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Valvular Heart Disease

Audrey White

Aortic stenosis

Chloe Carr

Etiology

  • Degenerative calcification of the aortic cusps
    • Congenital bicuspid aortic valve
    • Chronic deterioration (calcific)
    • Prior rheumatic fever/inflammation

Presentation

  • Usually asymptomatic
  • Angina, syncope, exertional dyspnea, heart failure (HF carries worse prognosis) when severe
  • Typically aged 70 – 80 y/o; if bicuspid aortic valve expect 10-20 yrs earlier
  • Physical exam: Systolic crescendo-decrescendo murmur that radiates towards the carotids
    • Late peaking murmur, faint or absent S2, or delayed carotid upstroke suggest severe AS

Evaluation

  • TTE with doppler is test of choice
Severity   Valve Area (cm2)  Mean Gradient (mmHg) 

Velocity  

(m/s) 

 Indexed Valve Area (cm2/m2)  
Mild   >1.5  <20  2.0-2.9  >0.85 
Moderate   1.0-1.5  20-39  3.0-3.9  0.60-0.85 
Severe  <1.0  >40  >4.0  <0.6 
Critical   <0.5  --  --  -- 

AS stages

  • A: at risk of AS (those with bicuspid anatomy or calcification
  • B: Asymptomatic non-severe AS
  • C: asymptomatic AS
    • C1: normal EF
    • C2: abnormal EF
      • This stage might benefit from exercise or stress testing to elicit symptoms

  • D: Symptomatic AS

  • Some with symptomatic AS might not have enough LV reserve to produce high velocities and gradients (ex EF of 10% w/ critical valve area) = low flow/low gradient AS; consider dobutamine to unmask AS

Management

  • No proven effective medical therapy
  • Definitive treatment is valve replacement for:
    • Stage D
    • Stage C with inducible symptoms on stress testing, low EF, or undergoing other cardiac procedure
    • Rapid progression (increase in velocity >0.3m/sec per year)
  • Consult cardiac surgery for determination of SAVR vs TAVR
    • In general, high risk surgical patients benefit most from TAVR
    • At VUMC: If determined to be intermediate to high operative risk by Cardiac Surgery, they will often recommend contacting the TAVR team for evaluation
  • Avoid rapid hemodynamic shifts and aggressive changes in preload or afterload
  • Aim for normotension
    • Avoid preferential vasodilators such as hydralazine or nitroglycerin
    • Significant vasodilation may ↓ coronary filling pressures -> myocardial ischemia

Monitoring

  • Severe AS: TTE q 6-12 months
  • Moderate AS: TTE q 1-2 years
  • Mild AS: TTE q 3-5 years

Post AVR anticoagulation

  • All patients will get 3-6 months of AC s/p AVR
  • Continued duration based on type of AVR
    • Bioprosthetic (TAVR and some SAVRs): antiplatelets alone after Initial AC
    • Mechanical: lifelong AC with warfarin only

Aortic Regurgitation

Hannah Granger 

Etiology

  • Primary valve disease (rheumatic disease, bicuspid aortic valve, infective endocarditis, syphilis)
  • Primary aortic root disease (medial degeneration, aortic dissection, Marfan’s syndrome, bicuspid aortic valve, syphilis, non-syndromic familial)

Presentation

  • Acute AR: LV cannot respond to increased volume to maintain stroke volume pulmonary edema and cardiogenic shock
  • Chronic AR: indolent presentation, often patient will develop symptoms of heart failure including DoE, orthopnea, PND
  • Physical exam: “Water-hammer” pulses, wide pulse pressure, laterally displaced PMI, high pitched “blowing” decrescendo murmur best heard at third intercostal space at left sternal border, S3

Management

  • Acute severe AR
    • Page cardiac surgery for urgent surgical repair
    • Vasodilators such as nitroprusside and diuretics can be used to stabilize patient
    • Though pts with aortic dissection are often treated with beta blockers, these should be used with caution if there is concomitant severe AR as it may block compensatory tachycardia and lead to marked hypotension

  • Chronic severe AR

    • Medical management
      • Early symptoms of exercise intolerance can be treated with diuretics
      • Systolic BP should also be controlled with goal SBP \< 140 in chronic AR

  • Repeat imaging should be performed 3-6 month to assess for depressed LVEF or LV dilation

  • Stages of Chronic AR: Ranging from Stage (A): Asymptomatic but “At Risk” AR to Stage (D) Symptomatic Severe AR

    • If symptoms are present, automatically Stage D, otherwise Progression through stages is determined by AR Jet Width
  • Class I indications for Valve Repair:
    • Stage D (Symptomatic)
    • Stage C (Asymptomatic Severe AR) with LVEF \< 55%
    • Severe AR and undergoing cardiac surgery for other indication

Mitral Regurgitation

Krissie Lobon

Etiology

  • Primary MR – caused by direct involvement of the valve apparatus (leaflets or chordae tendineae)

    • Most common cause: Degenerative/myxomatous mitral valve disease (mitral valve prolapse with flail leaflet, mitral annular calcification, chordal rupture)
    • Rheumatic fever
    • Infective endocarditis
    • Papillary muscle rupture following acute (inferior) MI

  • Secondary MR (also called functional MR)- caused by changes of the LV that lead to valvular incompetence

