Valvular Heart Disease

1. Valvular Heart Disease John G. McGinnity M.S., P.A.-C. Associate Professor Wayne State University Detroit, Michigan jmcginnity@wayne.edu

2. Heart Valve disease • Aortic • Mitral • Tricuspid • Pulmonic • 95% of all hospital discharges related to valvular heart disease is left sided. • AVD 50.1% • MVD 44.9%

3. Normal Aortic Valve P A T M

4. Aortic Stenosis • Congenital- • Bicuspid Aortic valve • 2% of population Non-Congenital Rheumatic Heart Disease Degenerative Calcific • Uncommon in industrialized countries • Leading cause of AS in Third world • Most common acquired valvular stenosis • Majority of patients are men (80%)

5. Aortic Valve and Aging Normal pressure in aorta High pressure LV Pressure gradient across narrowed aortic valve

6. From Sokolow M, McIlroy MB, Cheitlin MD: Clinical Cardiology, Appleton & Lange, 1990

8. Aortic Stenosis Etiologies Bicuspid Rheumatic Calcific

9. Aortic Stenosis • Typically develops 60’s, 70’s, 80’s • As the disease progresses calcification and fibrosis lead to leaflet stiffness and reduced systolic opening • The area must be reduced to one-fourth its original size before clinically significant obstruction occurs

10. Aging of the Population

11. Aortic Stenosis • Hallmark Symptoms • Angina, syncope, and exertional dyspnea

12. Aortic Stenosis • Physical Examination • Systolic ejection murmur radiating to the neck • As severity increases murmur peaks later in systole and can become softer as CO decreases • Diminished carotid upstroke • Loss of A2 component of second heart sound***

13. Diagnostic Work-up • ECG • CXR • Echo ***Stress testing is contraindicated in symptomatic patients and should be used only with caution in asymptomatic patients.See the guidelines established by the American College of Cardiology

14. Echocardiogram in AS • Determine left ventricular dimensions and note hypertrophy • Measure transvalvular pressure gradient • Estimate valve area • Estimate ejection fraction

15. Mild AS <30 mmHg gradient AVA >1.5cm2 Encourage patient to live a normal life Moderate AS > 30 mmHg gradient AVA 1.0 – 1.5 cm2 Moderate to severe exertion and competitive sports should be avoided Aortic Stenosis Severe AS • AVA < .75 cm2 • Cautious approach to diuretics, nitrates, and ACE inhibitors • Require high ventricular filling pressures to sustain CO • Sinus rhythm imperative

16. Treatment of AS • Prophylaxis against subacute bacterial endocarditis no longer required • No standard medical therapy proven to improve outcome • The only effective treatment is Aortic valve replacement **AVR in the elderly with LV dysfunction has been shown to lower mortality. Hinchman Cardiol Clin 1999

17. Severe AS is Lethal • Once symptoms occur: • 3 year mortality 30-50% • 5 year mortality 60-80% • 10 year mortality 80-90% Braunwald E; Heart Disease: A textbook of cardiovascular Medicine

18. Acute Infective Endocarditis Trauma Dissection Valve rupture Hypertension Chronic Idiopathic dilation Bicuspid valve Rheumatic heart disease Connective tissue disorders Aortic Regurgitation

19. Chronic AR LV dilation Produces a larger stroke volume Increasing pulse pressure Causing systolic HTN Usually asymptomatic – until end stage diesease Acute AR No time to mount adaptive response to larger end diastolic volumes Increased end diastolic pressures causing inadequate contractile response resulting Decreased stroke volume Usually symptomatic Aortic Regurgitation

20. Chronic Aortic Regurgitation • When adaptive response exhausts • Ejection fraction will begin to fall • CHF symptoms may develop • Dyspnea, orthopnea, paroxysmal nocturnal dyspnea, and fatigue

