ADULT CONGENITAL HEART DISEASE. Stuart Lilley

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1 ADULT CONGENITAL HEART DISEASE Stuart Lilley

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3 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!!

4 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

5 Congenital Heart Disease VSD PDA ASD PS AS COARCTATION TGA TETRALOGY Percentage liveborn

6 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

7 Important Anatomical features

8 Left SVC

9 ADULT TYPES L-R SHUNTS OBSTRUCTIONS Muscular/membrane, valve and supravalve,and Arterial REGURGITATION VENTRICULAR FUNCTION

10 ASD L-R Shunt at Atrial level Right heart enlargement Late development Pulmonary Artery Hypertension Arrythmias

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12 Echo Appearances

13 ASD aneurysmal

14 ASD

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16 ASD Sinus Venosus - LPAPVD

17 TOE

18 DEVICE CLOSURE

19 TOE device placement

20 PFO - TOE CONTRAST STUDY VALSALVA

21 EDGE IDENTIFICATION SIZE SINGLE/MULTIPLE ANEURYSM IDENTIFICATION

22 VSD PERIMEMBRANOUS MUSCULAR/TRABECULAR SUB AORTIC SUB ARTERIAL DOUBLY COMITTED ANTERIOR MUSCULAR POSTERIOR APICAL INLET

23 VSD

24 VSD SMALL ANEURYSM MUSCULAR ENLARGED LV AND LA PULMONARY PRESSURE ENDOCARDITIS RISK

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29 TOE

30 AVSD 1 PARTIAL L- R ATRIAL SHUNT 2 COMPLETE L- R ATRIAL + VENTRICULAR SHUNT AV VALVE ABNORMALITY CHORDAL ARRANGEMENT SUB AORTIC STENOSIS DOWNS SYNDOME

31 AVSD

32 AVSD

33 PARTIAL AVSD

34 COMMON ORIFICE AVSD

35 Calculations RV /PA pressure Doppler Tricuspid regurgitation/ VSD signal L-R Shunt size Doppler mean velocity

36 PA PRESSURE ASD Tricuspid regurgitation ( TR) spectral VSD TR or VSD spectral Arterial Duct (AD) - TR or AD spectral NEED QUALITY SIGNALS

37 TR 2M/S = 16mmHg

38 VSD Spectral Doppler RVp = mmHg

39 EISENMENGER SYNDROME

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41 SHUNT SIZE ESTIMATE PULMONARY FLOW / SYSTEMIC FLOW 1:1 DOPPLER CALCULATIONS

42 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

43 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

44 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

45 ARTERIAL DUCT DESC AO LPA L-R SHUNT LEFT HEART ENLARGEMENT LARGE SHUNTS PRODUCE PAH CONTINOUS SHUNT LEFT PARASTERNAL SUPRASTERNAL

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48 DEVICE CLOSURE

49 PAH - EISENMENGER

50 AORTIC STENOSIS VALVE

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52 RE GROWTH

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54 BICUSPID AO VALVE ECCENTRIC AO FLOWS. WALL ANEURYSM. ENDOCARDITIS

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57 COARCTATION of AORTA AO NARROWING AT DUCTAL AREA PROXIMAL HYPERTENSION LV HYPERTROPHY BICUSPID AO V association DUCTAL TISSUE INVOLVEMENT POOR/DELAYED LEG PULSES SUPRASTERNAL

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61 TOE

62 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

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64 FALLOTS TETRALOGY VSD, PS, RVH, DEXTROPOSITION of AO

65 Fallots tetralogy LARGE VSD, OVERIDING AORTA, PULMONARY OBSTRUCTION

66 CONGENITALLY CORRECTED TRANSPOSITION

67 VENTRICLES SIDE BY SIDE

68 CRUX APPEARS REVERSED GREAT ARTERIES ARE PARALLEL AO IS ANTERIOR + TO LEFT TR RV is systemic

69 VSD, PS, TR, RV DYSFUNCTION

70 UNIVENTRICULAR HEART RV or LV TYPE ONE or TWO AV Valves OUTLET OBSTRUCTION HEART BLOCK PACEMAKER DYSFUNCTION

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73 POST OPERATIVE and OTHER ISSUES

74 FALLOTS Dis-synchrony, Free PR

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77 FALLOTS RV DIS-SYNCHRONY RV DILATATION PR ARRYTHMIAS RVOT VT Long QRS SUDDEN DEATH

78 CCTGA SYSTEMIC RV and TR HEART BLOCK

79 TGA with atrial baffle

80 SYSTEMIC RV HEART BLOCK ATRIAL ARRYTHMIA PUMP FAILURE LONG STANDING TR

81 UNIVENTRICULAR REPAIR ATRIAL/ SYSTEMIC VENOUS PLUMBING FONTAN TYPE OP (requires low LA pressure) ARRYTHMIAS DIS SYNCHRONY AV VALVE REGURGITATION PUMP FAILURE

82 Restricted to older patients. Connects right atrial Classical Fontan appendage directly to main pulmonary artery. Any ventriculo-pulmonary connection is divided.

83 Present day situation Univentricular repair

84 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

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86 Re synchronisation

87 VENTRICULAR FUNCTION

88 VENTRICULAR FUNCTION 3D

89 VALVE REPAIR 3D

90 MORE LIKE MRI

91 3D STRAIN

92 GUCHD GOOD PATIENT PROCEDURE HISTORY DETAILED DESCRIPTION OF ANATOMY MULTI SPECIALITY APPROACH (ECHO,CATH,MRI,ELECTROPHYSIOLOGY) VENTRICULAR FUNCTION DRUGS PREGNANCY LIFESTYLE

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