1. Valvular Heart Disease
J.B. Handler, M.D.
Physician Assistant Program
University of New England
STUDY PE FINDINGS! MOST IMPORTANT FOR US

2. Abbreviations VHD- valvular heart disease RF- rheumatic fever
MR- mitral regurgitation
AR- aortic regurgitation
HF- congestive heart failure
MS- mitral stenosis
LAP- left atrial pressure
PuVR- pulmonary vascular resistance
RV- right ventricle
CO- cardiac output
TR- tricuspid regurgitation
PI- pulmonic insufficiency
NSST-T- non specific ST-T
PAH- pulmonary artery hypertension
SV- stroke volume
RVH- right ventricular hypertrophy
AoV- aortic valve
MVA- mitral valve area
PSVT- paroxysmal supraventricular tachycardia
MVR- mitral valve replacement
MVP- mitral valve prolapse
AS- aortic stenosis
SEM- systolic ejection murmur
LVEDP- left ventricular end diastolic pressure
PND- paroxysmal nocturnal dyspnea
LSB- left sternal border
ACE- angiotensin converting enzyme
BE- bacterial endocarditis
RF- rheumatic fever

3. Etiologies of VHD Rheumatic valve disease
Congenital, including bicuspid aortic valve.
Coronary heart disease: MI, papillary muscles
Dilation of the aorta: Aortic root disease
Chronic “wear and tear”: aortic sclerosis/stenosis
Dilation of the LV- from any cause: MR
Endocarditis
MV prolapse
Others
RVD – consequence of Rheum fever
In cor ds can lead to ischemia and cause damage to papillary muscles
Root disease- b/c of dilation valves don’t close all the way and leak

4. Acute Rheumatic Fever: IO
2/3 all cases - developing countries
Episodes of RF are quite uncommon in U.S., except in immigrants.
Epidemiology - Identical to that of Group A Streptococcus; children 5-15
Pathogenesis- oropharyngeal infection; RF follows the sore throat; usually within 2-3 wks.
Mechanism - systemic immune process involving Group A strep. antigens; abnormal immune response. Preventable with adequate Rx of streptococcal pharyngitis.
Not seen much in this country.
IO – Interest Only

5. Diagnosis - Jones Criterion
Carditis - Pancarditis involving valves, endocardium, myocardium and pericardium
Healing of Rheumatic valvulitis - fibrous thickening resulting in valvular stenosis or insufficiency
Migratory polyarthritis
Sydenham’s Chorea
Subcutaneous nodules
Erythema marginatum IO
When valves get inflammed they get damaged. Usually localized to valves on the left side of the heart

6. Treatment
Antistreptoccal Rx until regimen finished; Penicillin IM or oral (10 day course) Erythromycin and others are alternatives
Arthritis/fever - Salicylates
Severe carditis- Glucocorticoids
HF, MR, AI - specific Rx.
Secondary prophylaxis to prevent recurrences- PCN or alternative until adult. IO
Must assume strep infection and treat it

7. Cardiac Pressures Images.google.com 4-12 4-12 4-12
Valvular ds deals with changes in pressures within the heart.
4-12
8-15
4-12
4-12
Images.google.com

8. Mitral Regurgitation-Etiology
Chronic Rheumatic heart disease- ing frequency
LV dilatation from any cause
Coronary Heart Disease: Papillary muscle dysfunction with ischemia/infarction
Mitral Valve Prolapse
Infective endocarditis
Two most common are mitral regurg and aortic valve stenosis.

