Post Midterm: via Final review + more?

Describe systemic diseases related to cardiac manifestations: diabetes

Description:

• hypo-secretion of insulin

Cardiac involvement:

• poor systolic and diastolic function

• LV remodelling, concentric LVH

Describe systemic diseases related to cardiac manifestations: Neuromuscular diseases (2)

Description:

• Two main ones:

  • Friederich’s Ataxia

  • Ducheme muscular Dystophy

• inherited, causes muscle weakness include heart muscle

Cardiac involvement:

• associated with dilated cardiomyopathy

Describe systemic diseases related to cardiac manifestations: Ankylosing spondylitis

Description:

• chronic inflammatory disease of the spine

Cardiac involvement:

• aortic dilation

• arrhythmias/atrial fibrillation

Describe systemic diseases related to cardiac manifestations: Rheumatoid arthritis

Description:

• inflammation of joints

Cardiac involvement:

• atherosclerosis risk

• MI, stroke

• atrial fibrillation

• hypertension

• heart failure

Describe systemic diseases related to cardiac manifestations: Scleroderma

Description:

• autoimmune disease of the connective tissues → thickening and tightening of the skin, organs (lungs and heart)

Cardiac involvement:

• pulmonary hypertension → can lead to right heart failure

Describe systemic diseases related to cardiac manifestations: Lupus Erythematosus

Description:

• autoimmune disease

  • joints, skin, kidneys, blood, brain, heart, and lungs

Cardiac involvement:

• effusions/pericarditis

• myocarditis

• valve thickening

• heart failure

• CAD

Describe acquired diseases related to cardiac manifestations: Chagas Cardiomyopathy - “kissing bugs”

• Chagas Cardiomyopathy is dilated cardiomyopathy that forms 20-30 years post Chagas infection

• can also lead to heart failure

Describe systemic diseases related to cardiac manifestations: hereditary connective tissue disorders

Description:

• 3 main types:

  • Marfan syndrome

  • Ehlers-Danlos syndrome

  • Loeys-Dietz syndrome

• connective tissue diseases → leaky →weakens blood vessels

Cardiac involvement:

• aortic dilation and dissections

What are the main characteristics of Marfan Syndrome? What heart abnormalities mainly occur in (3)

• Characteristics

  • tall/scoliosis/hypermobility of joints

  • high palate/poor vision/ long limbs

• Diseases:

  • mitral valve prolapse

  • aortic dilation or dissection

  • aortic regurgitation

Describe the main anatomy of aorta (5)

• Aortic root: area between LVOT and ST junction

• Ascending aorta: extends from ST junction to origin of brachiocephalic artery, roughly ~5cm long

• Aortic arch: extends brachiocephalic artery to ligamentum arteriosum, which lies near the subclavian artery, roughly ~4cm long

• Aortic isthmus: located at site of ligamentum ateriosum, where aorta is fixed to thoracic cage (cannot move bc attached to bone but vulnerable to trauma)

• Descending aorta: extends from the aortic isthmus to diaphragm

What are the layers of the aorta? (3)

• tunica externa

• tunica media

• tunica intima

Describe anatomy of aortic valve (4 components)

• Annulus: provides structural support to cusps

• Cusps: 3 half-moon (semilunar) in shape; right, left, non-coronary

• Commissures: where the cusps come together

• Interleaflet triangles: extensions of the ventricular outflow tract

Describe anatomy of Aortic Root/ Sinuses of Valsalva, includes 4 components

• section between the LVOT and Asc. Ao

  • specifically the inferior attachment of the aortic cusps to the ST junction

• Includes:

  • aortic cusps

  • Sinuses of Valsalva

  • Comissures

  • Interleaflet triangles

Describe the diseases of the great vessels/aorta (6)

• Atherosclerosis – Plaque builds up in artery wall

• Aortic aneurysm – Weakening or degeneration layers of wall → dilation

• Aortic dissection – Intimal tear → blood splits wall

• Intramural hematoma – Bleeding inside wall (no tear)

• Blunt chest aortic trauma – Sudden force → aortic tear on isthmus

• Coarctation of the aorta – Congenital narrowing of descending aorta

What are the 2 types of aortic aneurysm

• Saccular: occurs due to weakening of vessel wall at one point, leading to an out pouching of vessel wall

• Fusiform: uniform and symmetrical dilation of entire circumference of the vessel (most common)

What are some etiologies for aortic aneurysms?

