Congenital Heart Diseases PDF

8/9/2019 Congenital Heart Diseases PDF

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RADIOLOGIC INTERPRETATION OFRADIOLOGIC INTERPRETATION OF

CONGENITAL HEART DISEASECONGENITAL HEART DISEASE

1. Vascularity1. Vascularity

2. Cyanotic or Non-cyanotic?2. Cyanotic or Non-cyanotic?

3. Specific chamber enlargement3. Specific chamber enlargement

4. Great Vessels4. Great Vessels 5. Ancillary findings5. Ancillary findings



NON-CYANOTIC CYANOTIC

•ASDASD

•VSDVSD

•PDAPDA

•PAPVRPAPVR

• TGA TGA

• TAPVR w/o obstruction TAPVR w/o obstruction

•Complete endocardialComplete endocardial

cushion defectcushion defect

•PTAPTA

•Single ventricleSingle ventricle

•DORV w/o pulmonary stenosisDORV w/o pulmonary stenosis

Swischuk 3rd edition



• Tetralogy of Fallot Tetralogy of Fallot

•Ebstein’s anomalyEbstein’s anomaly

•Uhl’s DiseaseUhl’s Disease

•Hypoplastic Right Heart SyndromeHypoplastic Right Heart Syndrome

• Trilogy of Fallot Trilogy of Fallot



•Coarctation of aortaCoarctation of aorta

•Aortic insufficiencyAortic insufficiency

•Mitral stenosisMitral stenosis

•Mitral insufficiencyMitral insufficiency

•Pulmonary stenosisPulmonary stenosis

•Pulmonary insufficiencyPulmonary insufficiency

• Tricuspid insufficiency Tricuspid insufficiency

•Cor triatriatumCor triatriatum



INCREASEDINCREASEDPULMONARYPULMONARY

VASCULARITYVASCULARITY

CYANOTICCYANOTIC



Total Anomalous Pulmonary Total Anomalous Pulmonary

Venous ReturnVenous Return

1.5% of all congenital cardiac defect1.5% of all congenital cardiac defect Pulmonary veins connect to systemicPulmonary veins connect to systemic

veins or the RA rather than to the LA.veins or the RA rather than to the LA. TAPVC occurs when all PV connect TAPVC occurs when all PV connect

anomalouslyanomalously Anomalous venous return maybeAnomalous venous return maybe

obstructed or non obstructed.obstructed or non obstructed.





Pulmonary venous return + systemicvenous return

Right side of theheart



PA

RA LA

RV LV

Systemicveins

Lungs

Aorta To systemic circulation

PV



Total Anomalous PulmonaryTotal Anomalous Pulmonary





Venous ReturnVenous Return Increased vascularityIncreased vascularity

CardiomegalyCardiomegaly

Chamber prominence:Chamber prominence:

right atriumright atrium right ventricleright ventricle

Enlarged systemic veinEnlarged systemic vein

into which drainageinto which drainageoccursoccurs




Venous Return ( Type I )Venous Return ( Type I )

http://radiographics.rsnajnls.org/cgi/content/full/27/5/1323/F2D

http://radiographics.rsnajnls.org/cgi/content/full/27/5/1323/F2C





Type I (Supracardiac) Type I (Supracardiac)

left-sided vertical veinleft-sided vertical veinconnects pulmonaryconnects pulmonary

venous confluence to thevenous confluence to theleft innominate vein,left innominate vein,right SVC or azygos veinright SVC or azygos vein

““Snowman appearance”Snowman appearance” 50%50%





Type II (Intracardiac) Type II (Intracardiac)

connections to theconnections to the

right atrium orright atrium orcoronary sinuscoronary sinus

30%30%





Type III (Infra Type III (InfraDIAPHRAGMATICDIAPHRAGMATIC)) Connection is below theConnection is below the

diaphragm; to the portal vein,diaphragm; to the portal vein,ductus venosus or hepatic veinductus venosus or hepatic vein

