Most commonly inflammation with resultant calcification. Also bicuspid, congenital and less likely rheumatic.

Physical exam:

• Harsh crescendo-decrescendo systolic murmur heard best at the right upper sternal border, radiating to the supraclavicular fossa and carotids.
• Narrow pulse pressure, parvus et tardus pulse.
• S2 softens and murmur peaks later as aortic stenosis progresses.
• A single S2 (inaudible A2) are signs of critical AS (best heard over the left infra-clavicular space).
• Gallivardin’s phenomenon is AS murmur heard in the mitral position.

Clinical manifestations and natural history:
 

 

Severity of AS by ACC/AHA:

TTE parameter	Sclerosis	Mild AS	Moderate AS	Severe, high gradient AS
Peak velocity Vmax, m/s	<2.5	2.5-3	3-4	>4
Mean gradient, mmHg	Normal	<20	20-40	≥40
AVA, cm2	3-4	>1.5	1-1.5	≤1

• Stage C: asymptomatic, severe. Patients should undergo stress testing to confirm they are are asymptomatic.
• Stage D1: symptomatic, severe, high gradient.
• Stage D2: symptomatic, severe, low flow, low gradient. Due to LV dysfunction (LVEF <50), resting V_max <4m/s or gradient <40mmHg. Dobutamine stress testing results in V_max >4m/s.
• Stage D3: symptomatic, severe, paradoxical low flow, low gradient. Due to small LV chamber and stroke volume with LVEF ≥50, resting V_max <4m/s or gradient <40mmHg. Stroke volume index <35mL/m².

Management:

• The mainstay of therapy for severe AS (stage D, or C in some circumstances) is replacement. Patients with stage B disease sometimes undergo SAVR if they are undergoing other cardiac surgery. There is no known medical therapy known to slow progression of AS. Once symptoms develop in patients with severe AS there is a dramatic reduction in lifespan when treated medically, rather than surgically. 
• Type of valve replacement:

• SAVR is recommended if age <65 or life expectancy >20 years.
  • Mechanical valves are typically favored for age <50.
  • Mechanical or bioprosthetic valves can be considered for ages 50-65.

• TAVR or SAVR is recommended:
  • In patients who are symptomatic and age 65-80 with no contraindication to transfemoral access.
  • In patients who are asymptomatic with LVEF <50 or abnormal exercise test, elevated BNP, or at risk for rapid progression.

• TAVR is recommended if age >80, life expectancy <10 years and no contraindication to transfemoral access.

• Mimics mild AS on physical exam but is due to thickened and calcified valve leaflets which open normally (no stenosis).
• Factors that favor aortic stenosis over aortic sclerosis include late peaking S2 sound, diminished S2 intensity, and delayed carotid upstroke. Aortic sclerosis usually does not radiate to the carotids.
• Aortic sclerosis is an indicator of generalized atherosclerosis and a marker of increased risk for cardiovascular events; 2% progression per year to aortic stenosis.

• Systolic murmur heard best at the left upper sternal border, transmitted to the neck or left shoulder.
• Diminished A2, delayed P2. May be associated with a click.
• Parasternal lift from resulting RVH.
• Murmur may increase with inspiration.

Etiology:

Carpentier classification (based on leaflet motion):

• Type 1 has normal leaflet motion. Caused by endocarditis, dilated annulus from atrial fibrillation, or congenital causes.
• Type 2 has excessive leaflet motion. Associated with MVP or flail leaflet (from papillary muscle rupture or trauma) and typically isolated disease.
• Type 3a has leaflet restriction in systole and diastole. From rheumatic valve disease, SLE, or radiation.
• Type 3b (“functional” or “secondary”) has leaflet restriction in systole only. From ischemic causes, LV dysfunction, or dilated cardiomyopathy. Mitral valve apparatus is otherwise normal. LA dilation from atrial fibrillation can also cause annular dilation and secondary MR.

