2. Valvular heart disease Aortic Valve Diseases Dr. Hussam Al-Faleh Med 341 course

3. Lecture outline  General principles :  Pressure overload and volume overload  Heart murmurs  Aortic valve disease  Mitral valve disease

4. VALVULAR STENOSIS Pressure in upstream chamber IS HIGHER than Pressure in downstream chamber during time of flow (when valve is normally open). Hemodynamic abnormality = "PRESSURE GRADIENT" Hemodynamic abnormality = "PRESSURE GRADIENT" UpstreamDown stream High pressurelow pressure

5. VALVULAR REGURGITATION UpstreamDown stream Volume overload Retrograde flow of blood "upstream" during time when valve is normally closed. Hemodynamic abnormality = "VOLUME OVERLOAD"

6. Left Ventricular Hypertrophy “Pressure and Volume overload”

7. LA RV RA LV Vena Cava Aorta Pulm Artery Pulm Vein

8. RA RV LV Aortic stenosis LV Normal

9. LA RV RA LV Vena Cava Aorta Pulm Artery Pulm Vein Aortic Insufficiency

10. RA RV LV ↑↑LV Example: Aortic regurgitation

12. AI AS

13. Heart murmurs

14.  Produced by turbulent blood flow  Turbulence is mainly determined by velocity of blood flow across a structure  Timing of murmurs (either systolic, or diastolic) can give helpful information regarding the valve lesion

15. Systolic Murmurs  Aortic stenosis  Mitral insufficiency  Mitral valve prolapse  Tricuspid insufficiency Diastolic Murmurs  Aortic insufficiency  Mitral stenosis S1 S2 S1 Common Murmurs and Timing

16. Outline for every valve lesion  Etiology  Pathphysiology  Symptoms and signs  Natural history  Investigations  Management

17. Aortic stenosis

18. Etiology  Supra-Valvular  Valvular - Congenital - Congenital - Acquired - Acquired Sub-Valvular Sub-Valvular - Discreet - Discreet - Tubular - Tubular

19. Etiology  Valvular Congenital Congenital 1. Uni-cusped - Rare 1. Uni-cusped - Rare - Ages affected 2-30yrs - Ages affected 2-30yrs 2. Bi-cusped - 2% of the population 2. Bi-cusped - 2% of the population - More common in males - More common in males - Associated with coarctation - Associated with coarctation in 6% of patients in 6% of patients - Ages affected 40-50yrs - Ages affected 40-50yrs

21. Etiology  Acquired 1. Rheumatic 1. Rheumatic - Adhesion and fusion of valve commissures leads to - Adhesion and fusion of valve commissures leads to stiffening of the free borders as well as calcification stiffening of the free borders as well as calcification 2. Degenerative (senile) 2. Degenerative (senile) - Results from mechanical stress - Results from mechanical stress - Associated with traditional risk factors for - Associated with traditional risk factors for CAD such as HTN, Dyslipidemia and smoking CAD such as HTN, Dyslipidemia and smoking

22. A.Normal Trileaflet AV B.Congenital AS C.Rheumatic AS D.Calcific AS E.Degenerative AS

23. Pathphysiology

25. Symptoms  Cardinal symptoms: 1. Chest pain 1. Chest pain - Occurs due to ↑ O2 demand - Occurs due to ↑ O2 demand (LV hypertrophy) and ↓ O2 delivery (LV hypertrophy) and ↓ O2 delivery - Is often related to concomitant CAD. - Is often related to concomitant CAD. 2. Presyncope/Syncope 2. Presyncope/Syncope - Caused by transient ↓ cerebral blood flow - Caused by transient ↓ cerebral blood flow - May also be related transients VF or AF - May also be related transients VF or AF 3. Dyspnea 3. Dyspnea - Late manifestation of severe AS - Late manifestation of severe AS

26. Signs  Central Pulse : - Slow rising, low volume ( Pulsus Parvus et tardus) - Slow rising, low volume ( Pulsus Parvus et tardus) - Coarse systolic vibrations at Carotid artery (Carotid Shudder) - Coarse systolic vibrations at Carotid artery (Carotid Shudder)  JVP: - Prominent a wave - Prominent a wave  Apex: - Sustained - Sustained - Systolic thrill - Systolic thrill - Displaced (late with LV failure) - Displaced (late with LV failure)

27. Slow rising pulse Aortic pulse

28. Signs ( Auscultation)  S2 may be soft and single  Paradoxical splitting of S2 in severe AS S1S2 Inspiration Expiration

29. Signs (Auscultation)  Aortic ejection sound with Bicuspid AV  S4 S1 Ejection Click S2 S4S1

30. Auscultation S1 S2 Mild-Moderate S1 S2 Severe

31. Natural history

32. Investigations  ECG - LAD - LAD - LVH - LVH

33. Investigations  CXR - Aortic Calcification - Aortic Calcification - Post stenotic dilation of Ascending Aorta - Post stenotic dilation of Ascending Aorta  Echocardiography - Routinely used to diagnose and estimate the severity of AS - Routinely used to diagnose and estimate the severity of AS - Peak and mean gradients are measured - Peak and mean gradients are measured - Valve area is measured - Valve area is measured Mild AS (area >1.5 cm 2 ) Mild AS (area >1.5 cm 2 ) Moderate (area >1.0 to 1.5 cm 2 ) Moderate (area >1.0 to 1.5 cm 2 ) Severe (area <1.0 cm 2 ) Severe (area <1.0 cm 2 )

34. Management  No place for medical therapy if severe AS is associated with symptoms.  Surgery is the treatment of choice.  Generally speaking,if the patient has symptoms with severe AS Surgery is indicated.

