Basics of adult congenital heart disease assessment
1. BASICS OF ADULT
CONGENITAL HEART DISEASE
ASSESSMENT
Christina Attenhofer Jost
Cardiovascular Center Klinik Im Park
C di l C t Kli ik I P k
and
Children‘s University Hospital Zurich (ACHD)
EUROECHO CONGRESS - COPENHAGEN -
TEACHING COURSE 2010
2. BAD NEWS: EVERYONE GETS OLDER
OLDER……………..
EUROECHO CONGRESS - COPENHAGEN -
TEACHING COURSE 2010
3. GOOD NEWS: EVERYONE GETS OLDER
NEWS
After Khairy et al. JACC 2010;56:1149
EUROECHO CONGRESS - COPENHAGEN -
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4. A congenital echo cardiologist
• assumes that every vein, artery,
chamber or valve is abnormal until it is
shown to be normal
• will use an organized method when
examining the CV system
P.O. Leary
P O Leary, Mayo Clinic
EUROECHO CONGRESS - COPENHAGEN -
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5. Segments - Connection
Great veins
Veno-atrial
V ti l
Atria
Atrio-ventricular
Ventricles
Ventriculo-arterial
Great arteries
6. Atria
• Atrial i
A i l situs solitus: mRA anterior and right of mLA
li RA i d i h f LA
• Atrial situs inversus: mRA to the left of mLA
• True atrial situs ambiguous = rare
• Thicker limbus of the foramen ovale on the same side as
morphologic RA (mRA), thinner valve of the foramen ovale on
the same side as morphologic LA (mLA)
• Connection CS and suprahepatic IVC to mRA
• Morphology atrial appendages
- mRA: course, muscular appearing walls
RA l i ll
with pectinate muscles and crista terminalis
- mLA: smooth surface
7. Ventricular morphology:
Cardiac crux
Normal
N l Ventricular
morphology
RA LA
– TV committed
to RV
– MV committed
to LV
Valve morphology
RV LV – TV apically displaced
– MV superior insertion
W.D. Edwards, Mayo Clinic
8. Ventricular morphology:
Cardiac crux
Normal
N l CCTGA Ventricular
morphology
RA LA RA LA
– TV committed
to RV
– MV committed
to LV
Valve morphology
RV LV – TV apically displaced
LV RV
– MV superior insertion
W.D. Edwards, Mayo Clinic
9. Left Ventricle
• smooth endocardial
surface
• large discrete
large,
papillary muscles not
inserting into septum
• more round shaped
• mitral valve
W.D. Edwards
10. Right Ventricle
• coarse
trabeculation
• septoparietal
t i t l
muscle bundle
• multiple small
papillary muscles
with septal and
free wall
attachments
W.D. Edwards
11. What is that?
EUROECHO CONGRESS - COPENHAGEN -
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12. What is that?
1. CC G
CCTGA
2. HCM
3. Noncompaction
4.
4 Endocardial fibroelastosis
5. Double chambered LV
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13. What is that?
W.D. Edwards
W D Ed d
EUROECHO CONGRESS - COPENHAGEN -
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14. What is that?
Congenitally corrected transposition
EUROECHO CONGRESS - COPENHAGEN -
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16. Subaortic stenosis
• 8 30 % of LVOT obstruction
8-30 f b t ti
• In 80 %, discrete obstruction by fibrous ring
• In 20 %, diffuse tunnel-like narrowing by a
fibromuscular band
• In 60 % associated with multilevel LVOT
obstruction, VSD, coarctation of the aorta,
Shone syndrome, PDA, left superior vena
Sh d PDA l ft i
cava, HCM, PS, bicuspid aortic valve etc.
F. Walker. Diagnosis and management of adult CHD 2003
17. Discrete subaortic stenosis
• Aortic valve usually tricommissural
• Hypertrophy of muscular ventricular septum
yp p y p
in up to 75 %
Muscle
membrane
P. O’Leary Mayo Clinic
21. Supravalvular aortic stenosis
• 6 % of congenital LVOT obstruction
• Most often due to elastin
arteriopathy
• Association with coarctation, PDA,
ASD, VSD, tetralogy of Fallot,
coronary artery abnormalities,
bicuspid aortic valve
23. Coarctation
• Prevalence 0.4/1000 live
births
• 7 % of CHD
f
• Severe heart failure,
hypertension etc
etc.