Physical exam:

• Holosystolic murmur heard best at the apex, radiating to the left axilla (especially with anterior leaflet incompetence) or left sternal border (with posterior leaflet incompetence).
• Soft S1.
• Timing is early in acute/severe MR.
• MVP classically has mid-systolic click followed by late systolic murmur (from MR).

Clinical manifestations and natural history:

• Symptoms of volume overload and LV dysfunction typically only occur in severe disease.
• Atrial fibrillation is common.
• When assessing symptoms of secondary MR, heart failure management including revascularization should be optimized first.
• Symptoms of MVP are nonspecific and not reliable predictors of MVP or MVP severity. MVP in the absence of MR is associated with increased risk of endocarditis but not convincingly associated with increased rates of PAC, PVC, or atrial fibrillation.

Evaluation:

• TTE is used to quantify severity, LV size and function, PASP, and valve anatomy.
  • Severity is assessed by measuring the effective regurgitant orifice area, regurgitant volume and regurgitant fraction.
  • Since MR functionally increases preload and decreases LV afterload, the “normal” EF is 70% and LVEF <60% or LVESd <40mm indicate LV dysfunction. These criteria are used to grade MR, with different cutoffs for primary and secondary disease. Can be done with exercise.
• TEE should be completed if TTE inadequately assesses severity or anatomy. Helps to guide intervention.
• Ischemic evaluation should be pursued in secondary MR.

Management:

• Surveillance TTE q6 month to yearly (or with change in symptoms) to assess LV function and PASP in moderate to severe disease.
• Guideline-directed medical therapy (GDMT) for LVEF <60, see section Heart Failure for details. Unlike acute MR, vasodilator therapy in normotensive patients has not been shown to decrease MR severity.
• Revascularization for ischemic MR.
• Surgical intervention is indicated for primary MR with stage D (severe, symptomatic) and/or LV dysfunction (though data is lacking for LVEF <30) or in patients undergoing cardiac surgery for other reason. Intervention is reasonable in asymptomatic patients with severe MR and normal LV if the chances of success are high. Intervention can be considered in chronic severe secondary MR with persistent NYHA III-IV symptoms despite GDMT or when undergoing cardiac surgery for other reason.
  • Valve repair preferred over replacement if possible, especially when dysfunction is limited to posterior leaflet, due to lower operative risk and avoidance of risk from prosthetic heart valve (thromboembolism, need for anticoagulation, eventual need for another replacement).
  • Valve replacement typically performed for patients who are not candidates for or who have failed valve repair.

• Transcatheter intervention (Mitra Clip) is reasonable in secondary MR with LVEF 20-50%, LVESD <70mm, PASP <70mmHg and NYHA II-IV symptoms despite GDMT for HFrEF. Benefit is less in more severe LV dysfunction and less severe MR.

• Blowing holosystolic murmur best heard along the left lower sternal border radiating to the right sternal border; increasing intensity with inspiration.

Introduction:

The presentation, clinical findings and management of AR are highly dependent on the severity and tempo of onset. In either acute or chronic AR, which are discussed separately below, the etiology can be broken down into leaflet abnormalities or aortic root/ascending aorta abnormalities.

 

CHRONIC AORTIC REGURITATION  

Etiology:

• Leaflet abnormalities: infective endocarditis, myxomatous degeneration, rheumatic, congenital (e.g. bicuspid/unicuspid), inflammatory degeneration (e.g. SLE, rheumatoid), drug-induced valvulopathy (MDMA, phentermine), perimembranous VSD (high velocity resulting in leaflet destruction and poor coaptation).
• Aortic root or ascending aorta abnormalities: age related dilation, systemic hypertension, aortic root dissection, trauma, connective tissue disorders (Marfan syndrome, Ehlers-Danlos), chronic inflammatory/infectious (syphilis, giant cell arteritis, Bechet's).

Physical exam:

• Wide pulse pressure (large stroke volume SV).
• The apical impulse is displaced laterally and caudally.
• Diastolic decrescendo murmur at left sternal border, 3rd or 4th interspace, heard best with patient sitting, leaning forward, and holding breath at end-expiration.
• Other exam signs include water hammer or Corrigan’s pulse (bounding, full radial pulse with rapid downstroke), Quincke’s sign (capillary pulsation in nailbed with gentle pressure), Musset’s sign (head bobbing), Muller’s sign (pulsating uvula). All are related to a dilated left ventricle and increased stroke volume.

