Imaging Assessment of the Pulmonary Valve in Stenosis/Atresia and Regurgitation
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1 Imaging Assessment of the Pulmonary Valve in Stenosis/Atresia and Regurgitation Craig E Fleishman, MD FACC FASE The Heart Center at Arnold Palmer Hospital for Children SCAI Fall Fellows Course 2014 Las Vegas
2 Disclosure Information Imaging assessment of the pulmonary valve in stenosis/atresia and regurgitation Craig E Fleishman MD, FACC, FASE As a faculty member for this program, I disclose the following relationships with industry: (GRS): Grant/Research Support (C): Consultant (SB): Speaker s Bureau (MSH): Major Stock Holder (AB): Advisory Board (E): Employment (O):Other Financial or Material Support None
3 Anatomy
4 Echocardiography - Focus RVOT region(s) of stenosis Infundibular hypertrophy Pulmonary regurgitation Flow reversal in branch pulm arteries RV size, thickness, and function TV competency TR severity Estimated RV pressure Hepatic vein systolic flow reversal RA size LV size and function
5 RV Outflow Obstruction Imaging and Doppler evaluation Dimensions Assess for multiple levels of obstruction and max/mean gradient Infundibulum Valve Supravalve Branch PA s
6 Anatomy Pulmonary Valve Stenosis Before After
7 Pulmonary Valve Stenosis Prevalence = 8-12% of all CHD Familial recurrence rate = 2-4% in 1st degree relatives Noonan s syndrome 50% of patients have form with PS autosomal dominant transmission 25% offspring affected
8 Pulmonary Valve Stenosis Domed 42% Tricuspid - 6% Bicuspid - 10% Unicommissural - 16% Hypoplastic annulus - 6% Dysplastic - 20%
9 Pulmonary Valve Stenosis Post-stenotic dilatation Eccentric Flow Not related to PS severity May extend into LPA May not be present in newborn Not usual with dysplastic PV
10 Pulmonary Valve Stenosis - Severity Severity Trivial Peak Doppler (mmhg) <25 Mild Moderate Severe >60 (70) Ductal dependent, R->LCorrelation, atrial shunt, GoodCritical Echo/Cath variable RV but debate on MIG vs Mean
11 Pulmonary Valve Stenosis - Severity Both MIG and Mean Doppler grad had good correlation with Cath gradient Mean Doppler gradient had significantly less bias Mean Doppler bias -5 mm Hg MIG bias mm Hg J Am Soc Echocardiogr 2005;18:
12 RV Evaluation 2D Echo vs Cath Mean Doppler Gradient Correlated Best with Cath Brown DW et al. JASE 2012;25:383-92
13 Pulmonary Valve Stenosis - Severity
14 Pulmonary Valve Stenosis Natural History PS may progress rapidly during infancy Mild PS rarely progresses after 2 y.o. Valve thickness/mobility does not correlate with progression
15 Pulmonary Valve Stenosis - Progression
16 Pulmonary Regurgitation Assessment from multiple views Width of PR jet Color flow for extent of diastolic flow reversal
17 TOF Pulmonary Regurgitation 52 adult PTS with repaired TOF Echo-Doppler compared to CMR Pulmonary Regurgitation index (PRi)= Time PR/Time of diastole (B/A) PRi correlated to CMR The smaller the ratio, the worse the PR Li W et al. Am Heart J 2004;147:165-72
18 Right Ventricle Size relative to LV RV free wall thickness Function RV fractional area Δ Volume 3D Echo MRI Key vol ml/m(2) RVEDVi RVESVi 80-90
19 Real Time 3D Echo RV Volume Limitations of 3DE RV Vol Visualization of endocardium Field of view Volumes/sec
20 3DE RV Volume Freehand 2DE Dragulescu A et al. JASE 2011;24:
21 CMR RV Dilatation Geva Journal of Cardiovascular Magnetic Resonance :9
22 CMR PR Fraction Geva Journal of Cardiovascular Magnetic Resonance :9
23 CMR RV Evaluation Geva Journal of Cardiovascular Magnetic Resonance :9
24 RV Evaluation 2D Echo vs CMR Good correlation of 3 Point PR scale with CMR Brown DW et al. JASE 2012;25:383-92
25 RV Evaluation 2D Echo vs CMR RV Dias Area 30 cm2/m2 Brown DW et al. JASE 2012;25: CMR RVEDV 160 ml/m2
26 RV Evaluation 2D Echo vs CMR RV Dias Area 20 cm2/m2 Alghamdi MH et al. JASE 2012;25: CMR RVEDV < 170 ml/m2
27 Pulmonary Atresia/Intact Ventricular Septum /1000 live births Heterogenous presentation Variable RV size and function Variable TV size and competency TR associated with RV failure Variable PV and MPA morphology Variable coronary anatomy
28 Echocardiography PA/IVS - Goal Identify patients for RV decompression vs shunt alone (?vs transplant) Biventricular, 1½ ventricular, vs Fontan physiology RV size/morphology/growth TV size/morphology/growth Initial procedure ultimately depends on identifying RV dependent coronary circulation - angiography
29 Echocardiography - Focus PV size and anatomy Architecture of pulmonary arteries Infundibular anatomy RV to coronary artery sinusoids/fistulas Other coronary artery abnormalities up to 30-50% RV size and morphology (?tri-, bi-, uni-partite) TV size absolute and relative to MV TV competency RV wall thickness RA size Source(s) of pulmonary blood flow Usually single left PDA Tortuosity ASD
30 PA/IVS Pulmonary Valve and Infundibulm
31 PA/IVS Right Ventricle and Tricuspid Valve
32 PA/IVS Right Ventricle and Tricuspid Valve 30 Patients No patients with TV Z -score > -2.5 had RVDCC RV decompression?preop Angiogram Addition of color Doppler identified all with coronary fistula Satou GM et al. Am J Cardiol 2000;85:
33 PA/IVS Right Ventricle and Tricuspid Valve 36 PTS Low TV Z-scores and TV/MV ratio associated with failed biventricular repair TV/MV > 0.5 best predictor of successful biventricular repair Minich L et al. Am J Cardiol 2000;85:
34 PA/IVS Doppler and Coronary Arteries Coronary flow pattern Abnormal branching Ventriculocoronary connections/sinusoids
35 PA/IVS Tricuspid Competency
36 PA/IVS PDA Size Course Branch PA s Candidate for stenting?
37 PA/IVS ASD
38
39 PA/IVS Fetal Evaluation
40 PA/IVS Fetal Evaluation 27 live-born followed prenatal 19 bivent / 8 nonbivent TV/MV diam < 0.7 TV inflow duration (% cardiac cycle) <31.5% RV/LV length < 0.6 RV sinusoids 3/4 Nonbiventricular Roman KS et al. Am J Cardiol 2007;99:
41 PA/IVS Fetal Evaluation Fetal echo 28 PTS TV z-score <-4 differentiated outcome groups TV growth - groups already differentiated by 23 weeks gest age RV:LV width and length also predictive Peterson RE et al. J Am Soc Echocardiogr 2006;19:
42 Biventricular vs Single Ventricle Path Coronary artery stenoses/rv dependent coronary circulation RV size, morphology, and growth potential Absence/muscularization of infundibulum TV size and function
43 Thank You
44 Tricuspid Valve Severity of regurgitation RA size Maximal velocity Check multiple sites Hepatic vein systolic flow reversal
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