Valvular Lesions of the Heart

Valvular Lesionsof the Heart

© William Herring, MD, FACR

Aortic Stenosis

Aortic StenosisFrequency of Causes

1.Degeneration of bicuspid aortic valve

2.Degeneration of tricuspid aortic valve inperson > 65 years old

3.Rheumatic heart disease in a tricuspidvalve

Aortic Stenosis3 Locations

● Supravalvular

● Valvular

● Subvalvular

SupravalvularAortic Stenosis

Supravalvular Aortic StenosisGeneral

●Uncommon

●Types

■Hourglass

■Membrane

■Hypoplasia of entire ascending aorta

●Associated with other lesions in ⅔

■William’s syndrome

• William’s syndrome

• Supravalvular Ao stenosis

• Hypercalcemia

• Elfin facies

• Pulmonary stenoses

• Hypoplasia of aorta

• Stenoses in

• Renals, celiac, SMA

Supravalvular Aortic Stenosis

Valvular Aortic StenosisCongenital

Congenital Valvular Aortic StenosisGeneral

●Bicuspid aortic valve is the mostcommon congenital cardiac anomaly

■ 0.5 –2%

●Usually not stenotic during infancy

●More prone to fibrosis and calcificationthan normal valve

Society of Thoracic Surgeons

●Many malformations of aorta and/or LVare associated with bicuspid valve

■In 50% of coarctation of aorta

■Hypoplastic left heart syndrome

■Interruption of aortic arch

Congenital Valvular Aortic StenosisAssociations

Congenital Valvular Aortic StenosisCalcification

●Calcification begins earlier (4th decade)than in degeneration of a tricuspid Aovalve (>65)

Unopacified jetstream through abicuspid aorticvalve

Acquired aorticstenosis wouldnot demonstratethis jet becausesevere deformityof valve turbulent flow

Leaflets are“domed” onsystole

What other lesion does this patient have?

Congenital Valvular Aortic StenosisAngiographic findings

2, not 3,aorticsinuses

Valvular Aortic Stenosis Acquired

Acquired Valvular Aortic StenosisCauses

●Fusion, thickening or calcification of atricuspid aortic valve

■Degenerative process (>65 years old)

■Rheumatic heart disease (younger)

Valvular Aortic StenosisDifferentiating Features

Etiology/Findings

Calcification

Other clues

Congenital BicuspidValve

30’s

Jet effect onaortogram

Degeneration ofTricuspid Valve

> 65

No jet effect;Commissures don’tfuse

Rheumatic dz inTricuspid Valve

30’s here; teens in3rd world countries

MS or MR almostalways present;commissures fuse

Aortic StenosisImaging Findings

● Depends on age patient/severity of disease

■ In infants, AS  CHF/pulmonary edema

● In adults

■ Normal heart size

● Until cardiac muscle decompensates

■ Enlarged ascending aorta 2° post-stenoticdilatation 2° turbulent flow

■ Normal pulmonary vasculature

Aortic stenosis

Prominenceof ascendingaorta frompost-stenoticdilatation

Prominenceof ascendingaorta frompost-stenoticdilatation

Normal-sizedheart

Aortic stenosis

Post-stenotic Dilatation of Aorta

●From turbulent flow just distal to anyhemodynamically significant arterialstenosis

■Jet effect also plays role

●Occurs mostly with valvular aorticstenosis

■May occur at any age

Aortic stenosis

Prominenceof ascendingaorta frompost-stenoticdilatation

Prominenceof ascendingaorta frompost-stenoticdilatation

Aortic StenosisCalcification of Valve

● In females, usually indicateshemodynamically significant AS

■ Calcification of valve usually indicates gradientacross valve of > 50mm Hg

● In males, calcified valve may not =significant aortic stenosis

Calcification of Aortic Valve

Patient with prosthetic mitral and aortic valves

A

M

Calcification of Aortic Valve

SubvalvularAortic Stenosis

Subvalvular Aortic StenosisSubaortic Stenosis

●Associated with

■Hypoplastic left heart syndrome

■Idiopathic Hypertrophic Subaortic Stenosis

●Hypertrophic cardiomyopathy

■Subaortic fibrous membrane

Aortic Regurgitation(Aortic Insufficiency)

Aortic RegurgitationCauses

●Rheumatic heart disease

●Marfan’s

●Luetic aortitis

●Ehlers-Danlos syndrome

●Endocarditis

●Aortic dissection

Aortic Regurgitation 2° Rheumatic Dz

Thickening of thecusps

Commissuralfusion (doesnot occur indegenerativeform of AR)

Aortic RegurgitationImaging Findings

●Hallmarks

■Left ventricular enlargement (very large)

■Enlargement of entire aorta

●Cine MRI (gradient refocused MRI)

■Signal void coming from aortic valve to LV

Aortic RegurgitationCine MR image during diastole shows signal voidemanating from the aortic valve

