Transcript Adult Congenital Heart Disease and Echocardiography

Congenital Heart
Disease and Cardiac
Imaging Modalities
Riya Chacko, MD
May 2008
Overview
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Classification of congenital heart disease
Brief overview of common simple adult
congenital heart diseases: ASD, VSD, PDA, and
coarctation
Transthoracic echocardiography
Fetal echocardiography
Transesophageal echocardiography
3D echocardiography
Cardiac CT
Cardiac MRI
Relevance
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Congenital heart disease is the most common major
birth defect affecting 6-8/1000 live births (Gardiner)
Accounts for 40% of perinatal deaths from congenital
anomalies, 20% of deaths in 1st month of life, and
majority of congenital defect deaths in childhood
(Randall)
With improved surgical and medical therapies, now more
adults live with congenital heart disease. In 1950s, 20%
of congenital heart disease patients lived to adulthood
and now 80%. (Crean)
Adult population in US with congenital heart disease now
1 million (Russell)
Table 1. Survival Rate from Year of Birth (1940-2000) by Complexity of Congenital Heart
Disease
Russell, I. A. et al. Anesth Analg 2006;102:694-723
Copyright restrictions apply.
Classification of Congenital Heart
Diseases
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Simple or complex
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Simple includes ASD, VSD, or singular valvular
abnormalities (Ebstein’s anomaly)
Complex includes those with multiple defects, AV
canal defects, or “single” ventricle physiology.
Cyanotic or non-cyanotic
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Non-cyanotic: ASD, VSD, sinus venosus defect,
patent ductus arteriosus, aortic stenosis, pulmonary
stenosis, aortic coarctation
Cyanotic: Tetralogy of Fallot, Ebstein’s anomaly,
transposition of the great arteries, Eisenmenger’s
syndrome, truncus arteriosus, tricuspid atresia, total
anomalous pulmonary venous return “5 Ts and 2 Es”
The heart is formed
and septations
occur at 50
embryonic days.
Atrial Septal Defects
 One
third of all congenital defects detected
in the adult are ASDs.
 2-3 times more likely to occur in women
 3 varieties:
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Ostium primum
Ostium secundum
Sinus venosus
Ostium Primum
 15%
of all ASDs
 Occurs in the lower part of the atrial
septum
 Associated with a left axis deviation on
EKG
 Also associated with cleft in anterior mitral
valve leaflet
Ostium Secundum
 Defect
in fossa ovalis
 Represents 75% of all ASDs
 Associated with mitral valve prolapse
Sinus Venosus Defect
 Located
in the upper atrial septum
 Represents 10% of all ASD defects
 Associated with anomalous pulmonary
venous drainage into the right atrium or
venae cavae
Patent Ductus Arteriosus
PDA
 10%
of congenital heart disease
 Rarely closes spontaneously after infancy
 May become aneurysmal or calcified
leading to rupture
 1/3 of patients die of heart failure,
pulmonary hypertension or endarteritis by
age 40. 2/3 die by age 60.
Aortic Coarctation
 Usually
distal to left subclavian at site of
ligamentum arteriosum.
 2-5 times more common in men
 Associated with Turner’s syndrome,
bicuspid aortic valve, ventricular septal
defect, patent ductus arteriosus, mitral
stenosis or regurgitation, or aneurysms of
the circle of Willis
Imaging Protocols for Congenital
Heart Disease
 Position
of the cardiac apex and aortic
arch•
 Levocardia Left-sided cardiac apex•
Dextrocardia Right-sided cardiac apex•
Mesocardia Midline/indistinct cardiac
apex
 Aortic arch Left or right aortic arch,
branches
Defining Atria and Ventricles
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Left atrium– by finger-like appendage
Right atrium – larger, wider appendage
Usually concordance with abdominal situs 7080% (IVC on right, RA on right)
Right ventricle – triangular with 3 components
(inlet, trabeculated, outlet). Moderator band,
septal attachments of TV
Left ventricle – elliptical, fine trabeculae, no
septal attachment of MV (inlet, tension
apparatus, and outlet zone for AV valve)
 Situs
of the atrium
 Situs solitus Normal atrial arrangement
 Situs inversus Reverse atrial arrangement
(LA–RV, RA–LV)
 Situs ambiguous Often LA isomerism
(usual with abnormal venous drainage
from azygous/hemi-azygous veins)
 Atrioventricular
(AV) arrangement
 AV concordance
RA–RV, LA–LV
 AV discordance
RA–LV, LA–RV
 Double inlet
RA and LA to LV or
RV
 Absent
Right or left AV valve
atresia
 Ventriculoarterial
(VA) arrangement
 VA concordance RV–PA, LV–aorta
 VA discordance
RV–aorta, LV–PA
 Double outlet
RV or LV giving off
both PA and aorta
 Solitary or common arterial trunk Truncus
or A–P window
 Ventricular
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morphology
Normal
Single ventricle
physiology
Rudimentary
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Other defects
ASD
VSD
AVSD
Common in Trisomy 21 (Down's
syndrome)
Valvular defects, atresia, abnormal number of
cusps
Shunts
Blalock–Taussig or Glenn
shunts•
Venous drainage
Anomalous systemic or pulmonary venous
connections
Vascular rings
Nicol ED. Clin Rad 2007;62(6
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Coronary anatomy
 Normal configuration
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Rotation
discordance
 Aberrant origin
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Single coronary artery
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Malignant course
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Abnormal morphology
Normal or congenital
heart
Rotation common in AV
and VA
i.e., RCA off left
coronary sinus
Beware left main
coronary artery atresia
i.e., between RVOT and
aorta
i.e., Kawasaki's disease
Echocardiography
 Preferred
method for initial assessment of
congenital heart disease
 Also the preferred imaging modality in
babies
 Limitations – Chest wall deformities,
median sternotomies, relation to
extracardiac structures and post-surgical
assessment
 Poor assessment of RV size and function
Fetal Echocardiography
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Two forms: basic and extended
“Four chamber view” the most basic
Color-flow and spectral Doppler imaging
M-mode available
Sensitivity from 60-100%
Limited by body habitus and fetal age (11 to 14
weeks  transvaginal ultrasound). 16 weeks by
abdominal.
