WATCHING AND WAITING: WHEN TO REFER THE VALVE PATIENT

JOHN V OLSEN MD SWEDISH MEDICAL CENTER 7/12/19 A RECENT LAWSUIT

• 75 YO male with 1 month progressive DOE and chest discomfort. • “Systolic murmur” noted at local ED and transferred to downtown hospital for cardiology evaluation. • III/VI murmur and exam findings of AS. Echo confirmed AS with 6 m/sec velocity. Cardiac cath showed no CAD. • Discharged x 10 days for outpatient dental workup and pre-op PFTs (normal). • Died quietly at home 3 days before scheduled AVR. • Record review disclosed insurance exam 4 years earlier by ARNP with finding of “II/VI murmur”. CHARACTERIZATION OF MURMURS

• SYSTOLIC OR DIASTOLIC • Timing • Point of maximum intensity • Loudness • Character/Quality • Radiation

GGrading: I Soft IV Loud with thrill II Definite V Very loud with thrill III Loud VI Heard without chest contact FUNCTIONAL MURMURS

• 50-100% of patients of all ages (highest in young). • Systolic, grade II/VI intensity or less. • Peak intensity early in (peak in first 0.2 seconds after onset of QRS). NOT holo/pansystolic • Loudest in pulmonic area (ULSB) and LSB at 3rd-4th interspaces (can be apical in elderly). Mostly of aortic origin. • Twangy or musical “Still’s murmur” of childhood or murmur in neck • No other abnormal findings of heart sounds, diastolic murmurs, etc. STAGES OF VALVULAR DISEASE:

Stage A: At risk for disease

Stage B: Progressive disease

Stage C1: Severe (asymptomatic) – Compensated LV Stage C2: Severe (asymptomatic) – Decompensated LV

Stage D: Severe disease (symptomatic)

AORTIC AORTIC REGURGITATION MITRAL REGURGITATION

• Etiology: 30-40 yo Rheumatic, 50-60 yo Bicuspid, 70-90+ Sclerotic. Average progression 0.3 m/sec/year. • “Cardinal symptoms”: Chest pain, , CHF • Exam: Systolic, harsh/cooing, crescendo-decrescendo (late peaking proportional to degree of stenosis) systolic murmur, URSB/LLSB/apex (15%), radiating to carotids. A2 muffled. Pulses parvus et tardus. Sustained PMI. • PRESSURE OVERLOAD

HJ – 5/29/2019 - 12 HJ – 5/29/2019 - 38 HJ – 7/24/2018 - 23 HJ – 7/24/2018 - 61 Planimetry AV Area 1.1 cm2 DOPPLER FLOW VELOCITY Aortic Stenosis: Maximal Doppler Velocity Peak Gradient = 4 (Vmax AV)2 – Peak Instantaneous Gradient Mean Gradient – Integration of velocity over time – Estimate – 0.7 * Peak Grad. – Correlates with cath Peak-to-Peak gradient Valve Area by continuity equation

Assessment by: Peak Velocity Mean Gradient Valve Area Mild stenosis: 2.0 – 2.9 m/s < 20 mmHg > 1.5 cm2 Moderate stenosis: 3.0 – 3.9 m/s 20 – 39 mmHg 1.0 – 1.5 cm2 Severe stenosis: > 4.0 m/s ≥ 40 mmHg < 1.0 cm2 “Critical” stenosis: > 5.0 m/s < 0.7 cm2

Time for an Update: The Asymptomatic “Slide” Pellikka PA, et al. Circulation (2005) 111:3290

Why?? Progression of Stenosis Worsening LV Hypertrophy Subclinical Diastolic Dysfunction Subclinical Systolic Dysfunction LA Pressure Overload and Congestion Pulmonary Hypertension Patients in denial Doctors in denial When to Operate on an Asymptomatic Patient:

Risk of Operation Consequences of Intervention: • Prosthetic hemodynamics • Prosthetic deterioration • Bleeding / Thrombosis risk • • Death

Risk of Observation Consequences of Medical Rx: • LV dysfunction / CHF • Progression rate • Pulmonary HTN • Stroke • Death

Assessed Comorbidities, “Scores” (STS, Euro), Frailty, Pt. Preference

TRANSCUTANEOUS REPLACEMENT EDWARDS SAPIEN 3 VALVE / PARTNER 3 TRIAL

COREVALVE / EVOLUT TRIAL

Low risk patients non-inferior to SAVR 2014 ACC / AHA GUIDELINES:

