Basics of adult congenital heart disease assessment
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Transcript of Basics of adult congenital heart disease assessment
BASICS OF ADULT CONGENITAL HEART DISEASECONGENITAL HEART DISEASE
ASSESSMENTChristina Attenhofer Jost
C di l C t Kli ik I P kCardiovascular Center Klinik Im Parkand
Children‘s University Hospital Zurich (ACHD)
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
BAD NEWS: EVERYONE GETS OLDERBAD NEWS: EVERYONE GETS OLDER……………..
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
GOOD NEWS EVERYONE GETS OLDERGOOD NEWS: EVERYONE GETS OLDER
After Khairy et al. JACC 2010;56:1149
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
A congenital echo cardiologistA 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• will use an organized method when examining the CV system
P O Leary Mayo ClinicP.O. Leary, Mayo Clinic
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
Segments ConnectionSegments - Connection
Great veins
V t i l
Atria
Veno-atrial
Ventricles
Atrio-ventricular
Ventricles
Ventriculo-arterialGreat arteries
AtriaAtriaA i l i li RA i d i h f LA• Atrial situs solitus: mRA anterior and right of mLA
• Atrial situs inversus: mRA to the left of mLA• True atrial situs ambiguous = rareTrue 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)the same side as morphologic LA (mLA)
• Connection CS and suprahepatic IVC to mRA• Morphology atrial appendages
RA l i ll- mRA: course, muscular appearing walls with pectinate muscles and crista terminalis- mLA: smooth surface
Ventricular morphology: Cardiac crux
N l Ventricular morphology
Normal
LARA– TV committed
to RVMV committed
LARA
– MV committed to LV
Valve morphologyValve morphology– TV apically displaced– MV superior insertion
RV LVMV superior insertion
W.D. Edwards, Mayo Clinic
Ventricular morphology:Cardiac crux
N l CCTGA Ventricular morphology
Normal CCTGA
LARA– TV committed
to RVMV committed
LARA LARA
– MV committed to LV
Valve morphologyValve morphology– TV apically displaced– MV superior insertion
RV LV LV RVMV superior insertion
W.D. Edwards, Mayo Clinic
Left VentricleLeft Ventricle• smooth endocardial
Left VentricleLeft Ventricle• smooth endocardial
surface• large discrete• large, discrete
papillary muscles not inserting into septuminserting into septum
• more round shaped• mitral valve• mitral valve
W.D. Edwards
Right VentricleRight Ventricle• coarse
Right VentricleRight Ventricle• coarse
trabeculationt i t l• septoparietal
muscle bundle• multiple small
papillary muscles with septal and free wall attachments
W.D. Edwards
What is that?What is that?
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
What is that?What is that?
1. CCTGACC G2. HCM3. Noncompaction4 Endocardial fibroelastosis4. Endocardial fibroelastosis5. Double chambered LV
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
What is that?What is that?
W D Ed dW.D. Edwards
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
What is that?What is that?
Congenitally corrected transposition
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
Overview of Adult CHDOverview of Adult CHD
• Outflow obstruction assessmentOutflow tract, aortic valve, coarctation, pulmonary valve
• Abnormal intracardiac communicationsASD, VSD, PDA, anomalous pulmonary veins, persistent left superior vena cava
• Complex malformationsEbstein’s anomaly TOF CCTGA completeEbstein s anomaly, TOF, CCTGA, complete transposition, univentricular heart
Subaortic stenosisSubaortic stenosis
8 30 % f LVOT b t ti• 8-30 % of LVOT obstruction• 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, Sh d PDA l ft iShone syndrome, PDA, left superior vena cava, HCM, PS, bicuspid aortic valve etc.
F. Walker. Diagnosis and management of adult CHD 2003
Discrete subaortic stenosisDiscrete 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
Congenitally Abnormal Aortic ValveCongenitally Abnormal Aortic Valve
Leonardo Da Vinci
Bicuspid aortic valveBicuspid aortic valve
• Incidence 1%, familial in 25 %• Males : females = 3:1 to 5:1• Associated congenital cardiovascular lesions
include coarctation, Turner syndrome, PDA,include coarctation, Turner syndrome, PDA, William’s syndrome, VSD, Shone’s complex, abnormal coronary arteriesabnormal coronary arteries
Bicuspid aortic valveBicuspid aortic valve
Schaefer et al. Heart 2008 94: 1634-1638
Supravalvular aortic stenosisSupravalvular aortic stenosis
• 6 % of congenital LVOT obstruction• Most often due to elastin
arteriopathy• Association with coarctation, PDA,Association with coarctation, PDA,
ASD, VSD, tetralogy of Fallot, coronary artery abnormalities,coronary artery abnormalities,bicuspid aortic valve
15 year old with Williams Beuren syndrome
CoarctationCoarctation
• Prevalence 0.4/1000 live births7 % f CHD• 7 % of CHD
• Severe heart failure, hypertension etchypertension etc.
