Valvular Heart Disease

1. Valvular Heart Disease Division of Cardiology, Shanghai Sixth Hospital Zhi-Gang Lu

2. Introduction • Valvular heart disease: abnormal structure or function of single or multiple valves result in the valve orifice stenosis or/and incompetence. • Etiology: commonly caused by inflammation, mucoid degeneration, regressive change, congenital malformation, ischemic necrosis, trauma and so on. • Severe dilation of the ventricles, aortic artery and pulmonary artery also lead to relative incompetence of the corresponding valves. • The mitral valve is most often involved, next the aortic valve • Most cases of valvular disease in China are due to rheumatic heart disease

3. Mitral Stenosis Etiology • Rheumatic fever:rheumatic heart disease • Valve ring calcification: degenerative change in the aged • Congenital • Connective tissue disease：SLE, scleroderma • Multiple myeloma

4. Pathology • Rheumatic fever results in fibrosis, thickening, stiffening, calcification, adhesion, shortening and fusion of mitral valve structure (leaflet juncture, free margin of leaflet, chordae tendineae and papillary muscle) , which produce mitral stenosis. • Left atrial dilation is companied by parietal thrombus, thickening of pulmonary artery wall, hypertrophy and dilation of right ventricle.

5. Valve orifice areanormal 4-6cm2mild stenosis 1.5--2.0cm2moderate stenosis1.0—1.5cm2severe stenosis< 1.0cm2

6. Two types:1．Septum type2．Funnel type

7. 1．Septum type·only leaflets involved, without obvious shortening·rare complicated with incompetence due to good elasticity of leaflets·no dilation of LV·prominent S1, with opening snap·percutaneous balloon valvuloplasty is preferred to relieving the stenosis

8. 2．Funnel type·leaflet, chordae tendineae and papillary muscle are all invloved·common complicated with incompetence due to poor elasticity of leaflets·S1 decrease·valvular replacement is preferred to relieving the stenosis

10. [Pathophysiology]1．Stage of left atrial compensation·early stage，mild stenosis·the pressure gradient across mitral valve < 20mmHg，left atrial mean pressure < 25mmHg·clinical asymptomatic, with cardiac murmur

11. [Pathophysiology]1．Stage of left atrial decompensation·moderate and severe stenosis·the pressure gradient across mitral valve> 20mmHg，left atrial mean pressure> 25mmHg·elevated left atrial pressure, pulmonary congestion and pulmonary hypertension · with apparently clinical symptoms

12. Mechanisms of clinical manifestation:pulmonary venous congestion, pulmonary edema – similar with ↗ left heart failureLA failure ↘pulmonary hypertension, right ventricular hypertrophy – right heart failure↗down transfer of elevated LA pressuremain causes → reactive constriction of pulmonary arteriolesof pulmonary →obstructive changes of pulmonary vascular bed hypertension ↘recurrent pulmonary infection and overfatigue

13. Pathophysiology • LA compensation ---- influences of MS on pressure gradient across the valve and left atrial pressure • LA decompensation ---- influences of elevated left atrial pressure onpulmonary circulation: pulmonary hypertension • Right heart involved ---- influences of pulmonary hypertension on right ventricle

14. Clinical manifestion • symptomsorifice area <1.5cm2 • Dyspnea: exertional dyspnea, resting dyspnea, orthopnea and paroxysmal nocturnal dyspnea • Hemoptysis: blood; bloody sputum; blood-streaked sputum; pink frothy sputum; pulmonary infarction with hemoptysis • Cough • Hoarseness (horner syndrome)

15. Clinical symptoms • Signs: “mitral face” is common in severe MS patients • Cardiac signs: normal or weakened apical impulse; prominent apical S1 and opening snap; apical middiastolic crescendo rumble low in pitch; diastolic tremor • Cardiac signs of pulmonary hypertension and right ventricular dilation: right ventricular systolic heave pulsation, prominent P2; short systolic spurting murmur along left sternal borderand high-pitched decrescendo early-diastolic sighing murmur (Graham-Stell murmur); tricuspid pansystolic murmur (relative tricuspid incompetence)

16. Auxiliary examination • X ray: left atrial enlargement • ECG: mitral p wave; right axis deviation and right ventricular hypertrophy • UCG: confirms diagnosis and estimates severity • Cardiac catherization

