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 !!

Device closure of ASD & PFO – TTE/TOE The systemic right ventricle - Univentricular repair Pulmonary regurgitation & RV assessment Bicuspid aortic valves Eisenmenger patients - PAH

Important issues in adult echo

Congenital Heart Disease

0 5 10 15 20 25 30 35

TETRALOGY

TGA

COARCTATION

AS

PS

ASD

PDA

VSD

Percentage liveborn

ASD or aneursym 39

VSD or aneurysm 15

Pulmonary stenosis 8

Valve or subaortic stenosis 5

APVD 4

AVSD 4

Arterial duct 4

Coarctation 3

Ebstein’s 1

Tetralogy of Fallot <1

UVH <1

Cor triatriatum <1

CCTGA <1

New diagnosis

Adults with congenital heart disease

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

VALSALVA

CONTRAST STUDY

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

HEART BLOCK ATRIAL ARRYTHMIA PUMP FAILURE LONG STANDING TR PA Banding resynch therapy Transplant

SYSTEMIC RV

UNIVENTRICULAR REPAIR

ATRIAL/ SYSTEMIC VENOUS PLUMBING – FONTAN TYPE OP

(requires low LA pressure)

ARRYTHMIAS

DIS SYNCHRONY

AV VALVE REGURGITATION

PUMP FAILURE

Classical Fontan Restricted to older patients.

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