• Up to 50 % associated lesions (VSD abnormallesions (VSD, abnormal AV valve, subvalvular stenosis, AV septal pdefects, etc)Juxtaductal coarctation
by W.D. Edwards
CoarctationCoarctation
P.O.Leary
Patel, Young fromEchocardiographic in Ped and Adult CHD
• BP MEASUREMENT ARMS AND LEGS• Systolic pressure gradient with Bernouilli equation (4(V 2-v 2)
Echocardiographic in Ped and Adult CHD
• Systolic pressure gradient with Bernouilli equation (4(V2 -v1 )• Doppler assessment of abdominal aortic flow• Imaging aorta by MRI/CT compulsory
Pulmonary stenosisPulmonary stenosis
W D Ed dW.D. Edwards Mayo Clinic
Pulmonary stenosisPulmonary stenosis
• 7 to 10 % of CHD• 90 % valvular (often isolated),90 % valvular (often isolated),
10 % supravalvular or subvalvularS l l PS ft i Willi• Supravalvular PS: often in Williams syndrome
• Noonan syndrome: 2/3 have PS due to valve dysplasiavalve dysplasia
25 year old woman with PS only?25 year old woman with PS only?
V max 4.8m/sec
25 year old woman with PS only?25 year old woman with PS only?
25 year old woman with PS25 year old woman with PS
25 year old woman with PS only?25 year old woman with PS only?
PS PR di t 56 HPS, PR, mean gradient 56mmHgAS, AR, mean gradient 52mmHg
PDANoncompaction of the LV
PSLVC
A congenital echo cardiologistA congenital echo cardiologist
• Is only amazed when a heart is completely normal.p y
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
Overview of Adult CHDOverview of Adult CHD
• Outflow obstruction assessmentOutflow tract, aortic valve, coarctation, pulmonary valve
• Abnormal intracardiac communicationsASD, VSD, PDA, anomalous pulmonary veins, persistent left superior vena cava
• Complex malformationsEbstein’s anomaly, TOF, CCTGA, completeEbstein s anomaly, TOF, CCTGA, complete transposition, univentricular heart
Atrial septal defects: 10% of CHDAtrial septal defects: 10% of CHD
Ostium primum ASD =partial AVSD 15%
Sinus venosusASD 5%
SecundumASD 80%
InferiorSinus venosusASD<1% Coronary sinus
ASD<1% After Perloff JK. Clinical Recognition of Congenital Heart Disease 2003
Simple secundum ASD?Simple secundum ASD?
• Dyspnea on exertion NYHA II• Dizziness, occasional faintingDizziness, occasional fainting• First degree AV block (PR interval 396 ms)• Holter intermittent 2nd and 3rd degree
AV blockb oc
HOLT ORAM SYNDROME = HEART HAND SYNDROMEHEART HAND SYNDROME
Female with 5 children working 100%Female with 5 children, working 100%
Female with 5 children working 100%Female with 5 children, working 100%
IVC RAC RA
SVC
Female with 5 children working 100%Female with 5 children, working 100%
IVC RAC RA
SVC
Female with 5 children working 100%Female with 5 children, working 100%
Female with 5 children working 100%Female with 5 children, working 100%
A congenital echo cardiologistA congenital echo cardiologist
• Knows that women don‘t tire easily… there is always an explanationy p
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
Sinus venosus atrial septal defectSinus venosus atrial septal defect
• Description in 1858• 5% of ASDs5% of ASDs• Common association with
PAPVC i 97 %PAPVC in 97 % -TAPVC rare
Ventricular septal defectsVentricular 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;135:812f C lli d ll A J C di l 1983after Capelli and colleagues: Am J Cardiol 1983
Ventricular septal defectVentricular septal defect
VSD in adults• VSD operated in childhood with or without p
residual VSD• Small VSD with insignificant L-R shuntSmall VSD with insignificant L R shunt• VSD with significant L-R shunt, pulmonary
hypertension and various degrees of LVhypertension, and various degrees of LV volume overloadEisenmenger syndrome: large VSD with large• Eisenmenger syndrome: large VSD with large L-R shunt
VSD and aortic regurgitationVSD and aortic regurgitation
Early systole, blood through Right coronary cusp and aortic Unsupported right or NC cusp VSD
Ammash, Warnes. Ann Int Med 2001;135:812after Tatsuno and colleagues. Circulation 1973;48:1028
sinus driven into RV (Venturi effect)
pushed down away from other cusp: AR
after Tatsuno and colleagues. Circulation 1973;48:1028
VSD: 45 year old man no symptomsVSD: 45 year old man, no symptoms
VSD: 45 year old man no symptomsVSD: 45 year old man, no symptoms
A congenital echo cardiologistA congenital echo cardiologist
• Knows that good advice may go down the drain occasionallyy
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
Normal pulmonary venous connectionsNormal pulmonary venous connections
Ammash et al. JACC 1997;1351-8
Normal pulmonary venous connectionsNormal pulmonary venous connections
Right PV Left PV
Ammash et al. JACC 1997;1351-8
Scimitar syndrome – what the hell is that??
