Download ADULT CONGENITAL Heart disease

ADULT CONGENITAL
HEART DISEASE
Stuart Lilley
• More
adults than children have congenital heart disease
•Huge variety of congenital lesions from minor to major
• Heart failure, re-operation and arrhythmia are
inevitable in this group of patients
• Good imaging is the key to diagnosis, functional
assessment and effective follow-up
•Know the limitations of the imaging technique
and the imager !!
Important issues in adult echo
Device closure of ASD & PFO – TTE/TOE
The systemic right ventricle Univentricular repair
Pulmonary regurgitation & RV assessment
Bicuspid aortic valves
Eisenmenger patients - PAH
Congenital Heart Disease
VSD
PDA
ASD
PS
AS
COARCTATION
TGA
TETRALOGY
0
5
10
15
20
25
Percentage liveborn
30
35
New diagnosis
ASD or aneursym
VSD or aneurysm
Pulmonary stenosis
Valve or subaortic stenosis
APVD
AVSD
Arterial duct
Coarctation
Ebstein’s
Tetralogy of Fallot
UVH
Cor triatriatum
CCTGA
Adults with congenital heart disease
39
15
8
5
4
4
4
3
1
<1
<1
<1
<1
Important Anatomical
features
Left SVC
ADULT TYPES
 L-R SHUNTS
 OBSTRUCTIONS – Muscular/membrane,
valve and supravalve,and Arterial
 REGURGITATION
 VENTRICULAR FUNCTION
ASD
 L-R Shunt at Atrial level
 Right heart enlargement
 Late development Pulmonary Artery
Hypertension
 Arrythmias
Echo Appearances
ASD aneurysmal
ASD
ASD – Sinus Venosus -
LPAPVD
TOE
DEVICE CLOSURE
TOE device placement
PFO - TOE
CONTRAST STUDY
VALSALVA
EDGE IDENTIFICATION
SIZE
SINGLE/MULTIPLE
ANEURYSM IDENTIFICATION
VSD
 PERIMEMBRANOUS
 MUSCULAR/TRABECULAR
 SUB AORTIC
 SUB ARTERIAL
 DOUBLY COMITTED
 ANTERIOR MUSCULAR
 POSTERIOR
 APICAL
 INLET
VSD
VSD
 SMALL
 ANEURYSM
 MUSCULAR
 ENLARGED LV AND LA
 PULMONARY PRESSURE
 ENDOCARDITIS RISK
TOE
AVSD
 1 PARTIAL
L- R ATRIAL SHUNT
 2 COMPLETE L- R ATRIAL +
VENTRICULAR SHUNT




AV VALVE ABNORMALITY
CHORDAL ARRANGEMENT
SUB AORTIC STENOSIS
DOWNS SYNDOME
AVSD
AVSD
PARTIAL AVSD
COMMON ORIFICE AVSD
Calculations
 RV /PA pressure – Doppler
Tricuspid regurgitation/
VSD signal
 L-R Shunt size –Doppler mean
velocity
PA
PRESSURE
 ASD – Tricuspid regurgitation ( TR)
spectral
 VSD – TR or VSD spectral
 Arterial Duct (AD) - TR or AD spectral
 NEED QUALITY SIGNALS
TR
2M/S = 16mmHg
VSD Spectral Doppler
RVp = 120 – 100mmHg
EISENMENGER SYNDROME
SHUNT SIZE ESTIMATE
PULMONARY FLOW / SYSTEMIC FLOW
1:1
DOPPLER CALCULATIONS
L-R SHUNTS
Normal heart shunt is 1:1
QP – Pulmonary flow
Qs – Systemic flow
ASD/VSD means increased
Pulmonary blood flow -shunt will be
greater than 1 : 1
We then can calculate shunt size
from calculating QP and dividing it
by QS
AO stroke volume
 Mean Velocity x time = stroke
distance - SD
 Calculate AO root area from Radius
(pr ) – AREA
 Measure mean velocity
 Stroke volume = SD X AREA
SHUNT = SV PA/SV AO
PA stroke volume
• Mean Velocity x time = stroke
distance - SD
• Calculate PA root area from
Radius (pr ) – AREA
• Measure mean velocity
• Stroke volume = SD X AREA
SHUNT = SVPA/SVAO
ARTERIAL DUCT
 DESC AO – LPA
 L-R SHUNT
 LEFT HEART
ENLARGEMENT
 LARGE SHUNTS
PRODUCE PAH
 CONTINOUS SHUNT
 LEFT PARASTERNAL
 SUPRASTERNAL
DEVICE CLOSURE
PAH - EISENMENGER
AORTIC STENOSIS
VALVE
RE GROWTH
BICUSPID AO VALVE
 ECCENTRIC AO
FLOWS
. WALL ANEURYSM
. ENDOCARDITIS
COARCTATION of AORTA
 AO NARROWING AT