• Connective tissue disease

• Bicuspid AoV

• Aortic stenosis

• Atherosclerosis → intimal layer thickened due to fatty plaque and they destroy elastic fibres and muscle cells in medial layer → weaken

Describe Sinus of Valsalva Aneurysm (SVA)

• rare congenital anomaly seen on PSAX or PLAX

  • not medical emergency because born with it! → still report

• weakening or absence of the media layer

• weakened sinus dilates and forms an aneurysm, can cause holes

What are some complications of SVA (3)

1. LSOVA

   • may rupture into LA or RA

2. NCSOVA

   • may rupture into LA, RA, LV, or ventricular septum

3. RSOVA (most common)

   • may rupture into LV, RA, RV (across septum), pericardium, adjacent main pulmonary artery

   • can cause most problems: obstruction, dissection, compression, acute MI, heart block, tamponade

4. Overall: more blood is being moved back into the heart instead of being pumped out

Describe aortic dissection

• dissection is a tear in the intimal layer that allows blood to enter the media layer

  • creates a blood filled lumen separated from the true lumen by an intimal flap

What are the 2 classifications of aortic dissections, and their sub types?

• DeBakey:

  • Type I: originates in the proximal ascending aorta. Involves ascending aorta, arch, and variable lengths of descending and abdominal aorta

  • Type II: ascending aorta only

  • Type III: begins in the descending aorta

• Standard:

  • Type A: any dissection that involves ascending aorta

  • Type B: any dissection that does not involve ascending aorta

Describe Kawasaki disease origin and complications

• Etiology: usually via viral infection, auto-immune disease, inflamed blood vessels (coronary)

• Complications:

  • vasculitis: inflammation of blood vessels, usually coronary arteries

  • coronary artery dilation

  • giant aneurysm

  • myocardial infarction

    • blood clots within coronary

    • coronary artery aneurysm

Describe the two main pulmonary artery disease and etiology (2)

• dilation of pulmonary artery → Marfan syndrome

• stenosis of pulmonary artery → congenital

Differentiate AoV stenosis and sclerosis

• Aortic Stenosis: reduced (restricted) opening of the aortic valve in systole via calcium build up over time

  • valve appears brighter than normal, does not open well, has velocity over 2.5 m/s

• Aortic Sclerosis: thickening of the valve leaflets with no restriction of blood flow

  • valve appears brighter than normal, still opens well, has velocity less than 2.5 m/s

Describe pathology causes of aortic stenosis (3) and how common

1) Congenital: roughly 30-40% of cases

• bicuspid (but not all are stenotic)

• unicuspid or quadricuspid

• subvalvular or supravalvular

2) Acquired: Calcific: > 50% of cases

• calcium deposits overtime (age) prevent opening

3) Acquired: Rheumatic: <10% of cases

• history of rheumatic fever, the scar tissue creates rough surface → narrow opening + place for calcium to collect

Echo differentiation from calcific versus rheumatic valves?

• calcific:

  • commissural fusion commonly absent

  • “chunks” of brightness (calcium) seen, uneven brightness

• rheumatic/age:

  • commissural fusion is triangular systolic orifice

  • slightly brighter than normal, uniformly thickened leaflets

What are the two types of congenital bicuspid AoV?

• Without a raphe

  • rare

  • cusps usually equal in size

• With a raphe (seam/union)

  • more common

  • cusps unequal in size

  • RCC/LCC → RCC/NCC → NCC/LCC

What are the consequences of congenital bicuspid AoV? (4)

1) Aortic root dilation: higher risk for aortic aneurysm or dissections

2) Coarctation of aorta: narrowing in desc. ao

3) Supravalvular aortic stenosis

4) Ventricular septal defects (VSD)

Describe subvalvular stenosis

• fibrous membrane or muscular ring in LVOT (below AoV) → obstruction in the outflow

• Leads to: narrow LVOT → septal hypertrophy = thicker IVS → dynamic obstruction → mitral valve may get “sucked up”

What is dynamic obstruction?