• Up to 50 % associated
lesions (VSD, abnormal
(VSD
AV valve, subvalvular
stenosis, AV septal
p
Juxtaductal coarctation defects, etc)
by W.D. Edwards
24. Coarctation
P.O.Leary
Patel, Young from
Echocardiographic in Ped and Adult CHD
• BP MEASUREMENT ARMS AND LEGS
• Systolic pressure gradient with Bernouilli equation (4(V22-v12)
• Doppler assessment of abdominal aortic flow
• Imaging aorta by MRI/CT compulsory
26. Pulmonary stenosis
• 7 to 10 % of CHD
• 90 % valvular (often isolated),
10 % supravalvular or subvalvular
• S
Supravalvular PS: often in Williams
l l PS ft i Willi
syndrome
• Noonan syndrome: 2/3 have PS due to
valve dysplasia
27. 25 year old woman with PS only?
V max 4.8m/sec
33. Atrial septal defects: 10% of CHD
Ostium primum ASD =
partial AVSD 15%
Sinus venosus
ASD 5%
Secundum
ASD 80%
InferiorSinus
venosus
ASD<1% Coronary sinus
ASD<1% After Perloff JK. Clinical Recognition of
Congenital Heart Disease 2003
34. Simple secundum ASD?
• Dyspnea on exertion NYHA II
• Dizziness, occasional fainting
• First degree AV block (PR interval 396 ms)
• Holter intermittent 2nd and 3rd degree
AV block
b oc
41. A congenital echo cardiologist
• Knows that women don‘t tire easily…
there is always an explanation
y p
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42. Sinus venosus atrial septal defect
• Description in 1858
• 5% of ASDs
• Common association with
PAPVC i 97 % -
in
TAPVC rare
43. Ventricular septal defects
1 = membranous (70-80%); 2 = double committed/subarterial
or supracristal (5-7%), 3 = muscular (5-20%); 4 = inlet (8%)
Ammash, Warnes. Ann Int Med 2001;135:812
after C
f Capelli and colleagues: A J C di l 1983
lli d ll Am Cardiol
44. Ventricular septal defect
VSD in adults
• VSD operated in childhood with or without
p
residual VSD
• Small VSD with insignificant L-R shunt
LR
• VSD with significant L-R shunt, pulmonary
hypertension,
hypertension and various degrees of LV
volume overload
• Eisenmenger syndrome: large VSD with large
L-R shunt
45. VSD and aortic regurgitation
Early systole, blood through Right coronary cusp and aortic Unsupported right or NC cusp
VSD sinus driven into RV (Venturi pushed down away from
effect) other cusp: AR
Ammash, Warnes. Ann Int Med 2001;135:812
after Tatsuno and colleagues. Circulation 1973;48:1028
52. Scimitar syndrome
• PAPVC of th right pulmonary
f the i ht l
vein or veins to the IVC
• Anomalous systemic arterial
supply to the right lung
l t th i ht l
• Varying degrees of hypoplasia of
the right lung with or without
pulmonary sequestration
• 25 % associated CHD: VSD, ASD,
PDA, coarctation, TOF
M. Vogel in Adult Congenital Heart Disease 2003
From the website: Children’s H
F th b it Child ’ Hospital B t
it l Boston
53. Scimitar syndrome: 1st Description
.. If you think it is sufficiently interesting
for insertion in your valuable journal
journal,
it is much at your service. – I have
the honour to remain, Sir,
Your very obedient servant
GEORGE COOPER
Breatford, 27th June, 1836
58. 64 year old female with brain
abscess age 10 years
59. 3D echo
in Ebstein’s anomaly
35 year old patient with
ld ti t ith
Ebstein‘s anomaly and
severe TR
60. Tetralogy of Fallot
• Most common cyanotic
CHD
• 4 10 % of all CHD
4-10 f ll
• 15 % deletion
chromosome 22q11
(CATCH 22 syndrome)
• Unoperated patients
rarely present the 1st
time as adults
P.O. Leary
61. Surgical repair of TOF
• VSD closure
• Resection of infundibular muscle
• P l
Pulmonary valvotomy +/- monocusp (G
l t / (Goretex)
t )
• RVOT patch
• Transannular patch if needed or
RV to PA conduit (anomalous CA), rarely
Pulmonary valve replacement not for infants
Pulmonary valve homograft
Pulmonary arterioplasty
• Correction of other lesions
62. A congenital echo cardiologist
• Never starts with the echo before
reading the operative notes and last
g p
exams
EUROECHO CONGRESS - COPENHAGEN -
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63. Tetralogy of Fallot: echo after repair
• Residual l
R id l pulmonary regurgitation
it ti
• Residual RVOT obstruction
• RV dysfunction
• Residual shunting
• AR with or without aortic root dilatation
• LV dysfunction
64. Conclusion
• E h
Echocardiography in adult CHD
di h i d lt
encompasses a huge variety of
possible problems and unique
situations
• It is hard to adequately diagnose and
t eat e e C
treat even CHD of simple or moderate
o s peo ode ate
severity
• Complex CHD belongs to a tertiary
referral center