Valvular Heart Disease: Diagnosis and Treatment

1. MUDr. Jan Tomis 5/2016 Valvularheartdisease

2. Normal valve function • Maintain forward flow and prevent reversal of flow. • Valves open and close in response to pressure differences (gradients) between cardiac chambers.

3. Abnormal valve function • Valve Stenosis • Obstruction to valve flow during that phase of the cardiac cycle when the valve is normally open. • Hemodynamic hallmark -“pressure gradient” • Valve Regurgitation, insufficiency, incompetence • Inadequate valve closure → reverse flow of the blood, back leakage • Combined – a single valve can be both stenotic and regurgitant; combinations of valve lesions can coexist • Single disease process • Different disease processes • One valve lesion may cause another • Certain combinations are particularly common(AS & MR, MS & TR)

4. Valvular heart disease • Common cause of cardiovascular morbidity, 2nd most common indication for heart surgery (after ischemic heart disease) • In the Czech Republic (2012): 8644 heart surgeries, about 46% involved valve intervention • Combined myocardial revascularisation and valve surgery particularly common for some diagnosis – CABG + AVR (common risk factor), CABG + MRV (in ischemic mitral regurgitation)

5. Diagnosis • History • Physical examination • ECG • Chest x-ray • Echocardiography • Invasive evaluation, CT, MRI

6. Diagnosis • History • Physical examination • ECG • Chest x-ray • Echocardiography • Invasive evaluation, CT, MRI

7. History • Other known heart diseses • Congenital heart disease, bicuspid aortic valve • Ischemic heart disease • Rheumatic fever • Hypertrophic/dilated cardiomyopathy • Aortic diseases – Marfans, Ehlers-Danlos

8. History • Generally: symptoms of heart failure and low cardiac output • Breathlessness • Chest pain or dyscomfort • Syncope • Fatigue • Periferal or pulmonary oedema • Palpitations

9. Diagnosis • History • Physical examination • ECG • Chest x-ray • Echocardiography • Invasive evaluation, CT, MRI

10. Physical examination

11. Physical examination

12. Physical examination • MURMURS!!! • Periferaloedema • Lungcrackles • Elevated JVP • Displaced apex beat, irregularheart beat… • Hundredsofeponymoussignsfrom past millenium (↓importance in dailyroutine, ↑importanceforpassingexam)

13. Heart murmurs • Sounds produces by turbulent blood flow (in valve diseseses, artery stenosis, abnormal chamber or AV communication) • Localization,grade,propagation, • timing, quality

14. Heart murmurs

15. Diagnosis • History • Physical examination • ECG • Chest x-ray • Echocardiography • Invasive evaluation, CT, MRI

16. ECG • Not specific • Findings might be caused or altered by other concomitant heart disease (hypertensive heart disease, ischemic heart disease) • Left ventricular hypertrophy (aortic valve disease) • Left atrial enlargement (mainy MS, but any left heart valve disease) • Atrial fibrilation • Bundle branch block • Arrytmias (atrial fibrilation, ectopic beats)

17. Diagnosis • History • Physical examination • ECG • Chest x-ray • Echocardiography • Invasive evaluation, CT, MRI

18. Chest x-ray in valvular disease • Different heart shapes in different valvular heart diseses, ↓specificity, ↓significance • Cardiomegaly, pulmonary congestion • Widened mediastinum • Valve calcifications, prosthetic valves

20. Diagnosis • History • Physical examination • ECG • Chest x-ray • Echocardiography • Invasive evaluation, CT, MRI

21. Echocardiography • Mainstay ofvalvediseasediagnosisandfollow-up • Allowsreal-timemeasurementofchamberandwalldiameters, ejectionfractionassessmentandfunctionalvalveevaluation • Easilyavaiableandrepeated • Essential in acute valvediseasediagnosis • No radiationharm • Trans-esophagealechocardiographyavaiableforpatientswithpoortransthoracicsonographicwindow

