1. Valvular Heart Disease: Ambulatory Monitoring and Surgical Referral Dr. Shane Shapera Previous version: Dr. Wassim Saad for AIMGP October 2006

2. Valvular Heart Disease Focus on: Aortic stenosis Chronic mitral regurgitation Mitral Stenosis (extra slides if time permits) Who should have an Echo? At first contact? Follow up? How do we interpret the results? How should we follow patients? How often should they be seen in clinic? Are there options for medical management? When should we refer to a surgeon?

3. References New ACC/AHA Practice Guidelines: ACC/AHA 2006 Practice Guidelines for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Management of Patients With Valvular Heart Disease) JACC. 2006;48(3):598-675.

4. Reminder Class I: There is evidence and/or general agreement that a given procedure or treatment is useful and effective Class II: There is conflicting evidence and/or a divergence of opinion about the usefulness/efficacy of a procedure or treatment IIa: Weight of evidence/opinion is in favor of usefulness/efficacy IIb: Usefulness/efficacy is less well established by evidence/opinion. Class III: There is evidence and/or general agreement that the procedure/treatment is not useful and in some cases may be harmful.

5. A new referral 62 year old man RFR: Family MD “heard a murmer” PMH: HTN and obesity Meds: ASA and Norvasc HPI: Told he has a murmer 2 years ago No symptoms of CVS or respiratory nature Limited physical activity, but no limitations

6. A new referral O/E: HR: 88 BP: 150/85 JVP normal Harsh midsystolic ejection murmer (3/6) over aortic area – radiates to the clavicle Normal pulses Remainder of physical exam normal

7. Questions to think about: Is a 2D-Echo appropriate in this patient? Which patients require an echo? Are there any symptoms that need to be considered when deciding whether to order an echo?

8. Recommendations for Echocardiography in Asymptomatic Patients With Cardiac Murmurs Indication Class Diastolic, Continuous, Holosystolic or late Systolic murmurs I Grade 3 or greater midsystolic murmursI Murmurs with ejection click or radiation to neck or back I Murmurs associated with abnormal physical findings on cardiac palpation or auscultation IIa Murmurs associated with an abnormal ECG or chest x-ray IIa ≤Grade 2 midsystolic murmur (Innocent)III

9. Recommendations for Echocardiography in Asymptomatic Patients With Cardiac Murmurs Indication Class Diastolic, Continuous, Holosystolic or late Systolic murmurs I Grade 3 or greater midsystolic murmursI Murmurs with ejection click or radiation to neck or back I Murmurs associated with abnormal physical findings on cardiac palpation or auscultation IIa Murmurs associated with an abnormal ECG or chest x-ray IIa ≤Grade 2 midsystolic murmur (Innocent)III

10. Recommendations for Echocardiography in Symptomatic Patients With Cardiac Murmurs IndicationClass Signs / symptoms of CHF, MI, ischemia or syncopeI Signs / symptoms of endocarditisI Signs / symptoms of thromboembolismI Signs/ symptoms likely due to noncardiac disease but cardiac disease not excluded by standard cardiovascular evaluation IIa

11. Back to the case… CXR: normal ECG: LVH (aVL > 11mm) ECHO: AVA 1.4cm 2 with a mean gradient 30mmHg Mild concentric LVH Grade I LV Final interpretation: moderate AS

12. Aortic Stenosis: Causes Two most common causes of AS: Calcification of the valve Older patients Very similar to an atherosclerotic process Bicuspid aortic valve Younger patients Mechanical abnormality leading to degeneration

13. Aortic Stenosis: Natural History Prognosis mnemonic: A-S-D

14. Aortic Stenosis: Classification AVAGradientJet Velocity Mild AS< 1.5 cm 2 < 25 mmHg< 3 m/s Moderate AS1.0 – 1.5 cm 2 25 – 40 mmHg3 – 4 m/s Severe AS< 1.0 cm 2 > 40 mmHg> 4 m/s Critical AS< 0.75 cm 2 variableVariable, but often > 5 m/s

15. Questions to think about for our patient with moderate AS What is his expected prognosis? How should he be followed? When should we offer a surgical intervention? Is there medical therapy we can offer?

