Valvular Heart Disease and the Cardiac Exam

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					Valvular Heart Disease and
    the Cardiac Exam
        Charlotte Bai, M.D.
  Internal Medicine Board Review
           May 28, 2009
                          Overview
   Clinical syndromes
   Overview of cardiac murmurs and maneuvers
   Left sided valvular lesions
    – Aortic stenosis and sclerosis
    – Mitral stenosis
         Rheumatic fever prophylaxis
    – Acute and chronic aortic regurgitation
    – Acute and chronic mitral regurgitation
   Right sided valvular lesions
    – Tricuspid valve disease
   Prosthetic valves
   Endocarditis prophylaxis
   Questions
                   General Appearance
   Marfan Syndrome                     Fredreich ataxia
    – Tall, long extremities             – Lurching gait, hammertoe, pes
    – Associated with: aortic root         cavus
      dilitation, MV prolapse            – Associated with: hypertrophic
   Acromegaly                             cardiomyopathy
    – Large stature, coarse facial      Duchenne type muscular
      features, “spade” hands            dystrophy
    – Associated with: Cardiac           – Pseudohypertrophy of the
      hypertrophy                          calves
   Turner Syndrome                      – Cardiomyopathy
    – Web neck, hypertelorism,          Ankylosing spondylitis
      short stature                      – Straight back syndrome, stiff
    – Associated with: Aortic              (“poker”) spine
      coarctation, pulmonary             – Associated with: AI, CHB
      stenosis                             (rare)
   Pickwickian Syndrome                Lentigines (LEOPARD
    – Severe obesity, somnolence         syndrome)
    – Associated with: Pulmonary         – Brown skin macules that do
      hypertension                         not increase with sunlight
                                         – Associated with: HOCM, PS
“Spade” hands in acromegaly
                General Appearance- 2
   Hereditary hemorrhagic                 Sarcoidosis
    telangiectasia (Osler-                  – Cutaneous nodules, erythema
    Weber-Rendu)                              nodosum
    – Small capillary hemangiomas           – Associated with: Secondary
      on the face or mouth                    cardiomyopathy, heart block
    – Associated with: Pulmonary           Tuberous Sclerosis
      arteriovenous fistula                 – Angiofibromas (face; adenoma
   Lupus                                     sebaceum)
    – Butterfly rash on face,               – Associated with:
      Raynaud phenomenon- hands,              Rhabdomyoma
      Livedo reticularis                   Myxedema
    – Associated with: Verrucous            – Coarse, dry skin, thinning of
      endocarditis, Myocarditis,              lateral eyebrows, hoarseness
      Pericarditis                            of voice
   Pheochromocytoma                        – Associated with: Pericardial
    – Pale diaphoretic skin,                  effusion, LV dysfunction
      neurofibromatosis- café-au-lait
      spots
    – Associated with:
      Catecholamine-induced
      secondary dilated CM
       Grading the Intensity of Cardiac
                  Murmurs
   Grade 1
    – Murmur heard with stethoscope, but not at first
   Grade 2
    – Faint murmur heard with stethoscope on chest wall
   Grade 3
    – Murmur hears with stethoscope on chest wall, louder than grade
      2 but without a thrill
   Grade 4
    – Murmur associated with a thrill
   Grade 5
    – Murmur heard with just the rim held against the chest
   Grade 6
    – Murmur heard with the stethoscope held away and in from the
      chest wall
                     Cardiac Murmurs

   Most mid systolic murmurs of grade 2/6 intensity
    or less are benign
    – Associated with physiologic increases in blood
      velocity:
        Pregnancy
        Elderly
   In contrast, the following murmurs are usually
    pathologic:
    – Systolic murmurs grade 3/6 or greater in intensity
    – Continuous murmurs
    – Any diastolic murmur
Maneuver                      Hemodynamic Effect              Murmur Effect
Normal respiration            Transient ↑ in venous filling   ↑ right-sided murmurs
                              during inspiration
Passive leg elevation         ↑ venous return (transient      ↑ right-sided murmurs,
                              ↑ in LV size and preload)       ↓murmur of HOCM and MVP
Stand to squat                ↑ venous return (transient      ↑ right-sided murmurs,
                              ↑ in LV size and preload)       ↓murmur of HOCM and MVP