19. Diagnosis and differential diagnosis • Diagnosis: apical diastolic rumble with X ray or ECG evidence of left atrial enlargement; UCG confirms diagnosis. • Differential diagnosis: increased blood flow across mitral valve; Austin-Flint murmur; left atrial myxoma

20. complication • Atrial fibrillation • Acute pulmonary edema • thromboembolism • congestive heart failure • infective endocarditis • pulmonary infection

21. Treatment • General treatment • Prophylaxis for rheumatic fever: benzathine benzylpenicillin 1 200 000 units, per 4 weeks IM. • Prophylaxis for infective endocarditis • To avoid intense physical activity and take regular examination for asymptmatic patients • To reduce physical activity, sodium restriction, oral diuretics and refrain from provocative factors

22. Treatment • Treatment of complication • Severe hemoptysis：to lower pulmonary venous pressure • Acute pulmonary edema: to avoid dilating arterioles and using cardiac inotropic agents • Atrial fibrillation: ventricular rate control, conversion and maintenance of sinus rhythm, anticoagulation • Prevention from embolism • Right heart failure

23. Treatment • Intervention and operation • Percutaneous balloon mitral valvuloplasty (PBMV) • open surgical valvotomy • Prosthetic valve replacement

25. Mitral Incompetence • Mitral valve closure depends on the integrity of mitral valve structure (leaflet juncture, free margin of leaflet, chordae tendineae and papillary muscle) and left ventricular structure and function. Any abnormal factor from above can result in mitral incompetence.

26. Etiology and pathology • Chronic • Rheumatic heart disease • Mitral valve prolapse: mucoid degeneration • Coronary heart disease • Rupture of chordae tendineae • Calcification of mitral ring • Infective endocarditis • Severe enlargement of left ventricle • Rare reasons: congenital deformity, SLE, rheumatoid arthritis, HOCM, endomyocardial fibrosis and left atrial myxoma

27. Etiology and pathology • Acute • Rupture of chordae tendineae • Impairment of leaflet or rupture of chordae tendineae due to infective endocarditis • Acute ischemia, necrosis and repture of papillary muscle due to acute myocardial infarction • Traumatic impairment of mitral valvular structure • Impairment of prosthetic valves

28. Pathophysiology • Acute: some blood reflow from LV to LA in systole + blood from pulmonary vein to LA filling LV in diastole volume overload of LA and LV LV end-diastolic pressure LA pressure pulmonary congestion pulmonary edema pulmonary hypertension and right heart failure • Chronic: with a longer period of compensation

29. Clinical manifestion • Symptoms • Acute: mildexertional dyspnea severe acute left heart failure, acute pulmonary edema or cardiac shock • Chronic: mild asymptomatic for lifetime severe pulmonary venous congestion

30. Signs • Chronic: • Apical impluse: hyperdynamic, left inferior displacement in case of LV enlargement • Cardiac sound: rheumatic heart disease S1 mitral valve prolapse, coronary heart disease normal S1 • Cardiac murmur：pansystolic blowing murmur, radiating to the axilla and occasionally to the base • Acute hyperdynamic apical impulse, P2 non-pansystolic murmur

31. Auxiliary examination • X ray: enlarged LA and LV, pulmonary congestion and interstitial edema are common in case of left heart failure • ECG: hypertrophy of LA and LV, atrial fibrillation is common • UCG: confirmation of diagnosis, semiquantitative estimates of the severity of regurgitation • Radionuclide ventriculography: evaluation of LV systolic function; evaluation of regurgitation degree by stroke volume ratio between LV and RV (>2.5 suggests severe regurgitation) • LV ventriculography: the gold standard for semiquantitative evaluation of regurgitation degree

33. Diagnosis and differential diagnosis • Diagnosis: • In acute cases, dyspnea onset, apical systolic murmur, no enlarged cardiac shadow with obvious pulmonary congestion by X ray, easy to diagnose • In chronic cases, apical typical murmur with enlarged LA and LV help to diagnose and final diagnosis should be confirmed by UCG.

34. Diagnosis and differential diagnosis • Physiological murmur • Relative mitral incompetence • Ventricular septal defect • Aortic valve stenosis • Tricuspid incompetence

35. complication • Atrial fibrillation • Infective endocarditis • Systemic circulation embolism • Heart failure • Complication of mitral prolapse: infective endocarditis, brain embolism, arrhythmia, sudden death, severe mitral incompetence and heart failure

36. treatment • acute • Purpose: to lower pulmonary vein pressure, to improve cardiac output and to treat the underlying pathologic process.Medical therapy is usually preoperative measure. • Operation is usually necessary.

