Valvular Heart Disease

Aortic Stenosis

Michelle Chintanaphol

Etiology

  • Fibrosis and degenerative calcification of the aortic cusps
    • Congenital bicuspid aortic valve
    • Chronic deterioration (calcific) of trilcuspid aortic valve
    • Prior rheumatic fever
    • Less common causes: SLE, Fabry disease, radiation, inflammation

Presentation

  • Usually asymptomatic, though could have exertional dyspnea, decreased exercise tolerance, exertional dizziness/lightheadedness, syncope, exercise-induced angina, heart failure (worse prognosis) when severe
  • Typically, aged 70–80 yo; if bicuspid aortic valve expect 10-20 yrs earlier

Physical exam

  • Loud, late-peaking systolic crescendo-decrescendo murmur in right intercostal space that radiates towards the carotids
  • Signs of severe AS: late peaking murmur, faint or absent S2, or “parvus et tardus” (delayed and reduced/low volume carotid upstroke)

Evaluation

  • TTE with doppler is test of choice for diagnosis and evaluation

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 -- -- --

Management

  • No proven effective medical therapy. Definitive treatment is valve replacement for:
    • Stage D (symptomatic AS)
    • Stage C (asymptomatic 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 pts 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, nitroglycerin, or peripheral alpha blockers
    • 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 pts will get 3-6 months of AC s/p AVR depending on bleeding risk
  • Continued duration based on type of AVR
    • TAVR: Aspirin 75-100mg daily following initial AC
    • SAVR: Aspirin 75-100mg daily following initial AC (usually warfarin)
    • Mechanical: lifelong AC with warfarin only

Aortic Regurgitation

Faria Khimani

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, leading to pulmonary edema and cardiogenic shock
  • Chronic AR: indolent presentation, often pt 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, do not delay
    • Vasodilators such as nitroprusside and diuretics can be used to stabilize pt
    • Use beta blockers with caution with concomitant severe AR and dissection and may block compensatory tachycardia leading to marked hypotension.
  • Chronic severe AR
    • Surgical management: Aortic valve replacement (AVR) in severe AR (Stage D), asymptomatic severe AR with LV ejection fraction (LVEF) ≤55% (Stage C2), and severe AR in patients undergoing other cardiac surgery
    • Medical management
      • For patients with severe AR or LV systolic dysfunction with prohibitive surgical risk, optimize GDMT for HFrEF
      • Systolic BP should also be controlled with goal SBP < 140 in chronic AR
  • Imaging and Monitoring:
    • Echo primary modality for monitoring AR severity. CMR used with echo data inconclusive
    • Regular follow-up every 3-6 months to monitor LV function and dimensions

Mitral Regurgitation

Faria Khimani

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

Presentation

  • Acute MR- Sudden onset reduction in forward cardiac flow, dyspnea with flash pulmonary edema, left-sided heart failure.
  • Chronic MR- Progressive symptoms due to cardiac remodeling, worsening heart failure, left ventricular dilation, left atrial remodeling leading to atrial fibrillation.

Auscultation

  • Holosystolic murmur best heard at apex with radiation to the axilla. Associated S3 filling sound. Murmur may be absent in acute MR due to large regurgitant orifice/low velocity regurgitant jet

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 (pmitrale). 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: At risk for MR due to risk factors (i.e. mild valve thickening or leaflet restriction)
  • B: Progressive MR w/o hemodynamic changes or symptoms
  • C: Asymptomatic severe MR
  • C1: preserved EF and normal LV size
    • C2: reduced EF (<60%), dilated LV (LVESD > 40mm)
  • D: Symptomatic severe MR

Management

  • Asymptomatic severe MR (stage C)
    • - Follow-up echo every 6-12 months to monitor LV function/size and pulmonary pressure
  • Acute hemodynamically significant MR
    • - Urgent surgical repair or replacement
    • - Medical stabilization as a bridge to surgery:
      • o Afterload reduction with vasodilation (nitroprusside, nitroglycerine) is key to promote forward flow
      • o Diuresis to reduce preload and improve pulmonary edema

Chronic severe primary MR -> Surgical repair favored over valve replacement

Mitral Stenosis

Faria Khimani

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 Regurgitation Management Algorithm

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