1. Valvular Heart Disease:
An Update in Management
Speaker: Prof.Malik Tariq Mahmood
Case:
An active 75 yo farmer comes to your office after experiencing a fainting
spell while baling hay. The episode occurred without warning and he had no
symptoms following the episode. However, on close questioning he admits to some
breathlessness and vague chest heaviness with his usual heavy exertion over the past
few months and a very unwelcome tendency to want to slow down which he
reluctantly attributed to his age. He has been healthy all his life, doesn’t smoke and
has not seen a doctor in 30 years. He served in the army in 1942; no abnormalities
were reported during his induction physical
Physical Exam:
Robust looking older man with a laceration on his forehead from falling on the
handle of his pitchfork.
BP 135/90 P 68 bpm, regular RR-12 T-98.6° F
JVP 6 cm with normal “a” and “v” waves
Carotids: Difficult to palpate, delayed upstroke
Lungs: Clear
Heart: Palpation:
Palpable “thrill” over the mid LSB. PMI 5 ICS, 2 cm lateral to the MCL.
Palpable presystolic impulse followed by a sustained ventricular lift.
Auscultation:
Loud S4. S1 is normal. A single S2 (P2) is heard at the upper left sternal border
but no S2 is heard at the lower left sternal border. There is a 4/6 systolic ejection
murmur (crescendo-decrescendo) heard best at the R 2nd interspace but radiating
widely to the LSB, and to the neck. No diastolic murmurs.
Abdomen and extremities are unremarkable.
Aortic Stenosis:
Aortic Stenosis: Etiology:
♥ Congenital bicuspid aortic valve
♥ Rheumatic aortic valve disease
♥ Calcific (senile) aortic stenosis
Aortic Stenosis
Physiologic Principles-Natural History
2. • Normal aortic valve area is 3.0 - 4.0 cm2
• Circulation affected when valve area is reduced by ~ 75% (i.e. 0.75 - 1.0 cm2)
valve area (cm sq) mean gradient (mm Hg)*
Mild > 1.5 < 25
Moderate 1.0 - 1.5 25 - 50
Severe < 0.75 > 50
Aortic Stenosis
Physiologic Principles-Natural History:
• Primary adaptation is concentric hypertrophy
• Latent phase usually lasts decades
• Risk of sudden death is very low during this phase
• Rate of progression ranges from 0-0.3 cm2/yr. (average rate is 0.12 cm2/yr)
• 50% of patients with severe AS do not progress
• Cannot predict who will
Key Physical Findings in Severe
Aortic Stenosis:
♥ Carotid impulse: “parvus et tardus”
♥ JVP: Prominent “a” wave
♥ Heart: Systolic thrill
Palpable presystolic impulse (S4)
Sustained apical systolic impulse S4
Coarse late peaking systolic ejection murmur
(may radiate to neck and/or LSB)
Attenuated/absent aortic component of S2
Natural History of Aortic Stenosis:
♥ Long asymptomatic “latent” period
♥ “Cardinal” symptoms of severe aortic stenosis
Dyspnea
Angina
Syncope
♥ Sudden death
♥ Left ventricular dilatation and contractile failure
♥ Endocarditis
♥ Arrhythmias
Ventricular tachycardia
Conduction system disease
Atrial fibrillation
Natural History of AS:
3. Aortic Stenosis
Physiologic Principles-Natural History:
• Once symptoms develop, average survival is 2-3 yrs
• With LV systolic dysfunction, there may be increased risk of sudden death and
permanent LV dysfunction
Ross J Jr, Braunwald E: Aortic stenosis. Circulation 38[Suppl V]:61, 1968
Aortic Stenosis
Management Guidelines:
Initial Diagnostic Testing:
Lipids, renal fxn, Ca, P---all patients
CXR, ECG, Echocardiography---all patients
Cardiac catheterization with angiography
If clinical and echo data are discordant
To assess coronary circulation prior to surgery
Aortic Stenosis
Management Guidelines:
Initial Diagnostic Testing (cont.)
