Richard L. Hallett, MD
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1 Richard L. Hallett, MD Chief, Cardiovascular Imaging Northwest Radiology Network Indianapolis, IN Adjunct Assistant Professor of Radiology Stanford University Hospital and Clinics Stanford, CA NASCI 2016 Baltimore, MD October 15, 2016
2 DISCLOSURES None HANDOUT: Choose folder: NASCI 2016
3 CASE 1 5 yo male with murmur, FTT
4 SYSTOLE DIASTOLE ECG synchronized CTA, HR > 100
5 CASE 1
6 CONGENTIAL UNICUSPID AORTIC VALVE SYS DIAST Line drawings: Petit CJ, et al Am J Cardiol 2016; 117(6)
7 CONGENITAL UNICUSPID AORTIC VALVE
8 CASE 1 QUESTION: WHICH OF THE FOLLOWING IS TRUE REGARDING UNICUSPID AORTIC VALVE? A. Most cases present in adulthood B. UAV have better results from balloon valvuloplasty than bicuspid aortic valve (BAV) C. UAV shares some overlap with BAV patients, including risk of aortopathy and coarctation D. Have larger valve opening area than BAV
9 CASE 1 QUESTION: ANSWER WHICH OF THE FOLLOWING IS TRUE REGARDING UNICUSPID AORTIC VALVE? A. Most cases present in adulthood B. UAV have better results from balloon valvuloplasty than bicuspid aortic valve (BAV) C. UAV shares some overlap with BAV patients, including risk of aortopathy and coarctation D. Have larger valve opening area than BAV Mookadam F, et al. J Heart Valve Dis 2010, 19(6)678-83
10 CONGENITAL UNICUSPID AORTIC VALVE Unicuspid unicommisural (one lateral commisural attachment, slit-like orifice) Unicuspid acomissural (no lateral attachments, pinhole orifice, neonates) Mean age of diagnosis 14 months #1 presentation: CHF #1 lesion: isolated AS (37%) Singh S, et al. Texas Heart J. 2015; 42: Mookadam F, et al. J Heart Valve Dis 2010; 19(6)678-83
11 CONGENITAL UNICUSPID AORTIC VALVE Associated anomalies: Coarctation (37%) VSD (12%) PDA (5%) Aortic Aneurysm (5%) Coronary artery anomalies Singh S, et al. Texas Heart J. 2015; 42: Mookadam F, et al. J Heart Valve Dis 2010; 19(6)678-83
12 CONGENITAL UNICUSPID AORTIC VALVE Unicuspid (or functionally unicuspid) valves 1,2 : Have lower associated LVEF than BAV Lower opening area (AVOA) Greater fusion length Higher incidence of leaflet tears and resultant aortic insufficiency after balloon v-plasty 1. Gao, K, et al. J Invasive Cardiol. 2016; 28(9): Petit CJ, et al Am J Cardiol 2016; 117(6)
13 CASE 2
14
15 BICUSPID AORTIC VALVE
16 SAM QUESTION: Bicuspid aortic valve (BAV) is best characterized as: A. An isolated defect causing aortic valve abnormalities B. A systemic disorder with aortic tissue showing decreased levels of matrix metalloproteinases C. A systemic disorder that shares some histopathologic findings with Marfan syndrome D. A systemic disorder with sporadic heritance
17 SAM QUESTION: ANSWER Bicuspid aortic valve (BAV) is best characterized as: A. An isolated defect causing aortic valve abnormalities B. A systemic disorder with aortic tissue showing decreased levels of matrix metalloproteinases C. A systemic disorder that shares some histopathologic findings with Marfan syndrome D. A systemic disorder with sporadic heritance Hanneman K, et al. Pre- and Postoperative Imaging of the Aortic Root. Radiographics. 2016;36:19 37.
18 BICUSPID AORTIC VALVE 1-2% of the population Decreased Fibrillin-1 levels (M) Increased matrix metalloproteinases (M) NOTCH1 and ACTA2 genes Some inherited - autosomal dominant
19 BICUSPID AORTIC VALVE Aortic Dilatation / Aneurysms Aortic Dissection Aortic Coarctation Family members of BAV patients can show aortic dilatation even though they have trileaflet valves
20 BAV: CLASSIFICATION Sievers and Schmidtke 2007 Classification based on 304 surgical BAV specimens Raphe: conjoint or fused portions of two adjacent leaflets that become a malformed commissure
21 SIEVERS CLASSIFICATION OF BAV
22 SIEVERS 0, LATERAL
23 SIEVERS 1, L-R DIASTOLE SYSTOLE Hallett RL, et al. Curr Radiol Rep (2016) 4:1-14
24 CASE 3 50 M w hx BAV, cerebral abscess, new heart failure
25 CASE 3 50 M w BAV, cerebral abscess
26 INFECTIOUS ENDOCARDITIS (IE) WITH SUBVALVULAR ABSCESS AND AORTO-RIGHT ATRIAL FISTULA HACEK endocarditis
27 CASE 3 QUESTION Regarding HACEK infectious endocarditis (IE), which is true? A. HACEK are gram positive bacteria that are part of normal skin flora B. HACEK organisms cause a majority of infectious endocarditis (IE) cases C. HACEK IE patients tend to be younger than other IE patients D. Outcomes are poorer than other IE patients
28 CASE 3 QUESTION - ANSWER Regarding HACEK infectious endocarditis (IE), which is true? A. HACEK are gram positive bacteria that are part of normal skin flora B. HACEK organisms cause a majority of infectious endocarditis (IE) cases C. HACEK IE patients tend to be younger than other IE patients D. Outcomes are poorer than other IE patients Chambers ST et al. HACEK Infective Endocarditis: Characteristics and Outcomes from a Large, Multi-National Cohort Abbate A, editor. PLoS ONE. 2013;8:e63181.