Squat to stand                ↓ venous return (transient ↓    ↑ murmur of HOCM, moves
                              in LV size and preload)         midsystolic click of MVP
                                                              closer to S1 and ↑ MVP
                                                              murmur, ↓ AS murmur
Valsalva                      ↓ venous return (transient ↓    ↑ murmur of HOCM, moves
                              in LV size, preload, and        midsystolic click of MVP
                              relative systemic               closer to S1, and ↓ murmur
                              hypotension)                    of MVP
Isometric handgrip exercise   ↑ afterload                     ↑ murmur of MR and VSD,
                                                              ↓the murmur of HOCM, ↓AS
                                                              murmur
Inhaled amyl nitrate          ↓ afterload                     ↓ murmur of MR and VSD,
                                                              no change in AS murmur
                     Diagnostic Testing
   ECHOCARDIOGRAM
   Exercise testing
    – To assess the clinical severity of valvular heart disease
         Those with inconsistent resting hemodynamics
         Equivocal history of symptoms
    – Exercise testing in AS patients
         Should be ended promptly if:
            – Cardiac symptoms provoked
            – Decrease or minimal increase (<20 mmHg) in blood pressure
         Prior history of angina, congestive heart failure, or exertional
          syncope absolute contraindications to exercise testing
   Cardiac catheterization
    – Usually not needed for primary evaluation
                      Aortic Stenosis
   Most common cause is calcific degeneration
    – Active disease process with risk factors of male sex, smoking,
      HTN, DM, older age, hypercholesterolemia
   2% of the general population have bicuspid aortic valves
    – Symptomatic or severe AS occurs earlier (age 40-60 years)
   AS less commonly from rheumatic heart disease
    valvulitis
    – Invariably MV involved first
    – Associated AV involvement in <1/2 patients
   AV sclerosis
    – Valve thickening without obstruction
    – Present in >20% of people >65 years
    – Associated with 50% increased risk of MI and CV death
        Progression of Aortic Sclerosis

   Hemodynamic progression usually slow
    – Average rate of increase in aortic jet velocity of 0.3
      m/s per year
    – Increase in mean transaortic gradient of 7 mmHg
    – Decrease in AVA of 0.1 cm2 per year
   Severe AS
    – Aortic jet velocity > 4 m/s
    – Mean transvalvular pressure gradient > 50 mmHg
    – AVA < 1.0 cm2
     Pathophysiology of Aortic Stenosis

