ECHO FOR ACHDHOW DOES IT DIFFER

Dr Haifa Abdul Latiff

Consultant Pediatric Cardiologist

Institut Jantung Negara Kuala Lumpur

Malaysia

7th TSC 2019

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

482

257

190

182

123

107

91

71

47

53

42

35

30

30

17

15

16

13

13

12

12

13

10

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

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

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

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

Standard acquisition protocol for TTE ACHDISACHD International J of Cardiology 272(2018) 77-83

Echocardiography for ACHD

Requires systematic approach

I. Anatomy : situs, position and connections of cardiac segments

II. Structural functions (stenosis, regurgitation)

III. Ventricular functions

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

SEGMENTAL APPROACH: Atrial and Abdominal situs

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.

SEGMENTAL APPROACH: VA connection

SEGMENTAL APPROACH: VA connection

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

Pulmonary hypertension: look for secondary cause

TR gradient 144mmHgPR gradient 64mmHg

Large perimembranous VSD with bidirectional shunt

TR gradient 144mmHgPR gradient 64mmHg

Large PDA with birectional shunt

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

40 year old with cyanosis

VSD bidirectional shunt - Eisenmenger’s?

Tetralogy of Fallot

Post atrial switch complications

Severe TR due to RV failure or pathology of TV

Baffle leak

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

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

TOF free flow PR

Non functioning monocusp valveResidual PS/conduit stenosisBranch PA stenosis

Assessment of RV systolic function

Eur Heart J (2016)37:1182-1195

Altered LV geometry

29Years, 10 years Post Ebstein’s repairReliability of standard Simpson?

Adult: asymptomatic previously, pinkC/O palpitations on exertion

Congenitally Corrected Transposition of Great Arteries (CTGA)

CCTGA – assessment of systolic function

Speckle tracking (?for systemic RV)

Simpson ? reliable

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

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

Thank you