ECHO FOR ACHD HOW DOES IT DIFFER - Tam Duc...
Transcript of ECHO FOR ACHD HOW DOES IT DIFFER - Tam Duc...
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ECHO FOR ACHDHOW DOES IT DIFFER
Dr Haifa Abdul Latiff
Consultant Pediatric Cardiologist
Institut Jantung Negara Kuala Lumpur
Malaysia
7th TSC 2019
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INTRODUCTION• Adults with CHD (ACHD) is a newly emerging, fast growing
population
• Improve survival rates of patients with complex CHD –early diagnosis, better treatment results and perioperative care
• 2 groups
– Long term complications from treatment received during childhood (e.g. Post TOF repair, Fontan)
– Presenting in adulthood with complications (defaulted treatment, undiagnosed) e.g. ASD with PHT
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17
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0 100 200 300 400 500 600
ASD
Post TOF Repair
PHT/Eisenmengers
VSD
Others
TOF
Post ASD Closure
Post VSD Closure
PDA
Pulmonary Stenosis
Ebstein's Anomaly
CRHD
ccTGA
Post Rastelli
Post Mustard/Senning
Post Fontan
Post PDA Closure
Post Ebstein Repair
AVSD
PAVSD
Aortic Stenosis
Normal Heart
Coarctation repair
DIAGNOSIS ON REFERRAL
Adult Congenital Heart Disease
IJN NEW CASES - OUTPATIENT
3
OTHER DIAGNOSIS Frequency
CORTRIATRIUM 3
HOCM 5
KAWASAKI DISEASE 3
PAPVD 2
POST IAA REPAIR 2
POST PAPVD REPAIR 1
POST PAVM OCC. 2
POST ROSS / MR 1
TRICUSPID ATRESIA WITH PS 2
TRICUSPID STENOSIS 1
MITRAL VALVE PROLAPSE 5
POST TRUNCUS REPAIR 4
SINGLE VENTRICLE 4
POST COA REPAIR 4
POST GLENN SHUNT 3
POST A. SWITCH 9
PULMONARY ATRESIA 4
POST CAF OCCLUSION 1
VPC'S 1
POST PTBV - PS 2
POST MVR 3
UNIVENTRICULAR HEART 7
AORTIC REGURGITATION 2
PFO 8
PULMONARY EMBOLISM 1
POST ALCAPA 1
COMPLETE HEART BLOCK 2
TRICUSPID REGURGITATION 4
SINUS TACHYCARDIA 2
SVC 1
SVT 1
PULMONARY REGURGITATION 2
POST AVR 1
POST RSOV REPAIR 1
MITRAL REGURGITATION 4
PAIVS 2
CARDIOMYOPATHY 4
DORV-TGA 2
TGA 8
BICUSPID AORTIC VALVE 1
RIGHT ISOMERISM 1
ATRIAL ISOMERISM 1
TGA-IVS 1
DCRV 3
MITRAL STENOSIS 2
POST PPM 1
TRUNCUS ARTERIOSUS 1
INFUNDIBULAR STENOSIS 1
2008 – FEB 2018
ACHD CASES RANGE FROM SIMPLE TO COMPLEX LESIONSPOST SURGICAL REPAIR OR DEVELOPED COMPLICATIONS OF DISEASE Most common: ASD and post TOF repair
Courtesy Dr Geetha
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5
8
26
20
45
60
172
429
427
0 50 100 150 200 250 300 350 400 450 500
Pulmonary Thrombosis/Embolism
PLE/PE
Others
Hemotypsis
Neurological
Infective Endocarditis
Heart Failure
PHT
Arrythmia
COMPLICATION
4
NONE = 3280
OUTPATIENT
Adult Congenital Heart Disease
Hypercyanotic spells 1
Thallasemia trial B 1
G6PD 1
Bilateral ankle edema 1
PPCM 1
Vocal cord dysfunction 3
Residual 2
Asthma 2
Right subclavian aneurysm 2
Mechanical 1
Mechanical valve dysfunction 1
LAA Clot 2
Gastritis 1
Others 1
PV preserved 1
Mediastinitis 1
Free flow PR 3
2008 – FEB 2018
Significant numbers (1/3) suffer from complications of CHD
Courtesy Dr Geetha
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Roles of ECHO in ACHDACHD patients require a lifelong surveillance
Echocardiography is important first line in imaging assessment, easily available non invasive tool
• Determine the diagnosis (explain cause of symptoms) in newly referred patient
• Assess the status of previous intervention/surgery (?functioning)
• Look for potential complications of disease or previous surgery intervention -decide further diagnostic investigations (e.g. TOF PS/PA: free flow PR post TOF correction,
conduit stenosis, dilated aortic root and AR, RV dysfunction, ventricular failure)
• Progression of disease/complications (e.g. conduit stenosis, failure) that may require intervention or further investigation
• Response to treatment/intervention
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ECHO in ACHD: how does it differ?• Nature of cardiac problem:
– Pediatric only structural and Adult only functional abnormality– ACHD: Structural and functional abnormalities
• Technical problem : challenging window, modified views– Chest deformity, scars– Artificial materials causing acoustic shadows – conduits, patch– Important structures not in usual position e.g. placed behind the sternum : RVOT in post
ASO, conduits/baffles – need modified echo view
• Type of Ventricular failure– Adults – mainly LVF. ECHO assessment, parameters and references are well established– ACHD – mainly RVF. Functional assessment involves a complex ventricular geometry - not
well established
• Normal reference value for echo parameters– Pediatric – Z score– ACHD? Outgrown pediatric age group but adult standard value base on normal heart
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Standard acquisition protocol for TTE ACHDISACHD International J of Cardiology 272(2018) 77-83
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Echocardiography for ACHD
Requires systematic approach
I. Anatomy : situs, position and connections of cardiac segments
II. Structural functions (stenosis, regurgitation)
III. Ventricular functions
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I. Anatomical Assessment
ATRIA SITUS
CARDIAC POSITION
ATRIO-VENTRICULAR CONNECTION
VENTRICULO-ARTERIAL CONNECTION
At least once for a new patient unless therapeutic intervention performed
REQUIRES SWEEP TECHNIQUESNon conventional window /modified viewsInverted image for subcostal and apical 4 chamber
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SEGMENTAL APPROACH: Atrial and Abdominal situs
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SEGMENTAL APPROACH: Cardiac Position
Base-apex axis (ventricular apex): levocardia, mesocardia, dextrocardia
Cardiac apex is independent of cardiac situs
Position in the mediastinum : levoposition, dextroposition, mesoposition, can be determined by lung
pathology e.g. collapsed/hypoplastic lung, diaphragmatic hernia etc.
