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7/29/2019 Cyanotic Heart Dz

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Cyanotic HeartDisease

Cyanotic HeartDisease

William Herring, M.D. 2003

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Cyanosis With Decreased Vascularity

O Tetralogy

O Truncus-type IV

O Tricuspid atresia*O Transposition*

O Ebstein's

* Also appears on DDx of Cyanosis with Vascularity


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Cyanosis With Increased Vascularity

O Truncus types I, II, III

O TAPVR

O Tricuspid atresia*

O Transposition*

O Single ventricle

* Also appears on DDx of Cyanosis with Vascularity


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Tetralogy of FallotTetralogy of Fallot


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Tetralogy of FallotComponents

High VSD

Pulmonic stenosis, i.e. right

ventricular outflow obstruction Usually infundibular, sometimes

valvular

Overriding of the aorta

Right ventricular hypertrophy


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1

4

2

3

Frank Netter, MD Novartis

Tetralogy of Fallot


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Tetralogy of FallotOther anomalies

Right aortic arch in 25% Mirror image type

Left superior vena cava

ASD

Tricuspid valve abnormalities

Anomalies of coronary arteries Aberrant left anterior descending

coronary artery arising from right

coronary artery


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Tetralogy of FallotOther anomalies

Abnormalities of the pulmonary arteryand its branches

Peripheral PA coarctations, unilateral

Absence or hypoplasia of pulmonary artery

Usually left

Absence of pulmonic valve

Bicuspid pulmonic valve


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Tetralogy of FallotCritical Component

Degree of pulmonic stenosis

Regulates degree of R L shunt

Regulates overriding of aorta

Greater the stenosis, the greater the

aortic overriding


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Tetralogy of FallotClinical findings

Squatting

Dyspnea

Failure to thrive Cyanosis-usually

Severe cases at birth severe PS

Mild cases much later mild PS Pink tets (acyanotic) and Blue

tets (cyanotic)



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Tetralogy of FallotImaging Findings

Heart size normal Rarely enlarged

Cardiac apex displaced upward coeren sabot

PA segment concave

Decreased vasculature

R aortic arch in 25%


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Tetralogy of Fallot


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Tetralogy of Fallot


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Tetralogy of Fallot


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Trilogy of Fallot

Pulmonic valvular stenosis

ASD Right ventricular hypertrophy


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TruncusArteriosusTruncus

Arteriosus


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Truncus ArteriosusEmbryology

Uncommon anomaly 2failure ofprimitive common truncus arteriosus todivide into aorta and pulmonary artery


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Truncus ArteriosusGeneral

The truncal valve is usually tricuspid

Main pulmonary artery segment isconcave in types II, III, and IV

Pulmonary vasculature is shunt type intypes I, II and III


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Truncus ArteriosusRight sided aortic arch

Right-sided arch in about 33%

Usually mirror image type But because truncus is so rare, it

accounts for only 6% of all right

arches


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Truncus ArteriosusTriad

Right aortic arch Increased flow

Cyanosis


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Truncus ArteriosusAssociations

VSD

Always

Anomalies of the coronary arteries


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Truncus ArteriosusClinical Findings

Infants and small children demonstrateL R shunt

Minimal cyanosis

CHF

Respiratory infections

Growth disturbances

Majority are dead by 6 mos


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Truncus ArteriosusClinical Findings

z Cyanosis is worse in II and IIIz Cant tell them apart clinically

z Associated anomaliesz Bony

z Renal

z Lung

z Cleft palate


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Truncus ArteriosusX-ray Findings

Cardiomegaly

Right aortic arch (33%)

Concave pulmonary artery segment

Enlarged left atrium in 50%

Displacement of hilum Elevated right hilum in 20%

Left hilum in 10%


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Truncus-Type I

Convexpulmonaryartery

segment

Shunt

vessels

PA arises onleft via short

common stem

Most common (75%)

Ao


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LPA

Truncus

Ao

Ao

LV

LPARight Ao Arch

Truncus Type 1


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Truncus Type 1


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Truncus-Type II

Shunt vessels

Concave main

pulmonaryartery

Pulmonaryarteries ariseposteriorlyfrom aorta

Uncommon (25%)