16. Aortic Stenosis: Follow-up Expect prolonged latency period Low M&M while asymptomatic Progression of stenosis is highly variable Treatment based largely on symptoms Average survival 2-3 yrs once symptoms Risk of sudden cardiac death once symptoms

17. Aortic Stenosis: Follow-up ACC Guidelines suggest “frequent monitoring” of asymptomatic patients looking for: Symptoms of angina, syncope and SOB Signs of CHF (raised JVP, SOA, crackles)

18. Aortic Stenosis: Follow-up When should you repeat an ECHO? Changing signs or symptoms Patient becomes pregnant Routine follow-up Mild AS:q 3-5 years Moderate AS:q 1-2 years Severe AS:q 1 year

19. Aortic Stenosis: When to operate? Indication for Aortic Valve RepairClass Severe AS (AVA <1.0cm 2 ) with symptomsI Severe AS undergoing CABG or CVS surgeryI Severe AS and impaired LV function (LVEF < 50%)I Moderate AS undergoing CABG or CVS surgeryIIa Asymptomatic patients: - critical AS if operative mortality very low (<1%) - severe AS with exercise induced hemodynamic changes - severe AS and risk of rapid progression (age, calcified, CAD) IIb

21. Aortic Stenosis: Medical Therapy Baloon Valvotomy Not an alternative to valve replacement Many complications (10%) Most get restenosis in 6 – 12 months Can be used as a bridge to OR Can be used for palliation in non-operative pts

22. Aortic Stenosis: Medical Therapy No medical therapy prolongs life Theoretical benefit of statins, but trials –ve so far Treatment of CHF can reduce symptoms Diuretics, ACEi, Digoxin have all been used Atrial fibrillation worsens symptoms Needs aggressive rate control or cardioversion All patients should be considered for OR Age is NOT a contraindication to surgery, but increases risk of complications

23. Back to the case… 62M with asymptomatic moderate AS Annual history and physical exam Repeat Echo q 1-2 years or if symptoms Discuss prognosis and possibility of valve replacement in future if symptoms develop Cover with endocarditis prophylaxis for dental and surgical procedures

24. Next referral 68 year old woman RFR: Murmur heard on routine physical exam PMH: previous smoker x 20 pk yrs Meds: Vitamin D and Calcium HPI: Very poor physical fitness Occasional SOB while running for the bus No chest pain or pre-syncope Mild SOA and a couple of episodes of PND

25. Next referral O/E: HR: 92 BP: 138/78 JVP normal Soft S1 with holosystolic murmer at the apex (2/6) that radiates to the axilla Mild SOA Remainder of physical exam normal Is a 2D-Echo appropriate in this patient?

26. Recommendations for Echocardiography in Asymptomatic Patients With Cardiac Murmurs Indication Class Diastolic, Continuous, Holosystolic or late Systolic murmurs I Grade 3 or greater midsystolic murmursI Murmurs with ejection click or radiation to neck or back I Murmurs associated with abnormal physical findings on cardiac palpation or auscultation IIa Murmurs associated with an abnormal ECG or chest x-ray IIa ≤Grade 2 midsystolic murmur (Innocent)III

27. Recommendations for Echocardiography in Symptomatic Patients With Cardiac Murmurs IndicationClass Signs/symptoms of CHF, MI, ischemia or syncopeI Signs / symptoms of endocarditisI Signs / symptoms of thromboembolismI Signs/ symptoms likely due to noncardiac disease but cardiac disease not excluded by standard cardiovascular evaluation IIa

28. Back to the case… CXR normal ECG normal PFT’s normal ECHO Grade II LV with regional variability Severe MR Slightly dilated LA (42mm) LV end systolic dimension ~42mm RVSP 65mm Hg