Treadmill stress testing
Dangerous in symptomatic pts
Not useful for dx of CAD
May be used to assess functional significance of severe AS in pts who deny
symptoms (e.g. bp response)
Aortic Stenosis
Management Guidelines:
Scheduled Follow-up:
4. office intervalecho interval
Mild AS 12 mos 5 yrs
Moderate AS 6 mos 2 yrs
Severe AS 6 mos 1 yr
Aortic Stenosis
Management Guidelines:
Low Gradient AS
• Special case
• Minimal valve mobility and low cardiac output
• Calculated valve area is small but pressure gradient is also small
• Functional vs. fixed AS?
• Consider dobutamine stress test (DSE) to clarify
ACC Classification of Recommendations:
Class I:
Conditions for which there is evidence and/or general agreement that a given
procedure or treatment is useful and effective.
Class II:
Conditions for which there is conflicting evidence and/or a divergence of
opinionabout the usefulness/efficacy of a procedure or treatment.
IIa. Weight of evidence/opinion is in favor of usefulness/efficacy IIb.
Usefulness/efficacy is less well established by evidence/opinion.
Class III:
Conditions for which there is evidence and/or general agreement that the
procedure/treatment is not useful/effective, and in some cases may be harmful.
Aortic Stenosis
Management Guidelines:
Recommendations for AVR
Class I
Severe AS and symptoms
Severe AS (with or without sxs) and need for CABG, other valve replacement
or aortic surgery
Class IIa
Moderate AS and need for other cardiac surgery
Asymptomatic severe AS and diminished LVEF or hypotensive response to
exercise
Aortic Stenosis
Management Guidelines:
Recommendations for AVR (cont.)
Class IIb
Asymptomatic AS and VT, severe LVH (>15mm)
or valve area <0.6 cm2
Class III
Asymptomatic AS with none of the above
CASE:A 52 yo salesman is referred to you for evaluation of a heart murmur. He
had applied for a pilot’s license and was denied because of the murmur. He is
5. asymptomatic and physically active. He denies chest pain, dyspnea or dizzy
spells and gives no history of a murmur being mentioned during his last physical
exam five years ago. He has no family history of heart disease. He has never had
high blood pressure or diabetes, doesn’t smoke, and takes no medications. A
lipid profile done five years ago was reported to be “OK”.
Physical Exam:
BP - 145/45 P - 78 reg RR - 12 Temp:98.6F
Carotids: Very brisk with sharp collapse
JVP: 5 with normal ‘a’ and ‘v’ waves
Lungs: Clear
Heart: Palpation: PMI is enlarged (4fb), in the anterior
axillary line
Auscultation:
S1 normal, S2 soft. A 2/6 early peaking systolic ejection murmur at
the upper RSB and a 3/6 holodiastolic blowing murmur, heard best
at the lower LSB when you ask the patient to hold his breath in
expiration and lean forward. There is a different 2/6 low-pitched
diastolic murmur at the apex.
Pulses are all very prominent and brisk; audible pulse over
the femoral arteries
Major Causes of Aortic Regurgitation:
Leaflet Dysfunction Aortic Root Dilation
Rheumatic fever Systemic hypertension
Endocarditis Dissecting aneurysm
Trauma Aortitis (syphilis)
Bicuspid aortic valve Reiter’s syndrome
Rheumatoid arthritis Ankylosing spondylitis
Myxomatous degeneration Ehlers-Danlos
Ankylosing spondylitis Osteogenesis imperfecta
Marfan’s syndrome Pseudoxanthoma elasticum
Fenfluramine-phentermine Marfan’s syndrome
Annulo-aortic ectasia
Physical Findings in Aortic Regurgitation:
Wide pulse pressure:
Bounding pulses
Soft aortic second sound (A2)
Early diastolic murmur (blowing) immediately after A2
Upper RSB with root dilation
Mid to lower LSB with leaflet dysfunction
Systolic murmur at base (similar to aortic stenosis)
Austin Flint murmur: mid to late diastolic “rumble” at apex
Some Really Neat Physical Findings in Severe Chronic Aortic Regurgitation:
♥ deMusset’s sign: Head bob with each systolic pulsation
♥ Corrigans’s pulses: “Pistol shot” pulses over femoral artery
♥ Mueller’s sign: Pulsation of the uvula
♥ Duroziez’s sign: Systolic/diastolic bruit over femoral arterY
6. ♥ Quincke’s pulses: Capillary pulsations seen in the nailbeds
♥ Becker’s sign: Pulsation of retinal arteries and pupils
♥ Hill’s sign: Popliteal BP exceeds brachial BP by > 60 mmHg
Pathophysiology of Chronic
Aortic Regurgitation:
♥ Slowly progressive diastolic volume overload
♥ Augmented stroke volume with rapid runoff
Increased systolic pressure with low
diastolic pressure: wide pulse pressure
♥ Progressive left ventricular dilation, some hypertrophy
♥ Increased diastolic compliance with maintenance of normal diastolic
pressures initially
♥ Late systolic failure with reduced ejection fraction and CHF
7. Additional Testing:
ECG: LVH with massive voltage in the lateral precordial leads (V4-V6)
Chest X-Ray: Large heart, predominant left ventricular enlargement. No
congestive heart failure.