Scimitar syndromeScimitar syndromePAPVC f th i ht l• PAPVC of the right pulmonary vein or veins to the IVC
• Anomalous systemic arterial l t th i ht lsupply to the right lung
• Varying degrees of hypoplasia of the right lung with or without pulmonary sequestration
• 25 % associated CHD: VSD, ASD,25 % associated CHD: VSD, ASD, PDA, coarctation, TOF
M. Vogel in Adult Congenital Heart Disease 2003
F th b it Child ’ H it l B tFrom the website: Children’s Hospital Boston
Scimitar syndrome: 1st DescriptionScimitar syndrome: 1st Description
.. If you think it is sufficiently interestingfor insertion in your valuable journalfor insertion in your valuable journal,it is much at your service. – I havethe honour to remain, Sir,
Your very obedient servant
GEORGE COOPER
Breatford, 27th June, 1836
Overview of Adult CHDOverview of Adult CHD
• Outflow obstruction assessmentOutflow tract, aortic valve, coarctation, pulmonary valve
• Abnormal intracardiac communicationsASD, VSD, PDA, anomalous pulmonary veins, persistent left superior vena cava
• Complex malformationsEbstein’s anomaly TOF CCTGA completeEbstein s anomaly, TOF, CCTGA, complete transposition, univentricular heart
Ebstein‘s anomalyEbstein s anomaly• 1:200’000 live births1:200 000 live births• <1 % of CHD• 80-90 % interatrial communication• 80-90 % interatrial communication• Bicuspid or atretic aortic valve• PA or hypoplastic PA• PA or hypoplastic PA• Coarctation• MVP accessory mitral valve tissue• MVP, accessory mitral valve tissue• left ventricular dysplasia
resembling noncompactionAttenhofer Jost, Connolly et al. Circulation 2007;115:277
resembling noncompactionW.D. Edwards, Mayo Clinic
W.D. Edwards, Mayo Clinic
15 year with heart failure due to associated LV noncompaction
RV
LV
Attenhofer Jost, Connolly et al. JASE: 2004:17: 677-680
Ebstein‘s anomaly mild and severe
64 year old female with brain abscess age 10 years
3D echo in Ebstein’s anomaly
35 ld ti t ith35 year old patient withEbstein‘s anomaly and
severe TR
Tetralogy of FallotTetralogy of Fallot
• Most common cyanotic CHD4 10 % f ll CHD• 4-10 % of all CHD
• 15 % deletion chromosome 22q11chromosome 22q11(CATCH 22 syndrome)
• Unoperated patients• Unoperated patients rarely present the 1st
time as adults
P.O. Leary
Surgical repair of TOFSurgical repair of TOF• VSD closure• VSD closure• Resection of infundibular muscle
P l l t / (G t )• Pulmonary valvotomy +/- monocusp (Goretex)• RVOT patch• Transannular patch if needed or
RV to PA conduit (anomalous CA), rarelyPulmonary valve replacement not for infantsPulmonary valve homograftPulmonary arterioplasty
• Correction of other lesions
A congenital echo cardiologistA congenital echo cardiologist
• Never starts with the echo before reading the operative notes and last g pexams
EUROECHO CONGRESS - COPENHAGEN -TEACHING COURSE 2010
Tetralogy of Fallot: echo after repairTetralogy of Fallot: echo after repair
R id l l it ti• Residual pulmonary regurgitation • Residual RVOT obstruction• RV dysfunction• Residual shunting• Residual shunting • AR with or without aortic root dilatation• LV dysfunction
ConclusionConclusion
E h di h i d lt CHD• Echocardiography in adult CHD encompasses a huge variety of possible problems and unique situations
• It is hard to adequately diagnose and treat even CHD of simple or moderate t eat e e C o s p e o ode ateseverity
• Complex CHD belongs to a tertiary• Complex CHD belongs to a tertiary referral center
End thank youEnd – thank you