Clinical manifestations and natural history: 

• Notably these patients are often asymptomatic for years. Around 75% of patients with moderate to severe AR remain without symptoms at 7 years after initial diagnosis. However, the LV begins to change with increased wall tension resulting in increased LV volume (i.e. Law of Laplace’s).
• Eventually, the LV function declines and symptoms related to increased LV filling pressures including orthopnea, PND, and DOE begin.
• Once symptoms develop there is usually a rapid decline in functional status. With development of angina and heart failure most patient die within 4 and 2 years respectively without surgical intervention.

Evaluation:

• Echocardiography: 2D and M-mode transthoracic (TTE) and transesophageal echo (TEE) are used to diagnose severity, LV dysfunction and etiology of severe AR.
• CMR is helpful in cases where severity remains inconclusive after TEE and can more accurately assess regurgitation fraction and chamber volumes.

Management:

• Surveillance: screening TTE intervals based on severity.
  • At risk or mild AR: TTE every 3-5 years.
  • Moderate AR: TTE every 1-2 years.
  • Severe AR: TTE every 3-6 months while awaiting surgery.

• Medical therapy:
  • Vasodilators are controversial in chronic AR, especially in asymptomatic patients. The only class I indication for vasodilators is in patients who are nonsurgical candidates with severe, chronic AR and LV dysfunction or symptoms.
  • Beta blockers are indicated in those with reduced LV function. However, caution should be used as this can increase regurgitation time and symptoms.
  • Blood pressure and other co-morbidities should be aggressively treated per AHA/ACC guidelines.

• Class I surgical indications:
  • Severe symptomatic AR.
  • Severe asymptomatic AR if LVEF <50% or LVESD >5cm.
  • Moderate AR if undergoing other cardiac surgery.

ACUTE AORTIC REGURITATION

Etiology:

• Leaflet abnormalities: traumatic rupture/injury, acute infective endocarditis, prosthetic valve dysfunction or post aortic balloon valvuloplasty.
• Aortic root or ascending aorta abnormalities: acute aortic dissection, perivalvular leak, aortic graft failure or prosthetic valve dehiscence.

Physical exam:

• Signs of hemodynamic compromise such as tachycardia, hypotension, cool extremities, and pulmonary edema.
• The murmur of acute AR may be very short or not heard due to a rapid diastolic pressure equilibration between the aorta and the “unprepared,” non-compliant LV.
• Auscultation can reveal an Austin-Flint murmur (diastolic murmur in mitral area due to AR flow impinging/vibrating anterior mitral leaflet).

Clinical manifestations and natural history:

• In contrast to chronic AR, acute AR results in acute hemodynamic compromise.
• Severe AR carries high mortality if left untreated & requires aggressive supportive measures and prompt surgical evaluation.

Evaluation:

• ECG: tachycardia. Non-specific ST-T wave changes suggestive of LV strain.
• CXR: pulmonary congestion; normal LV size; widened mediastinum in acute aortic dissection (this is neither sensitive nor specific).
• Echocardiography: the mainstay of urgent diagnosis of acute AR with 2D and M-mode echo allowing for diagnosis of severity and often the etiology.

Management:

• Medical therapy: hemodynamic stabilization is the goal of medical therapy while awaiting urgent/emergent, definitive surgical interventional.
  • Antibiotics should be started early in cases of suspected endocarditis.
  • Vasodilators such as nitroprusside (which drop LVEDP and afterload) or inotropic agents such as dobutamine or isoproterenol (which can increase HR and drop afterload) are ideal options for management of acute AR.
  • Patients with evidence of congestion should also undergo aggressive diuresis.
• Surgical therapy: prompt surgical evaluation and intervention is mandatory for survival especially in the setting of acute hemodynamic instability. The timing of surgery depends on cause and severity of hemodynamic compromise. In most cases, including acute endocarditis, earlier surgery has been associated with better outcomes in multiple trials.

