IMAGING OF CONGENITAL HEART DISEASE
CLINICAL HISTORY
Newborn
Infant
Child
Cyanosis
Surgical history
Family history
Multiorgan systems
ANATOMY
Pathologists view- AP view aligned with septum
Radiologists view- 45 degrees off orthogonal planes
KEY ANATOMIC FEATURES RIGHT ATRIUM
v
Appendage- broad based,
dog ear configuration
KEY ANATOMIC FEATURES RIGHT VENTRICLE
Inflow portion- posterior
Outflow portion-infundibulum, anterior
separated from inlet portion by crista supraventricularis
Apical portion- heavily trabeculated
KEY ANATOMIC FEATURES LEFT ATRIUM
Appendage narrow, elongated
KEY ANATOMIC FEATURES LEFT VENTRICLE
Fibrous continuity between:
Mitral valve anterior leaflet and aortic valve
(Mitral valve- miter double peaked headgear worn by Catholic Bishops.)
KEY ANATOMIC FEATURES AORTIC VALVE
Right cusp
Left cusp
Posterior(noncoronary) cusp
Posterior and left cusps form fibrous attachment to the MV
30 degree tilt from vertical:
posterior tilt normal young children(exaggerated in TGV)
anterior tilt older persons(exaggerated in DORV)
KEY ANATOMIC FEATURES CORONARY ARTERIES
LCA- circumflex and LAD
RCA- is a circumflex vessel
Crux- junction of AV and posterior interventricular grooves
Dominance- origin of the posterior descending artery
KEY ANATOMIC FEATURES CORONARY VEINS
Left system drainage- coronary sinus
Right system- Thebesian veins
CINE ANGIOGRAPHY
Reduced motion unsharpness- 1-5 msec recording time
Resolution- 3.5 line pairs/mm
High visual contrast sensitivity- 60 fps
CINE ANGIOGRAPHY
Grid controlled X ray tubes- intermittent (-) bias
Stops e- flux to anode
CINE ANGIOGRAPHY
35 mm film conventional motion picture camera
16 mcR/ frame minimum exposure to prevent quantum mottle
U.S.- 60/30/15 fps, 50 fps Europe
CINE ANGIOGRAPHY
Biplane capabilty
Single injection of contrast material
Tubes must fire out of phase to prevent cross fogging
CINE ANGIOGRAPHY
Angulation- relative to position of the image intensifier
Cranial-looking down
Caudal-looking up
CINE ANGIOGRAPHY
Hepatoclavicular or 4 chamber view-
20-30 deg LAO, 30 deg cranial
Septum- 60-70 deg LAO, 20-30 deg cranial
Main PA- AP, 45 deg sitting up
Branch PAs- 30 deg RAO, 30 deg cranial
CARDIAC MRI
Inflow enhancement- white blood, unsaturated protons entering the imaging volume
High velocity signal loss- black blood, protons rapidly move through the
imaging volume and do not receive the full 90 and 180 degree RFpulses
.
CARDIAC MRI
Velocity required for black blood= slice thickness/0.5*TE
180 degree pulse occurs at midportion of TE
CARDIAC MRI
Anatomic imaging ECG-gated multislice technique
TE=20-60 msec
TR=R-R interval
# slices obtained=TR(or R-R)/TE
CARDIAC MRI
Cine studies
Gradient echo-white blood, turbulence decreased signal
TR=20-30 msec, TE=4-17 msec
16-30 images...cine display
Plain Film Approach CHD
Chest X-ray
Skeleton
Situs
Apex
Cardiac size/ configuration
Ao arch/descending Ao
Pulmonary vessels
Lungs/Pleura
Skeleton
Ribs- number, postop change
Sternum- Manubrial ossification centers
hypersegmentation-80% Downs(20%) normals
sternotomy
Spine- VATER
Situs
Solitus- 0.6-0.8% CHD
Situs
Inversus- 0.01%
3-5% CHD, often l-TGV
Reversal sides with concordance of structures
Ambiguous- 95-98% CHD, cardiosplenic syndromes
Cardiac Position
Apex:
right-dextrocardia
incomplete-mesocardia
left-levocardia
Dextrocardia
Dextroposition, extrinsic factors or Scimitar
Dextroversion-nearly 100% CHD, 1/29,000
TGV, d- or l-
Dextroversion
With s. inversus
mirror image dextrocardia
1/10,000
SITUS
Solitus
Inversus
Ambiguous
Cardiac Malposition
Amplatz
1. R/O extrinsic factors
2. catheter position
3. Stomach/Ao Right= S.inversus
4. Stomach/Ao Left, apex right=dextroversion
Cardiac Malposition
Amplatz
5. Stomach/Ao Right, apex left=ambiguous or S.inv/levoversion
