hsci 335 valvular heart diseases

• valve stenosis with or without regurgitation

• pure regurgitation (no stenosis)

what types of valvular disorders are classified functionally

valvular

what types of disorders can be classified functionally and anatomically

rheumatic or non-rheumatic

acquired valvular heart disease can be…

non-rheumatic

_______ disease can be:

• calcific

• degenerative

• functional

• inflammatory (endocarditis)

rheumatic

what type of heart disease:

• affects almost 40 million people, with almost 300,000 deaths/year

• is a consequence of an autoimmune disorder from β-hemolytic strep infection either untreated or undertreated

• usually has affected mitral valve, sometimes aortic too

• has murmurs of aortic and mitral insufficiency

rheumatic fever

autoimmune disorder from β-hemolytic strep infection either untreated or undertreated; it’s thought that lymphatic channels from tonsils transmit group A strep to heart

murmurs of aortic and mitral insufficiency

• High-pitched, blowing, pansystolic murmur may be heard with mitral valve affected

• Short, low-pitched mid-diastolic murmur (Carey Coombs murmur) may be heard at apex; may be due to swelling and stiffening of mitral valve leaflets, increased gradient over valve, a decreased in compliance of left ventricle

infective endocarditis

• Acute & subacute bacterial, viruses, fungi, other microorganisms

• Cases have increases since 1990, with a little over 1 million cases

• Acute is most often caused by Staphylococcus aureus

• Subacute most often caused by streptococcal bacteria

• Three processes involved in pathogenesis

heart valves must be primed (•Bacterial do not adhere to normal endothelium •Surface damaged from trauma, inflammation, ECF disruption, platelet/fibrin deposition)

1st process involved in pathogenesis of infective endocarditis

bacterial adhere to fibrin-platelet matrix

2nd process involved in pathogenesis of infective endocarditis

antibodies may block adherence, but bacteria may be resistant

3rd process involved in pathogenesis of infective endocarditis

degenerative valve disease

• Historically both aortic (2% of population) and mitral (18 million) valve disease

• Most common mitral valve disease is mitral valve prolapse

• Women disproportionately affected; DALY and mortality rates higher in women

• Two types of processes

Myxomatous degeneration

• Accumulation of proteoglycans, collagen degradation, elastin fragmentation

• Results in prolapse and regurgitation from “floppy” valve

1st process of degenerative valve disease

Fibrotic degeneration

• Accumulation of collagen, proteoglycan degradation, elastin fragmentation

• Restriction of leaflet motion and stenosis (stiff) valve

2nd process of degenerative valve disease

calcific heart disease

• Major disease is calcific aortic stenosis; approx 1 in 8 people over 75 have moderate to severe aortic stenosis

• Thought to be similar to atherosclerosis – lipoprotein deposition, chronic inflammation, active calcification

• Patients have higher prevalence of coronary artery disease, peripheral artery disease, hypercholesterolemia, and hypertension

stage A

AHA/ACC Guideline for the Management of Patients with Valvular

Heart Disease:

• At risk, patients w/risk factors for development of VHD

stage B

AHA/ACC Guideline for the Management of Patients with Valvular

Heart Disease:

• Progressive, patients w/progressive VHD (mild ot moderae severity and asymptomatic)

stage C

AHA/ACC Guideline for the Management of Patients with Valvular

Heart Disease:

• asymptomatic severe, asymptomatic patients who have the criteria for severe VHD

• 1: LV or RV remains compensated

• 2: decompensation of the LV or RV

stage D

AHA/ACC Guideline for the Management of Patients with Valvular

Heart Disease:

• symptomatic severe, patients who have developed symptoms as a result of VHD

Left ventricular hypertrophy

What does a sustained apical impulse indicate?

prolonged outward movement with a diffuse apical impulse

How does left ventricular hypertrophy affect the apical impulse?

hyperdynamic apical impulse

thrusting of increased amplitude from increased stroke volume or

volume overload (mitral/aortic regurgitation)

Mitral stenosis (a less sustained impulse)

What does a tapping apical impulse indicate?

hyperkinetic

What type of apical impulse is seen during pregnancy?

it’s louder than normal

how does s1 change during pregnancy

it may be widely split

how does s2 change during pregnancy

A left ventricular S3 (present in most pregnant patients)

What additional heart sound is commonly heard in pregnant women?

A soft middiastolic murmur

What kind of murmur may be heard at the upper left sternal border during pregnancy?

Venous hum or mammary soufflé

What continuous murmurs may occur during pregnancy?

pulmonary

what valve has 3 cusps (right, left, anterior), is thinner and more delicate than aortic, and is part of right ventricular outflow tract; is also anterior and superior to 3 other valves

pulmonary stenosis

• Congenital in 95% cases

• Different forms based on level of outflow tract narrowing (valvar, subvalvar, suprevalvar)

valvar pulmonary stenosis

doming leaflets with total fusion and single orifice in middle

• Uncommon for valves to calcify with time

• Usually association with a degree of R vent hypertrophy and dysfunction

subvalvar pulmonary stenosis

commonly caused by infundibular stenosis or 2-chambered right

ventricle

supravalvar pulmonary stenosis

in the proximal segment of pulmonary artery

• Single or multiple narrowing

No, mild stenosis is typically asymptomatic

Do patients with mild pulmonary stenosis usually have symptoms?