Pulmonary edemaPulmonary edema Normal sized heartNormal sized heart Prominence of the right atrium &Prominence of the right atrium &

less often the right ventricleless often the right ventricle 15%15%





Type III (Infracardiac) Type III (Infracardiac) Connection is below theConnection is below the

diaphragm; to the portal vein,diaphragm; to the portal vein,ductus venosus or hepatic veinductus venosus or hepatic vein

Pulmonary edemaPulmonary edema Normal sized heartNormal sized heart Prominence of the right atrium &Prominence of the right atrium &

less often the right ventricleless often the right ventricle 15%15%





Mixed typeMixed type

with variouswith various

connections to theconnections to theright side of theright side of the

heartheart



Scimitar SydromeScimitar Sydrome

an anomalous pulmonary vein (an anomalous pulmonary vein (scimitar vein)scimitar vein) that drains any or all of the lobes of the rightthat drains any or all of the lobes of the rightlung.lung.

Band-like shadow adjacent to the right heartBand-like shadow adjacent to the right heartborder on chest x-rayborder on chest x-ray (hallmark)(hallmark)

Hypogenetic lungHypogenetic lung

Decreased caliber of the pulmonary artery Decreased caliber of the pulmonary artery

Cooley 3rd edition



Scimitar SyndromeScimitar Syndrome

http://radiographics.rsnajnls.org/content/vol27/issue5/images/large/g07se15c03b.jpeg

http://radiographics.rsnajnls.org/content/vol27/issue5/images/large/g07se15g03a.jpeg




Persistent TruncusPersistent Truncus

ArteriosusArteriosus

FAILURE OF DIVISION OFFAILURE OF DIVISION OF THE TRUNCUS THE TRUNCUSARTERIOSUS INTO THEARTERIOSUS INTO THEAORTA AND PULMONARYAORTA AND PULMONARYARTERYARTERY

A SINGLE VESSEL DRAINSA SINGLE VESSEL DRAINSBOTH VENTRICLES ANDBOTH VENTRICLES ANDSUPPLIES THE SYSTEMIC,SUPPLIES THE SYSTEMIC,PULMONARY, CORONARYPULMONARY, CORONARYCIRCULATIONCIRCULATION

HIGH VENTRICULARHIGH VENTRICULAR

SEPTAL DEFECTSEPTAL DEFECT





Pulmonary is concavePulmonary is concave Dilatation of the aortaDilatation of the aorta

(truncus)(truncus)

wide mediastinum due towide mediastinum due tolarge “aortic shadow”large “aortic shadow”

High position of theHigh position of the

transverse arch of the aortatransverse arch of the aorta

Elevation of the leftElevation of the left

pulmonary arterypulmonary artery right aortic arch (in 35%)right aortic arch (in 35%)

i





Increased vascularityIncreased vascularity Pulmonary venous congestionPulmonary venous congestion

or edema is frequent in Type Ior edema is frequent in Type I CardiomegalyCardiomegaly Chamber prominence:Chamber prominence:

either or both ventricleseither or both ventricles left atriumleft atrium

Concave main pulmonaryConcave main pulmonaryartery segment (Prominent inartery segment (Prominent in Type I) Type I)



Types of Persistent TruncusTypes of Persistent Truncus


1 – the ascending aorta and a single pulmonary trunk arise1 – the ascending aorta and a single pulmonary trunk arisefrom the truncusfrom the truncus

2 – the right and left pulmonary arteries arise close2 – the right and left pulmonary arteries arise closetogether from the dorsal wall of the truncus (posterior)together from the dorsal wall of the truncus (posterior)

3 – one or both pulmonary arteries arise independently3 – one or both pulmonary arteries arise independentlyfrom either side of the truncus (lateral origin )from either side of the truncus (lateral origin )

4 – absence of the pulmonary arteries, circulation to the4 – absence of the pulmonary arteries, circulation to thelungs by means of the bronchial arteries.lungs by means of the bronchial arteries.

COOLEY 3RD



Persistent Truncus Arteriosus




ArteriosusArteriosus ““Indeed, persistent truncus is the onlyIndeed, persistent truncus is the only

condition in which trulycondition in which truly concaveconcave

pulmonary artery pulmonary artery along with generallyalong with generally

increased pulmonary vascularity increased pulmonary vascularity isisseen.”seen.”