21. Aortic Regurgitation • Physical examination • Widening pulse pressure • Bounding peripheral pulses • Hyperdynamic apical pulse • Diastolic blowing murmur best heard along LSB • Rare But Interesting: • Musset’s sign - bobbing of head with each heart beat • Traube’s sign – pistol-shot sound over femoral artery • Quincke’s pulse – capillary pulsations detectable on nail beds

22. Aortic Regurgitation • Echocardiogram • Assist in assessing the cause • Define valve morphology • Estimate severity • LV end systolic/diastolic dimensions, mass, and aortic root size • Ejection fraction

23. Aortic Regurgitation • Treatment • SBE prophylaxis not required Mild AR No therapy needed and do not need to restrict activity Moderate AR No therapy needed but should avoid heavy physical exertion Vasodilator therapy has shown some benefit for moderate to severe AR (Symptom reduction and improved ventricular function)

24. Aortic Regurgitation Indications for Surgery • Symptomatic patients – more than mild symptoms • EF falls below 55% • End diastolic dimension exceeds 55 mm • “The 55 Rule” • Patients with Severe AR in the absence of symptoms • Should be followed closely every 4-6 months, including serial noninvasive testing

25. Mitral Valve Prolapse The Most common form of valvular heart disease. Affecting 2-15% of the population. A 1999 report from the Framingham database a prevalence of 2.4%. Freed et al. NEJM

26. Mitral Valve Prolapse • Primary Valve disorder • Idiopathic • Redundant mitral apparatus • Myxomatous degeneration of the valve First reported in 1913, by Gallavardin in Europe describing a somewhat variable sound of short duration and coin the term “Click”

27. Secondary MVP Myxomatous degeneration Duchenne muscular dystrophy Ruptured Chordae tendineae Ischemic heart disease Papillary muscle rupture Lupus erythematosis Marfan’s Polyarteritis nodosa Rheumatic fever Von Willebrand’s disease Trauma Cardiomyopathy

28. Clinical Syndrome Palpitations Nonexertional CP Fatigue Dyspnea Syncope/lightheadedness Anxiety Physical examination Midsystolic click (mult.) Late systolic murmur Mitral Valve Prolapse In cases where MVP has progressed or MR increased the murmur is longer and becomes more pansystolic. Control-matched patient population studies have not substantiated an increase in these symptoms in MVP.

29. Low Risk MVP Morphological normal valve that bulges into LA +/- Systolic click No MR Intermediate-Risk MVP Systolic click Intermittent or persistent Mild to Moderate MR Mitral Valve Prolapse • High-Risk MVP • Leaflet redundancy >5mm • Chordal elongation • Moderate to severe MR • Male >45 y/o • LA/LV enlargement • Holosystolic murmur

30. Mitral Stenosis • In virtually all cases it is secondary to rheumatic heart disease • Only approximately 50% of patients with acute rheumatic carditis develop chronic disease • Patients reporting h/o RHD is unreliable Rheumatic MV Normal MV

31. Disease Prevalence

32. Mitral Stenosis • Pathological process • Commissural fusion • Leaflet thickening • Calcification • Choral fusion This pathological process can often cause both stenosis and regurgitation lesions in the same valve

33. Mitral Stenosis Symptoms • Typical for that of CHF or COPD • Progression of symptoms is often subtle • As severity increases: • Fatigue rather than dyspnea • C/o Dependent edema • PND • RUQ pain 2° venous congestion • Palpitations / A Fib

34. Mitral Stenosis Physical exam • Opening snap with diastolic rumble • Loud S1 • Loud P2 • JVD • Edema • Hepatic congestion • Malar butterfly rash

35. Mitral Stenosis Medical Treatment • SBE prophylaxis not required • HR control “mainstay of therapy” • Beta-blockers • Calcium channel blockers • Digitalis only if LV/RV dysfunction • Diuretics if pulmonary congestion • warfarin if in atrial fibrillation • Controversy remains as to giving warfarin to MS patients in sinus rhythm

36. Mitral Stenosis • Closed commissurotomy • Open commissurotomy • Percutaneous Mitral Balloon valvotomy • FDA approved 1994