37. Therapy • Chronic • Medical therapy • Prevention from infective endocarditis; prevention from rheumatic fever for rheumatic heart disease • No special therapy for asymptomatic patients with normal cardiac function, but regular follow-up is required. • Treatment of atrial fibrillation in mitral incompetence is the same as in mitral stenosis, but maintaining sinus rhythm is less important than in mitral stenosis • Treatment of heart failure: sodium restriction, ACEI, diuretics and digitalis

38. Treatment • Chronic • Surgical therapy: the fundamental measure to restore competence • Prosthetic valve replacement: better than medical therapy • Mitral valve repair: with lasting effect and fewer postoperative complication (infective endocarditis, thromboembolism); long time anticoagulation is unnecessary; left ventricular function is better preserved.

40. Aortic Stenosis (AS)

41. AS Etiology and pathology ① • 1. Rheumatic heart disease: rheumatic inflammation →valvular fusion, fibrosis, stiffening and contracture →orifice stenosis; little pure AS, usually associated with aortic incompetence and mitral valvular impairment. • 2. Agedregressive calcified AS: the most common cause of pure AS patients >65; no juncture fusion, calcified tubercle on the aortic side, usually associated with mitral annular calcification

42. AS Etiology and pathology② • 3. congenital deformity: ① congenital bilobed calcified AS, approximately 1％－2％ of patients have congenital bicuspid deformity, male>female, no stenosis on birth due to no juncture fusion, with 1/3 of these developing AS in adulthood; underlying mechanism: deformity → turbulent flow → valvular impairment → fibrosis and calcification → developing AS in adulthood, the most popular cause of isolated AS in adult

43. AS • 3. congenital deformity: ②congenital AS: stenosis is present on birth • 4. Other rare causes：large vegetations obstructing the valve orifice, such as SBE, SLE Etiology and pathology③

45. AS pathophysiology ① • Normal valve area ≥3cm2, mild stenosis 1.5－3cm2, moderate stenosis 1.0－1.5cm2, severe stenosis ＜0.8cm2。 • A valve area decreases to 1/2 of the original, a normal pressure gradient acrossLV- aortic artery in systole being preserved (＜5mmHg)。 • A valve area ≤1.0cm2 (decrease to 1/4 of the original),LV systolic pressure ↑, with an significant pressure gradient(＞50mmHg)。

46. AS Pathophysiology ② • Aortic stenosis → obstructedLV ejection → LV afterload↑（resistence increased）→ CO↓ • LV afterload↑→LVEDP↑→LV hypertrophy and slightly dilationso as to preserve normal CO → progressive ongoing →LA pressure↑→ pulmonary congestion →LV dysfunction (late stage) • LA pressure↑→ LA hypertrophy → cardiac stroke preserved, with pulmonary congestion aggravated

47. AS Pathophyiology ③ AS（severe stenosis）→the mechanism of myocardial ischemia • LV wall thickening：LV systolic pressure↑, ejection time↑, myocardial oxygen comsumption ↑ • LV hypertrophy：myocardial capillary density relatively↓ • LVEDP↑→ oppressing the subendocardial arteries→ myocardial blood supply↓ • LVEDP↑→ the pressure gradient acrossarotic arter－LVin diastole ↓→ decreased perfusion pressure of coronary artery→coronary artery blood supply↓ • AS→blood flow of aortic root↓→ coronary artery blood supply↓→angina pectoris

48. AS Pathophysiology ④ • Left cardiac output ↓→ insufficientbrain blood supply → syncope • Exercise increases myocardial work and oxygen consumption ↑→aggravated myocardial ischemia

49. AS Clinical manifestion • Introduction: AS is usually gradually progressive disease ＋ strong compensation ability of LA and LV, therefore symptoms always occur at the age of more than 20 in cases of congenital AS; while in cases of mild AS, patients may be asymptomatic for lifetime, but sudden death sometimes happens, male:female=3:1

50. AS Clinical manifestation symptoms: AS triple symptom complex • Angina pectoris：present in 60％ of symptomatic patients, provoked by exercise, relieved by rest, or not definitely related with physical exercise • dyspnea：present in 90％ of symptomatic patients, resulted from pulmonary congestion in advanced stage • sycope：occurs during standing up, exercising or right after exercises