They can tolerate it for years without major symptoms

How do patients with moderate pulmonary stenosis typically present?

Fatigue and dyspnea (shortness of breath)

What are common symptoms of severe pulmonary stenosis?

systolic crescendo-decrescendo murmur loudest at the 2nd intercostal space near the sternal border

Describe the murmur associated with pulmonary stenosis

To the neck or left shoulder

Where can the pulmonary stenosis murmur radiate?

An ejection click

What sound may precede the murmur in pulmonary stenosis?

Based on the pressure gradient across the valve

How is pulmonary stenosis graded?

pulmonary regurgitation

• A small amount of regurgitation is common is most cases

• Significant regurgitation is very rare

• pulmonary htn and dilation of right ventricular outflow tract may

demonstrate a degree of regurgitation

• Soft, diastolic murmur best heard in left parasternal region; Begins after pulmonary component of S2 and may also have systolic murmur from increased stroke volume

right-ventricular dilation

Complications of Pulmonary Regurgitation

• Long-standing regurgitation usually well tolerated , possibly due to low pressure differences between pulmonary artery and right ventricle

• May remain normal size even in severe regurgitation, but may show different degrees of dilation (remodeling)

right-ventricular dysfunction

Complications of Pulmonary Regurgitation

• Myocardium may become stiff

• Compromised filling time, limited stroke volume, exercise intolerance

arrythmia

Complications of Pulmonary Regurgitation

• either atrial or ventricular

tricuspid

what valve has 3 cusps, resembling curtain-like, billowing flaps (large anterior, septal, and posterior (inferior), combined surface area of cusps larger than area of valvular orifice

tricuspid stenosis

• Less prevalent than mitral stenosis

• Causes:

• Rheumatic fever: most common; cusps thickened and fuse – valve area less and domes toward RV in diastole; atrium dilates and congests as progresses

• Carcinoid disease: colonic tumor secreting substance usually affecting right heart, leaflets become fibrosed and fuse

• Right ventricular pacing: a pacing wire perforating a leaflet can cause inflammation

rheumatic fever

most common; cusps thickened and fuse – valve area less and domes toward RV in diastole; atrium dilates and congests as progresses

carcinoid disease

colonic tumor secreting substance usually affecting right heart, leaflets become fibrosed and fuse

right ventricular pacing

a pacing wire perforating a leaflet can cause inflammation

functional tricuspid stenosis

A form with normal valve leaflets but raised ventricular inflow resistance

• Large atrial septal defect – left to right shunt increases right atrial stroke volume

• Localized pericardial effusion behind R atrium – increased perfusion pressure may

collapse right atrial free wall and narrow inflow to R vent

• Right atrial myxoma – tumor narrows right ventricular inflow tract; rare in terms of incidence compared to L atrium

• Right atrial secondaries – different types of tumors may spread to R atrium

rare in comparison

How common is tricuspid stenosis compared to left atrial (mitral) stenosis?

It’s similar to mitral stenosis but heard closer to the sternum and intensifies with inspiration

How does the murmur of tricuspid stenosis differ from mitral stenosis?

Venous congestion, liver dysfunction, and ascites

What are complications of long-standing tricuspid stenosis?

No, mild regurgitation is a normal finding in about 50% of healthy people

Is mild tricuspid regurgitation always abnormal?

functional (due to right ventricular enlargement)

Is tricuspid regurgitation usually functional or structural?

Ebstein anomaly

What is the most common congenital cause of tricuspid regurgitation?

The hinge points of the septal and inferior cusps are displaced, causing regurgitation

What happens in Ebstein anomaly?

Right ventricular and tricuspid ring dilation, often due to pulmonary hypertension

What causes functional tricuspid regurgitation?

rheumatic disease

severe regurgitation noticed following mitral valve replacement for rheumatic disease

endocarditis

isolated tricuspid endocarditis less common than other valves

Endomyocardial fibrosis

rare; inflow tract of right ventricle distorted

pacemaker insertion

lead perforating leaflet can lead to fibrosis and retraction

leaflet prolapse

mid-systolic tricuspid prolapse may be associated with mitral valve

• Seen in Marfan syndrome, but insignificant compared to mitral

cardiomyopathy

progressive ischemic deterioration of R vent can dilate basal tricuspid ring: similar may be seen in idiopathic dilated cardiomyopathy

radiotherapy

uncommon. May be long-term radiotherapy to chest

A pansystolic murmur heard at the right sternal border, radiating to the epigastrium

Describe the murmur of tricuspid regurgitation.

increases in intensity due to increased venous return

How does inspiration affect the murmur of tricuspid regurgitation?

mitral

Which heart valve is most affected in almost all cases of rheumatic heart disease?

at risk

Stage A of AHA/ACC Guidelines for Valvular Heart Disease is defined as

congenital

Most cases of pulmonary stenosis are?

20 years

Mitral valve stenosis occurs approximately how long after rheumatic fever?

Decreases forward output

What is the effect of mitral regurgitation?

apex

Where is the murmur from mitral valve prolapse best heard?

valvar

What is the most common form of aortic stenosis?

C and D

Subclasses for aortic stenosis occur in which stages

1/2

mild tricuspid regurgitation is a normal finding in what proportion of people?