurgery Predictors of Postoperative PrognosisPredictors of Postoperative Prognosis

LV systolic functionLV systolic function LV End Systolic Size ( LV ESD)LV End Systolic Size ( LV ESD)

1 Bonow, et al. Circulation 1998;98:1949-84

Bonow, et al. JACC Nov 1998

Postoperative MortalityPostoperative 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

Summary of Surgical TimingSummary of Surgical Timing

Asymptomatic, nl LV size/fxnAsymptomatic, nl LV size/fxn Asymptomatic, ESD >55 EF < 50-55%Asymptomatic, ESD >55 EF < 50-55%

serial exam/ measurements q 2-4 mosserial exam/ measurements q 2-4 mos Symptomatic, mild-mod LV dysfxnSymptomatic, mild-mod LV dysfxn Symptomatic, severe LV dysfxnSymptomatic, severe LV dysfxn

Hi surgical risk, but worse with med Rx Hi surgical risk, but worse with med Rx (mortality 20-50%)(mortality 20-50%)

individualizeindividualize

Post Operative ConsiderationsPost Operative Considerations

Preload kept high immediate postop Preload kept high immediate postop period to fill dilated LVperiod to fill dilated LV

temporary IABP use may be necessary temporary IABP use may be necessary until LV fxn improves early post opuntil LV fxn improves early post op

Surgical OptionsSurgical Options

Ao Root diseaseAo Root disease annuloplasty or other valve sparing surgery annuloplasty or other valve sparing surgery

possible if pure Ao Root dzpossible if pure Ao Root dz Primary AV diseasePrimary AV disease

valve replacementvalve replacement

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.)

AV sparing conduitAV sparing conduit

Braunwauld 6th ed

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

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.

Key PointsKey Points

Severe AR is clinical dxSevere AR is clinical dx murmur, pulse pressure, etcmurmur, pulse pressure, etc ECHO – flow reversal desc AoECHO – flow reversal desc Ao

Serial clinical/ noninvasive followupSerial clinical/ noninvasive followup Med Rx: ACEI/Vasodilator; avoid exertion/BBMed Rx: ACEI/Vasodilator; avoid exertion/BB Predictors of worse prognosis in Severe AR Predictors of worse prognosis in Severe AR “55” rule“55” rule

LV ESD >55mmLV ESD >55mm LV EF < 50-55%LV EF < 50-55%

Mortality HF/Severe AR 20-50% 1yrMortality HF/Severe AR 20-50% 1yr Individualize therapy and tailor to pt presentationIndividualize therapy and tailor to pt presentation

55Saves Lives