Swischuk 3rd

edition



Most common CHD presenting withMost common CHD presenting with

cyanosis in the first 24 hours of lifecyanosis in the first 24 hours of life

Transposition of the Great Transposition of the Great

ArteriesArteries




ArteriesArteries

Aorta originates from the rightventricle

PA originates from the LV

Normal position of atria and

ventricles 2 independent/ closed circulations

incompatible with life unless withassociated anomalies that permitmixing of the two circulations ( ASD,VSD, PDA)

http://radiographics.rsnajnls.org/cgi/content/full/27/5/1323/F1D



HemodynamicsHemodynamics




Arteries (d-TGA)Arteries (d-TGA)

Increased vascularityIncreased vascularity


Cardiac silhouette:Cardiac silhouette:

““Egg on its side”Egg on its side” ““Apple on a stem”Apple on a stem”

Narrow vascularNarrow vascular

pediclepedicle PA – FLATPA – FLAT AORTA - ANTERIORAORTA - ANTERIOR




Arteries (TGA)Arteries (TGA)



TRICUSPID ATRESIATRICUSPID ATRESIA

(Absent Right AV connection)(Absent Right AV connection)



TRICUSPID ATRESIATRICUSPID ATRESIA

Decreased pulmonaryDecreased pulmonary

vascularityvascularity

Flat or concaveFlat or concavepulmonary arterypulmonary artery

Small asd – r atrial eSmall asd – r atrial e

Large asd – r a (min orLarge asd – r a (min or

normal in size)normal in size)



Systemicveins

PA

RA LA

RV LV

PV

Lungs

Aorta

To systemic circulation

Type I Tricuspid Atresia



AssociationsAssociations

Patent foramen ovale or ASD are alwaysPatent foramen ovale or ASD are always

presentpresent

Complete transposition of great arteries,Complete transposition of great arteries,

35%35% VSD, commonVSD, common

Pulmonary atresiaPulmonary atresia

Hypoplastic right heartHypoplastic right heart Extracardiac anomaliesExtracardiac anomalies

(GIT, bones )(GIT, bones )



Radiographic featuresRadiographic features

Maybe normalMaybe normal No TGANo TGA

similar as tetralogy of Fallotsimilar as tetralogy of Fallot With TGAWith TGA

increased pulmonary blood flowincreased pulmonary blood flow

cardiomegalycardiomegaly

narrow vascular pediclenarrow vascular pedicle



Pulmonary AtresiaPulmonary Atresia

Congenital absence of the pulmonaryCongenital absence of the pulmonaryartery.artery.

Atretic pulmonary valve withAtretic pulmonary valve with

underdeveloped pulmonary artery distally.underdeveloped pulmonary artery distally. Maybe associated with hypogenetic lung.Maybe associated with hypogenetic lung. Passive congestionPassive congestion



INCREASEDINCREASEDPULMONARYPULMONARY

VASCULARITYVASCULARITY

Non- CYANOTICNon- CYANOTIC



Atrial Septal DefectAtrial Septal Defect

Increased vascularityIncreased vascularity


Chamber prominence:Chamber prominence: right atriumright atrium

right ventricleright ventricle

Enlarged main andEnlarged main and

central pulmonarycentral pulmonary

arteriesarteries

Small aortic knobSmall aortic knob



Anatomical Classification of ASDAnatomical Classification of ASD

OSTIUM SECUNDUM defect – OSTIUM SECUNDUM defect – most commonmost common

Majority adjacent to or involve the foramen ovaleMajority adjacent to or involve the foramen ovale

Foramen ovale defectsForamen ovale defects SINUS VENOSUS defectSINUS VENOSUS defect

FREQUENT association withFREQUENT association with partial anomalous venous partial anomalous venous

returnreturn

Located in the inflow portion of the atria that is near theLocated in the inflow portion of the atria that is near the

insertion of either the superior or inferior vena cavainsertion of either the superior or inferior vena cava