Reported specificity up to 100% in babies
without congenital heart disease
Transabdominal view of the 4 chambers of the heart at 13
weeks' gestation.
Transvaginal view of atrioventricular septal
defect at 13 weeks' gestation in a fetus with
trisomy 21 (case 2).
(A) A full examination of the fetal heart may be
obtained by five transverse sections through
the abdomen and chest of the fetus. The first
section shows abdominal situs (B) with the
aorta (Ao) to the left of the spine and the
inferior caval vein (IVC) anterior and to the
right. The normal fetal stomach (St) and
heart lie on the left side. The second section
(C) illustrates the four chambers of the heart
with the left atrium (LA) in front of the spine
and the right ventricle (RV) just below the
sternum. The third cut (D) shows the aorta
arising centrally in the heart from the left
ventricle (LV) and the fourth the pulmonary
trunk (PV) arising from the anteriorly placed
right ventricle and crossing to the fetal left
over the ascending aorta (E). The fifth
section shows the anteriorly positioned
ductal arch (D) and the transverse aortic
arch (Ao) to be of equal size traversing back
to the fetal spine (F). A normal variant "three
vessel" view is shown with a right sided
aortic arch and persistent left superior caval
vein (LSVC). The trachea (T) can be seen
lying between the aortic (Ao) and ductal (D)
arches (G).
Gardiner.
Transvaginal 3-vessel view of the great arteries at 13 weeks'
gestation. PA, Main pulmonary artery; Ao, aorta; SVC, superior
vena cava; BPA, right branch pulmonary artery.
McAuliffe F.
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In 1992, only 8% of infants with congenital heart
disease undergoing cardiac surgery had a
prenatal diagnosis. With fetal echo, this number
rose to 57% in 2002.(Mohan)
 In one study assessing use of early fetal echo
(prior to 14 weeks gestation) in a high-risk
population, sensitivity of 70% and specificity of
98%
 This resulted in 79% of patients terminating their
pregnancy prior to their 18-20 week follow up.
Transesophageal
Echocardiography
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Good for evaluation of venous return, atria, AV
valves, and the left ventricular outflow tract
Mid-esophageal (ME) four-chamber and bicaval
views good for atrial septum by 2D
ME 4-chamber and transgastric mid-short axis to
assess for VSDs
Also essential in intra-operative repair
Limited for evaluation of the right ventricular
outflow tract and pulmonary arteries
 Preferred
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method in the following cases:
ASD, secundum
Mitral valve regurgitation
Ebstein’s anomaly
Fontan – assessing for right atrial thrombus,
obstruction
Coronary sinus
defect
Inferior
sinus
venosus
defect
Superior sinus
venosus
defect
AV septal
defect
3D Echocardiography
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In a small, non-randomized comparison of 3D
echo evaluation of congenital abnormalities as
compared to anatomical specimens, Vogel et al.
found a close association. The key difference is
in thickness of supporting structures
(overestimated on 3D echo).
 Salustri et al reported additional information
gathered by 3D versus 2D echo on 36% of
patients screened with known congenital heart
disease.
 Advantage of being able to view en face both
sides of a septal defect versus 2D echo
--Live 3D imaging examples of how 3D imaging is better than 2D imaging for evaluating
structures such as septal defects
Houck, R. C. et al. Am. J. Roentgenol. 2006;187:1092-1106
Copyright © 2006 by the American Roentgen Ray Society
--Live 3D imaging examples of how 3D imaging is better than 2D imaging for evaluating
structures such as septal defects
Houck, R. C. et al. Am. J. Roentgenol. 2006;187:1092-1106
Copyright © 2006 by the American Roentgen Ray Society
Cardiac CT
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10% of MDCT patients at Royal Brompton
Hospital in London have congenital heart
disease.
