Curr Probl Cardiol

Mitral Stenosis: Evaluation and Management; I Am Interested in Knowing How you Manage Stenosis When it is Secondary to Annular Calcification

Sunil Mankad, MD, FACC, FCCP, FASE Associate Professor of Medicine Mayo Clinic College of Medicine Director, Transesophageal Echocardiography Associate Director, Cardiology Fellowship Mayo Clinic, Rochester, MN [email protected] @MDMankad Disclosures

Relevant Financial Relationship(s) None

Off Label Usage Valve in Valve Procedures Objectives

• Pearls and pitfalls in the assessment of mitral stenosis • 2D echo • Doppler • 3D echo •Mitral Annular Calcification Mitral Stenosis

“Fish-mouth” appearance Mitral Stenosis: Symptoms

• Left sided heart failure (although contractility of the LV is usually normal) • Dyspnea on exertion • Orthopnea • Paroxysmal nocturnal dyspnea • Right sided heart failure • Patients often asymptomatic until he/she becomes pregnant or develops atrial fibrillation Mitral Stenosis: “Disease of Plateaus”

Bruce CL and Nishimura R. Curr Probl Cardiol 1998 Once class III or IV symptoms develop, the prognosis is grim with a 15% 10-year survival with medical therapy M-mode Echocardiography Mitral Stenosis • 2D TTE and TEE • Planimetry • Doppler Pressure half-time • Color Doppler Methods • Formula for Elipse • PISA • Continuity equation • Measurements required • LVOT diameter [2-D] • LVOT velocity, TVI [PW] • Peak MV TVI [CW] TTE: 2D and color Doppler

• Doming of AML • “Hockey-stick” appearance Mitral Valve Area by Planimetry

• Hatle L et al. Noninvasive assessment of pressure drop in mitral stenosis by Doppler ultrasound. Br Med J 1978 • Concept first described by Libanoff and Rodbard in 1966 Pressure Half-time

5 220 MVA = =Deceleration Time x 0.29 PHT 4 •the time required for the velocity to 3 Velocity drop to ½ the peak pressure (m/s) 2

0 MV Mean Gr = 4 mmHg Case MVA Pressure ½ Time = 1.2 cm2 The Doppler Angle of Interrogation was Sub-optimal

• Question 1. This will overestimate the MVA by PHT 2. This will underestimate the MVA by PHT 3. This will not effect the MVA calculation by PHT Doppler Angle of Incidence Does Not Influence MVA by PHT

Stoddard MF, Prince CR, Tuman WL, Wagner SG. Am Heart J 1994. 27:1562 Where should we draw the pressure half-time ? B A C

- Figure from Davidoff R.

PHT by Doppler

• Measurement Problems • PHT is problematic when velocity profile is curvilinear A B

A=130 ms B= 232 ms • 62 year old female; history of rheumatic fever • Dyspnea on exertion, NYHA functional class III • PND/Orthopnea Apical 3 Chamber

Mean MV Gradient = 20 mmHg at HR of 72 BPM MVA by PHT = 2.5 cm2 Coexistent Aortic Regurgitation What is the Effect of Aortic Regurgitation on MVA Calculation by PHT ? A. No effect B. Overestimation of MVA C. Underestimation of MVA Controversy in the 1980’s

• AR lengthens PHT (underestimate of MVA) • Hoffman A. et al. Br Heart J 1986;55:283. • AR shortens PHT (overestimate of MVA) • Nakatani et al. Circulation 1988;77:78. • AR has little effect on PHT • Grayburn PA et al. Am J Cardiol 1987;60:322. PHT and AR

Flachskampf F.....Thomas JD, JACC;16;1990:396-404 PHT and AR: Importance of LV Compliance

Flachskampf F.....Thomas JD, JACC;16;1990:396-404 What is the Effect of Aortic Regurgitation on MVA Calculation by PHT ?

A. No effect B. Overestimation of MVA C. Underestimation of MVA Planimetry

Planimetry MVA = 1.2 cm2

PHT MVA = 2.5 cm2

-Gonzalez MA, Child JS, Krivokapich. Am J Cardiol 1987;60:327-32 -Loperfido D et al. Br Heart J 1987;57:348-55 -Robson DJ et al. Eur Heart J 1985;6:791-4 -Riggs TW et al. J Am Coll Cardiol 1983;1:873-8. MVA by Pressure ½ Time Method: Pitfalls • Suboptimal Doppler signal • Marked Tachycardia • Aortic Regurgitation • Very High Left Atrial Pressure • Acute changes in LA compliance (i.e., immediately post valvuloplasty) • AV block/arrhythmias – alters timing of atrial contribution and E-wave configuration • Nonlinear (convex/concave) pressure decay- use mid-diastolic slope and extrapolate Formula for Eliptical Area • Color Doppler Flow Analysis

Not affected by: 1)MR 2)AR 3)LV Function 4)Atrial Fibrillation

Kawahara T et al. J Am Coll Cardiol 1991;18:87 Color Doppler Method

π * 0.9 * 2.0= 1.4 cm2 4 PISA Method EOA=2πr2v/Vmax Mitral Valve Area by PISA Rodriguez L et al. Circulation 1993;88:1157-1165

2 α° MVA = 6.28 x r x alias velocity x Peak MS velocity 180° α = 100 MVA By Continuity Equation May not be used if significant regurgitation of aortic or mitral valve present 2

x 0.785 x MVA = Doppler Assessment Mean Gradient • Dependent on • Heart Rate • Cardiac Output • Mitral Regurgitation • Trace MV inflow signal (CW)