Echo: Marked left ventricular dilation, estimated EF 65%. The end diastolic
dimension is 65 mm and the end diastolic dimension is 55 mm. Aortic valve:
bicuspid and thickened.
Doppler: Severe aortic regurgitation. The aorta is slightly enlarged (4.2 mm).
Acute Aortic Regurgitation:
♥ Sudden diastolic volume overload without LV dilation:
- Acute elevation in left ventricular diastolic pressure◊ pulmonary
edema
- Acute LV systolic failure ◊ hypotension
♥ Provide inotropic support, vasodilator therapy if tolerated, urgent valve
replacement
Natural History of Chronic
Aortic Regurgitation:
♥ Long asymptomatic phase; may be decades long.
♥ Left ventricular systolic dysfunction ( decline in EF)
NOTE!! LV dysfunction may occur in the absence of symptoms
♥ Symptoms associated with LV dysfunction:
- Exercise intolerance
- Dyspnea on exertion
♥ Angina (rare)
♥ Sudden death (rare)
Natural history of aortic regurgitation:
8. Aortic Regurgitation
Physiologic Principles-Natural History:
• LV faces combined pressure and volume load
• Primary adaptation is dilatation (eccentric hypertrophy)
• Since this adaptation takes time, AR classified as acute or chronic
• Acute AR results in sudden increase in LVEDP >>> pulmonary edema and
cardiogenic shock
Aortic Regurgitation
Physiologic Principles-Natural History:
• Latent phase of AR, like AS, may last decades
• Decompensation when
• LV systolic function begins to fail
• Progressive LV dilatation occurs
• Spherical geometry develops
• Initially this is reversible
• LV systolic function and ESD are the most important predictors of postop
survival and LV function
Aortic Regurgitation
Physiologic Principles-Natural History
• In asymptomatic pts with severe AS and nl LV systolic function, progression
is slow
• 4.3%/yr develop symptoms of LV systolic dysfunction
• 1.3%/yr progress to LV dysfunction without symptoms†
pooled data from 7 series. 490 pts with mean follow-up of 6.4 yrs
Aortic Regurgitation
Management Guidelines:
Scheduled Follow-up (office and echo)
Severe AR without symptoms
• q 4-12 month depending on pace of change and current LV ESD/EDD
Moderate AR without symptoms
• 1st follow-up in 2-3 months to establish pace, then ~ q 12 months
9. Aortic Regurgitation
Management Guidelines:
Vasodilator Therapy
• Expected to ι afterload, η stroke volume and
ι regurgitant volume
• Hemodynamic benefit shown with hydralazine and nifedipine, less consistent
results with ACEi
• Improvement in clinical outcomes in trial of LA nifedipine vs. digoxin (need
for AVR in 143 pts followed for 6 yrs--- 15% vs 34%)
• Dose titrated to achieve ι in SBP, not normalization
Aortic Regurgitation
Management Guidelines:
Vasodilator Therapy Indications
Class I
• Severe AR with symptoms or severe LV dilatation but contraindications to
surgery
• Severe AR without symptoms but LV dilatation and elevated SBP
• Any degree of AR with hypertension
• Persistent LV systolic dysfunction s/p AVR (ACEi)
• Short term therapy prior to AVR
Aortic Regurgitation
Management Guidelines:
Vasodilator Therapy Indications
Class III
• Mild to mod AR without sxs and nl LV function
• In lieu of AVR in pts without contraindications