   Obstruction of LV outflow increases intracavitary
    systolic pressures and leads to LV pressure
    overload
   Initial compensatory mechanism is myocardial
    hypertrophy with preservation of systolic
    function
   Diastolic function impaired as a consequence of
    increased wall thickness and abnormal
    myocardial relaxation
   Increased wall stress and afterload causes
    eventual decrease in ejection fraction
                  Pseudostenosis
   Occurs in patients with impaired systolic function
    and aortic stenosis
    – Unable to generate transvalvular gradient
   Careful diagnostic testing with dobutamine
    infusion protocols can aid in differentiating
    between true AS and pseudostenosis
   If the calculated AVA increases with
    augmentation of cardiac output, then
    pseudostenosis present
   If AVA does not increase with dobutamine, then
    obstruction fixed and true AS present
Clinical Presentation of Aortic Stenosis
   Cardinal symptoms:
    – Angina
         Occurs in >50% of patients, not sensitive due to prevalence of CAD
    – Syncope
    – CHF
   Sudden cardiac death rare, <1% per year
   In earlier stages, AS presentation more subtle
    – Dyspnea
    – Decreased exercise tolerance
   Rarely, AS diagnosed in the setting of GI bleeding
    – Heyde’s syndrome
         Bleeding caused by AVM
         Concurrent AS occurs at prevalence rate of 15-25%
         Associated with an acquired von Willebrand syndrome due to
          disruption of vW multimers through a diseased AV
        Management of Aortic Stenosis
   Prognosis in asymptomatic disease excellent
   Conservative approach with monitoring for symptoms
    recommended
   When severe stenosis present-
    – 38% of asymptomatic patients develop symptoms within 2 years
    – 79% are symptomatic within 3 years
   Once symptoms occur, AVR needed
   LV dysfunction and severe AS have increased
    perioperative mortality with AVR
    – But outcomes still favorable with surgery
   Nitroprusside may transiently improve cardiac function
    as a bridge to valve replacement
    – Does not supplant AVR in symptomatic patients
Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51
               Aortic Valve Replacement
   Prophylatic AVR in asymptomatic patients not routinely
    performed due to surgical risks
    – Thromboembolism, bleeding associated with anticoagulation,
      prosthetic valve dysfunction, and endocarditis
    – Occurs at a rate of 2-3% annually
    – Only should be considered:
           If other cardiac surgery (such as CABG) planned
           Severe LVH or systolic dysfunction
           Women contemplating pregnancy
           Patients remote from health care
   Surgical valve replacement with operative morbidity and
    mortality of 10%
   Percutaneous balloon aortic valvotomy rarely used
                Mitral Stenosis
 Usually associated with history of
  rheumatic fever
 >40% of cases of RHD result in mitral
  stenosis
    – Women affected more than men (2:1)
   Presentation 20-40 years after the initial
    episode of rheumatic fever
    – If infected at a young age, latent period is a
      few years
    Clinical Presentation of Mitral Stenosis
   Significant MS leads to ↑LA pressure and pulm HTN
   Symptoms include dyspnea with ↑ cardiac demand
     – Exercise
     – Pregnancy
   Survival excellent with asymptomatic or minimally
    symptomatic patients
     – >80% survival at 10 years
   Survival in symptomatic patients much worse
     – 10 year survival drops to 15% or lower (if pulm HTN present)
   Findings consistent with severe MS:
     – Transvalvular diastolic pressure gradient >10 mmHg
     – MVA <1.0 cm2
     – Severe pulmonary hypertension (>60 mmHg)
        Management of Mitral Stenosis

   Atrial fibrillation
    – Prevalence >30% in symptomatic patients and
      associated with poorer long term outcome
    – Warfarin indicated:
         In patients with AF and MS
         Patients without history of AF but with MS and embolic CVA
    – In patients with prior history of AF who have mitral
      valve surgery, decreased postoperative AF observed if
      MAZE performed concominantly
                  Mitral Valve Repair
   Percutaneous valvotomy
    – Therapeutic intervention of choice if:
        LAA thrombus excluded
        MR less than moderate
        Valvular characteristics favorable
           – Pliable leaflets, minimal commisural fusion, minimal valvular or
             subvalvular calcification
    – Pulmonary HTN not contraindication to valvotomy
    – Major complications include: severe MR (1-8%),
      systemic embolization (1-3%), and tamponade (1-
      2%)
        Periprocedural mortality- 1%
   Surgical commissurotomy or MVR can be
    performed in unfavorable anatomy
Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51
         Rheumatic Fever Prophylaxis
   Primary prophylaxis
    – If living in an endemic area, with pharyngitis and a
      +test for group A strep or positive throat culture
    – Given once, may be repeated as needed:
        PCN G 1.2 million U IM or PCN V 500 mg TID x 10d
        Azithromycin 500 mg on day 1, 250 mg daily for 4d

