Valvular Heart Disease

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Valvular Heart Disease

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Mitral Stenosis

• The MV leaflets thicken, the commissures fuse,
  and the chordae tendineae thicken and shorten.
• Almost always the result of rheumatic fever.
• Less common causes include congenital MS, SLE,
  RA, atrial myxoma, and bacterial endocarditis.

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Pathophysiology -1

• The normal area of the MV orifice is 4-6 cm2.
• Pure MS develops in approximately 40 of all pts
  with RHD.
• A latency period of 10-20 years, or more, after
  an episode of rheumatic fever therefore, the
  onset of MS symptoms does not occur until then.
• The orifice ? 2 cm2, an increase in LA pressure
  (LAP) is necessary for normal transmitral flow to
  occur.
• Critical MS - the opening lt 1 cm2, at this stage,
  a LAP of 25 mm Hg is required to maintain a
  normal cardiac output.

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Pathophysiology -2

• ? LAP raises pulmonary venous and capillary
  pressures, resulting in exertional dyspnea.
• As the disease progresses, chronic ? LAP leads to
  pulmonary hypertension, tricuspid and pulmonary
  incompetence, and eventual right heart failure.

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• Frequency - In the US The prevalence of MS has
  decreased because of the decline in the
  occurrence of rheumatic fever in the US and
  developed countries.
• Mortality/Morbidity - Without surgical
  intervention, the progressive nature of the
  disease results in an 85 mortality rate 20 years
  after the onset of symptoms.
• Sex - 2/3 of all patients with MS are female.
• Age - the onset of symptoms usually is between
  the third and fourth decades.

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Clinical

• History - Hx of acute rheumatic fever.
• History of murmur
• Effort-induced dyspnea
• - Most common complaint.
• - Often triggered by exertion, fever, anemia,
  onset of Afib, or pregnancy.
• Orthopnea
• Chest pain due to RV ischemia, concomitant
  coronary atherosclerosis, or a coronary embolism.
• Thromboembolism may be the first symptom of MS.
• Palpitations
• Recumbent cough

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Physical

• Peripheral and facial cyanosis
• Jugular venous distention
• Resp. distress, evidence of pulmonary edema (eg,
  rales)
• Digital clubbing
• Systemic embolization
• Signs of right heart failure in severe MS include
  ascites, hepatomegaly, and peripheral edema.
• If pulmonary hypertension is present, there may
  be a RV lift an increased pulmonic S2 sound and
  a high-pitched, decrescendo, diastolic murmur of
  pulmonary insufficiency (ie, Graham Steell's
  murmur).

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Heart Murmur

• Diastolic thrill that is palpable over the apex
• A loud S1, followed by an S2, and the opening
  snap are best heard at the left sternal border.
• This is succeeded by a low-pitched, rumbling,
  diastolic murmur, which is heard best over the
  apex while the patient is in the left lateral
  decubitus position.
• This may diminish in intensity as the stenosis
  increases.
• The duration, not the intensity, of the diastolic
  murmur correlates with the severity of the mitral
  narrowing.
• The holosystolic murmur of MR may accompany the
  valvular deformity of MS.

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Imaging Studies

• Chest x-ray
• - Signs of pulmonary overload include
• (1) prominence of pulmonary arteries, (2)
  enlargement of RV, and (3) evidence of CHF (eg,
  interstitial edema with Kerley B lines).
• - LA enlargement with straightening of the left
  heart border
• - Double density
• - Elevation of the left main stem bronchus
• - Pulmonary venous pattern changes with
  redistribution of the flow toward the apices
• - Prominent pulmonary arteries at the hilum,
  then they rapidly taper.

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Normal Cardiac Shadow
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Mitral Stenosis
- LA border is prominently convex
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• Echocardiography
• - the most sensitive and specific noninvasive
  method
• - the size of the mitral orifice can be
  measured
• - color doppler can evaluate the transvalvular
  gradient,
• pulmonary artery pressure, and accompanying
  MR.
• - Transesophageal echocardiography (TEE) is
  useful for
• detecting vegetations that are smaller than 5
  mm or thrombi
• in the left atrium, which are not seen with
  transthoracic
• echocardiography.

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Echocardiographic Score
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• Electrocardiogram
• - an enlarged left atrium is signified by a
  broad notched P wave, which is most prominent in
  lead II, with a negative terminal force in V1.
• - With severe pulmonary hypertension, right
  axis deviation and RV hypertrophy can be seen.
• - Atrial fibrillation is a common but
  nonspecific finding in MS.