Class I

For patients in whom TAVR or high-risk surgical AVR is being considered, a Team consisting of an integrated, multidisciplinary group of healthcare professionals with expertise in VHD, , interventional cardiology, cardiac anesthesia, and should collaborate to provide optimal patient care. (Level of Evidence: C)

Class IIa

TAVR is a reasonable alternative to surgical AVR in patients who meet an indication for AVR and who have high surgical risk for surgical AVR (Level of Evidence: B)

Dentition, dentition, dentition! AORTIC REGURGITATION

• Etiology: , aortic diseases (dissection), bicuspid valve, with AS. Acute vs chronic signs and effects vary. • Symptoms: Dyspnea. • Exam: Murmur is diastolic, high pitched, “blowing”, decrescendo (ends before mid ), at LMSB/LLSB (L 2nd to 4th interspace) or to apex. Heard best leaning forward at end expiration with firm pressure. Widened pulse pressure with abnormal pulses: Corrigan’s (visible) or “water hammer” carotids, Quincke’s (nailbed pulsation), Duroziez’s “to and fro” or “pistol shot of Traube” femoral pulses. PMI displaced.

• VOLUME OVERLOAD

MITRAL REGURGITATION

• Etiology: Myxomatous/MVP (“degenerative”), enlarged LV/annular dilation/altered LV geometry (“functional”), endocarditis, HOCM. • “Mitral regurgitation begets mitral regurgitation” • Symptoms: DOE, CHF, A Fib. • Exam: Murmur is apical radiating to axilla (or in direction of jet). Displaced PMI. Murmur is either: – Late systolic with mid systolic click (MVP) and “snapping” S1S2 or – blowing, holo- or pan-systolic (obliterates S1 and/or S2)

• VOLUME OVERLOAD

LL – 10/14/2014 LL – 5/2/2016 - 3 LL – 5/2/2016 - 59 LL – 10/5/2018 - 25 LL – 10/5/2018 - 32

To measure regurgitant flow rate: Flow rate = 6.28 x r2 x Va Flow rate = 6.28 x .682 x 30 Flow rate = 87 cc

To measure effective regurgitant ERO = flow rate/VMR orifice area: ERO = 87/523 ERO = 0.17 cm2

To measure RV = ERO x TVIMR regurgitant volume: RV = 16 cc

2014 ACC / AHA GUIDELINES: Class I surgery is recommended for symptomatic patients with chronic severe primary MR (stage D) and LVEF greater than 30%

Class I is recommended in preference to MVR when surgical treatment is indicated for patients with chronic severe primary MR limited to the posterior leaflet (Level of Evidence: B)

Class I Mitral valve repair is recommended in preference to MVR when surgical treatment is indicated for patients with chronic severe primary MR involving the anterior leaflet or both leaflets when a successful and durable repair can be accomplished (Level of Evidence: B) Class IIa Mitral valve repair is reasonable in asymptomatic patients with chronic severe primary MR (stage C1) with preserved LV function (LVEF >60% and LVESD <40 mm) in whom the likelihood of a successful and durable repair without residual MR is greater than 95% with an expected mortality rate of less than 1% when performed at a Heart Valve Center of Excellence (Level of Evidence: B) Class IIa Mitral valve repair is reasonable for asymptomatic patients with chronic severe nonrheumatic primary MR (stage C1) and preserved LV function (LVEF >60% and LVESD <40 mm) in whom there is a high likelihood of a successful and durable repair with 1) new onset of AF or 2) resting pulmonary hypertension ( systolic arterial pressure >50 mm Hg) (Level of Evidence: B) STAGES OF VALVULAR DISEASE / MANAGEMENT:

Stage A: At risk for disease OBSERVE Echo Q 3-5 yrs

Stage B: Progressive disease OBSERVE Echo Q 1-3 yrs

Stage C1: Severe (asymptomatic) – Compensated LV ??? Echo Q 6-12 mos

Stage C2: Severe (asymptomatic) – Decompensated LV INTERVENE

Stage D: Severe disease (symptomatic) INTERVENE 2017 AND 2019 FOCUSED GUIDELINE UPDATES:

IE prophylaxis: Antibiotic prophylaxis before dental procedures now is also recommended for patients with transcatheter prosthetic valves, and for patients with prosthetic material used in valve repair (including an annuloplasty ring or artificial chords) (Class IIa, Level of Evidence [LOE] C-LD).