35. Aortic Regurgitation

36. Etiology

37. Etiology (chronic AR)  Two major causes: A. Intrinsic structural valve problem A. Intrinsic structural valve problem 1. Congenital : Bicuspid valve 1. Congenital : Bicuspid valve 2. Acquired : - Inflammatory (Rheumatic, 2. Acquired : - Inflammatory (Rheumatic, Connective tissue diseases) Connective tissue diseases) - Infectious (IE) - Infectious (IE) - Degenerative - Degenerative

38. Etiology( chronic AR) B. Abnormality of the Ascending Aorta 1. Congenital : Marfans disease 1. Congenital : Marfans disease 2. Infectious : Syphilis (15-25yr after infection) 2. Infectious : Syphilis (15-25yr after infection) 3. Inflammatory : Connective tissue diseases 3. Inflammatory : Connective tissue diseases (RA, AS, GCA) (RA, AS, GCA) 4. Idiopathic : progressive dilation (cystic medial 4. Idiopathic : progressive dilation (cystic medial necrosis) necrosis)

39. Etiology (Acute AR)  Trauma  Aortic dissection  Infective endocarditis

42. Aortic regurgitation Excess volume to the LV ↑LV end diastolic pressure Stretching of Myocardium ↑ wall stress Eccentric LV hypertrophy ↓ effective Stroke volume ↑ Myocardial O2 demand (Ischemia) LV failure ↓ Myocardial O2 supply (Ischemia)

43. Symptoms  Gradual development of Dyspnea, Orthopnea, and PND  Angina  Palpitations  With Acute AR, abrupt development of dyspnea.

44. Signs of Chronic AR  Peripheral signs - De Musset sign (head movment with pulse) - De Musset sign (head movment with pulse) - Water hammer pulse (abrupt distention and quick collapse) - Water hammer pulse (abrupt distention and quick collapse) - Duroziez sign - Duroziez sign - Muller’s sign (systolic pulsation of Uvula) - Muller’s sign (systolic pulsation of Uvula) - Pistol shot/ Traube sign - Pistol shot/ Traube sign - Quincke sign - Quincke sign - Hill’s sign (Popliteal pressure at least 20 mmHg higher than brachial pressure - Hill’s sign (Popliteal pressure at least 20 mmHg higher than brachial pressure

45. Signs of chronic AR  Wide pulse pressure  Central pulse: - Large volume pulse - Large volume pulse - Bisferines pulse - Bisferines pulse - Corrigan pulse - Corrigan pulse  JVP may be normal or elevated  Displaced and hyperdynamic apex

46. Auscultation  S2 may be soft or accentuated  S3 indicates severe AI  Ejection click  High pitched, blowing, decrescendo diastolic murmur at LSB, best heard at end-expiration & leaning forward

47. Auscultation  Length of murmur correlates with severity.  In Acute AR diastolic murmur is low pitched and short.  Austin-Flint murmur indicates severity (mid to late diastolic murmur)  Systolic murmur related to high flow state S1 S2 S1

48. Investigations  ECG – LVH, LAD  CXR- may show ↑cardiothoracic ratio, and dilated aorta

49. Investigations  Angiography: - Would aid in diagnosis and grading of severity - Would aid in diagnosis and grading of severity  Echocardiography: - The easiest and fastest way of diagnosing - The easiest and fastest way of diagnosing and grading the severity of AR. and grading the severity of AR. - Detection of the underlying mechanism of AR. - Detection of the underlying mechanism of AR.

50. Asymptomatic %/Y Asymptomatic %/Y Normal LV function (~good prognosis) Normal LV function (~good prognosis) Progression to symptoms or LV dysfunction < 6 Progression to symptoms or LV dysfunction < 6 Progression to asymptomatic LV dysfunction < 3.5 Progression to asymptomatic LV dysfunction < 3.5 Sudden death < 0.2 Sudden death < 0.2 Abnormal LV function Abnormal LV function Progression to cardiac symptoms > 25 Symptomatic (Poor prognosis) Mortality > 10 Natural history

51. Management  Any patient with severe AR and any of the following should have aortic valve replacement: 1. Symptomatic patients 1. Symptomatic patients 2. Patients without symptoms, but with 2. Patients without symptoms, but with LV systolic dysfunction (EF<50%), or LV systolic dysfunction (EF<50%), or marked dilation of the LV. marked dilation of the LV.

52. Management  Vasodilator therapy ( ACE I, or CCB’s) for: 1. Patients not candidates for surgery 1. Patients not candidates for surgery 2. Short term to Improve hemodynamics 2. Short term to Improve hemodynamics 3. Treatment of hypertension 3. Treatment of hypertension