Report HR @ time of mean gradient measurement Echo/Doppler: Historical Severity of Mitral Stenosis

MVA PHT Mn Gradient (cm2) (msec) (mmHg)

Normal 4.0 - 6.0 40 - 70

Mild 1.6 - 2.0 90 - 150 5

Moderate 1.1 - 1.5 150 - 219 6 - 10

Severe < 1.0 > 220 > 10+ Grading the Severity of Mitral Stenosis

Stage Definition Anatomy Hemodynamics Consequences Symptoms A At risk Doming Normal None None B Progressive Doming, MVA >1.5cm2 Mild-mod LAE None Commissural PHT <150ms fusion C Severe Doming, MVA ≤1.5cm2 Severe LAE None Asymptomatic Commissural MVA ≤ 1cm2 (very RVSP > 30 mmHg fusion severe) PHT ≥ 150ms PHT ≥ 220ms (very severe) *MG > 5-10mmHg D Severe Doming, MVA ≤ 1.5cm2 Severe LAE Decrease Symptomatic Commissural MVA ≤ 1cm2 (very RVSP > 30mmHg exercise fusion severe) tolerance PHT ≥ 150ms Dyspnea PHT ≥ 220ms (very severe) *MG > 5-10mmHg

Nishimura RA et al. ACC/AHA Guidelines Circ 2014 Use of 3D Echo in Mitral Stenosis 3D Guided 2D Planimetry Mitral Stenosis: Different Results Depending on Method Sugeng L…Lang RM. J Am Soc Echocardiogr 2003;1292-1300 Mitral Annuloplasty Score Score < 8 associated with a more favorable outcome

- Wilkins GT et al. Br Heart J 1988 - Oh JK et al. Echo Manual 2007 3rd Edition Echocardiographic Assessment of Commissural Calcium

Abascal score ≤ 8 Calcification absent

Abascal score > 8 Calcification present

No commissural calcium statistically significant Probability Probability of Survival (%) survival (86% vs 40%) Probability of Survival (%) free of all events combined (82% vs 38%) vs pts with commissural calcification P<0.001 0 0 0 0.5 1 1.5 2 2.5 3 0 0.5 1 1.5 2 2.5 3 Years from Procedure Years from Procedure

Cannan CR, Nishimura RA, Reeder GS et al. J Am Coll Cardiol 1997;29:175-80 Assessment of Commissural/Leaflet Calcification 2D Echo 3D Live TEE

View from LV JACC Imaging 2009;2:1-7. Real-time 3-D TTE and Mitral Balloon Valvuloplasty

Sugeng L…Lang RM. J Am Soc Echocardiogr 2006;19:413-421 View from LV Perspective

Pre Post LA Appendage Thrombus • May account for up to 45- 50% of all cardiogenic thromboemboli • TEE shows evidence of LA thrombi in 14% of patients with acute AF, 27% in chronic AF, 43% with AF and clinical thromboembolism • Appear free-floating with distinct edges • Associated with depressed LAA Doppler velocities and spontaneous echo contrast (smoke) • Associated with depressed LV function • Generally near the distal end of the LAA Case • 86 y/o with class IV CHF • S/P AVR→Core-Valve (Germany) • S/P MVR with a CE valve (2000) • Severe mitral prosthesis stenosis

MG = 13 mmHg Melody Valve • Bovine jugular venous valve segment • Platinum-Iridium stent Mitral Valve-in-Valve Therapy Transapical Approach Echo Guided Procedure Melody in Mitral Valve-in-Valve

Mean Gradient 5 mmHg Mitral Annular Calcification (MAC)

• A chronic, degenerative process in the fibrous base of the mitral valve • Associated with an increased incidence of cardiovascular disease, mitral valve disease, arrhythmias, and mortality • Influences the outcomes of cardiac surgery and interventions Mitral Annular Calcification

LV RV

Mean Gradient 6 mmHg, HR 70 BPM Case • 85 year old female with class IIIB heart failure • Hx of CABG and mechanical AVR at age 75 • Bypass grafts patient; normal AVR • Now with severe mitral stenosis and moderate mitral regurgitation • LV EF 75% • Turned down for redo surgery at Mayo Clinic • Referred valve in valve procedure TEE

Mean Doppler Gradient 18 mmHg at HR 64 BPM 3D TEE

LA VIEW LV VIEW 23 mm Sapien Bioprosthesis Implantation with ECMO Support TEE: Post-Implantation

Mean Doppler Gradient 4 mmHg at HR 63 BPM 3D TEE: View from LA 64 Year Old Woman s/p CVA → Echo for SOE

-Courtesy Gerard P. Aurigemma, MD, FASE MV Mean GR = 3 mmHg at HR 60 BPM

Caseous MAC Caseous Calcification of the Mitral Annulus ‘Toothpaste’ tumor • Rounded, echodense, and immobile • Posterior aspect of the mitral annulus • A solid mass, containing toothpaste-like, white material • The central areas of lucency represent liquefaction necrosis • Found in 0.63% of autopsies of patients with mitral annular calcification (Harpaz et al) Caseous MAC

Courtesy of Dr. Roberto Lang Conclusions • Use a multi-modal approach when assessing mitral stenosis by 2D/3D echocardiography and Doppler → Don’t rely on a single method • Correlate multiple techniques to increase diagnostic confidence • Planimetry • Pressure Half-Time • Proximal Flow Convergence • Continuity Equation • Color Flow Area • Consider functional test • Mitral Annular Calcification Thank You! [email protected] @MDMankad