   Secondary prophylaxis
    – PCN G 1.2 million units IM every 4 weeks or PCN V
      250 mg PO BID or erythromycin 250 mg BID
        RHD without carditis- At least 5 years or until >21 y of
         age
        RHD with carditis, no valvular HD- At least 10 y or well
         into adulthood
        RHD with carditis and valvular HD- At least 10 years
         from last episode or until patient is older than 40 years
            Acute Aortic Regurgitation
   Causes of acute aortic regurgitation:
    – Aortic dissection
    – Valve distruction from endocarditis
    – Traumatic rupture
   Classic physical exam findings may be absent in the
    acute presentation
    – Diastolic murmur may not be present due to sudden increase of
      LVEDP
   TTE, along with TEE, cath, CT or MRI may be used for
    diagnosis
   Surgical AV repair or replacement should be performed
    emergently
   Afterload reducing medications and inotropes may help
    to acutely stabilize the patient
   IABP contraindicated
          Acute Mitral Regurgitation
   Most often occurs in:
    – Chordae tendineae rupture due to myxomatous valve
      disease or endocarditis
    – Myocardial infarction with papillary muscle
      dysfunction or rupture
   Symptoms almost always occur
    – Dyspnea and pulmonary edema
   Systolic function may occur normal or
    hyperdynamic
   IABP or afterload reducing drugs to temporize
   Surgical intervention for treatment
        Chronic Valvular Regurgitation
   Cardiac chamber size and function have time to
    compensate for dysfunction
    – May allow patients to remain asymptomatic for a long time
   Both preload and afterload increases
   Once increase in cardiac output insufficient→ systolic
    function declines → pulmonary HTN may develop and
    symptoms develop
   LV enlargement and progressive systolic dysfunction are
    associated with significant morbidity and mortality
   Serial echocardiography and evaluation by a cardiologist
    is indicated
             Chronic Aortic Regurgitation
   Occurs most often in bicuspid AV
   Other causes include ascending aortic aneurysm and Marfan’s
    Disease
   Risk factors for poorer outcome:
     –   Age
     –   Cardiac symptoms
     –   Atrial fibrillation
     –   LV enlargement
     –   Lower LVEF
   Asymptomatic patients with normal LV size and function do not
    require prophylatic surgery
   Surgery should be considered if:
     – LVESD > 55 mm
     – Ejection fraction <60%
     – Symptoms develop
   Oral afterload reduction (nifedipine or ACE-I) may slow rate of LV
    dilation
Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51
         Chronic Mitral Regurgitation