9. Mitral Regurgitation Images.google.com
Blood going to LA backwards from LV and from Pulm veins, makes LA stretch to handle blood, and now have increase in blood that goes into LV.
Images.google.com

10. Pathophysiology of MR Blood regurgitates from LV into LA.
LV volume increases progressively as severity of MR increases.
Increased blood return to LA: pulmonary veins + regurgitant volume from previous beat.
LV function- well preserved initially; often deteriorates in later stages as does cardiac output (CO). LV compensates for volume overload via the Starling mechanism.
Left atrium (and LV) dilates over time - LAP and LVEDP gradually risepulmonary congestion
Afib. common.
With inc volume in LV – inc CO. Handles well as long as its not acute. Eventually LV will weaken and begin to fail.
When LA pressures go up, Pul veins go up, pul cap goes up, leaks blood into lungs = pulm congestion.
Any ds that inc La vol or pressure can lead to arrhythmias.

11. Symptoms Often asymptomatic for years
Fatigue, DOE, orthopnea- symptoms of left sided heart failure (detailed discussion later in CV system).
With chronic severe MR –Elevation of pulmonary venous pressure leads to  PuVR  PAH and subsequent Rt Heart failure: hepatic congestion, peripheral edema, etc.
One of the causes is anything that causes LV to dilate – has a pos feedback cyle. Mitral regurg begets mitral regurg
Eventually can get to HF
Pulm cap pressures leak – pul arterioles constrict to prevent additional bloodflow into caps (protection) – not good for pulm circulation as it causes inc pulm vasc resistance – inc pulm artery pressure – hypertrophy RV – ultimately will fail.
Problem starts on L side of ht and eventually gets to R side.

12. Physical Exam
Palpation: Systolic thrill may be present at apex depending on turbulence.
Auscultation: S1 soft or absent; S3 gallop if significant MR; Systolic Murmur is hallmark - Gr. II-IV/VI holosystolic in most cases -radiates to axilla (exception is MVP); murmur is high pitched and blowing.
Holosystolic – goes through systole.

13. Additional Findings ECG: LAE; Atrial arrhythmias (Afib).
Echo/Doppler: LA & LV size; LV function. Can estimate severity of MR; LV often dilates with progressive MR.
CxR: Late findings - Progressive LVE; HF; pulmonary edema.
Best way to dx valvular ht ds p exam is with cardiac ultrasound.
Usually develops chonically.
LVE- left ventricular enlargement
CxR- chest x-ray

14. Treatment of MR
Medical - Treatment depends on severity. Once symptomatic: Decreased physical activity and Na restriction. Drug therapy often significantly improves symptoms and patients may do well for many years.
ACE inhibitors or other vasodilators: decrease afterload and preload.
Diuretics: decrease preload, Na and volume overload
Inotropic agents: digoxin- limited role
Usually slowly progressive problem that can lead to late sx of HF (SOB, orthopnea, peripheral edema, etc).
Vasodilators – relax smooth muscle in arterioles and arteries. Dec systemic vascular resistance – lowers systemic BP – allows ventricle to contract against less resistance so it ejects better. Less blood will go back into the atrium.

15. Treatment of MR Surgical- Indications:
Severe MR with Sx
Dilating LV with progressive dysfunction  EF (even with mild symptoms).
Timing of surgery is important; needs to be done before significant deterioration of LV function/EF.
Surgical result dependent on pre-existing LV function. Mitral valve repair is preferred to MV replacement.

16. Mitral Valve Prolapse
Very common (3-5% adults) - Excessive redundant MV tissue from abnormal connective tissue:
MVP - most common form involves MV without major connective tissue disease elsewhere in body. Familial form also exists- autosomal dominant.
MVP as part of major CT disease (Marfan’s, Ehler’s-Danlos) or variations; these disorders are uncommon.
Pathology- myxomatous degeneration of MV leaflet tissue.
Associated deformities: high arched palate; pectus excavatum.
Another cause of mitral regurge. Common.
W>M
Have abnormal tissue in valve (born with it). Leaflets become redundant and floppy.
Buckles when valve closes.
Pectus – bowing inward of sternum.
Most of the time is a benign condition. Some can develop sig mitral regurg over life- mostly men and may require tx

17. Mitral Valve Prolapse
Images.google.com

18. MVP: Pathology
Mitral regurgitation can develop due to redundant floppy valve leaflets and/or involvement of the MV supporting structures – chordae tendineae.
Stress on Papillary muscles or chordae is presumed reason for localized and atypical chest pain.
Abnormal valve structure and MR can predispose to infective endocarditis but incidence is very low antibiotic prophylaxis no longer indicated.
CP – not the type that comes with CHD. Sharp, lancing, jabbing , very well localized. Fleeting, atypical CP.