Anatomical Classification of ASDAnatomical Classification of ASD

OSTIUM PRIMUM defect – OSTIUM PRIMUM defect – Lowermost portion of the septum in the region of Lowermost portion of the septum in the region of

the primitive ostium primumthe primitive ostium primum

COMMON ATRIUMCOMMON ATRIUM Persistent atrioventricular canalPersistent atrioventricular canal

Gooseneck deformity of the left ventricular canalGooseneck deformity of the left ventricular canal



Ventricular SeptalVentricular Septal

DefectDefect

DEFECT IN THE MUSCULAR ORDEFECT IN THE MUSCULAR OR

MEMBRANOUS PORTION OF THEMEMBRANOUS PORTION OF THE

SEPTUMSEPTUM



Ventricular Septal DefectVentricular Septal Defect





DefectDefect Increased vascularityIncreased vascularity

Normal or enlarged cardiacNormal or enlarged cardiac

sizesize


either or both ventricleseither or both ventricles left atriumleft atrium

Enlarged main and centralEnlarged main and central

pulmonary arteriespulmonary arteries

AORTA IS NORMAL OR LARGERAORTA IS NORMAL OR LARGER



Patent DuctusPatent Ductus


WHEN THE DUCTUSWHEN THE DUCTUS

ARTERIOSUS FAILS TOARTERIOSUS FAILS TO

CLOSECLOSE THERE IS THERE IS

COMMUNCATIONCOMMUNCATION

BETWEEN THE GREATBETWEEN THE GREAT

VESSELS PERSISTSVESSELS PERSISTS



Patent Ductus ArteriosusPatent Ductus Arteriosus



Patent DuctusPatent Ductus

ArteriosusArteriosus Increased vascularityIncreased vascularity

Normal on enlargedNormal on enlarged

cardiac sizecardiac size


left ventricleleft ventricle

left atriumleft atrium

Enlarged main andEnlarged main and

central pulmonarycentral pulmonary

arteriesarteries

Prominent aortic knobProminent aortic knob



Decreased PulmonaryDecreased Pulmonary

VascularityVascularity



most common cyanoticmost common cyanotic

congenital heart disease of congenital heart disease of childhoodchildhood

Tetralogy of Fallot



Tetralogy of FallotTetralogy of Fallot

CONSISTS OF THEFOLLOWING:

Tetrad

1. Pulmonic stenosis

2. Interventricular septaldefect

3. Aorta overriding theventricular septum withdextroposition

4. Hypertrophy of the rightventricle.




ra

RA LA

RV

L V

P

V

Lungs

Aorta

PA STENOSIS

Pulmonic stenosis

Increased pressure inthe right ventricle

• septal defect

Right to left shunt

Cyanosis

TETRALOGY OF FALLOT



Tetralogy of Fallot

Decreased vascularityDecreased vascularity Normal or enlargedNormal or enlarged

cardiac sizecardiac size right ventricularright ventricular

prominenceprominence

Concave main pulmonaryConcave main pulmonaryartery segmentartery segment

Prominent aortaProminent aorta right aortic archright aortic arch (in 20-25%)(in 20-25%) Boot shaped heartBoot shaped heart ( enlarged RV ):( enlarged RV ): ceour en sabotceour en sabot

http://radiographics.rsnajnls.org/cgi/content/full/27/5/1323/F5B



Clinical FindingsClinical Findings

Squatting when fatiguedSquatting when fatigued

Episodic loss of consciousnessEpisodic loss of consciousness Cyanosis by 3 – 4 monthsCyanosis by 3 – 4 months

b i l



Ebstein’s AnomalyEbstein’s Anomaly

Decreased vascularityDecreased vascularity

Pulmonary arteryPulmonary artery

segment is flatsegment is flat

Marked cardiomegalyMarked cardiomegaly

right atrialright atrial


““squared-” orsquared-” or

““box-shaped”box-shaped”

http://radiographics.rsnajnls.org/cgi/content/full/27/5/1323/F9B

http://radiographics.rsnajnls.org/cgi/content/full/27/5/1323/F9A



Ebstein’s AnomalyEbstein’s Anomaly

Downward displacement of theDownward displacement of the

septal, frequently, the posteriorseptal, frequently, the posterior

leaflets of the tricuspid valve.leaflets of the tricuspid valve.