    • Dilated Cardiomyopathy
    • HOCM with systolic anterior motion
    • Coronary Artery Disease or prior MI leading to papillary muscle tethering

Presentation

  • Acute MR- sudden onset reduction in forward cardiac flow and left atrial/pulmonary vein volume overload
    • Dyspnea with flash pulmonary edema
    • Left-sided heart failure
  • Chronic MR- progressive symptoms d/t cardiac remodeling to compensate for mitral flow reversal
    • Progressively worsening heart failure: dyspnea, orthopnea, PND
    • LV dilation from volume overload
    • LA remodeling/dilation leading to afib

Auscultation

  • Holosystolic murmur, best heard at Apex, Radiation to the Axilla
    • Frequently associated with S3
    • Murmur may be absent in acute MR due to large regurgitant orifice/low velocity regurgitant jet
    • Increases w/ increased preload or afterload

Evaluation

  • CXR: assess for pulmonary edema, typically normal cardiac silhouette in acute MR. Cardiomegaly and LA enlargement in chronic MR.
  • ECG: often non-specific if chronic LA enlargement notable on p wave morphology (p-mitrale). Chronic MR often c/b development of atrial fibrillation.
  • Echocardiography needed for confirming diagnosis
    • TEE, CMR, or cardiac catheterization performed when insufficient or discordant information from TTE. TEE used to guide MV interventions

Chronic MR stages

  • A: No symptoms
  • B: >mild MR w/o hemodynamic changes or symptoms
  • C: Severe MR w/o symptoms
    • C1: preserved EF and normal LV size
    • C2: reduced EF (\<60%), dilated LV (LVESD > 40mm)
  • D: Severe/symptomatic

Management

Acute hemodynamically significant MR

  • Urgent surgical repair or replacement
  • Medical stabilization as a bridge to surgery
    • Afterload reduction is key to promote forward flow
    • Vasodilators (nitroprusside, nitroglycerin) reduce afterload
    • Diuresis to reduce preload and improve pulmonary edema
    • IABP placement can be used as mechanical afterload reduction

Chronic severe primary MR

  • Surgical repair favored over valve replacement
  • Class I:
    • Asymptomatic patients w/ LVEF 30-59% or LVESD > or equal 40mm
    • Symptomatic patients w/ EF > 30%
  • Class II:
    • A: asymptomatic patients with progressive EF decline or LV dilation on serial monitoring; or very severe MR
    • B: new onset AF
  • Secondary MR can consider MV repair with persistent class III-IV symptoms while on guideline directed medical therapy
  • In HFrEF, consider MitraClip after volume optimization (see Heart Failure section)

Mitral Stenosis

Krissie Lobon

Etiology

  • Characterized by thickened mitral valve leaflets and fused leaflet tips.
  • Rheumatic Fever (leading cause worldwide)
  • Calcification of the mitral valve annulus (common in high income countries)
  • Autoimmune Diseases: SLE, Rheumatoid arthritis

Presentation

  • Progressive symptoms: Asymptomatic Heart Failure
    • Orthopnea
    • PND
    • Hoarseness/Dysphagia (compression of recurrent laryngeal nerve/esophagus by enlarged left atrium from pressure overload)
    • Symptoms of Right Heart Failure
  • Acute Symptoms may present in settings of increased cardiac output (pregnancy, sepsis, or exercise) or tachyarrhythmias
    • Dyspnea
    • Fatigue
    • Palpitations

Physical exam

  • Low-pitched rumbling, diastolic Murmur, best heard at apex, low-pitched, rum
    • Loud S1, opening snap after S2
    • Prominent P2 if pulmonary HTN develops
  • Pulmonary Rales

Stages of MS

  • A: At risk of MS, characterized by mild valve doming during diastole, asymptomatic

  • B: Progressive MS, characterized by commissural fusion, increased transmitral flow velocities, asymptomatic

  • C: Asymptomatic Severe MS, characterized by above + mitral valve area \<1.5cm2

  • D: Symptomatic Severe MS, characterized by above criteria + decreased exercise tolerance

Evaluation

  • CXR: LA enlargement, increased pulmonary vasculature
  • Echocardiography: thickening of mitral valve leaflets, decreased area of valve leaflets, left atrial enlargement

Management

  • Varies between rheumatic MS and calcific MS (in general, intervention of calcific MS is very challenging and high risk)
  • Severe, symptomatic rheumatic MS:
    • Percutaneous mitral balloon commissurotomy (PMBC)
    • Surgical repair/replacement if patient failed PMBC or undergoing other cardiac surgery

  • Calcific MS has a poor prognosis with 5-year survival \<50%, Intervention is higher risk and should be reserved for severely symptomatic patients

  • No role for commissurotomy with calcific MS

  • Surgical valve replacement may be considered for severely symptomatic patients (technically challenging)

Anticoagulation

  • Anticoagulation is indicated if:
    • Mechanical prosthetic mitral valve
      • Warfarin, goal INR 3-4 lifelong
    • Bioprosthetic mitral valve replacement
      • Warfarin, goal INR 2-3 for first 3-6 months
    • Atrial Fibrillation regardless of CHADS2VASC score

2020 ACC/AHA Heart Valve Disease Guidelines: Mitral Stenosis Management Algorithm