DUCTAL AREA
PROXIMAL
HYPERTENSION
LV HYPERTROPHY
BICUSPID AO V association
DUCTAL TISSUE
INVOLVEMENT
POOR/DELAYED LEG
PULSES
SUPRASTERNAL
TOE
EBSTEINS
 Failure of TV leaflets to form of
endocardium
 Large sail-like leaflets –
regurgitation
 Abnormal tethering – stenosis
 Small RV, huge RA
 Reduced PA flow
 Arrythmias
 LV dysfunction
 Cyanosis if PFO present
FALLOTS TETRALOGY
VSD, PS , RVH , DEXTROPOSITION of AO
Fallots tetralogy
LARGE VSD, OVERIDING AORTA, PULMONARY
OBSTRUCTION
CONGENITALLY CORRECTED
TRANSPOSITION
VENTRICLES SIDE BY SIDE
CRUX APPEARS REVERSED
GREAT ARTERIES ARE PARALLEL
AO IS ANTERIOR + TO LEFT
TR
RV is systemic
VSD, PS, TR , RV DYSFUNCTION
UNIVENTRICULAR HEART
 RV or LV TYPE
 ONE or TWO AV Valves
 OUTLET OBSTRUCTION
 HEART BLOCK – PACEMAKER
 DYSFUNCTION
POST OPERATIVE
and OTHER ISSUES
FALLOTS
Dis-synchrony , Free PR
FALLOTS
 RV DIS-SYNCHRONY
 RV DILATATION
 PR
 ARRYTHMIAS – RVOT VT
 Long QRS
 SUDDEN DEATH
CCTGA
SYSTEMIC RV and TR
HEART BLOCK
TGA with atrial baffle
SYSTEMIC RV
HEART BLOCK
ATRIAL ARRYTHMIA
PUMP FAILURE
LONG STANDING TR
UNIVENTRICULAR REPAIR
 ATRIAL/ SYSTEMIC VENOUS
PLUMBING –
FONTAN TYPE OP
(requires low LA pressure)
 ARRYTHMIAS
 DIS SYNCHRONY
 AV VALVE REGURGITATION
 PUMP FAILURE
Restricted to older patients.
Classical Fontan
Connects right atrial
appendage directly to main
pulmonary artery.
Any ventriculo-pulmonary
connection is divided.
Present day situation
Univentricular repair
UNIVENTRICULAR REPAIR
 REQUIRES LOW LA PRESSURE
 TRANSPULMONARY GRADIENT IS
MAINTAINED
 LV function MUST BE GOOD
 NO DIS SYNCHRONY
 MINIMAL AV VALVE REGURGITATION
 ECHO 4 F’S
Re synchronisation
VENTRICULAR FUNCTION
VENTRICULAR FUNCTION 3D
VALVE REPAIR 3D
MORE LIKE MRI
3D STRAIN
GUCHD
 GOOD PATIENT PROCEDURE HISTORY
 DETAILED DESCRIPTION OF ANATOMY
 MULTI SPECIALITY APPROACH
(ECHO,CATH,MRI,ELECTROPHYSIOLOGY)
 VENTRICULAR FUNCTION
 DRUGS
 PREGNANCY
 LIFESTYLE