• degree of stenosis varies depending on loading conditions, or variable blockage of blood flow instead of fixed narrowing

  • severity changes based on: cardiac cycle, HR, volume, movement of leaflets

Describe supravalvular stenosis

• uncommon narrowing of aorta just above AoV

• Dysplasia (abnormal) aortic wall → hour glass type

• membrane with central orifice

• Hypoplasia (underdeveloped) ascending aorta

Describe hemodynamic consequences of aortic stenosis (2)

• Concentric LVH

  • narrowed AoV → increased pressure load → LV wall thickens to compensate (concentric LVH) → LV stiff + lower compliance

    • lead to diastolic dysfunction → LV does not relax well → poor filling

    • increased LAP → back into lungs → increase pulmonary pressures → SOB

  • Usually, normal EF, but will drop in severe cases

• Ischemia

  • increased muscle mass due to LVH → increase oxygen demand

  • compressed coronary vessels due to LVH → decrease oxygen supply

  • angina: decrease oxygen → decrease LV contractility → systolic dysfunction

• Dilated ascending aorta

  • high velocity jet hitting aortic wall → weakens and stretches wall

Signs and symptoms of aortic stenosis (4)

• Systolic Ejection Murmur (SEM)

• Angina (may be w/ CAD)

  • myocardial ischemia (LVH →↑ demand + ↓ supply)

• Syncope (fainting)/ presyncope (feeling light-headed)

  • fixed cardiac output → ↓ cerebral perfusion during exertion

  • forward failure → not enough blood flow to brain

• Shortness of breath and fatigue

  • reduced cardiac output → ↓ systemic perfusion

  • backward failure → lung problem cause by pressure backup

Role of Sonography in Aortic Stenosis (6)

1. Determine presence of aortic stenosis vs sclerosis

2. Determine etiology

3. Assess LV wall thickness

4. Measure aorta

5. Estimate severity of aortic stenosis

6. Identify associated abnormalities (regurgitation)

How to estimate severity of aortic stenosis (3)

1. Mean gradient (mean PG)

   • AoV VTI trace (from CW in 5Ch)

2. Maximum Jet Velocity (Vmax)

   • AoV VTI trace (from CW in 5Ch)

3. Continuity equation for AVA:

   • LVOT diameter

   • LVOT VTI (from PW in 5Ch)

   • AoV VTI (from CW in 5Ch)

Extra: double check with PEDOF to find peak Vmax through the valve in different windows (small enough that you can go anywhere)

What is the continuity principle? How is it related in normal vs stenotic valve?

• states what flows in, must flow out

• in normal valves: the velocity should be the same before and after the valve

• in stenotic valves: blood will have to speed up past the valve due to narrowing

What is VTI and why do we use it?

• What: area under Doppler curve

  • Unit is cm because VTI = velocity/time

• Why: In human body, velocity is not constant at any given time and varies at different parts of the vessel

  • VTI sums up all the individual velocities over time to find a representative overall velocity

What are the normal values for the Vmax, meanPG, and AVA (VTI)?

Can you use continuity equation of AoV when there is LVOT obstruction?

• no, bc equation compares normal flow to stenotic flow

• with LVOT obstruction → velocity is already high in LVOT and the velocity in AoV will still be high since there was no time to slow down

• Have to assess visually or perform a valve planimetry in SAX

What is 2D Planimetry of AVA

• PSAX view, trace opening in mid systole

• not routinely performed due to many pitfalls (hard to be consistent)

• could be used to double check the AVA if clearly seen

What is dimensionless velocity ratio (DVI)?

• removes error associated with LVOT diameter by removing CSA from the continuity equation

  • velocity ratio = V_LVOT/ V_AoV

• closer to 0 = more severe

• closer to 1 = more normal

What is aortic insufficiency (AI) / aortic regurgitation (AR) ?

• inability of the aortic valve leaflets to remain closed during diastole, resulting in some stroke volume leaking back into LV

  • increase in left ventricular end-diastolic volume

Role of Sonography in Aortic Insufficiency

1. Determine etiology

2. Assess LV size and systolic function

3. Measure aorta

4. Estimate severity of regurgitation

List the common etiologies of AI/AR (4)

• aortic annulus/ aortic root dilation: congenital, AS, athero. infection, trauma

• cusp abnormalities: bi/quad-cuspid, calcific, infection, rheumatic fever

• annular or aortic root distortion: aortic root inflammation

• loss of aortic cusp (commissural) support: VSD or dissection

Whats are some causes of acute vs chronic AI?