29 HACEK ENDOCARDITIS Haemophilus spp. Aggregatibacter spp. Cardiobacterium hominus Eikenella corodens Kingella spp.
30 HACEK ENDOCARDITIS Heterogenous group of gram negative fastidious bacteria Frequently colonize oropharyngeal Cause < 5% of IE cases Better outcomes 11% 1-year mortaility (vs 39%) Chambers ST et al. HACEK Infective Endocarditis: Characteristics and Outcomes from a Large, Multi-National Cohort PLoS ONE. 2013;8:e63181.
31 AORTO-RIGHT ATRIAL FISTULA 1,2 Congenital Aneurysm / Dissection Infective endocarditis Aorto-cavernous fistulae in only 1-2% IE pts Staphlyococcus: worse outcomes John ES, et al. Journal of Cardiothoracic Surgery 2014, 9:124 Nadgji GRJ, et al. Heart : 7-10.
32 CASE 4
33 CASE 4 32 yr old male Atypical CP, equivocal stress echo Cath: No vessel coming off R sinus, concern for anomalous coronary artery
34 CASE FOUR
35 QUADRICUSPID AORTIC VALVE
36 QUESTION The most common complication of Quadricuspid Aortic Valve (QAV) is: A. Valvular aortic stenosis B. Aortic regurgitation C. Atrial fibrillation D. Left ventricular hypertrophy Douglas, H., Moore, M., & Purvis, J. (2012). Comprehensive assessment of a quadricuspid aortic valve and coronary arteries by multidetector cardiac CT. Heart, 98(24),
37 QUESTION: ANSWER The most common complication of Quadricuspid Aortic Valve (QAV) is: A. Valvular aortic stenosis B. Aortic regurgitation C. Atrial fibrillation D. Left ventricular hypertrophy Douglas, H., Moore, M., & Purvis, J. Heart, 2012: 98(24),
38 QUADRICUSPID AORTIC VALVE (QAV) Rare, 1/6000 aortic valve surgery patients 1 M = F, avg. age at Dx ~ 50 Classification by size of cusps 2 Most common: 3 same size + 1 smaller cusp (type B) Echo: X -shaped SAX view CT/MR: confirmatory; perform planimetry and/or flow measurement 3 1. Douglas, H., Moore, M., & Purvis, J. (2012) Heart 2012; 98(24), Hurwitz L, et al. Am J Cardiol 1973; 31(5) Khan SK, Tamin SS, Araoz PA. J Comput Assist Tomogr. 35 (5):
39 CLASSIFICATION OF QAV 1 A B C D E F G 4 equal sized cusps 3 equal + 1 smaller (most common) 2 equal + 2 equal smaller 1 large + 2 intermediate + 1 smaller 3 equal + 1 larger 2 equal large + 2 smaller unequal sizes 4 unequal sized cusps 1. Hurwitz L, et al. Am J Cardiol 1973; 31(5)
40 QUADRICUSPID AORTIC VALVE (QAV) Usually isolated, but can be associated with: Single or Anomalous Coronary Arteries Displacement of coronary ostia (from addl cusp) HCM / Subaortic Stenosis PDA, VSD Endocarditis 1. Jagganath AD, et al. Echocardiography 2011; 28(9), Zhu J, et al. J Cardiothor Surg 2013; 8(1) Tutarel O. J. Heart Valve Dis. 2004;13 (4):
41 QUADRICUSPID AORTIC VALVE (QAV) Complications: Aortic Insufficiency (#1) Up to 75% at time of Dx LVH Conduction problems (BBB) TX: Reconstruction and/or Surgical valve replacement 1. Jagganath AD, et al. Echocardiography 2011; 28(9), Zhu J, et al. J Cardiothor Surg 2013; 8(1) Tutarel O. J. Heart Valve Dis. 2004;13 (4):
42 CONCLUSION Whether 1, 2, 3, or 4 cusps, aortic valve pathology can be well assessed by ECG synchronized CTA
43 THANKS FOR YOUR ATTENTION!! HANDOUT: Choose folder: NASCI 2016
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