37. Most frequently related to ischemic or degenerative process Can be acute or chronic Classified as functional or organic Causes MVP (Most common cause of severe MR) Prevalence Accounts for 90% chordae ruptures Ischemic heart disease Cardiomyopathy RHD Infective endocarditis Myocardial disease or tumors Connective tissue disorders Congenital defect Trauma Mitral Regurgitation

38. Acute MR Typically symptomatic Left heart failure Dyspnea on exertion PND Pulmonary congestion Cardiogenic shock Chronic MR Typically asymptomatic Even severe chronic MR is more often dx by murmur. Ling et al. NEJM 1996 Fatigue and mild DOE are the most common presentation. As disease progresses more severe DOE, PND, pulmonary edema, or hemoptysis may present Mitral Regurgitation Changes in symptoms in Chronic MR is often triggered by A Fib, endocarditis, ruptured chordae, or changes in LV function

39. Mitral Regurgitation • Diagnostic work-up • ECG usually normal or nonspecific • CXR may be normal or demonstrate LA enlargement • Echocardiogram • Determine valvular morphology, chamber dimensions, LA/LV Function, presence of Pulmonary HTN

40. Mitral Regurgitation • Medical Therapy • ACE inhibitors may provide clinical benefit by reducing afterload. However there are no long-tern large clinical trials supporting this. • ACE inhibitors have not been shown to reduce or delay the need for surgery. ACC/AHA guidelines J Am Coll Cardiol 1998. Surgery is the only definitive therapy for MR with LV dysfunction or a symptomatic patient

41. Mitral Regurgitation Timing of intervention • Should be considered for symptomatic patients and those with LV dysfunction • EF usually supernormal • Once EF falls below 60% the prognosis worsens. (Carabello BA Mod Concepts Cardiovasc Dis 1988.) • Once the End-Systolic dimension exceeds 45 mm the prognosis worsens.(Crawford et al. Circulation 1990.) When surgery is considered, it has been shown that MV repair has a lower operative mortality and better long-term outcomes. Patients >75 y/o will have a worse prognosis than their younger counterparts do, esp. if replacement vs. repair is performed.

42. Tricuspid Valve Disease • Rheumatic Heart Disease • Congenital • RA tumor • Carcinoid syndrome • Endomyocardial Fibrosis Majority of disease is mixed TS/TR

43. Tricuspid Stenosis Morphology • Fusion • Chordae shortening • Calcification- rare • Physical examination • TS usually missed 2° to MS • JVD • Hepatomegaly • Anasarca • OS heard best LSB • Diastolic rumble LSB • Treatment • Diuretics • Na+ restriction Surgery - >5mmHg gradient TVA < 2.0cm2 at time of MV repair/replacement Finger commissurotomy may not work 2° to TR

44. Tricuspid Regurgitation • Most common cause is RV dilatation • MVD • RV infarction • Congenital – Pulmonary stenosis/ HTN • Cor pulmonale • Infective endocarditis • Thyrotoxicosis • Tricuspid valve prolapse (1/3 pt’s with MVP) • Physical Exam • JVD • A Fib common • Pansystolic murmur • Jaundice TR in absence of Pulmonary HTN usually does not require surgery

45. Pulmonary Stenosis Congenital most common Rheumatic uncommon Carcinoid plaques in RV outflow tract and on Pulmonic valve Pulmonary Regurgitation Pulmonary HTN – annular ring dilation Idiopathic dilation of pulmonary artery Connective tissue disorders – Marfan syndrome Infective endocarditis Pulmonic Valve Disease

46. On The Horizon!?!

47. After Stent Implantation Anchoring test Longitudinal view

50. Balloon Aortic Valvuloplasty April 8, 2002 Gradient 30  13 mmHg AVA 0.60  1.06 cm² Follow-up : Transient improvement Day 4 : recurrence of cardiogenic shock Day 6 : impending death LVEF : 14%