6. Stomach Right/Ao Left=ambiguous
7. midline liver or prominent azygous=ambiguous
Cardiosplenic Syndromes
Cardiosplenic Syndromes
Dextroversion
With s. inversus
mirror image dextrocardia
1/10,000
Cardiac Chamber Enlargement
RA-AP
RV- clockwise rotation, elevation of apex
LA-normal right border 30%, splaying carina
LV-posterior IVC border, apex depressed
Aortic Arch
Side of tracheal deviation
Descending Ao
Right Aortic Arch
Truncus arteriosus- 31%
Tetralogy- 14-34%
Pulmonary atresia/VSD- 30%
DORV- 20%
TGV- 3-5%
Tricuspid atresia- 3-5%
Aortic Arch
Size increased in tetralogy, ascending
Descending enlarged in PDA, AP window,
Coarctation
Pulmonary Arteries
Pulmonary artery diameter =bronchial diameter
Right descending PA (interlobar =trachea at Ao knob)
Shunt vascularity (sharp margins)
Pulmonary Hypertension
Prominent central PA
Pruning
Tortuosity
Ca++
Bronchial Systemic Collaterals
Bizarre pattern of hilar vessels
Lack of definable central Hilar vessels
Assymetry
Main Pulmonary Artery
Straight or convex left upper cardiac border
concave- decreased flow or TGV
Prominent- L to R shunt, Pulmonary Stenosis
Pulmonary Veins
Pulmonary venous hypertension-passive congestion
Hazy vessel margins
Subpleural thickening /fissures
Septal lines
Classification Scheme
Overload,pressure-stenosis/ obstructions
Volume-shunts/valvar incompetence
myocardial dysfunction
Classification
Functional(Clinical, Radiologic, Physiologic)
Acyanotic-normal or increased vascularity
Cyanotic-increased or decreased vascularity
Venous hypertension
Cyanosis
*arterial O2 saturation less than 94%Physiologic
less than 85% visible
* assume normal HCT
anemia (less than 8 gm Hb) less than 60%
Acyanotic/ increased vascularity
VSD
ASD
PDA
AVC/endocardial cushion
Acyanotic/increased Vascularity
AP window
PAPVR
LV to RA
Anomalous LCA to PA
VSD
membranous-70-80%
muscular-10%
conal-5% DORV type II
posterior-5-10% endocardial cushion
VSD Radiology
Increased vascularity = 2:1 or greater shunt
Cardiomegaly, enlarged LA
VSD Prognosis
30-40% close spontaneously
CHF at 2-3 months
Surgery, Closure-RA approach( membranous)
Ventriculotomy (muscular)
ASD
PFO
Secundum type-60%
Primum, AV canal-35%
Sinus venosis type-5% (RUL PV to SVC)
Radiology-Secundum ASD
Increased pulmonary vascularity
Increased main PA
normal LA
ASD History
Repair age 4 to 5 years
Most common cause of secondary pulmonary hypertension(undiagnosed)
PDA
Orgin LPA to promixally descending Ao
Rx indomethacin-closure
PGE-dilates
lifeline in cyanosis
PDA Radiology
Chest X-ray-normal
Ca++ ligamentum arteriosum
Chest X-ray-increased vascularity, increased aorta size
surgery closure or coil embolization
AV Canal
synonymous with endocardial cushion defect
synonymous with atrioventricular septal defect
40% Downs (65% of trisomy 21)
Downs-33% AVC, 40% VSD
Anatomy
Primum ASD
Posterior VSD
Cleft anterior MV leaflet/ septal leaflet TV
Radiology AV Canal
Large shunt vascualarity
Cardiomegaly
Skeletal changes
left atrial enlargement=mitral regurgitation
AV Canal
ECG- left axis deviation
First degree AV block
AV Canal
Angiography
LV gram, AP projection
Gooseneck deformity
Subaortic region, prolaspe anterior MV leaflet
Elevation of LV by enlarged RV
Cyanosis with decreased vascularity
Tetrad physiology
Pulmonary atresia with intact septum
hypoplastic RV
Tricuspid atresia
Ebstein anomaly
Radiology of decreased pulmonary vascularity
Main PA concave-tetralogy, pulmonary atresia
Cardiomegaly-Ebstein, pulmonary atresia with intact spetum
Normal heart size-
Tricuspid atresia
complex disease with pulmonary atresia
Tetralogy of Fallot
VSD
Ao
RVH
Infundibular pulmonary stenosis
Stensen (Danish anatomist, 1671)
Fallot (French physican, 1888)
Tetralogy of Fallot
Pathologic Anatomy
Most common cyanotic lesion