Right ventricle is functionally andRight ventricle is functionally and

anatomically incorporated into theanatomically incorporated into the

right atriumright atrium



Displaced leaflet

Septal defect

EBSTEIN’S ANOMALY



PA

RA LA

RV LV

PV

Lungs

Aorta To systemic circulation

EBSTEIN’S ANOMALY



AssociationsAssociations

Maternal lithium intakeMaternal lithium intake

Patent foramen ovale or ASD, 80%Patent foramen ovale or ASD, 80%

cyanosis



ClinicalClinical

Tricuspid regurgitation Tricuspid regurgitation

ArrythmiasArrythmias

50% mortality in the 150% mortality in the 1stst

yearyear



UHL’S DISEASEUHL’S DISEASE

RARE CONDITIONRARE CONDITION

FOCAL OR COMPLETE ABSENCE OFFOCAL OR COMPLETE ABSENCE OF

THE RIGHT VENTRICULAR THE RIGHT VENTRICULAR

MYOCARDIUMMYOCARDIUM THE RIGHT VENTRICLE BECOMES THE RIGHT VENTRICLE BECOMES

THINWALLED, FIBROELASTIC BAG THINWALLED, FIBROELASTIC BAG



TRILOGY OF FALLOTTRILOGY OF FALLOT

1.1. PULMONARY STENOSIS WITHPULMONARY STENOSIS WITH

2.2. INTACT VENTRICULAR SEPTUMINTACT VENTRICULAR SEPTUM

3.3.

RIGHT-TO-LEFT ATRIAL SHUNTRIGHT-TO-LEFT ATRIAL SHUNT

(THRU A PATENT FORAMEN(THRU A PATENT FORAMEN

OVALE OR TRUE ATRIAL SEPTALOVALE OR TRUE ATRIAL SEPTAL

DEFECT)DEFECT)



ROENTGENOGRAPHICALLYROENTGENOGRAPHICALLY(CHARACTERISTIC):(CHARACTERISTIC): PULMONARY VASCULARITY DIMINISHEDPULMONARY VASCULARITY DIMINISHED

POSTSTENOTIC DILATATION OFPOSTSTENOTIC DILATATION OFPULMONARY ARTERYPULMONARY ARTERY

NORMAL HEART SIZE INITIALLY, BUT INNORMAL HEART SIZE INITIALLY, BUT IN TIME, RIGHT ATRIAL AND RIGHT TIME, RIGHT ATRIAL AND RIGHT

VENTRICULAR ENLARGEMENT DEVELOPVENTRICULAR ENLARGEMENT DEVELOP

TRILOGY OF FALLOTTRILOGY OF FALLOT



Normal VascularityNormal Vascularity



Aortic StenosisAortic Stenosis

Normal vascularityNormal vascularity


Left ventricularLeft ventricular


Dilated ascendingDilated ascending

aortaaorta



Aortic StenosisAortic Stenosis

Normal vascularity Normal vascularity


Left ventricular prominenceLeft ventricular prominence

Dilated ascending aortaDilated ascending aorta



Coarctation of the AortaCoarctation of the Aorta

Normal vascularityNormal vascularity


Left ventricularLeft ventricularprominenceprominence

““3” sign3” sign

Rib notchingRib notching



occurs with increased frequency amongoccurs with increased frequency among

patients with Turner syndrome, 20%–36%patients with Turner syndrome, 20%–36%

of whom are affected.of whom are affected.

NumberNumber 33 is formed by dilatation of theis formed by dilatation of theleft subclavian artery and aorta proximalleft subclavian artery and aorta proximal

to the site of coarctation, indentation of to the site of coarctation, indentation of

the site, and dilatation of the aorta distalthe site, and dilatation of the aorta distal

to the siteto the site





http://radiographics.rsnajnls.org/content/vol27/issue5/images/large/g07se15c07x.jpeg

http://radiographics.rsnajnls.org/content/vol27/issue5/images/large/g07se15g06a.jpeg