22. Diagnosis • History • Physical examination • ECG • Chest x-ray • Echocardiography • Invasive evaluation, CT, MRI

23. Invasive evaluation, CT, MRI • Methods usualy used for uncertain cases or repeat cardiac surgery / percutaneous inteventions planning • Angiography to assess regurgitation severity – direct transcatheter contrast medium administration into heart chambers – aortography, ventriculography • Hemodynamic measurment – measuring of intracardial pressures and gradients • CT aortography – method of choice in aortic dissection diagnosis • CMRI – very precise evaluation of cardiac tissues and function, but expensive, low avaiability, long examination time

25. Aortic stenosis • Most commonindicationforvalveintervention • Causes • Degenerativeaortic stenosis • Bicuspidaorticvalve • Congenitalaortic stenosis, unicuspidaorticvalve • Rheumaticdisease (alwayswithmitralvalveinvolvement) • Infectiveendocarditis (but severe stenosis due to massivevegetationsisextremely rare) • Other rare causes – post radiation, associatedwithsystemicdisease

26. Rheumatic vs. Degenerative (involves commissures) (spares commissures)

27. Aortic stenosis • Pathophysiology • Normal aortic valve area (AVA) – 3-4 cm2 • With a decrease of AVA ,apressure gradient develops between the left ventricle and the aorta (increased afterload) • LV function initially maintained by compensatory concentric hypertrophy (but without an adequete increase in vascularization) • When compensatory mechanisms are exhausted, LV function declines.

28. Aortic stenosis • Presentation • Anginapectoris(increased myocardial oxygen demand; demand/supply mismatch) • Dyspnea on exertion due to heart failure (systolic and diastolic) • Syncope (exertional) • Sudden death, mortality – whenasymptomaticwithpreservedleftventricleejctionfraction, thesuddendeath risk isabout 1%/y, whensymptomatic, however, the mortality increases to up to 50%/y

29. Aortic stenosis • Physical finding • Systolic crescendo-decrescendo murmur with maximum at right sternal border, 2nd-3rd intercostal space , propagated to the carotic arteries – the loundness of the murmur is not directly correlated to severity of stenosis • Slow rising carotid pulse (pulsus tardus) & decreased pulse amplitude (pulsus parvus) • Heart sounds- soft and split second heart sound, S4 gallop due to LVH…

30. Aortic stenosis • Therapy – medical therapy has no prognostic effect • Aortic valve replacement • Standard therapy for patients with low surgical risk or with indication for other procedure • Mechanical/biological prosthesis • TAVI (transcatheter aortic valve implantation) – patiens at unaccaptable surgical risk (elderly, comorbid) • Percutaneous aortic balloon valvuloplasty (for congenital stenosis, or as a bridging therapy for unstable patients)

31. Aortic stenosis • Indicationforreplacement • Severe aortic stenosis (AVA <1 cm2, mean PG > 40mmHg) • Symptomatic • LV functiondecreases • Otherindicationforsurgery • Moderate stenosis (AVA 1,5-1 cm2 ) • Withotherindicationforsurgery

32. Aortic stenosis

34. Aortic regurgitation • Causes • Chronicaorticregurgitation • Bicuspidaorticvalve • Rheumaticanddegenerative – alwayswithsomedegreeof stenosis • Aorticrootdilation (hypertension, Marfan’s, Ehlers-Danlos, syphyliticaortopathy) • Other rare causes (SLE, RA) • Acute aorticregurgiation • Infectiveendocarditis • Aorticregurgitation

35. Aortic regurgitation • Pathophysiology of chronic aortic regrgitation • Leakage of blood into LV during diastole due to ineffective coaptation of the aortic cusps • Combined pressure and volume overload • Compensatory Mechanisms: LV dilation, LVH. Progressive dilation leads to heart failure • Greatest mass of myocardium in any valve disease – „cor bovinum“ – over 500g