8% of all congential heart disease
Tetralogy of Fallot
Pathologic Anatomy
Van Praagh-hallmark infundibular stenosis
25% right aortic arch
VSD membranous/muscular beneath crista
PV anomalies bi/unicommissural, hypoplastic annulus
Tetralogy of Fallot
Coronary anomalies-
RCA from LAD or LAD from RCA, cross right ventricular
outflow tract
Pink Tet
VSD
PS with L to R shunt
Radiology/Tetralogy
Right arch-large aorta
Concave PA segment
coure en sabot-elevation of cardiac apex
thoracoctomy
Decreased vascularity/assymmetry
Angiography Tetralogy
Branch pulmonary stenoses
overriding aortic/VSD
RPA often larger than LPA
20 reorientation infundiblum, clockwise rotation
Correction Tetralogy
1944 Blalock-Taussig: SCA to PA
Central Shunt:
Waterston- descending aorta to LPA
Potts- ascending aorta to RPA
Graft
Tetralogy Repair
1954 Lilliehi, University of Minnesota
VSD closure
Outflow reconstruction
Unifocalization-bronchial artery to pulmonary artery
Tricuspid Atresia
1.5% CHD
absent tricuspid valve
ASD-PFO
VSD/PDA
associated transposition
Radiology Tricuspid Atresia
Small to normal heart size
Decreased vascularity
Correction Tricuspid Atresia
Glenn- SVC to PA
Fontan- RA to PA
Modified Fontan- RV to PA
Post-Op Fontan
Right pleural effusion
ascites
protein losing entrophy
Ebstein Anomaly
1866 Wilhelm Ebstein (German physcian)
Atrialization RV
Adherence redunant leaflets
False annulus
Ebstein Physisology
Newborn increased pulmonary artery pressure
Massive tricuspid regurgitation
Decreased forward flow to PA
Radiology Ebstein
Massive cardiomegaly
Decreased vascularity
Angiography-trilobed RVgram
Ebstein Treatment
Supportive care
ECMO-Newborn
? eventually tricuspid valve repalcement
Cyanosis and increased vascularity
TGV/TGV complex
Truncus Arteriosus
TAPVR
TA with TGV
Transposition of Great Vessels
Transposition-abnormal anteroposterior great vessel relationships
ventriculo-arterial con/discordance
d-or l-
d-TGV
Most common cyanotic lesion presenting in first 24 hours of life
ASD, VSD,or PDA
Parallel circulation, v/a discordance
Radiology TGV
Narrow superior mediastinum/
concave main PA segment
egg-on-a-string
increased pulmonary vascularity
Transposition Complex
DORV
type 1-Pulmonary stenosis
Type II (Taussig-Bing)-subpulmonic VSD incomplete VSD, supracristal
Corrected Transposition
l-TGV
L-Bulboventricular loop
Ventriculo-arterial concordance
Aorta-anterior and left
Associated complex disease
Radiology l-TGV
Prominent left upper cardiac border
boxlike configuration due to ventricular inversion
Correction TGV
Surgical
Rashkind-balloon atrial septostomy
Blalock-Hanlon-opertive atrial septecomy
Jantene-arterial switch
Mustard/Senning- atrial baffle
Persistent Truncus
Uncommon 0.4% CHD
Collett/Edwards orgins PAÕs:
I-short pulmonary trunk
II-closely spaced PA orgins
III-widely spaced PA orgins
IV-PAs from descending aorta-Ópseudo-truncusÓ
Truncus Variants
Hemitruncus- main PA, right or left PA
with interruption of aorta
Common Truncus
VSD
2-6 Cusps variable truncal valve
Radiology Truncus
Large truncus
Right arch
Increased vascularity
Hilar comma-LPA orgin-type II
Correction Truncus
Rastelli
RV to PA Conduit
TAPVR
Common pulmonary vein-failure of development
Site of connection:
supracardiac- 45%
cardiac-23%
infracardiac-21%
mixed-11%
Radiology TAPVR
Supra-figure 8 orsnowman
right medistinal border-SVC
left mediastinal border-left vertical vein
infra-:
venous hypertension pattern
Kerley B lines
Single Ventricle
True univentricular heart described by inlet type=
# AV valves
Usually RV hypoplasia, infundibulium(left ventricular type)
RV type
rundimentary septum -common ventricle
Radiology/ Single Ventricle
Varible pulmonary vascularity increased or decreased depends on shunt
surgery
shunt or PA band-initial
Glenn/Fontan
Coarctation of the Aorta