29. Mitral Regugitation: Natural History May be asymptomatic for many years Chronic severe MR tends to increase over time Usually leads to symptoms within 6 – 10 years Eventually develop overload with LV dysfunction  Chronic volume overload state  LV dysfunction  Increased LV end-systolic volume  LV dilatation and higher LV pressures  Pulmonary congestion  Symptoms of CHF

30. Mitral Regurgitation: Classification No specific numbers to memorize Multiple components go into determining severity (chamber sizes, LVEF, RVSP, visual assessment, etc…) Final report: Mild, Moderate or Severe

31. Questions to think about for our patient with moderate MR What baseline tests should we do? How should we follow her up? When should we reassess her symptoms? When should we repeat the Echo? How can we treat her medically? When should we refer her for surgery?

32. Mitral Regurgitation: Baseline Establish clear exercise tolerance Be sensitive to subtle changes suggesting CHF Baseline ECG and CXR Chamber enlargement and complications (Afib) ECHO Assess severity and look for possible anatomical causes of MR (Ischemic vs. functional) Exercise testing Consider exercise measurements of PAP and MR if exercise capacity can’t be established on history

33. Mitral Regurgitation: Follow-up Asymptomatic mild MR (normal LVEF, chamber sizes & RVSP) Annual history and physical No repeat Echo unless symptoms develop (Class III) Asymptomatic moderate MR Annual history and physical Repeat Echo annually (Class I)

34. Mitral Regurgitation: Follow-up Asymptomatic severe MR History and physical q6months Watch carefully for development of symptoms Repeat Echo q6months (Class I) Watch for Echo evidence of asymptomatic LV dysfunction which would be an indication for OR

35. Mitral Regurgitation: When to operate? General Principles (Class I evidence)* MV repair is preferred over MV replacement Acute severe symptomatic MR needs surgical repair Chronic severe MR should be referred to CVSx if: Symptomatic (NYHA≥II) and LV function preserved (LV ≥ Gr3) Symptomatic (NYHA≥II) and LV end-systolic dimension enlarged Asymptomatic with any of the following: LV dysfunction, LV dilatation, pulmonary HTN or new-onset atrial fibrillation Isolated MV surgery is not indicated for patients with mild to moderate MR (Class III) *please see guidelines for complete list of recommendations

36. Mitral Regurgitation: Medical Therapy Asymptomatic chronic MR No specific therapy recommended No evidence for vasodilators (despite the logical appeal) Chronic MR secondary to ischemic or dilated CM Preload reduction is beneficial (Lasix) LV dysfunction present Usual therapies for LV failure (BB, ACEi, Biventricular Pacing) Atrial Fibrillation Usual therapy with rate control and anticoagulation

37. Back to the case… 68 year old woman Minimal physical activity, but occasionally SOB on exertion Severe MR with Grade 2 LV, regional wall motion abnormalities and pulmonary hypertension

38. Back to the case… History, physical, ECG and CXR done Don’t add much to the picture Further workup? Possible ischemic etiology? Exercise tolerance unclear Regional wall motion abnormalities on Echo Stress echo MR worse with exercise with reversible inferior wall motion abnormality

39. Back to the case… Management Pt is a surgical candidate NYHA II with preserved LV function (Class I) Refer for CVSx opinion, TEE & Cath Treat medically while waiting for OR Rx for symptoms, underlying CAD and LV dysfunction with ASA, BB, ACEi, Statin & Lasix Hold ASA 5 days pre-op

40. Summary History and physical exam guide decision to do an Echo during first visit Many patients have valvular lesions for years before they develop symptoms Patients should be followed clinically and radiologically at intervals that vary according to the specific valve pathology and severity Symptomatic or severe asymptomatic valvular lesions requires an early surgical opinion

41. Extras… If time permits, continue with slides on mitral stenosis otherwise they can serve as a reference for residents

42. Last referral 57 year old man RFR: Murmur heard on routine physical exam PMH: No cardiac risk factors Meds: None HPI: Asymptomatic O/E: Loud S1, Low pitched rumbling diastolic murmur with pre- systolic accentuation at the apex. No opening snap.