Anticoagulation for : Among patients with atrial fibrillation and rheumatic mitral stenosis, anticoagulation with a vitamin K antagonist still is indicated (Class I, LOE B-NR). Anticoagulation should be used among patients with atrial fibrillation and a

CHA2DS2-VASc score ≥2 in the setting of native aortic valve disease, tricuspid valve disease, or MR (Class I, LOE C-LD). 2017 / 2019 FOCUSED GUIDELINE UPDATES:

Aortic stenosis: The recommendation for either surgical AVR or TAVR among high-risk patients with severe, symptomatic AS (stage D), after consideration by a heart valve team, was changed from Class IIa (LOE B) to Class I (LOE A)

After consideration by a heart valve team, TAVR is a reasonable alternative to surgical AVR for patients with severe, symptomatic aortic stenosis (stage D) and intermediate surgical risk (Class IIa, LOE B- R). 2017 / 2019 FOCUSED GUIDELINE UPDATES:

Primary MR: Among asymptomatic patients with severe primary MR with preserved left ventricular (LV) systolic function (LV ejection fraction [LVEF] >60%, LV end- systolic dimension <40 mm [stage C1]), mitral valve surgery is reasonable in the setting of serial imaging studies that reveal a progressive increase in LV size or decrease in LVEF (Class IIa, LOE C-LD).

Secondary MR: The definition of severe secondary MR is now the same as for severe primary MR (effective regurgitant orifice area ≥0.4 cm2, regurgitant volume ≥60 ml, regurgitant fraction ≥50%). 2017 / 2019 FOCUSED GUIDELINE UPDATES:

Prosthetic valve choice: Shared decision-making remains a Class I indication (LOE C-LD) in selecting a mechanical versus bioprosthetic valve. Among patients undergoing aortic or mitral , the age range was expanded from age 60-70 to age 50-70 for the Class IIa (LOE B-NR) indication for either a mechanical or bioprosthetic valve choice.

Prosthetic valve antithrombotic therapy: There are unchanged recommendations for use of a vitamin K antagonist (international normalized ratio [INR] 2.5 for bileaflet or current-generation tilting disk valves in the absence of additional thromboembolic [TE] risks, INR 3.0 for mitral mechanical prostheses or for aortic valve prostheses plus additional TE risks) plus aspirin 75-100 mg among patients with a mechanical valve prosthesis. There is a new consideration for a lower INR target of 1.5-2.0 for patients with an On-X bileaflet mechanical aortic valve and no additional TE risks (Class IIb, LOE B-R). The prior recommendation for use of a vitamin K antagonist after bioprosthetic valve replacement was changed to include both aortic and mitral bioprosthesis, for 3-6 months after surgery, in patients at low risk for bleeding (Class IIa, LOE B-NR). A vitamin K antagonist (INR target 2.5) may be reasonable for at least 3 months after TAVR in patients at low risk for bleeding (Class IIa, LOE B-NR). RESOURCES • Electronic stethoscopes with ability to record phonocardiograms (WAV file). • Heart Songs 5 (American College of Cardiology) training listening to heart sounds. $150. • Nishimura RA, Otto CM et al. 2014 AHA/ACC Guideline for the Management of Patients with . JACC 63(22): e57- 185 (June 2014). • Nishimura RA, Otto CM et al. 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients with Valvular Heart Disease. JACC 70(2): 252-289 (July 2017). • Nishimura, O’Gara et al. 2019 AATS/ACC/ASE/SCAI/STS Expert Consensus Systems of Care Document: A Proposal to Optimize Care for Patients with Valvular Heart Disease. JASE 32(6): 683-703.

RESOURCES (CONT’D) • Popma JJ et al. Transcatheter Aortic-Valve Replacement with a Self- Expanding Valve in Low-Risk Patients. NEJM 380(18):1706-1715 (May 2019). Evolut Low Risk Trial. • Mack MJ et al. Transcatheter Aortic-Valve Replacement with a Balloon- Expandable Valve in Low-Risk patients. NEJM 380(18): 1695-1705 (May 2019). Partner 3 Trial. • Don Michael, T. Anthony. Auscultation of the Heart: A Cardiophonetic Approach. McGraw Hill 1998. ISBN 0-07-018005-9. • Abrams, Jonathan. Synopsis of Cardiac Physical Diagnosis (2ed). Butterworth Heinemann 2001. ISBN 0-7506-7338-9. • Constant, Jules. Bedside Cardiology (3ed). Little Brown 1985. ISBN 0-316- 15323-0.