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• Cardiac Catheterization
• - Increased left atrial or pulmonary capillary
  wedge pressure (PCWP)
• - Increased left atrial or PCWP to left
  ventricular pressure gradient
• - Calculated mitral valve orifice area
• - Calcified mitral valve
• - Concomitant mitral regurgitations
• - Presence of coronary artery disease (CAD)

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Complications

• Mural thrombi
• - 20 of pts, at high risk for embolization are
  those aged over 35, those with Afib and a low
  cardiac output, and those having a large LA
  appendage.
• Development of Atrial fibrillation
• - up to 40 of pts.
• - Loss of atrial contraction with the
  development of Afib
• ? cardiac output by 20
• - Since cardiac output is related to the heart
  rate, Afib with a
• rapid ventricular response ? diastolic
  filling time
• and further compromises cardiac output

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Medical Treatment

• Rate control
• - Digitalis.
• - Beta-blocker.
• - Calcium channel blocker, in patients with a
  beta-blocker contraindication. (eg, diltiazem)
• Diuresis for signs of pulmonary edema.
• Anticoagulation Anticoagulation is helpful in
  preventing thrombus formation and embolization in
  pts with Afib.

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Interventional treatment

• Balloon valvulotomy
• - It most commonly is used in young pts without
• extensive valvular calcification, in pregnant
  women, and in
• pts who are unfavorable operative candidates
• Mitral valve replacement
• - performed if leaflets are immobile or heavily
  calcified.
• - It also is performed if there is severe
  subvalvular scarring.
• - Bioprosthetic or artificial mechanical valves
  can be used as replacements.

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Percutaneous Transluminal Mitral Valvuloplasty
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Mitral Regurgitation

• Causes
• - Acute rheumatic heart disease ( lt 40 y/o)
• - Mitral valve prolapse (ie, myxomatous
  degeneration)
• - Myxomatous degeneration, cause unknown, often
  is a slow process, with a complication being the
  rupture of the chordae tendineae.
• - Acute MR, can be caused by a chordae
  tendineae rupture or papillary m. dysfunction.
• - Ischemia is responsible for 3-25 of MR,
  severity is directly proportional to the amount
  of LV hypokinesis.

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• - Mitral annular calcification can contribute
  to regurgitation.
• - Impaired constriction of the annulus results
  in poor valve
• closure.
• - LV dilatation and heart failure can also
  produce annular dilatation and poor valve closure
  resulting in MR.
• - Tendineae rupture can be due to endocarditis,
  myocardial infarction, or trauma.
• - Papillary m. dysfunction usually is caused by
  infarction.

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Acute MR
Chronic MR
Lung edema with normal heart size
Diffuse dilatation of the heart
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• History and Physical Examination
• - A high-pitched "blowing" holosystolic murmur
  is common.
• - the intensity of the murmur does not
  correlate well with the severity of the MR.
• - A 3rd heart sound indicates a large LV
  filling volume, and its prevalence increases with
  increasing severity of the MR
• Echocardiography
• - determine the presence, severity, and
  mechanism of MR
• - size of the LA and LV and the degree of
  pulmonary HTN
• Catheterization is necessary only in pts being
  referred to surgery

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• Pathophysiology and Natural History
• Chronic MR
• - LV undergoes adaptive changes, such as
  chamber dilatation and eccentric hypertrophy.
• - EDV ? early, but the ESV EF initially
  remain normal
• - LV myocardial contractility ? slowly over
  time, while the pt remains asymptomatic. Later,
  symptoms develop and the depressed LV
  contractility becomes irreversible
• - The natural hx of chronic MR depends on its
  cause and severity and on LV function.
• - severe asympt. or minimally sympt. MR due to
  MV prolapse, dz progresses to OP is required at a
  rate of about 10/year.

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• Severe MR, with a flail leaflet and NYHA class
  III or IV suggest a high risk (an annual
  mortality rate of 34), which contrasts greatly
  with the 4 rate for pts with class I or II
  symptoms.

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• Medical Treatment
• - Optimum medical management of patients with
  severe asymptomatic MR is controversial.
• - If HTN or LV dysfunction(), treatment with
  afterload reduction is indicated.
• - In other pts, the use of afterload reduction
  is controversial, since there are no data to
  suggest that vasodilator therapy reduces the need
  for surgery.
• - ACEI may reduce the MR fraction and LV cavity
  size in some pts, especially if they have
  symptoms or an enlarged LV.

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• Surgical Treatment
• - The 1o indications for OP in severe MR are
  the development of symptoms or LV dysfunction.
• - The ideal time to operate is during
  transition from the chronic compensated state to
  the decompensated state.
• - The best results are achieved in pts with an
  EF gt 60 and an end-systolic size lt 4.5 cm.
• - Recent literature suggests a trend toward
  repairing the valve while the pt is still
  asymptomatic.