• causes of acute AI (emerg.):

  • trauma

  • dissection

  • endocarditis

• causes of chronic AI:

  • bicuspid AoV

  • rheumatic AoV

  • calcific AoV

Difference between aortic dilation and aneurysm

• Aortic aneurysm:

  • dilation involving all layers of the aorta 1.5x greater than normal diameter

• Aortic dilation:

  • dilation involving all layers of the aorta, larger than accepted normal values, but not large enough to be considered an aneurysm

What are some hemodynamic consequences for Acute AI?

if severe enough → medical emergency & immediate valve replacement needed

What are some hemodynamic consequences for Chronic AI- early stages?

• LVH also help reduce wall stress (LaPlace’s Law)

• wide pressure pulse bc still AI

• EF remain normal

What are some hemodynamic consequences for Chronic AI- long standing?

• even wider pulse pressure

  • low diastolic aorta pressure → coronary artery perfusion drops → decreased oxygen supply to heart muscles

How to estimate severity of Aortic Insufficiency? (5)

1. Visual assessment

2. Flow reversal in Desc. Ao

3. Pressure Half-Time (PHT)

4. Jet-width and Vena Contracta

5. PISA/EROA

What is PHT?

• time it takes for the initial pressure gradient to drop to half of its initial value

  • “how fast blood is flowing backward through the AoV”

• unit: msec

How do you measure PHT for Aortic Insufficiency?

• Obtain CW Doppler Trace in AI jet

  • 5 or 3-chamber as parallel as possible

• 2 points:

  • highest velocity on trace (around 5 m/s in early diastole regardless of severity)

  • line along the slope on the AI profile

• machine calculates PHT

What is the general idea of PHT for Aortic Insufficiency?

• Mild/Trival AI:

  • Long PHT → flat slope → slow pressure equalization

  • LV P rises slowly (blood from LA) → stays low → Ao P slowly falls → thus equalized slower

• Moderate/Severe AI:

  • Short PHT → steep slope → fast pressure equalization

  • LV P rises faster (blood from LA + Ao) + Ao P falls faster (less blood ejected) → thus equalized faster

How to determine Flow Reversal in descending aorta in Aortic Insufficiency?

• for moderate/severe AI → diastolic flow reversal seen in descending and/or abdominal aorta

• Assess PW from both suprasternal and subcostal window:

  • if only SSN → suggest moderate AI

  • if both SSN and subcostal → suggest severe

• Compare abnormal retrograde flow compare to normal antegrade flow

Describe the abnormal flow (flow reversal) in SSN and subcostal view for Aortic Insufficiency

• Suprasternal/SSN Window:

  • Flow reversal seen above the baseline during diastole

• Subcostal Abdominal Window:

  • Flow reversal seen below the baseline during diastole

How do you assess Jet Width to Height Ratio for Aortic Insufficiency?

• Jet width: width of AI jet as it travels through LVOT area

Steps:

1. Zoom in LVOT with colour

2. Freeze frame with AI jet

3. measure width of AI jet in LVOT area (same location as LVOT diameter)

4. Divide jet width by LVOT diameter to get percentage:

   • Calculate: (Jet width)/(LVOT diameter) * 100

How do you assess Vena Contracta for Aortic Insufficiency?

• Vena contracta: narrowest part of the jet in the area between the proximal flow convergence zone and the regurgitant jet

Steps:

1. measure in PLAX

2. Zoom in on the aortic valve and LVOT

3. store cine that shows flow convergence dome, VC, and regurgitant jet

4. freeze and measure the narrowest portion of the AI jet

How do you assess PISA/EROA for Aortic Insufficiency?

• Proximal Isovelocity Surface Area (PISA): measures the effective regurgitant orifice area (EROA), which is the size of the “hole” that the regurgitant blood flows through

  • bigger EROA, the worse regurgitation

Steps:

1. Find the regurgitant jet on CDI

2. Change Nyquist limit in same direction as jet

3. Align CW Doppler and measure aliasing radius of PISA:

   • r = radius of the regurgitant orifice to aliased velocity

4. Measure peak velocity of regurgitant jet (Vmax)

5. Calculate EROA using the 3 bolded values

What is flow convergence zone and isovelocities?