19. Clinical Features
Female > male; Sx, when present, commonly occur at ages
Most are asymptomatic; often detected on PE- characteristic murmur.
Most common symptoms when present: chest pain (*atypical) and palpitations.
Arrhythmias common: PAC’s, PVC’s, PSVT, non-sustained VTach.
Sudden death – exceedingly rare arrhythmias
*Atypical – CP unlike the pain/discomfort that is present with coronary heart disease

20. Physical Findings
Auscultation: Mid to late systolic click (tensing of chordal structures).
High pitched late systolic murmur best heard at apex.- click and murmur occur earlier and get louder with maneuvers that decrease LV volume: standing after squatting, valsalva. Maneuvers that increase LV volume delay the click and soften the murmur: isometric hand grip, squatting.
Not holosystolic. Most murmers on L ht get louder with maneuvers that make ht larger. This does opposite (gets louder with maneuvers that make ht smaller)

21. Additional Findings/Treatment
ECG: NST-TW changes. Usually in leads II, III, aVF.
Echo/Doppler: Diagnostic; shows MVP and identifies MR when present.
Treatment: Reassurance; ß-Blockers for chest pain or arrhythmias; additional anti-arrhythmics usually not necessary.
Infrequently, severe MR develops requiring MV repair (more common in men than women).

22. Mitral Stenosis 2/3 females, 1/3 males- only cause is RF.
About 40% of all cases of RF develop MS.
Valve leaflets thicken and calcify, commisures fuse; valve orifice narrows; subvalvular supporting apparatus involved.
Least common rheumatic valvular lesion.
Least common. Only cause is rheumatic ht ds.
Decreases flow from LA to LV.
LA enlargement due to higher pressures to try and push blood through. LV fxn is nl.

23. Mitral Stenosis
Images.google.com

24. Images.google.com

25. Pathophysiology
Normal MVA cm2. Valve leaflets fuse, decreasing valve area. Severe MS < 1 cm2.
LA pressure rises in order to propel blood across the stenotic valve- pressure gradient compared to LVEDP.
LAP reflected backwards into the pulmonary circulation results in pulmonary venous congestionpulm capillary congestion  interstitial fluid  dyspnea. Pulmonary arterioles subsequently constrict.
MVA gets narrowed.
Can lead to R ht failure

26. LV function usually normal. LVEDP normal.
Chronic severe MS - Elevation of pulmonary vascular resistance (PVR) and subsequent development of Pulmonary Artery Hypertension (PAH).
Chronic PAH RVH  RV dysfunction and failure.
CO at rest- usually normal but does not rise adequately with exercise. With severe MS and PAH, CO eventually falls.

27. Symptoms/Complications
DOE, orthopnea, PND pulmonary edema
Findings of Rt Ht failure - late
Atrial arrhythmias: PAC’s, Atrial Fibrillation and Flutter
Hemoptysis  ruptured pulmonary capillaries
Atrial Thrombi and embolization - (AFib)
Afib very common

28. Physical Exam Palpation: prominent RV impulse
Auscultation: S1 loud/accentuated; S2 loud if PAH present Opening Snap of MV-apex, follows S2. Diastolic Rumble- Follows OS; low pitched/apex; length correlates with severity; MR murmur often audible.
Need bell to hear rumble.