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Aortic Stenosis

• - Aortic stenosis (AS) is the obstruction of
  blood flow across the aortic valve.
• - AS has several etiologies congenital uni- or
  bicuspid valve, rheumatic fever, and degenerative
  calcific changes of the valve.
• Frequency
• - AS exists in up to 2 of those lt than 70
  yrs.
• - The etiology of AS in those aged 30-70 yrs
  can be rheumatic disease or calcification of a
  congenital bicuspid valve.
• - gt 70 yrs, degenerative calcification is the
  primary cause of AS.
• - Among people older than 75 years, 3 have
  critical AS.
• - Among children, 75 of cases of AS are in
  males.

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• History
• - usually has an asymptomatic latent period of
  10-20 years.
• - the classic triad of chest pain, heart
  failure, and syncope.
• - May have a higher incidence of
  nitroglycerine-induced syncope.
• Mortality/Morbidity
• - Sudden cardiac death occurs in 3-5 of
  patients with AS.
• - Adults with AS have a 9 mortality rate per
  year.
• - Once symptoms develop, the incidence of SCD
  increases to 15-20, with less than a 5-year
  survival rate.
• - Pts with exertional angina, syncope, and CHF
  have an average of 5, 3, 2 years survival
  respectively.

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• Pathophysiology
• - Stenosed AV, resistance to systolic ejection
  occurs and a systolic p. gradient develops
  between the LV and the aorta.
• - ? aperture that leads to a progressive ? LV
  systolic p.
• - ? pressure overload in the LV, causes an ?
  in ventricular wall thickness (ie, concentric
  hypertrophy), no chamber dilatation and
  ventricular function is preserved at this time.
• - Eventually, the LV dilates increase in LV
  end-diastolic pressure.
• - A sustained pressure overload eventually
  leads to myocardial decompensation.
• - ? myocardium contractility, which leads to a
  decrease in cardiac output, ? LVEDP, ? PCWP, ? EF
  C.O
• - Ultimately, congestive heart failure occurs.

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• Physical
• Palpation - laterally displaced apex (LVH)
  systolic thrill
• (at the base, the jugular notch, and along
  the carotid a.)
• Auscultation - Crescendo-decrescendo systolic
  ejection murmur (loudest at the base of the heart
  and most commonly is appreciated in the 2nd Rt
  intercostal space) S3 (LV dilates and fails) S4
  (LVH)
• Pulsus parvus et tardus
• - arterial pulse with a delayed and plateaued
  peak, decreased amplitude, and a gradual
  downslope.

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A, Normal aortic valve B, Congenital AS C,
Rheumatic AS D, Calcified AS E, Calcified
senile AS
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• Imaging Studies
• Chest x-rays
• - may show cardiac enlargement, more severe
  stages of AS, signs of LAE, PA enlargement,
  Rt-sided enlargement, and pul. congestion are
  evident.
• Echocardiographs
• - An echo-dense aortic valve with no cusp
  motion, ? max. aortic cusp separation (less than
  8 mm in the adult) is also indicative of severe
  AS, LVH, systolic P.G across the AV.
• Electrocardiogram
• - LVH ?/? strain pattern, 13 have conduction
  defects, LAE (mitral valve process)

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Calcified AS
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Aortic Stenosis - rounded LV border (LVH) -
prominent proximal ascending aorta (
post-stenosis dilatation)
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• Cardiac Catheterization
• - surgery, suspected of having CAD, and gt 40
  years (even without significant symptoms).
• - 50 incidence of underlying CAD.
• - OP is routinely recommended when a
  cross-sectional area is 0.8-1.9 cm.
• - unlike PBMC for treating mitral stenosis,
  valvuloplasty has been largely unsuccessful for
  treating aortic stenosis. (associated with a high
  risk of bleeding and embolic complications, and
  the 6-month success rate is dismally low)

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• Surgical Treatment
• - AS must be thought of as a surgical disease,
  since there are few medical alternatives to
  surgery and no medical treatments that positively
  affect survival.
• - Surgery is indicated when symptoms are
  present together with severe stenosis, as
  measured by echocardiography or catheterization.
• - The prognosis without surgery is poor.