Aortic Regurgitation
Management Guidelines:
Recommendations for AVR (chronic severe AR)
Class I
• NYHA functional class III or IV sxs
• NYHA functional class II sxs and progressive LV dilatation or declining
LVEF on serial studies
• CCS class II angina
• Mild or moderate reduction in EF (25-50%)
• Need for CABG or surgery on other valves
Aortic Regurgitation
Management Guidelines:
Class IIa
• NYHA class II sxs with nl LVEF (>50%) with stable EF, LV size and exercise
tolerance
• Asymptomatic pts with nl LVEF but severe LV dilatation (ESD > 55 mm or
EDD > 75 mm)
Class IIb
• LVEF < 25%
10. • Asymptomatic pts with nl LVEF and progressive LV dilatation with ESD 50-
55
Valvular Heart Disease
Mitral Stenosis
A 75 year old woman with loud first heart sound and mid-diastolic murmur:
• Chronic dyspnea Class 2/4
• Fatigue
• Recent orthopnea/pnd
• Nocturnal palpitation
• Pedal edema
Mitral Stenosis:
• Etiology
• Symptoms
• Physical Exam
• Severity
• Natural history
• Timing of Surgery
Mitral Stenosis: Etiology:
• Primarily a result of rheumatic fever
(~ 99% of MV’s @ surgery show rheumatic damage )
• Scarring & fusion of valve apparatus
• Rarely congenital
• Pure or predominant MS occurs in approximately 40% of all patients with
rheumatic heart disease
• Two-thirds of all patients with MS are female.
Mitral Stenosis:
Pathophysiology
• Normal valve area: 4-6 cm2
• Mild mitral stenosis:
• MVA 1.5-2.5 cm2
• Minimal symptoms
• Mod mitral stenosis
• MVA 1.0-1.5 cm2 usually does not produce symptoms at rest
• Severe mitral stenosis
• MVA < 1.0 cm2
Mitral Stenosis: Symptoms:
• Fatigue
• Palpitations
• Cough
• SOB
• Left sided failure
• Orthopnea
• PND
11. • Palpitation
• Afib
• Systemic embolism
• Pulmonary infection
• Hemoptysis
• Right sided failure
• Hepatic Congestion
• Edema
• Worsened by conditions that cardiac output.
• Exertion,fever, anemia, tachycardia, Afib, intercourse, pregnancy,
thyrotoxicosis
Recognizing Mitral
Stenosis
Palpation:
• Small volume pulse
• Tapping apex-palpable S1
• +/- palpable opening snap (OS)
• RV lift
• Palpable S2
ECG:
• LAE, AFIB, RVH, RAD
Auscultation:
• Loud S1- as loud as S2 in aortic area
• A2 to OS interval inversely proportional to severity
• Diastolic rumble: length proportional to severity
• In severe MS with low flow- S1, OS & rumble may be inaudible
Mitral Stenosis: Physical Exam
• First heart sound (S1) is accentuated and snapping
• Opening snap (OS) after aortic valve closure
• Low pitch diastolic rumble at the apex
• Pre-systolic accentuation (esp. if in sinus rhythm)
Common Murmurs and
Timing (click on murmur to play)
Systolic Murmurs
• Aortic stenosis
• Mitral insufficiency
• Mitral valve prolapse
• Tricuspid insufficiency
Diastolic Murmurs
• Aortic insufficiency
• Mitral stenosis
Auscultation-
Timing of A2 to OS Interval:
• Width of A2-OS inversely correlates with severity
12. • The more severe the MS the higher the LAP the earlirthe LV pressure falls
below LAP and the MV opens
Mitral Stenosis: Natural History:
• Progressive, lifelong disease,
• Usually slow & stable in the early years.
• Progressive acceleration in the later years
• 20-40 year latency from rheumatic fever to symptom onset.