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Truncus Type II


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Truncus-Type III

Shunt vessels

Concave mainpulmonaryartery

Right and leftpulmonaryarteries ariselaterally

Rare (5%)


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Truncus Type III


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Truncus-Type IV

Concave mainpulmonaryartery

Bronchialcirculation

No pulmonaryarteries

Rare to non-existent

TOF withpulmonary

atresia


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Truncus-Type IV(TOF with pulmonary atresia)


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Bronchial Circulation Increased flow


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I

III

II

IV


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Pulmonary artery Shunt vessels

I Convex Yes

II Concave Yes

III Concave Yes

IV ConcaveBronchialcirculation

Truncus Arteriosus


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Truncus ArteriosusAngiographic Findings

On angiogram, truncal valve (commonvalve) may have 2-6 cusps

Most often tricuspid

In lateral projection, plane of truncalvalve is distinctive

Tilts anteriorly toward patients toes Helpful to distinguish truncus from

aorticopulmonary window or TOF with pulmonic

atresia


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TricuspidAtresia

TricuspidAtresia


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Tricuspid Atresia

Fusion of dorsal and ventralendocardial cushions occurs too far to

the right obliteration of tricuspidvalve, and

Hypoplasia of right heart

Tricuspid valve, Right ventricle andpulmonary artery

T i id A i


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Tricuspid AtresiaShunts needed

Complete obstruction to outflow from RA Need R L shunt: Patent foramen ovale or ASD

Small ASD elevated RA pressures and

enlarged RA Large ASD lower RA pressures and minimal

enlargement of RA

Blood in L heart must get back to lungs Also have associated VSD or PDA

T i id At i


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Tricuspid AtresiaTransposition of Great Vessels

70% have normal relationships of great

vessels

30% have transposition of great arteries

T i id At i


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Tricuspid AtresiaTwo main types

No Transposition of the Great Arteries

Some degree of PS

Majority (70%)

Transposition of the Great Arteries

No pulmonic stenosis Minority (30%)


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Tricuspid atresiano transposition


N d R L h t


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Tricuspid atresiano transposition

Need R L shuntthrough patent

foramen ovale

Some

unsaturatedblood exitsaorta

Systemic

blood cannot enterRV

Blood

returns toright heartand thenlungs either

throughVSD or PDA

Systemic Need R L shunt


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Ao PA

Tricuspid atresiawith transposition

Systemicblood can

not enterRV

Need R L shuntthrough patent

foramen ovale

Un-oxygenatedblood flowsto lung via

transposedPA flow

Oxygenated

bloodreturns toLA

Need L Rshunt to getblood intobody

Tricuspid atresia


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Tricuspid atresiaX-ray Findings - No transposition

Normal-sized heart

Decreased pulmonary vessels (60-70%)

Flat/concave pulmonary artery

Small ASD enlarged RA

Large ASD normal or slightly enlargedRA


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Tricuspid atresiasome PS, no transposition

Tricuspid atresia


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Tricuspid atresiaX-ray Findings - Transposition

Mild cardiomegaly

Engorged pulmonary vessels

No characteristic appearance to heart


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Tricuspid atresiano PS, shunt vessels


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Transposition of

The Great Vessels

Transposition of

The Great Vessels

The TR Lesions


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The TR LesionsCyanosis with or vasculature

Tricuspid atresia or Transposition or

Truncus arteriosusType I, II, IIIType IV

Tetralogy

TAPVR

TrEbsteins


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The Rules

Since anatomic side (i.e. left orright) in complex lesions is frequentlyreversed or indeterminate

Naming conventions for Atria

AV valves

Ventricles

Ventricular outflow tracts

The Rules


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The RulesHow the atria are named

Anatomic right atrium is on the side

of the trilobed lung and liver

Trilobed lung=upper, middle and lower

The anatomic left atrium is on the

side of the bilobed lung and spleen Bilobed lung=upper and lower

The Rules


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The RulesMitral and tricuspid valves

The tricuspid valve belongs to theanatomic right ventricle

Not right atrium

The mitral valve belongs to the

anatomic left ventricle Not left atrium

The Rules


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The RulesHow the ventricles are named

The anatomic right ventricle is the

trabeculated ventricle The anatomic left ventricle is the

smooth-walled ventricle

The Rules


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The RulesAortic and pulmonic valves