   Often caused by myxomatous disease or MVP
    – Myxomatous mitral valve disease with progressive MR
      associated with poor long term outcome
        Higher risk of arrhythmias and sudden cardiac death
    – Mitral valve prolapse occurs in ~2% of the general
      population
        Consists of the buckling of the mid portion of the valve
         leaflets into the LA
        Usually asymptomatic, but may be associated with
         palpitations or chest discomfort
        Prognosis usually benign
        Antibiotic prophylaxis now not indicated
         Chronic Mitral Regurgitation
   Other causes include secondary or acquired
    leaflet dysfunction
    – Endocarditis
    – Rheumatic heart disease
    – Annular tethering from LV dilation
    – Tethering of the chordal apparatus from ischemic
      heart disease
    – Rare cause: Fenfluramine and phentermine, also
      associated with AI
   Compensatory increase in LV chamber size
    initially allows for increase in total stroke volume
    and restoration or total forward cardiac output
         Treatment of Chronic Mitral
               Regurgitation
   Mitral valve repair preferred over mitral valve
    replacement
    – Avoids risk of anticoagulation
    – Preservation of subvalvular apparatus
        Better postoperative LV function and long term survival
   When MR occurs in volume overloaded states,
    afterload reduction can be beneficial
    – Dilated CM
    – CAD
   Revascularization may improve dysfunction of
    the papillary muscle
   Biventricular pacing may improve LV geometry
   Timing of Intervention for Left-Sided Valvular Conditions
Aortic Stenosis                Mitral Stenosis                Chronic Severe AR               Chronic Severe MR
Intervention:                  Intervention:                  Intervention:                   Intervention:
AVR                            Percutaneous valvotomy if      Surgical AVR with aortic        Surgical mitral valve repair
                               anatomy amenable and           root replacement if needed      if anatomy amenable.
                               <moderate MR, and no                                           Otherwise, MVR
                               LAA clot. Otherwise, open
                               commissurotomy or MVR
IF:                            IF:                            IF:                             IF:
Patient is symptomatic         Patient has moderate or        Patient is symptomatic          Patient is symptomatic
(NYHA class II or greater,     more severe MS (MVA <          (NYHA class II or greater)      (NYHA class II or greater)
angina due to AS, or           1.5 cm2)                       OR                              OR
syncope)                       OR                             EF <60%                         EF <60%
OR                             Pulmonary hypertrension at     OR                              OR
Patient has symptomatic        rest (PAP > 60 mmHg)           ESD > 55 mm                     ESD > 45 mm
severe AS and needs other      OR
cardiothoracic surgery (i.e.                                  OR                              OR
                               Abnormal hemodynamic           Abnormal hemodynamic            Pulmonary hypertension or
CABG)                          response to exercise:          response to exercise            atrial fibrillation
                               PAP > 60 mmHg                  PAP increase by 25 mmHg
                               Mean gradient > 15 mmHg
OTHERWISE                      OTHERWISE                      OTHERWISE                       OTHERWISE
Depending on the severity      Clinical evaluation at least   Repeat TTE at least yearly,     Repeat TTE yearly, repeat
of AS, at least annual         annually, depending on the     repeat clinical evaluation at   clinical evaluation
clinical evaluation with TTE   severity of the mitral         least biannually depending      biannually
to monitor for symptom         stenosis                       on the severity of the LV
onset                                                         dilitiation
               Tricuspid Valve Disease
   Tricuspid stenosis is rare
    – Associated with rheumatic heart disease
   TR usually occurs secondary to:
    –   Pulmonary hypertension
    –   RV chamber enlargement with annular dilatation
    –   Endocarditis (associated with IV drug use)
    –   Injury following pacer lead placement
   Other secondary causes: carcinoid, radiation therapy,
    anorectic drug use, and trauma
   Primary causes: Marfan’s syndrome and congenital
    disorders such as Ebstein’s anomaly and AV canal
    malformation
   Echo is diagnostic in most cases
             Tricuspid Regurgitation
   Severe tricuspid regurgitation is difficult to treat
    and carries a poor overall clinical outcome
   Symptoms are manifestations of systemic
    venous congestion
    – Ascites
    – Pedal edema
   Surgical intervention usually considered if other
    cardiac surgery planned
   Surgical options include valvular annuloplasty or
    replacement
    – If replacement planned, bioprosthetic valve preferred
        Prosthetic Valves- Mechanical
   Three types:
    – Ball-cage valve
    – Single tilting disk valve
    – Bileaflet valve
   Durable but require life long anticoagulation
   For operative procedures, warfarin typically is
    discontinued for 48-72 hours and restarted
    postop as soon as possible, except for:
    – Mechanical mitral prosthesis
    – Atrial fibrillation
    – Prior thromboembolic events
Ball-cage valve




                             Single tilting disk valve




           Bileaflet valve
         Prosthetic Valves- Biological
   Biological Valves
    – Composed of autologous or xenograft biological
      material mounted on stents and a sewing ring
    – Warfarin therapy not required due to lower
      thromboembolic potential
    – Valve durability less when compared to mechanical
      valves
    – Newer stentless valves with increased longevity
Anticoagulation Guidelines for
      Mechanical Valves




                Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51
           Prosthetic Valve Complications
   Common complications include:
     –   Structural valve deterioration
     –   Valve thrombosis
     –   Embolism
     –   Bleeding
     –   Endocarditis
   Endocarditis prophylaxis required for patients with all types of
    prosthetic valves
   Suspect valve dehiscence or dysfunction in:
     –   Acute CHF in the immediate postop period
     –   New cardiac symptoms
     –   Embolic phenomena
     –   Hemolytic anemia
     –   New murmurs
   TEE is the diagnostic procedure of choice
   Postop TTE should be done 2-3 months after surgery
                  Valve Thrombosis
   Incidence with mechanical prosthesis of 2-4 % per year
   Suspect in patients with new murmur, change in
    cardiopulmonary symptoms, or an embolic event
   Diagnosis based on clinical presentation, TTE/TEE, and
    fluroscopy
   In small thrombus, treatment with heparin may be
    adequate
   Optimal treatment for left sided thrombosis is emergency
    surgery
   Consider thrombolytic therapy for right sided thrombosis
    or if surgery cannot be performed with left sided disease
Endocarditis Prophylaxis




       2007 AHA Prevention of Infective Endocarditis Guidelines