43. Does this patient need an Echo? Indication Class Diastolic, Continuous, Holosystolic or late Systolic murmurs I Grade 3 or greater midsystolic murmursI Murmurs with ejection click or radiation to neck or back I Murmurs associated with abnormal physical findings on cardiac palpation or auscultation IIa Murmurs associated with an abnormal ECG or chest x-ray IIa ≤Grade 2 midsystolic murmur (Innocent)III

44. Back to the case… The echo shows an MVA of 1.3cm 2. Chamber size and function are normal RVSP and gradient across valve normal CXR and ECG normal Pt is asymptomatic

45. Questions about MS? What should you do at your initial assessment? How should you follow up this patient? When would you refer to a surgeon? When would you repeat the Echo? How can you manage these patients medically?

46. Mitral Stenosis: Natural History Asymptomatic patient with MS 10 yr survival >80% 60% have no progression of symptoms Significant limiting symptoms 10 yr survival rate of 0-15% If severe pulmonary HTN Mean survival <3 yr

47. Mitral Stenosis: Classification MildModerateSevere Valve area> 1.5 cm 2 1.0 – 1.5 cm 2 < 1.0 cm 2 PAP< 30 mmHg30 – 50 mmHg> 50 mmHg Mean Gradient < 5 mmHg5 – 10 mmHg> 10 mmHg

48. Mitral Stenosis: Initial Exam Hx – dyspnea, hemoptysis, hoarseness, CHF, thromboembolism, endocarditis P/E – evidence of Afib, pulmonary HTN, CHF CXR – heart size, pulmonary edema ECG – rhythm, LAE, RVH due to pulmonary HTN

49. Mitral Stenosis: Initial Echo Baseline Echocardiogram Doppler to assess severity Determines valve area, RVSP and gradient Visualize leaflets and commissures Determines timing and type of interventions Assesses for other valvular lesions Chamber size & function Consider exercise Echo if clinical picture doesn’t fit with Echo findings

50. Follow Up - Asymptomatic Mild MS (MVA > 1.5cm 2) Patients remain stable for years Should be seen annually and have CXR, ECG Modearte-Severe MS (MVA ≤ 1.5cm 2 ) Initially, assess valve morphology & look for pulmonary HTN (PAP > 50mmHg) If intervention is not appropriate, then follow as for mild MS (above)

52. Follow up – When to re-Echo? Repeat any time symptoms change Routine follow-up Mild MS –repeat Echo q 3-5 years Mod MS – repeat Echo q 1-2 years Severe MS –repeat Echo q 1 year

53. Who Should be Referred to a Surgeon? IndicationClass NYHA ≥ II with moderate or severe MS (MVA≤1.5cm 2 ) I Asymptomatic patient with moderate or severe MS (MVA≤1.5cm 2 ) who have pulmonary HTN (PAP>50) I Asymptomatic patient with moderate or severe MS (MVA≤1.5cm 2 ) who have new onset atrial fibrillation IIb Interventions: balloon valvotomy, repair, replacement

54. Medical Management - MS CHF Salt restriction and intermittent diuretics See MD immediately if sudden onset SOB May have A-fib with flash pulmonary edema Exertional symtpoms Consider –ve chonotropic agents (BB,CCB) Atrial fibrillation Rate control and anticoagulation May consider anticoagulation if stroke w/o Afib

55. Back to the case… 57 year old man Asymptomatic with mild MS (MVA 1.3cm 2 ) Annual history and physical Repeat Echo q3-5yrs or sooner if symptoms Call EMS immediately if acute onset SOB Endocarditis prophylaxis for dental and surgical procedures

56. Questions?