LV

Ao

RA

Aortic Regurgitation

Enlarged leftventricle

Enlargementof entireaorta

Mitral StenosisLeft AtrialOutflow Obstruction

Mitral StenosisLeft AtrialOutflow Obstruction

Mitral StenosisRheumatic Valvular Heart Disease

● Rheumatic heart disease causesmitral stenosis in 99.8% of cases

●Acute phase (pancarditis) subsides

■Fibrosis alters leaflet and cusp structure

■Results in leaflet or cuspal thickeningalong valvular margins of closure

●Valves affected

■ Most often mitral valve alone

■ Then most often mitral and aortic together

■ Lastly aortic alone

Acute Rheumatic Valvulitis Pathophysiology

Normal mitral valve

Stenotic mitral valve

Fusion ofchordae

Thickeningof cusps

Chronic Mitral StenosisPathophysiology

● Mitral orifice becomes smaller leading to:

● Two circulatory changes

● Left atrial pressure to maintain LV filling acrossstenotic valve

● Overall, blood flow across mitral valve is  whichleads to  cardiac output

• Mitral stenosis occurs

• Left atrial pressure 

• Left atrium enlarges

• Cephalization

• PIE

• PAH develops

• PVR increases

• RV enlarges

• Pulmonic regurg develops

• Tricuspid annulus dilates

• Tricuspid insufficiency

• RV failure

Time course of MS in adult

Effect of Mitral Stenosis on LungsNormal chamber pressures

M 1-5

M 5-10

D 1-7

S 17-32

M 15

D 5-12

S 90-140

LA

RA

RV

LV

● Pulmonary venous and capillary pressure

Effect of Mitral StenosisOn Lungs

Imaging Findings of MSCardiac Findings

● Usually normal or slightly enlarged heart

■ Enlarged atria do not produce cardiacenlargement; only enlarged ventricles

● Straightening of left heart border

● Or, convexity along left heart border 2°to enlarged atrial appendage

■ Only in rheumatic heart disease

“Straightening” ofleft heart border

Normal-sizedheart

Cephalization

Mitral Stenosis

Mitral Stenosis

Straightening

Upright

Cephalization

Mitral Stenosis

Convexityfromenlargedleft atrialappendage

Mitral Stenosis

Convexity fromenlarged leftatrial appendage

Imaging Findings of MSCardiac Findings

● Small aortic knob from decreased cardiacoutput

● Double density of left atrial enlargement

● Rarely, right atrial enlargement fromtricuspid insufficiency

RA

LALA

Small aorta from  cardiac output

“Double density”

Mitral stenosis/regurgitation withtricuspid regurgitation

Right atrialenlargementfromtricuspidregurgitation

Enlarged Latrialappendagefrom mitralstenosis

Mitral stenosis/regurgitation withtricuspid regurgitation

Right atrialenlargementfromtricuspidregurgitation

Huge Latrium

Imaging Findings of MSCalcifications

● Calcification of valve--not annulus--seen best on CT and at angio

● Rarely, calcification of left atrial wall 2°fibrosis from long-standing disease

● Rarely, calcification of pulmonaryarteries from PAH

Calcification of mitral valve

Calcification of pulmonary artery2° long-standing PAH

Calcification of left atrial wall

Ao

Mitral

Mitral Annulus Calcification

●Calcification of mitral annulus does notsignify presence of mitral valve disease

■Occurs in older women

■Usually asymptomatic

■Rarely  Mitral Stenosis

Casseous Calcification of theMitral Annulus

●Uncommon variant ofmitral annular calcification

●Located on posteriormitral leaflet

●Putty-like “toothpaste”material surrounded bycalcified shell

●Usually asymptomatic

Imaging Findings of MSPulmonary Findings

● Cephalization

● Elevation of left main bronchus(especially if 90° to trachea)

● Enlargement of main pulmonary artery2° pulmonary arterial hypertension

■ Severe, chronic disease

● Multiple small hemorrhages in lung

■ Pulmonary hemosiderosis

Upper lobevessels equalto or largerthan size oflower lobevessels =Cephalization

Upper lobevessels equalto or largerthan size oflower lobevessels =Cephalization

Elevation of left mainstembronchus 2°  LA

Elevation of left mainstembronchus 2°  LA

Posterior view of heart

Elevation of left mainstem bronchus 2°  LA

Elevation of left mainstem bronchus 2°  LA

Mitral Regurgitation

Enlarged MPAsegment fromseverepulmonaryarterialhypertension

Straighteningof left heartborder from LA

Mitral Stenosis with severe PAH

● Chronic edema ofalveolar walls fibrosis

■ Pulmonaryhemosiderindeposited in lungs

■ Pulmonaryossification mayoccur

Effect of Mitral StenosisOn Lungs

Mitral StenosisCine MR image in axial plane demonstrates a diastolic signalvoid emanating from the mitral valve

LA

LV

RA

RV

Congenital Mitral Stenosis

● Exists as isolated abnormality 25% of time

● Coexists with VSD 30% of time

● Coexists with another form of leftventricular outflow obstruction 40% oftime — SHONE’S Syndrome