Localized (postductal or adult-type)aortic coarctation. Drawingshows a

(1)focal constriction of the aorta ( (2) just beyond the origin of the left

subclavian artery(3) and the ligamentum arteriosum(4)left common carotid artery(5) innominate artery,(6)right heart structures,(7)left heart structures,(8)pulmonary artery

The contour of the aorta isdeformed by both pre- andpoststenotic dilatation, and theleft subclavian artery is dilated.

http://radiographics.rsnajnls.org/content/vol27/issue5/images/large/g07se15c07x.jpeg




•Rib notching

•dilated and tortuous intercostal vesselsform deep grooves on the undersurfaces of the ribs (a process known as rib notching)

•fourth through the eighth ribs

MITRAL STENOSISMITRAL STENOSIS




Normal to slightlyNormal to slightlyenlarged heartenlarged heart Chamber prominence:Chamber prominence:

left atriumleft atrium right ventricleright ventricle

Equalization orEqualization orcephalization of cephalization of pulmonary blood flowpulmonary blood flow

Prominent mainProminent main

pulmonary arterypulmonary arterysegmentsegment

Small aortaSmall aorta




Normal to slightlyNormal to slightlyenlarged heartenlarged heart


left atriumleft atrium right ventricleright ventricle

Equalization orEqualization orcephalization of cephalization of pulmonary blood flowpulmonary blood flow

Prominent mainProminent mainpulmonary arterypulmonary arterysegmentsegment


MITRALMITRAL



MITRALMITRAL

REGURGITATIONREGURGITATION


Chamber Prominence:Chamber Prominence: left atrium left ventricleleft atrium left ventricle

Pulmonary venousPulmonary venous

congestioncongestion




AORTIC STENOSISAORTIC STENOSIS

Normal-sized heart orNormal-sized heart or

mild cardiomegalymild cardiomegaly


hypertrophyhypertrophy +/- pulmonary venous+/- pulmonary venous

hypertensionhypertension

Dilated ascending aortaDilated ascending aorta

AORTICAORTIC



AORTICAORTIC

REGURGITATIONREGURGITATION



enlargementenlargement Dilated ascendingDilated ascending

aorta and aortic archaorta and aortic arch

Normal pulmonaryNormal pulmonary

vascularityvascularity

l i



Pulmonary StenosisPulmonary Stenosis

Common anomalyCommon anomaly Isolated form or combinationIsolated form or combination

Pulmonary valve cusps fuse to form aPulmonary valve cusps fuse to form a

diaphragm with an orifice of variablediaphragm with an orifice of variable

sizesize

l i




Normal to decreasedNormal to decreasedvascularityvascularity

Normal or enlargedNormal or enlarged

cardiac sizecardiac size

right ventricularright ventricularprominenceprominence

Post-stenotic dilatationPost-stenotic dilatation

of the main pulmonaryof the main pulmonary

arteryartery




Normal to decreasedNormal to decreasedvascularityvascularity

Normal or enlarged cardiacNormal or enlarged cardiac

sizesize right ventricular prominenceright ventricular prominence

Post-stenotic dilatation of thePost-stenotic dilatation of the

main pulmonary arterymain pulmonary artery

C i i



Cor TriatriatumCor Triatriatum

RareRare

The pulmonary veins empty into a commonThe pulmonary veins empty into a common

vein, which is abnormally incorporated intovein, which is abnormally incorporated into

the left atrium.the left atrium.

A partial membrane creates an extra chamber A partial membrane creates an extra chamber

along the superior and dorsal aspect of the LAalong the superior and dorsal aspect of the LA

and variably obstructs venous emptying intoand variably obstructs venous emptying intothe LA.the LA.

TRICUSPID VALVETRICUSPID VALVE



TRICUSPID VALVETRICUSPID VALVE

DISEASEDISEASE

Right atrialRight atrial

enlargementenlargement +/- SVC or IVC+/- SVC or IVC




IN SUMMARYIN SUMMARY

IncreaseIncreasePULMONARYPULMONARYOUTFLOW TRACTOUTFLOW TRACTOUTPUTOUTPUT

DecreasePULMONARYOUTFLOW TRACTOUTPUT



Left to rightshunt

Right to leftshunt

Congenital Heart Diseases PDF

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