• Additional 10 years before disabling symptoms
Mitral Stenosis: Complications:
• Atrial dysrrhythmias
• Systemic embolization (10-25%)
• Risk of embolization is related to, age, presence of atrial fibrillation, previous
embolic events
• Congestive heart failure
• Pulmonary infarcts (result of severe CHF)
• Hemoptysis
• Massive: 20 to ruptured bronchial veins (pulm HTN)
• Streaking/pink froth: pulmonary edema, or infection
• Endocarditis
• Pulmonary infections
Mitral Stenosis: EKG:
• LAE
• RVH
• Premature contractions
• Atrial flutter and/or fibrillation
• freq. in pts with mod-severe MS for several years
• A fib develops in ≈ 30% to 40% of pts w/symptoms
A 75 year old woman with loud first heart sound and mid-diastolic murmer:
13. Mitral Stenosis: Role of Echocardiography:
• Diagnosis of Mitral Stenosis
• Assessment of hemodynamic severity
• mean gradient, mitral valve area, pulmonary artery pressure
• Assessment of right ventricular size and function.
• Assessment of valve morphology to determine
suitability for percutaneous mitral balloon valvuloplasty
• Diagnosis and assessment of concomitant valvular lesions
• Reevaluation of patients with known MS with changing symptoms or signs.
• F/U of asymptomatic patients with mod-severe MS
Mitral Stenosis:Therapy:
14. • Medical
• Diuretics for LHF/RHF
• Digitalis/Beta blockers/CCB: Rate control in A Fib
• Anticoagulation: In A Fib
• Endocarditis prophylaxis
• Balloon valvuloplasty
• Effective long term improvement
Recommendations for Mitral Valve Repair for Mitral Stenosis:
• ACC/AHA Class I
• Patients with NYHA functional Class III-IV symptoms, moderate or severe
MS (mitral valve area <1.5 cm 2 ),*and valve morphology favorable for
repair if percutaneous mitral balloon valvotomy is not available
• Patients with NYHA functional Class III-IV symptoms, moderate or severe
MS (mitral valve area <1.5 cm 2 ),*and valve morphology favorable for
repair if a left atrial thrombus is present despite anticoagulation
• Patients with NYHA functional Class III-IV symptoms, moderate or severe
MS (mitral valve area <1.5 cm 2 ),* and a non-pliable or calcified valve with
the decision to proceed with either repair or replacement made at the time of
the operation.
Recommendations for Mitral Valve Repair for Mitral Stenosis:
• ACC/AHA Class IIB
• Patients in NYHA functional Class I, moderate or severe MS (mitral valve
area <1.5 cm 2 ),* and valve morphology favorable for repair who have had
recurrent episodes of embolic events on adequate anticoagulation.
ACC/AHA Class III
Patients with NYHA functional Class I-IV symptoms and mild MS.
*The committee recognizes that there may be a variability in the measurement
of mitral valve area and that the mean trans-mitral gradient, pulmonary artery
wedge pressure, and pulmonary artery pressure at rest or during exercise should
also be considered.