• Iso-velocities: series of hemispheric shells of uniform velocity

• Flow convergence zone: where regurgitant blood rushes backwards towards the regurgitant orifice, there is flow acceleration just proximal to the orifice (just before the valve)

  • made up of iso-velocities

  • area of increased velocity

Describe the anatomy of mitral valve (5)

• annulus: structural support, D-shaped

• leaflets:

  • anterior: short & wide, 1/3 of annulus

  • posterior: long & narrow, 2/3 of annulus

  • Scallops: subdivision (A1-A3, P1-P3)

• commissures: where leaflets meet labeled by location

• chordae tendineae: connecting leaflets for pap muscles, prevent prolapse during systole

• papillary muscles: prevent prolapse during systole

  • anterolateral and postermedial

What are some etiologies of mitral stenosis (3)

1. congenital mitral stenosis

   • parachute mitral valve → chordae attached to single papillary muscle

   • supravalvular ring

2. rheumatic mitral valve stenosis

   • rheumatic fever earlier in life → hockey stick, commissural fusion

3. mitral annular calcification (MAC)

   • calcium build up as you age and starts at PMVL

What is one of the differentiation between MAC or rheumatic mitral stenosis?

• absence of commissural fusion in MAC differentiates it from rheumatic mitral valve stenosis

• it is fused in rheumatic !

Describe underlying hemodynamic changes associated with mitral stenosis

Role of Sonography for Mitral Stenosis (6)

1. Determine presence of MS

2. Determine Etiology

3. Assess LA: size (dilated?), presence/absence of thrombus

4. Identify associated lesions (MR or AS)

5. Estimate severity of stenosis

6. Other factors to consider → assess degree of hypertension bc as MS increases, pulmonary pressures increase

Two ways to determine presence of Mitral Stenosis

• Colour Doppler: LV inflow jets may be eccentric, looks like a candle flame

• M-Mode: mitral EF slope refers to the function of the rate of LA emptying and LV filling → hallmark is that slope more flat because blood flow is slower

How to assess severity of mitral stenosis? (3)

1. Mean pressure gradient

   • MV VTI Trace (from CW in 4Ch)

2. Mitral valve area (MVA) via:

   • PHT

     • Tip to baseline of trace (from CW in 4Ch) → MVA = 220/PHT

   • Continuity equation:

     • LVOT diameter

     • LVOT VTI (from PW in 5Ch)

     • MV VTI (from CW in 4Ch)

   • Planimetry (not BC)

3. PISA (not BC)

Describe PHT on normal versus stenotic mitral valve

• Flow through the MV is affected by both diastolic filling patterns and by the health of the MV

• normal valve → the predominant factor that affects flow through the valve is diastolic filling patterns.

  • Short PHT → steep slope

• stenotic valve → the predominant factor that affects flow through the valve is the health of the valve

  • Longer PHT → flatter slope

When is MVA via PHT not valid? (4)

• Tachycardia

  • shortens diastole/ changes shape of trace, E & A merge

• Significant aortic regurgitation

  • increases LV pressure / affects MV inflow patterns

• Changes in diastole

  • increases LV pressure / affects MV inflow patterns

• Post valve surgery

  • no longer “stenotic” and this measurement is no longer valid

Describe determination of PHT in Bimodal Doppler Spectrums for Mitral Stenosis

• deceleration slope is sometimes bimodal, the decline of mitral flow velocity being more rapid in early diastole than during the following part of the E wave

• recommended to trace deceleration slope in mid-diastole rather than early-diastole

Define mitral regurgitation

retrograde (backward) flow of blood from the left ventricle into the left atrium during systole

What differentiates primary/organic vs secondary mitral regurgitation?

• Primary/organic

  • mitral valve leaflets and/or the mitral valve apparatus is abnormal, leading to mitral regurgitation

• Secondary:

  • diseases that affect another part of the heart, but cause MR → example: dilated LV

  • valve itself is normal but leaks due to other problems with the heart

Describe flail leaflet

ruptured chordae tendineae causes loss of stability of leaflet → leaflet tip points in towards the LA when valve is closed

Describe ruptured pap

• rare

• pap muscle ruptures off the LV wall → causes flail leaflet with the entire pap muscle seen traveling between both the LA and LV every time the valve opens and closes

Describe cleft leaflet

• congenital hole in MV leaflet → usually in AMVL

Describe SAM

• systolic anterior motion of AMVL

• AMVL moves up into the LVOT during systole when valve is closed

• causes posteriorly directed MR

Describe tethered leaflet

Remodelling of the LV causes chordae tendineae to be mal-positioned and causes MR

Describe how dilated LV cause MR

leaflets can no longer fully close

Describe mitral valve prolapse