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……………..


GOOD NEWS EVERYONE GETS OLDERGOOD NEWS: EVERYONE GETS OLDER

After Khairy et al. JACC 2010;56:1149


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


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


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?



1. CCTGACC G2. HCM3. Noncompaction4 Endocardial fibroelastosis4. Endocardial fibroelastosis5. Double chambered LV



W D Ed dW.D. Edwards



Congenitally corrected transposition


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 PS25 year old woman with PS


PS PR di t 56 HPS, PR, mean gradient 56mmHgAS, AR, mean gradient 52mmHg

PDANoncompaction of the LV

PSLVC


• Is only amazed when a heart is completely normal.p y





• 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%


IVC RAC RA

SVC


IVC RAC RA

SVC




• Knows that women don‘t tire easily… there is always an explanationy p


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


• Knows that good advice may go down the drain occasionallyy


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




• 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


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


• Never starts with the echo before reading the operative notes and last g pexams


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

Date post:	30-Nov-2014
Category:	Education
Upload:	oussama-el-h
View:	1,404 times
Download:	5 times

Basics of adult congenital heart disease assessment

Education