29. Additional Findings ECG: LAE/LAA; RAD, RVH (over time)
Echo/Doppler: diagnostic-shows abnormal valve motion, estimates the gradient and MVA, defines LA size and LV function.
CxR: Pulmonary congestion; RVE.
Cardiac Cath: Documents gradient, MVA, presence or absence of MR and more.
RVH – R axis deviation

30. MS - Treatment Sodium restriction, diuretics.
Rate control of Afib or cardioversion.
Surgery -Mitral valvulotomy - marked symptomatic improvement. MVR only when repair cannot be done (mortality 3-5 %).
Percutaneous balloon valvuloplasty- alternative to surgery; if successful, avoids or delays need for surgery.
2 ways to approach:
Young pt – balloon across MV and open leaflets (percutaneous valvuloplasty) only when hasn’t fused
Other – Surgery p valve has fused.

31. Aortic Stenosis-Etiologies
Common: 20% all valvular disease; 80% males
Bicuspid valve leaflets thicken, fuse Rheumatic valvulitis leaflets thicken, fuse Idiopathic – Sclerocalcific: chronic wear and tear- develops in the elderly  leaflets thicken, fuse
Note: Thickening/calcification (without fusing) of the AoV often occurs with aging (Aortic Sclerosis) without progressing to significant aortic stenosis  Gr II/III murmur. Important to differentiate using history (asymptomatic), PE and echocardiogram if needed.
Idiopathic is most common. Sits in a high pressure environment. Get wear and tear.
Dilemma is trying to decide if they have aortic stenosis or sclerosis (noisy valve)
Sclerosis is asx, noisy
Need cardiac ultrasound to differentiate.

32. Aortic Stenosis Images.google.com
Thickened cusps. – increase in LV pressure – LVH (common causes are aortic stenosis and HTN)
Images.google.com

33. Pathophysiology Obstruction to LV outflow- pressure overload.
Systolic gradient between LV and Ao.
Obstruction gradual - initially well tolerated; LV hypertrophy is compensatory.
Cardiac Output often normal at rest - does not adequately rise with activity.
Late in course- LV failure, LVEDP rises, CO falls.
Difficulty w exercise b/c ht can’t increase anymore

34. Coronary blood flow is impaired from high LV pressures.
Myocardial oxygen consumption (MVO2) increases from LVH and high LV pressures.
Coronary blood flow is impaired from high LV pressures.
Myocardial ischemia can occur in the absence of *CHD severe LVH/ high pressures  outstrips coronary blood flow
Associated CHD may be present.
Normal AoV area: cm2 Critical AS: valve area <0.75 cm2
*CHD- coronary heart disease

35. AS Hemodynamics Images.google.com
Gradient between peak syst and peak aortic pressures.
Images.google.com

36. Symptoms of AS
Exertional dyspnea - elevation of LVEDP transmitted backward into pulmonary circuit.
Angina Pectoris-Increased MVO2 (pressure overload and hypertrophy) and decreased coronary reserve. *CHD may co-exist but does not have to be present for angina to develop.
Syncope - Peripheral vasodilation with inadequate forward CO with activity or from arrhythmia.
HF occurs late - very poor prognosis.
Pain mimicks pain of CHD.
CHD – coronary heart disease

37. Physical Exam Carotid pulse rises slowly; sustained peak.
Apex displaced laterally; +/- systolic thrill; systolic ejection sound (click ) variable.
Aortic valve closure is delayed - fixed or paradoxical splitting of S2.
S4 gallop common.
Murmur - SEM (crescendo-decrescendo)- peaks in mid to late systole depending on severity; harsh, low pitched, best heard at base and radiates to carotids – Grade II-IV/VI.
Carotids are reflexion of aorta pulse
Aplex displaced b/c heart enlarges.
Can hear atrial contaction – S4 gallop
Get low pitch sawing sound

38. Additional Findings ECG: LVH common; LAA.
Echo/Doppler: Diagnostic- identifies LVH, valve calcification and restriction; estimates gradient and aortic valve area.
CxR: LV prominence, displaced apex.
Cardiac Cath: Usually necessary prior to surgery; identifies gradient, valve area, LV function and presence or absence of CAD.
Dramatic LVH/will meet many criteria
Key in cath is to look for CAD