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• Aortic Regurgitation
• Background
• - AR is the diastolic flow of blood from the
  aorta into the LV.
• - incompetence of the AV or any disturbance of
  the valvular apparatus (eg, leaflets, annulus of
  the aorta), resulting in diastolic flow of blood
  into the LV chamber.
• Pathophysiology
• - The most common cause of chronic AR used to
  be RHD.
• - Presently, it is most commonly caused by
  bacterial endocarditis
• - In developed countries, it is caused by
  dilatation of the ascending aorta

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• - AR may be a chronic disease process or it may
  occur acutely, presenting as heart failure.
• - Diastolic reflux through the AV can lead to
  LV volume overload.
• - The severity of the AR is dependent on the
  diastolic valve area, the diastolic pressure
  gradient between the aorta and LV, and the
  duration of diastole.

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• Causes
• Acute AR
• - rheumatic, infective endocarditis, ruptured
  sinus of Valsalva, trauma, prosthetic valve
  surgery, aortic dissection, laceration of the
  aorta
• Chronic AR
• rheumatic, syphilis, aortitis (ie, Takayasu
  disease), Marfan syndrome, osteogenesis
  imperfecta, bicuspid aortic valve, VSD, sinus of
  Valsalva, ankylosing spondylitis, Reiter
  syndrome, Rheumatoid arthritis, SLE,
  Hypertension, Infective endocarditis

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• Mortality/Morbidity
• - 3/4 of patients with significant AR survive 5
  years after Dx. half survive for 10 years.
• - mild-to-moderate AR survive 10 years in
  80-95 of the cases.
• - Average survival after onset of CHF is less
  than 2 years.
• - Acute AR is associated with high morbidity,
  which can progress from pulmonary edema to
  refractory heart failure and cardiogenic shock.
• Age
• - Chronic AR often begins in the late 50s and
  is documented most frequently in patients older
  than 80 years.

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Clinical

• Physical
• The hallmark is a high-pitched decrescendo
  diastolic murmur at the left sternal border after
  the 2nd heart sound.
• Acute AR
• - severe AR associated with CHF and/or shock
  often will appear gravely ill.
• - Tachycardia, cyanosis, pulmonary edema,
  arterial pulsus alternans, early diastolic
  murmur, Austin-Flint murmur
• Chronic AR
• - All auscultatory phenomena indicate
  vasodilatation of peripheral circulation.
• - Decrescendo diastolic murmur

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• - Pulsus bisferiens increased pulse pressure
  visible, forceful, and bounding peripheral pulses
  (water hammer)
• - Corrigans pulse the carotid pulse has a
  rapid rise full upstroke with a rapid fall in
  diastole
• - de Musset sign Bobbing of the head
• - Quincke sign Capillary pulsations of the
  nail bed
• - Muller sign Pulsations of the uvula
• - Hill sign Systolic pressure in lower
  extremity greater than upper extremity by 20 mm
  Hg
• - Traube sign Loud systolic sound over femoral
  arteries
• - Duroziezs sign Compression of femoral
  artery with a stethoscope produces a
  systolic-diastolic murmur
• - Pistol-shot femoral pulses

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• Austin-Flint murmur - a low pitched diastolic
  rumble ( similar to MS murmur indicates mod. to
  severe AR.
• Diagnosis
• Electrocardiography - usually shows LVH
• CXR - proximal aorta dilated and LV dilatation
• Echocardiography
• Cardiac catheterization

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Aortic Regurgitation - a Marfan synd. pt -
prominent LV border (LV dilatation) - ascend.
aorta is convex - dilated descend. aorta
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Aortic Regurgitation
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• Medical Treatment
• - AR is the only valvular disease in which
  medical therapy (ie, afterload reduction) is
  proven to alter the natural history.
• - The agent most studied is nifedipine In a
  study comparing it with digoxin, nifedipine was
  shown to significantly delay the need for aortic
  valve replacement in pts with severe asymptomatic
  aortic insufficiency, also had relatively smaller
  ventricle size and higher ejection fraction.

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• Indications for Surgery
• - As in pts with severe MR, the preoperative
  size of the LV in pts with AR is directly related
  to the postoperative EF, but with two important
  differences.
• - Pts with AR may have larger ventricles
  preoperatively and maintain normal EF
  postoperatively.
• - Also, if the EF has been reduced for lt 12 to
  14 mo., it may return to normal postoperatively.
• - Asymptomatic pts with normal LV function have
  an excellent prognosis, even in the presence of
  mild cardiac enlargement.

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• - The indications for valve replacement in pts
  with severe AR include
• 1) the onset of symptoms,
• 2) () LV dysfunction (EF lt50), and
• 3) () severe LV dilatation (end-systolic
  size gt5.5 cm).
• - Once significant LV systolic dysfunction is
  present, surgical outcome is less satisfactory.