The pulmonic valve is part ofpulmonary artery

Not anatomic right ventricle

The aortic valve is part of aorta

Not anatomic left ventricle

The pulmonic infundibulum is partof anatomic right ventricle


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Normal heart

AnatomicRight ventricleis trabeculated

AnatomicLeft ventricle is

smooth

Pulmonicinfundibulum

always stays withthe anatomic R

ventricle

Normal relationship of aortic to pulmonic valves


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Pulmonic valve is

Anterior

Lateral

Superior

To the aortic valve

PALS



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In Transposition, pulmonic valve is

PosteriorMedial

Inferior

To the aortic valve

Corrected TranspositionNormal

Corrected Transposition


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Corrected TranspositionInversion of the Ventricles With Transposition

Physiologically flow is normal

Consistent with normal life, except

Frequently associated with VSD

Tricuspid insufficiency

Subpulmonic stenosis

Complete heart block

AcyanoticNormal

vasculature;


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Corrected Transposition (L-Trans)Inversion of the Ventricles with Transposition of the

Great Vessels

Aorta arisesfrom pulmonicinfundibulum

PA arises fromanatomic left

ventricle

Acyanoticvasculature;

or increasedwith VSD


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Corrected Transposition Frank Netter, MD Novartis


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p

General

Second most common cause ofcyanosis in infancy

Pulmonary and systemiccirculations form two separatecircuits

Must be mixing between twocircuits for life



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p

Associated abnormalities

About 1/3 have VSD Larger the shunt, more likely CHF

About to have patent ductus Some have ASD

Other major finding is obstruction to

blood entering pulmonary artery Usually subpulmonic stenosis

Cyanoticwith

Obligatoryshunt since


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Transposition of the Great Vessels (D-Trans)

with

increasedvasculature

there are 2separate

circulations

Aorta arisesfrom pulmonicinfundibulum

PA arises fromanatomic left

ventricle

ASD

VSD

PDA



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X-ray findings

Mild cardiomegaly

Concave pulmonary artery segment

Narrow mediastinum (Egg-on-string)

Shunt vessels

Depends on size of shunt and degree ofPS


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Transposition of the Great Vessels

Cyanotic with - vasculature


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Transposition of the Great Vessels -RVgram


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Transposition of the Great Vessels -LVgram


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LVgram with VSD butnormal Ao and PA

relationships

Corrected TranspositionWith VSD

Narrow waist inTransposition

Cyanotic


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Inversion of the VentriclesWithout Transposition


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Ebsteins AnomalyEbsteins Anomaly

Ebsteins Anomaly


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General

Rare Posterior and septal cusps of tricuspid valvedisplaced in to right ventricle

Right ventricle smaller or atrialized Tricuspid insufficiency right atrialpressure a R L shunt through foramen

ovale (or ASD)Cyanosis is present in neonate


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Ebsteins Anomaly


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Normal


Ebsteins Anomaly

Ebsteins Anomaly


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X-ray Findings

Cardiomegaly

One of few conditions cardiomegaly

first few days of life Unusual prominence to right heart border

Pulmonary flow is decreased

EbsteinsEbsteins


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AnomalyAnomaly

EbsteinsEbsteins


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AnomalyAnomaly

Ebsteins Anomaly


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Triad

Decreased flow

Cyanosis

MarkedCardiomegaly


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Single VentricleSingle Ventricle

Single Ventricle


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Surprise! There are usually two ventricles in this disease

Single ventricle: one ventricle with two atria

Three types of Single Ventricle

Morphologic LV with a rudimentary RV (common)

Morphologic RV with a rudimentary LV (rare) Morphologically indeterminate ventricle (rare)

Single Ventricle


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Most common Morphologic LV with rudimentary RV

Also called

Double-inlet left ventricle

Common ventricle

Univentricular heart

Frequently difficult to determinewhich anatomic ventricle is present

Single Ventricle


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Associated Findings

Pulmonic stenosis

Valvular or subvalvular (66%)

Pulmonary atresia

PAPVR

PDA


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Single Ventricle

I i Fi di


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Imaging Findings

No characteristic appearance

Concave pulmonary artery segment Shunt vessels


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Single ventricle


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The End