Localized juxtaductal
infantile-tubular hypoplasia
Radiology Coarctation
LV enlargment
CHF newborn, critical
3 sign
Rib notching, rare less than 1 year
Coarctation Repair
Subclavian flap
Interposition graft
Acyanotic with Normal Vascularity
Coarctation
AS
AI
PS/PI
Acyanotic with Normal Vascularity
Coarctation
Aortic stenosis
Aortic insufficiency
Pulmonic stenosis/insufficiency
Aortic Stenosis
Supra-hourglass configuration
Valvar- 60-75% total, uni/bicuspid. Most common form of congential heart disease
sub-membranous, muscular
Radiology Aortic Stenosis
Valvar
LV enlargement
Dilatation ascending Ao
Angiography thickening/doming
Williams Syndrome
Idiopathic hypercalcemia of infancy
Elfin facies
Nephrocalcinosis
Aortic/pulmonic stenosis
Pulmonic Stenosis
Unicommissural, fusion-95% classic
Thickening, doming
5% dysplastic-thickened leaflets
Pulmonic Stenosis Radiology
RV enlargement
Poststenotic dilatation main PA
Angioplasty
Pulmonary Venous hypertension
Normal heart size-lesion promixal to mitral valve
Cardiomegaly/LA enlargement distal to MV
Endocardial Fibroelastosis
Primary
Secondary to obstruction
LV enlargement
Glycogen Storage Disease
Cardiomegaly out of proportion to pulmonary vascularity
compression left lower lobe bronchus, collaspe left lower lobe
Pulmonary Venous Hypertension
Level of obstruction
PV- TAPVR
LA- Cor triatriatum, myxoma
MV-stenosis, insufficiency
Mitral valve
Stenosis
Supravalvar ring
Parachute mitral valve
Shone syndrome-multiple left sided obstructions
Cor Triatriatum
Incomplete incorporation of the common pulmonary vein
Rare
Membrane separates chamber from LA proper.
Radiology pulmonary venous hypertension
Coronary Artery anomalies
LCA from PA
Coronary flow-
RCA to LCA to PA, myocardial steal
Coronary to cardiac fistula
Aquired heart disease- Pediatric
Kawasaki-myocarditis, coronary aneurysms
Myocarditis-viral, coxsackie
Acute Rheumatic Fever
Aortic dissection- Marfans, homocystinuria
Hypoplastic Left Heart
Diminuitive LV
8% CHD- most common cause of death first week of life
Underdeveloped LV
Variable hypoplasia/ atresia... Ao, MV
Hypoplastic Left Heart
2 mm Ao annulus= aortic atresia
Common coronary artery
PDA
Coarctation
Hypoplastic Left Heart
ASD- 10% premature closure PFO
Alternate atrial egress:levoatrial cardinal v.-UPV to ScV
Fenestration of atrium to coronary sinus
Varying PV obstruction
Hypoplastic Left Heart
Radiology:
Cardiomegaly- usually without LA enlargement
Increased vascularity- arterial or venous
Hypoplastic Left Heart
Surgical correction:
Transplant
Norwood- reconstruction of Ao using main PA, central shunt
2nd stage-Glenn, 3rd stage Fontan
Modified Norwood, graft PA to Ao
Neonatal CHD
Decreased vascularity:
Pulmonary atresia
Tricuspid atresia
TGV with pulmonary stenosis
Neonatal CHD
Increased shunt vascularity:
TGV
Truncus
TAPVR
Critical coarctation(with cardiac enlargement)
Neonatal CHD
Decreased vascularity:
Pulmonary atresia
Tricuspid atresia
TGV with pulmonary stenosis
Neonatal CHD
Increased vascularity-venous hypertension:
TAPVR
Hypoplastic left heart
Coarctation of Ao
Summary/approach
Chest X-ray
Skeleton-surgery, Downs
Situs-abnormal, usually complex disease
Apex
Cardiac size/ configuration-enlarged
Ao arch/descending Ao-R, tet, truncus, tgv, tr atr.
Pulmonary vessels-decr. tet, tr atr, pulm atresia
References
Strife, J, and Bisset, G.,Cardiovascular system, Kirks, D.R.,Practical Pediatric
Imaging, pp 417-515, 1991
Amplatz, K., Moller, J., Radiology of Congenital Heart Disease, 1992.
Higgins, C., Essential of Cardiac Radiology and Imaging, 1992.
University of Utah cardiovascular pathology section:
http://www-medlib.med.utah.edu/WebPath/CVHTML/CVIDX.html
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Last modified 6-1-96 1420 CDT