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SEGMENTAL APPROACH: VA connection
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SEGMENTAL APPROACH: VA connection
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II. Structural functions
• Systemic veins – Anomalous drainage (heterotaxy) or post atrial switch/Glenn/Fontan circuit
• Pulmonary veins drainage : total/ partial anomalous pulmonary venous drainage
• Shunts and complications (PHT) : ASD, VSD, PDA
• AV valves abn. and function (stenosis/regurgitation): Ebstein’sanomaly, Cleft mitral valve, parachute etc, Common AVVs
• LV and RV Outflow tract stenosis/ regurgitation – native or conduit
• Pulmonary artery branches – stenosis, hypoplastic, disconnected
• Aorta – Right arch, aberrant subclavian, hypoplastic, coarctation
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Pulmonary hypertension: look for secondary cause
TR gradient 144mmHgPR gradient 64mmHg
Large perimembranous VSD with bidirectional shunt
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TR gradient 144mmHgPR gradient 64mmHg
Large PDA with birectional shunt
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29 YEARS OLD HEART FAILUREECHO SEVERE ECCENTRIC MR. TR GRADIENT 68mmHg. Moderate PEPREOP ECHO NO CLEAR SUPRASTERNAL VIEWUNDERWENT MV REPAIR 10/6/2016
Post op persistent HPT4/7/2016
10/8/2019
ECHO SIGNS FOR COARCNON PULSATILE ABDOMINAL AORTA ON SC VIEW(SHORT AND LONG AXIS )SMALL ITHMUS - < NECK VESSEL
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40 year old with cyanosis
VSD bidirectional shunt - Eisenmenger’s?
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Tetralogy of Fallot
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Post atrial switch complications
Severe TR due to RV failure or pathology of TV
Baffle leak
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III. Ventricular functionsLV and RV systolic and diastolic functions
CHALLENGES:
• Complex ventricular geometry in ACHD:
– Right ventricle, Single ventricle, altered LV geometry (RV dysfunction, subpulmonary RV (CCTGA, post atrial switch)
– Standard method (M-Mode, Simpson) not reliable
• Normal reference values are based on adults with structurally normal heart
– Important to record serial measurements as patient own baseline and reference to monitor progress or changes in the echo parameters
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Functional assessment for ACHD: challenges• RV systolic functions
– FAC (fractional area change) and TAPSE on 4CH view widely used, reproducible, easy to measure (represents longitudinal contractile function of RV) . TAPSE has prognostic value for Eisenmenger’s but angle dependent, may be influence by TR, abn. RV geometry, recent surgical procedures
– 3DE for function, volume – cannot be utilized in severely dilated heart
– TDI & speckle for regional and global deformation – but its clinical implication in CHD remains to be elucidated
• Diastolic dysfunction– lack of reliable parameters (E:E’ may not be accurate in all CHD as reduced E’ may result of
localized surgical scarring in the septum or free wall rather than impaired global LV diastolic function).
– Lack of standard echo criterial for diastolic function in systemic RV at present
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TOF free flow PR
Non functioning monocusp valveResidual PS/conduit stenosisBranch PA stenosis
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Assessment of RV systolic function
Eur Heart J (2016)37:1182-1195
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Altered LV geometry
29Years, 10 years Post Ebstein’s repairReliability of standard Simpson?
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Adult: asymptomatic previously, pinkC/O palpitations on exertion
Congenitally Corrected Transposition of Great Arteries (CTGA)
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CCTGA – assessment of systolic function
Speckle tracking (?for systemic RV)
Simpson ? reliable
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Systemic RVCCTGA develops severe TR
Post TVREyeballing – RV dysfunction
AV discordance wit severe TRSystemic RV dysfunction or valve abnormality?
Fractional Area Change to measure RV systolic function
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SUMMARY• Echocardiogram for ACHD patients is very challenging for adult and
pediatric cardiologists/CVTs
• Requires comprehensive assessment of cardiac morphology, physiology, pathophysiology and function a in all ACHD patients
• Systematic segmental analysis approach with ventricular function assessment using basic and advance echo modalities (e.g. speckle tracking, 3DE) is recommended in all patients
• A specialized ACHD echo specialist/echocardiographer trained in both adults and pediatric echo is important to complement ACHD program
• Plenty room for research in this field
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Thank you