Mitral Stenosis
Physiology/Natural History:
• Normal MVA 4 -5 cm2
• Symptoms not apparent until area < 2.5 cm2
• valve area (cm sq) mean gradient (mmHg)* Mild > 1.5
< 5
Moderate 1.0 - 1.5 5 -10
Severe < 1.0 > 10
Mitral Stenosis
Physiology/Natural History:
• Akin to severe diastolic dysfunction
• V = IR (electrical)
• P = QR (hydraulic)
• Q = P/Rvalve
15. Mitral Stenosis
Physiology/Natural History:
• LA pressure → PV pressure → interstitial edema → ± alveolar flooding
• Adaptations:
-pulmonary vascular constriction, intimal hyperplasia, medial hypertrophy
→ reversible pulmonary hypertension → ± fixed pulm htn
-downregulation of neuroreceptors, lymphatic drainage
Mitral Stenosis
Physiology/Natural History:
• Latent (subclinical) phase in RHD 20-40 yrs
• 10 yrs of symptoms before disabling
• With physically limiting symptoms
• 10 yr survival 0-15%
• 10-20% systemic embolism
• 30-40% develop AF
• With onset of severe pulm hypertension
• Mean survival < 3 yrs
Mitral Stenosis
Management Guidelines:
Initial Evaluation
• History
• Physical
• ECG
• CXR
• Echocardiogram
• ± Exercise echocardiogram
Mitral Stenosis
Management Guidelines:
Medical Therapy
• Rheumatic fever prophylaxis
• Infective endocarditis prophylaxis
• Limitation of strenuous physical activities
• Control of HR (negative chronotropes)
• Na restriction, intermittent diuretic use
• Prompt management of AF
Mitral Stenosis
Management Guidelines:
Interventional and Surgical Options
• Percutaneous mitral balloon valvotomy (PMBV)
• Closed commissurotomy (obselete)
• Open commissurotomy
• Mitral valve replacement
Mitral Stenosis
Management Guidelines:
16. Indications for PMBV (class I and IIa)
• Suitable anatomy, no LA clot, ≤ mild MR
• Symptomatic pts (NYHA class II-IV) with MVA <1.5 cm2
• Asymptomatic pts with MVA <1.5 cm2 and PASP 50 mmHg at rest, 60 with
exercise
Mitral Stenosis
Management Guidelines:
Indications for MVR (class I and IIa)
• Symptomatic pts (NYHA class III and IV) with MVA < 1.5 cm2 unsuitable
for PMBV
• NYHA class I and II pts with MVA < 1.0 cm2 and PASP >60 at rest
unsuitable for PMBV
Acute Mitral Regurgitation
Management Guidelines:
Medical Stabilization (while gathering OR team)
• If normotensive: nitroprusside
• If hypotensive: nitroprusside + dobutamine or
intra-aortic balloon pump (IABP)
Case 4
• On exam:
• obese, loquacious man with petite wife
• HR 86. BP 170/94. BMI 45.
• JVP 12 cm H2O. Nl carotid upstrokes
• diminished bs, no crackles
• apical impulse not palpable
• Neither S1 or S2 are well heard, obscured by a holosystolic blowing murmur
at apex and left parasternal border
Case 4:
• ECG:
• SR, RAD, LA abn, R>S in V1, NSSTT abn
• CXR:
• LA and LV enlargement
• Echo:
• severe LA enlargement, mild LV dilatation (ESD 45mm), nl LVEF (60%),
pulmonary hypertensio (est PASP 55 mmHg)
Chronic Mitral Regurgitation
Physiology and Natural History:
• Gradual development allows adaptation
• LA dilatation and increase in compliance
• LV dilatation and EF (via preload and ↓ afterload) → maintenance of
forward SV
• Compensation often adequate for vigorous exercise
• May last many years
17. Chronic Mitral Regurgitation
Physiology and Natural History:
• Eventually, volume overload → LV decompensation
• Preop LVEF (>60%) and LVESD (<45 mm) are primary predictors of
postop survival
Acute Mitral Regurgitation
Physiology and Natural History:
• Abrupt volume load---no time for adaptation
• Sudden ↓ in forward stroke volume
• Sudden in LA volume/pressure → PV pressure
• Rapidly fatal
Chronic Mitral Regurgitation
Management Guidelines:
Initial evaluation
• History
• Physical Exam
• ECG
• CXR
• Echo
• ± Exercise echo
Chronic Mitral Regurgitation
Management Guidelines
Scheduled Follow-up*
Instruct all pts to report any cv symptoms
office intervalecho interval
Mild MR 12 mos if sxs
Moderate MR 12 mos 1-2 yrs
Severe MR 6-12 mos 6-12 mos**
Chronic Mitral Regurgitation
Management Guidelines:
Medical Therapy
• No generally accepted rx in asymptomatic pts
• No long term studies suggesting benefit of afterload reduction in absence of
hypertension
• ACEi if hypertensive
• AF requires rate control, anticoagulation and 1 attempt at restoration of SR
Chronic Mitral Regurgitation
Management Guidelines:
Surgical Options
• Mitral valve repair
• Mitral valve replacement with preservation of subvalvular apparatus
• Mitral valve replacement with excision of subvalvular apparatus
• MVR with CABG (in ischemic MR)