36. Aortic regurgitation • Presentation • Dyspnea:exertional, orthopnea, and paroxsymal nocturnal dyspnea • Chestpain • Fatigue • Palpitations: due to increased force of contractionorarrytmias

37. Aortic regurgitation • Physical findings (the ones you might find) • Diastolic blowing murmur at the left sternal border – might be very discrete. Systolic ejection murmur might be present due to increased blood flow across the aortic valve of concomitant valve stenosis • Wide pulse pressure – caused by diastolic regurgitation of blood to LV and fast decrease of diastolic BP – „Corrigan’s pulse“ (160/30 mmHg…) • Heaving and laterally displaced apex beat – due to dilated heart with giant stroke volume

38. Aortic regurgitation • Physical findings (the ones you might not find…) • Quincke’s sign - pulsations of nail bed • Muller’s sign- pulsation of uvula • De Musset sign -  (head nodding in time with the heart beat) • Duroziez sign (systolic and diastolic murmurs heard over the femoral artery when it is gradually compressed with the stethoscope) • Austin Flint murmur (apex): Regurgitant jet impinges on anterior MVL causing it to vibrate

40. Aortic regurgitation • Acute aortic regurgitation • Caused by a leaflet perforation in infective endocarditis • In aortic dissection due to a change in aortic root geometry – dilation, extensive intimal tear with prolapse into LVOT and coaptation impairment • Presentation of acute aortic regurgitation itself is usually a pulmonary oedema accompanied by symptoms of the causing pathology • True emergency – mostly requires immediate cardiac surgery

41. Aortic regurgitation • Therapy –surgical • Isolated leaflet pathology - aortic valve replacement • Aortic root pathology - combined aortic root, ascendent aorta and aortic valve replacement – Bentall’s procedure

42. Aortic regurgitation • Indicationforreplacement • Severe aorticregurgitation (EROA – effectiveregurgitantorifice area >0,3 cm2) • Symptomatic • LV dilates (over 50 mm EDD) orfunctiondecreases (EF < 55%) • Otherindicationforsurgery • Acute • Moderate regurgitation (AVA 1,5-1 cm2 ) • Withotherindicationforsurgery

43. Mitral stenosis • Causes • Rheumatic heart disease in up to 99% of all cases • Other causes are rare - mitral annular calcification, obstructionwithmassiveendocarditisvegetations, leftatrialmyxoma, post radiation • Nowadays rare in developedcountries, stillprevalent in developingcountriesdue to rheumaticfever

44. Mitral stenosis • Pathophysiology: • Normal mitral valve area 4-6 cm2 – stenosis becomes severe with MVA < 1cm2 • Increased transmitral pressure gradient: leads to left atrial pressure increase, enlargement and atrial fibrillation → • Development of postcapillary pulmonary hypertension (there is no valve to isolate the increased left atrial pressure from pulmonary veins) → • Right heart failure symptoms - due to pulmonary HT, secondary right ventricle dilation and tricuspid regurgitation

45. Mitral stenosis • Presentation • Dyspnea • Syncope • Palpitations (atrialfibrilationiscommon) • In advancedcases - rightheartfailuresymptoms – periferaloedema, increased JVP, hepatomegaly, ascites…

46. Mitral stenosis • Physical finding • Diastolic murmur • Low-pitched diastolic rumble most prominent at the apex. • Heard best with the patient lying on the left side in held expiration • Intensity of the diastolic murmur does not correlate with the severity of the stenosis • Lungcrackles • Pleuraleffusion • Facies mitralis: When MS is severe and the cardiac output is diminished, there is vasoconstriction, resulting in pinkish-purple patches on the cheeks (mightbeseen in terminalheartfailureofany cause)

48. Mitral stenosis • Percutaneous therapy – PTMV – in caseswithsuitablemorphologyofmitralvalve • Surgical therapy -Mitralvalvereplacement • Medical therapy -Diureticsforoedema, ratecontrol therapy in atrialfibrillation, anticoagulant therapy (even in sinus rhytmwithgreatdilationofleft atrium)