Valvular Heart Disease

Etiology 

• Fibrosis and degenerative calcification of the aortic cusps 
• Congenital bicuspid aortic valve 
• Chronic deterioration (calcific) of tricuspid aortic valve 
• Elevated lipoprotein(a) 
• 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

Management 

• Definitive treatment is valve replacement for: 1) Stage D (symptomatic severe AS) 2) Stage C (asymptomatic with inducible symptoms on stress testing, low EF, or undergoing other cardiac procedure) 3) 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

Etiology 

• Primary valve disease (rheumatic disease, bicuspid AV, infective endocarditis, calcification, syphilis) 
• Primary aortic root disease (medial degeneration, aortic dissection, connective tissue disorders, chronic HTN, aortitis, thoracic aortic aneurysm)

Presentation 

• Acute AR: LV cannot respond to increased volume to maintain SV, leading to pulmonary edema and cardiogenic shock 
• Chronic AR: indolent presentation, often pt will develop sx of HF including DoE, orthopnea, PND 
• Physical exam: S3, “Water-hammer” pulses, wide pulse pressure, laterally displaced PMI, high pitched “blowing” decrescendo early diastolic murmur best heard at third/fourth ICS at left sternal border (valvular disease), right sternal border (aortic root disease), Austin Flint murmur (rumbling, low-pitched middiastolic/presystolic murmur)

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 - 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 q3-6 months to monitor LV function and dimensions

Etiology

• Acute: papillary muscle rupture (inferior MI), chordal rupture (prior MV disease), infective endocarditis. 
• Chronic: can be primary (degenerative) vs secondary (functional) 
• Primary MR: caused by direct involvement of the valve apparatus (leaflets or chordae tendineae) Most common cause: Degenerative/myxomatous mitral valve disease (MVP with flail leaflet, rheumatic fever, infective endocarditis, connective tissue disease 
• Secondary 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.

Evaluation 

• Auscultation: Holosystolic murmur at apex, S3, early diastolic rumble (may be absent/soft in acute MR) 
  • CXR: assess for pulmonary edema, typically normal cardiac silhouette in acute MR. Cardiomegaly and LA enlargement in chronic MR. 
  • ECG: Often non-specific in acute MR. In chronic MR, LA enlargement can present as pmitrale and often c/b development of Afib. 
  • TTE: to assess suspected MR, or any new-onset, changing sx with known MR. 
  • TEE, CMR, or cardiac catheterization can be planned when insufficient or discordant information from TTE.

Chronic primary 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: normal EF and LV size, with moderate to severe LA enlargement 
  • 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: Afterload reduction with vasodilation (nitroprusside, nitroglycerine) is key to promote forward flow. Diuresis to reduce preload and improve pulmonary edema 
• Chronic severe primary MR -> Surgical repair favored over valve replacement 
• Chronic severe secondary MR -> M-TEER can be considered in pt with persistent sx on maximal GDMT 

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

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 to 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). 
• Physical exam 
  • Diastolic murmur, best heard at apex, opening snap following s2

Evaluation 

• CXR: increased pulmonary vasculature, left atrial enlargement; typically normal cardiac silhouette in acute MS. 
• ECG: Often non-specific; chronic left atrial enlargement notable on P wave morphology (Pmitrale). Chronic MS often complicated by atrial fibrillation. 
• Echocardiography: thickening of mitral valve leaflets, decreased area of valve leaflets, left atrial enlargement

Stages of Chronic MS

Management 

• Varies between rheumatic MS and calcific MS (in general, intervention of calcific MS is challenging and high risk) 
• Severe, symptomatic rheumatic MS: 
• Percutaneous mitral balloon commissurotomy (PMBC) 
• Surgical repair/replacement if pt 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 pts. No role for commissurotomy with calcific MS 
  • Consider surgical valve replacement for severely symptomatic pts (technically challenging)

Anticoagulation 

• Anticoagulation is indicated if: 
  • Mechanical prosthetic mitral valve 
  • Lifelong AC with Warfarin, goal INR of 3.0 (range of 2.5-3.5) 
  • Bioprosthetic mitral valve replacement 
  • Warfarin, goal INR 2-3 for first 3-6 months