• Decrescendo diastolic murmur heard best at the left upper sternal border.
• Intensity may increase with inspiration.
• Often associated with pulmonary hypertension.

Etiology:

• Historically MS was typically rheumatic.
• With declining rates of rheumatic heart disease, nonrheumatic calcific MS (from MAC) has been increasing in older adults. Uncommon causes include infiltrative disease.

Physical exam:

• Low-pitched rumbling diastolic murmur heard best at the apex.
• Opening snap sometimes present if there is rheumatic heart disease.

Evaluation:

• History: symptoms include exertional dyspnea from increased LA and pulmonary pressures. New or worsening symptoms may be attributed to disease progression or systemic process such as pregnancy, fever, anemia, hyperthyroidism. Atrial fibrillation, thromboembolism, hoarseness, hemoptysis and right sided volume overload can also occur.
• Echocardiography: hemodynamic severity is estimated by mitral valve area that is calculated from the diastolic pressure half-time, with severe disease defined by MVA ≤1.5cm² or diastolic pressure half time ≥150ms (typically corresponds to gradient 5-10mmHg). These measurements can be affected by diastolic filling time (i.e., heart rate), LV and LA compliance. Other hemodynamic consequences include LA enlargement and elevated PASP. TEE is performed to evaluate for LA thrombus and is used prior to commissurotomy.
• Catheterization: typically used only to resolve discrepancy between TTE and symptom burden, ± exercise or dobutamine stress. LA and LV pressures are measured, which requires transseptal puncture; these are interpreted in context of typical right heart pressure and cardiac output.

Management:

• Surveillance TTE should be obtained with any change in symptoms.
• Medical therapy:
  • Indefinite anticoagulation if atrial fibrillation, prior embolic event or LA thrombus.
  • Rate control if atrial fibrillation with RVR.
  • Heart failure management (GDMT) is the cornerstone for calcific MS.
• Interventional therapy for rheumatic MS:
  • Percutaneous balloon commissurotomy indicated in stage D and reasonable in stage C. Can be considered in symptomatic patients with valve area 1.6-2.0 cm² if favorable valve anatomy, and no LA thrombus or moderate to severe MR.
  • Surgery (repair, commissurotomy, replacement) for those who are not high risk for surgery and who are not candidates or have failed commissurotomy. Also consider surgery for those undergoing CV surgery for other indications with MS that is stage C or D.

MANAGEMENT OF ACUTE VALVULAR LESIONS

Valve Lesion	Etiology	Identification	Initial Management
Symptomatic, severe AS	Inflammatory calcific (most common) Bicuspid Congenital Rheumatic	Cardiogenic shock	Consider early AVR intervention Cautious diuresis to relieve pulmonary congestion NE is first line in those with shock. Avoid vasodilator therapy unless under direction of advanced heart failure
Acute AR (may not have murmur)	Leaflet abnormalities Infective endocarditis Trauma Prosthetic valve dysfunction Post aortic balloon valvuloplasty Root or ascending aorta Aortic dissection Perivalvular leak Dehiscence of prosthetic valve	Signs of hyperdynamic circulation as in chronic AR often absent Pulse pressure may be normal or only slightly increased Suspect dissection in those with chest pain & differential blood pressures in both arms	Surgical emergency (especially if dissection associated) Medical management goals: increase HR (isoproterenol, dobutamine), reduce afterload (nitroprusside) and pulmonary decongestion Antibiotics for suspected endocarditis
Acute MR	Infectious endocarditis Spontaneous chordal rupture Papillary muscle rupture	Acute volume overload of LA, pulmonary congestion and low cardiac output Murmur may be short due to rapid rise in LA pressure and equalization of LA-LV gradient TTE typically adequate, rarely TEE required	Urgent or emergent surgery Vasodilators (nitroprusside, nicardipine) improve forward flow IABP as temporizing measure until surgery

 

 

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