39. Natural History: Untreated
Angina Pectoris -death within 3 years
Syncope - death within 3 years
Dyspnea - death within 2 years
CHF - death within 1.5 years

40. Treatment
Medical - Mild to moderate AS without symptoms: Careful F/U; serial echo/doppler studies. Limited role for meds once symptoms begin.
Surgical- severe or symptomatic AS. Valve replacement with tissue or mechanical valve - Op risk 4%; 60-70% 10 yr. survival; marked symptomatic improvement.
Ross procedure an option for young patients with AS.
Ballon valvuloplasty- palliative
Balloon doesn’t work here – closes back down.
Ross – removes pulmonic valve and puts it in aortic and puts tissue valve to pulm.

41. Aortic Regurgitation- Etiology
75% male
Rheumatic Heart Disease
Infective endocarditis on previously deformed valve
Aortic Root disease and dilatation
Bicuspic Aortic Valve (AS more common than AR)
Less common.

42. Aortic Regurgitation
Would hear diastolic murmur.

43. AR: Pathophysiology
Increase in LVEDV (preload): blood returning from LA + regurgitant blood.
LV dilates- allows increased SV (stroke volume) and adequate effective forward SV (Starling’s law).
Over time (years) LV function gradually declines and EF (ejection fraction) deteriorates.

44. Pathophysiology
LV deterioration often precedes symptoms (reason for serial echo/doppler exams).
As AR progresses, CO fails to rise adequately with exercise, LV dysfunction worsens  Increased LVEDP pulmonary congestion HF.
Lv deterioration makes dealing with pt’s tricky. Need to watch and tx when necessary.

45. AR: History Sometimes familial - Connective tissue disease.
History of RF or infective endocarditis.
Patient often asymptomatic for yrs. with significant AI.
Symptoms: Palpitations, exertional dyspnea, orthopnea; PND and HF occur later.
Atypical chest pain common.
Followed and watched to find out when and if they need valve replacement.

46. AR: Physical Exam
Arterial Pulse - Rapid rising “water-hammer pulse” and collapsing pulse. “Quinke’s pulse” - alternate flushing and palling of the skin at the nail root.
“Pistol shot” sound over femoral artery in systole.
Derosiez’ sign - to and fro murmur over femoral artery.
Arterial pulse pressure widened- elevated systolic pressure (often greater than 200mm) and lowered diastolic pressure.
Diastolic pressure is low b/c blood leaks back, lose the pressure

47. Physical Exam Palpation - apex displaced laterally/inferiorly.
Diastolic Thrill may be present along LSB.
Auscultation: S2 soft; S3 common; high pitched blowing diastolic decreshendo murmur (LSB).
Best heard with diaphragm – patient sitting upright/leaning forward.
Systolic ejection (increased flow across AoV) murmur.

48. Additional Findings ECG - Increased voltage/LVH develops over time.
Echo/Doppler: early on LV contractility normal or increased - later, LV dysfunction; AI jet detectable and semi quantitated by Doppler.
Cardiac Cath: Identifies severity of AI, degree of LV dysfunction and intra-cardiac pressures. Needed to assess coronary arteries in older adults. Cath may not be needed in younger patients.

49. Treatment of AR
Medical - very close follow-up; serial Echo/Doppler studies. Same Rx as for CHF: Afterload reduction with vasodilators (ACEI, hydralazine). Preload reduction with diuretics; digoxin may be useful in selected individuals.
Surgical - Timing of surgery is difficult as pts. with AI do not develop symptoms until after the development of LV dysfunction. Surgery indicated for progressive LV dilatation and dysfunction +/- symptoms.
Valve replacement is tx of choice

50. Surgery
Aortic Valve Replacement with bioprosthesis (tissue valve) or mechanical valve. Ross procedure an option if young.
Op mortality dependent on pre-op LV function (5% or greater mortality).