■ Supravalvular mitral ring

LA Myxoma

● Most common form of 1° cardiac tumor

● Most common benign cardiac tumor

● 86% of myxomas found in left atrium

● 90% of myxomas are solitary

● Usually occur around fossa ovalis

● Soft tissue, not fat, density

● They can calcify (10%)

Left Atrial MyxomaContrast-enhanced CT shows large fillingdefect in lumen of LA

Left Atrial MyxomaCine MRI shows soft tissue mass arising from wallof left atrium and projecting into lumen

LA

RA

LV

Ao

Left atrial myxoma

MitralRegurgitation

MitralRegurgitation

Mitral RegurgitationCauses

●Thickening of valve leaflets 2° rheumatic disease

●Rupture of the chordae

■Posterior leaflet more often-Trauma, Marfan’s

●Papillary muscle rupture or dysfunction

■Acute myocardial infarction

●LV enlargement  dilatation of mitral annulus

■Any cause of LV enlargement

●LV aneurysm  valvular dysfunction

■Acute myocardial infarction

Mitral RegurgitationGeneral

●The acute lesion of rheumatic fever ismitral regurgitation, not stenosis

●The largest left atria ever are producedby mitral regurgitation, not mitralstenosis

Mitral RegurgitationImaging Findings

●In acute MR

■Pulmonary edema

■Heart is not enlarged

●In chronic MR

■LA and LV are markedly enlarged

●Volume overload

■Pulmonary vasculature is usually normal

●LA volume, but not pressure, is elevated

Mitral Regurgitation

Markedly enlargedheart

Large Leftatrium

Mitral Regurgitation

Mitral RegurgitationCine MR image in axial plane during systole depicts asignal void emanating from the mitral valve

LA

LV

Mitral Stenosis

Mitral Regurgitation

Difference in heart size – MS and MR

Pulmonic Stenosis

Pulmonic StenosisGeneral

● Without VSD = 8% of all CHD

● Mostly asymptomatic

● When symptomatic

■ Cyanosis and heart failure

■ Cor pulmonale

● Loud systolic ejection murmur

Pulmonic Stenosis3 Types

● Supravalvular

● Valvular

● Subvalvular

Supravalvular Pulmonic StenosisAssociated Syndromes

●Williams Syndrome

■ Hypercalcemia

■ Pulmonic Stenosis

■ Supravalvular AS

■ Peculiar facies

● Post-rubella syndrome

● Carcinoid syndrome with liver mets

● Ehlers-Danlos syndrome

Valvular Pulmonic StenosisGeneral

● Classic pulmonic stenosis (95%)

■ Congenital in origin

■ Associated with metastatic carcinoid syndrome

● Tricuspid regurgitation as well

■ Associated with Noonan Syndrome

● ASD

● Hypertrophic cardiomyopathy

Valvular Pulmonic StenosisImaging Findings

● Enlarged main pulmonary artery

● Enlarged left pulmonary artery(jet effect)

● Normal to decreased peripheralpulmonary vasculature

● Rare calcification of pulmonary valve inolder adults

Pulmonic Stenosis

Normal-sizedheart

Enlargementof leftpulmonaryartery

Enlargementof leftpulmonaryartery

Pulmonic Stenosis

Subvalvular Pulmonic StenosisAssociations

● Typically in Tetralogy of Fallot

■ 50% of pts with TOF also have bicuspidpulmonic valves

■ 50% of patients with TOF also have valvularpulmonic stenosis

● Subvalvular pulmonic stenosis

■ Also associated with VSD (85%)

Tetralogy of Fallot with subvalvular pulmonic stenosis

Concavepulmonaryarterysegment

Trilogy of Fallot

● Severe pulmonic valvular stenosis

● RV hypertrophy

● ASD with R  L shunt

Identifying the Locationof Valve Replacementsby Their Position andDirection of Flow

T-A-M

T

T

A

A

M

M

T

T

M

M

A

A

M-A-T

Location and Orientation of theValves of the Heart

Mitral Valve

Direction of Flow

Frontal View Usual Orientations

T

T

A

A

M

M

Frontal View-Mitral Valve

T

T

A

A

M

M

Frontal View-Aortic Valve

T

T

A

A

M

M

Frontal View-Tricuspid Valve

T

T

A

A

M

M

Lateral View View Usual Orientations

T

T

M

M

A

A

Lateral View View Usual Orientations

T

T

M

M

A

A

T

T

M

M

A

A

Lateral View-Mitral Valve

Lateral View-Aortic Valve

T

T

M

M

A

A

Lateral View-Tricuspid Valve

T

T

M

M

A

A

Tricuspid Valve

5841191

Mitral and Tricuspid Valves

Mitral and Aortic Valves

Pulmonic Valve

Mitral, Aortic and Tricuspid Valves

Aortic Valve with Sternal Dehiscence

9

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The End