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 30 35 Percentage liveborn
New diagnosis 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 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 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. Connects right atrial Classical Fontan 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