 Multi-detector CT scans (MDCT) are able to
identify those with congenital heart disease and
anomalous coronary arteries.
 In a retrospective review of 85 patients with
congenital heart disease at Ohio State
University, authors Cook and Raman was found
that 19% had detectable anomalous coronary
anatomy by MDCT both 16 and 64 slice.
 Normal
coronary arteries
Figure 6 Anomalous LCA arising from a common RCA origin and coursing anterior to the RVOT
(arrow). Note the vestigial left anterior descending artery (LAD) (arrowhead).
Manghat, N E et al. Heart 2005;91:1515-1522
Copyright ©2005 BMJ Publishing Group Ltd.
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Pacemaker leads extending through ASD to left
atrium and terminating in left ventricle.
 Kawasaki’s
disease
Ebstein’s Anomaly with distal
displacement of the right AV valves
compared to the left AV valves
 Patent
ductus arteriosus
Angiography with
pigtail catheter
(A). (B) MRA. (C)
CT after stent
showing
contained aortic
rupture. (D) CT
showing
resolution of
pseudoaneurysm.
(E) MRA showing
drop out with
stent.
 Transposition
of the great vessels
 Not
as useful due to poorer image quality
if the patient has frequent beat-to-beat
variation or atrial fibrillation.
 Flow data is not available and thus
information on physiologic effects of
valvular abnormalities.
 Inadequate assessment of RV function
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wall motion as compared to TTE
may be inferior due to lower temporal
resolution.
 Radiation exposure should be considered
as compared to cardiac MR or TTE
especially if repeat examinations are
required throughout a lifetime.
Cardiac CT
 Advantages
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over cardiac MRI include:
Shorter breath-hold sequences that may not
be tolerated in a congenital heart disease
population
Better tolerability for those with
pacemakers/ICDs or those with
claustrophobia
More robust coronary artery imaging
especially when involvement of fistulous
communication
Rapid image acquisition time
Cardiac MRI
1st described to assess congenital heart disease
in 1984.
 Also utilizes contrast-enhanced MR angiography
(CE-MRA)
 Allows better visualization of mediastinal vessels
(small central pulmonary arteries) and better to
assess the left pulmonary artery than echo.
 Best to visualize proximal and mid pulmonary
arterial stenosis but not distal to the hila.
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(A and B) ALCAPA
syndrome
(anomalous left
coronary artery from
PA). (C) Large RCA
with fistulous
connection.
CT at bottom (E)
shows anomalous
LCX draining into
coronary sinus
Crean. Heart
2007.
Pulmonary vein
stenosis seen
on MRI
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Favored to evaluate coarctation in children.
 Accurately measures pulmonary blood flow and
assesses the aorta.
 Ultrasound better to assess valvular pathology but MR
better to assess systemic and venous malformations.
 Because of wider field of view, better to assess larger
cardiac chambers (ie Ebstein’s anomaly)
 May also be used to estimate left-to-right shunts by
measuring flow to calculate Qp:Qs
 Probably most effective to assess post-surgically as well
 Allows
better assessment of RV size and
function (critical in cases such as Tetralogy
of Fallot post-surgery).
 With delayed enhancement technique, it is
useful to assess prognosis in congenital
heart disease as well.
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Disadvantage compared to cardiac
catheterization: inability to measure pressures
and obtain O2 saturations
 Examination time is long (up to 90 minutes)
which is difficult in a pediatric population.
 Faster heart rates in infant population make MRI
less helpful and accurate.
 Images are obscured by metallic stents in those
with stent-corrected coarctation
Components of a congenital CMR exam
 Axial steady state free precession (SSFP) cine sequence from aortic
arch to diaphragm 10 mm thick, no gap
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Short axis oblique SSFP from ventricular apex to atrioventricular (AV)
groove, 8 mm thick, no gap
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Fast cine phase contrast through ascending aorta, main and left and
right pulmonary arteries
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Three dimensional contrast enhanced magnetic resonance
angiography (CE-MRA): coronal plane, sternum to spine, slice
thickness 2–2.5 mm, arterial and venous phase
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Additional thin slices (SSFP or black blood) and cine phase contrast
studies (for example, for flow through shunts, collaterals, anomalous
veins or arteries, etc) as directed by monitoring physician
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Multiplanar reformations, maximum intensity projections and volume
rendered image preparation (ideally by reporting physician)
Creon A.
Summary
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Classification of congenital heart disease
Overview of common adult congenital heart
diseases: ASD, VSD, PDA, and coarctation
Transthoracic echocardiography
Fetal echocardiography
Transesophageal echocardiography
3D echocardiography
Cardiac CT
Cardiac MRI
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Regardless of
imaging modality, one
requires the expertise
of an experienced
specialist…
References
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