Death is a Distant Rumor to the Young: The Bicuspid Aortic Valve. Hector I. Michelena, MD Assistant Professor of Medicine NO DISCLOSURES

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1 Death is a Distant Rumor to the Young: The Bicuspid Aortic Valve Hector I. Michelena, MD Assistant Professor of Medicine NO DISCLOSURES

2 Leonardo s notes Royal collection, Queen Elizabeth II

3 Leonardo s notes Royal collection, Queen Elizabeth II

4 After Leonardo s description 1844 Paget peculiar liability of BAV to disease pathologic curiosity 1858 Peacock BAV become thick ossified inducing obstruction and incompetency 1886 Osler BAV develop infective endocarditis 1927 Abbott BAV and aortic dissection

5 Circulation, 1978

6 Mayo Clinic Ao dissection 9 times more common in BAV than TAV

7 The facts MRI 20 patients with nonstenotic BAVs and 20 matched control patients Reduced aortic elasticity J Am Coll Cardiol 2007;49:1660

8 The facts Marfan-like BAV is associated with accelerated degeneration of the aortic media matrix disruption and smooth muscle cell loss MMP activity may be elevated in the aorta of patients with BAV Circulation 2002;106;900

9 Fact: BAV= dilatation of the aorta HEART 2006;92:1496 Ascending aorta Z score 0.4 year Mean age 35 years

10 Possibly the majority of patients with bicuspid aortic valve will develop serious complications The bicuspid valve may be responsible for more deaths and morbidity than the combined effects of all the other congenital heart defects

11 Questions What is the true incidence of aortic dissection in BAV? What is the true incidence of aortic bacterial endocarditis in BAV? Is BAV disease a life-threatening condition?

12 Olmsted County Retrospective-cohort study Geographically-defined population, all echos accounted for All patients with echo-bav included, long follow-up High echocardiography utilization Aorta outcomes ascertained: Medical records(surgical-medical), mailed surveys, telephone calls Deaths: Death certificates, autopsy reports, coroner s cases

13 Complications of the Aorta in Bicuspid Aortic Valve Disease: A 25 Year Follow-up Population Study Michelena, Khanna, Mahoney, Margaryan, Topilsky, Suri, Sundt, Enriquez-Sarano Residents of Olmsted County (Minn), all ages Echo diagnosed and confirmed BAV, 1980 to 1999 January 1980 to December ,230 echocardiograms Mayo Clinic, Rochester 41,687 (13%) Olmsted County 489 BAVs (1% of Olmsted County echocardiograms, 0.3% of the Olmsted County population for 2000) Total 416, 69% male, years, 79% 18 y 84% typical BAV, FU 16 7 years (range to 29 years). (until , death or 20 y) in 95% Survival, BE, AA, AD, ASx,

14 Baseline characteristics by total aorta events Variable Total N= 416 Aorta events Yes N= 84 Aorta events No N= 332 P value Age years 35±21 37 ± ± Males n (%) 289 (69) 66 (79) 220 (69) HTN n (%) 93 (22) 26 (31) 67 (20) 0.04 Smoking n 132 (32) 29 (35) 103 (31) 0.6 (%)* Diabetes n (%) 15 (4) 2 (0.2) 13 (4) 0.5 Coarctationn 30 (7) 12 (14) 18 (5) (%) Other 54 (13) 15 (18) 39 (12) congenital heart 0.15 disease n (%) EF % 62 ± 7 62 ± 7 63 ± Root / ascending aorta diametermm Typical BAV n (%) Aortic regurgitation n (%)** Complete FUn (%) BAV diagnosis ± 8 39 ± ± 7 < (84) 73 (87) 277 (83) (62) 49 (67) 197 (62) (95) 82 (98) 311 (94) (37) 39 (46) 113 (34) 0.04

15 BAV type There was no relation between BAV phenotype and aortic size, sex, aortic complications, AVR, stenosis or regurgitation

16 Survival 92 1 % and 80 3 % at 10 and 25 years after BAV diagnosis Compare survival matched for age and sex

17 P=0.98

18 Bacterial endocarditis % ± 0.5 % Time -Yr 5 ± 2% BE 11 patients (2 died, 9 required AVR) BE 1.4% of typical and 8% of atypical BAVs (p=0.003)

19 Aortic Dissections Variable Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Sex male female male male male Age BAV echo 47 N/A diagnosis - years Age at AD - years Type dissection Ascendi ng Aortic diameter at baseline - mm Time between AVR and dissection Aortic Coarctation Acute ascending Acute ascending 70 Not measured Acute ascending Acute ascending N/A 9 years 13 months 3 days N/A no no no no no Acute Descending Hypertension yes no yes?? yes Smoking yes no no no no BAV type typical typical typical typical typical BAV dysfunction at baseline echo Severe aortic regurgitation Severe aor tic stenosis Moderate aortic stenosis Moderate aortic regurgitation Moderate aortic stenosis

20 Sudden Cardiac Deaths Variable Patient 1 Patient 2 Patient 3 Patient 4 Pat ient 5 Se Age x BAV echo diagnosi - years sage at year death Autops s Cause y of death by death certificat e ascending aorta dimension - f e mal e n Cardiopulmonary o arrest 3 2 mal 7e n Cardiopulmonary o arrest mal 3e n Witnessed o ventricular fib illatio Suspected r n M I mal 6e ye Suspected s Arrhythmic death, no aortic dissectio 7 n 0 f e mal e n Cardiopulm o nary arrest, o suspected MI 4 4 AVR / AAR Yes / Comorbiditie CAD, no s HTN No / HTN, no CAD, CH F Yes / HTN, nocad, CH F No / o No / S/P n MVR, HTN, no CAD, CH CH F F

21 Risk of Aorta Surgery Relative to Aortic Valve Replacement AVR Total Ao Sx AA Sx Ao coar Sx 52 ± 4% Events % ± 4% 23 ± 4% Time years 3 ± 1%

22 Risk of Aortic Aneurysm and Aortic Dissection ± 5% 30 AA AD Events % Time years 1 ± 0.5%

23 Predictors of ALL aortic events Univariate Hazards Ratio Confidence interval P value Age > 50 years < Root or asc Ao > 40 mm baseline < Male sex HTN AR at baseline Multi variate Age > 50 years Root or asc Ao > 40 mm baseline <0.0001

24 Conclusions The largest, population-based, longest follow-up of BAV up to date Survival no different than general population Ao valve dysfunction common, AVR > 50% at 25 years Endocarditis is uncommon, risk 5% at 25 years, but highly morbid Aortic aneurysm is common, risk 40% at 25 years Aortic dissection is rare, risk 1% at 25 years Age (4x) and aorta diameter (7x) at baseline are independent predictors of aorta events Echo surveillance in >50 yo and >40mm

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26 Speculative conclusions Aortic dissection possibly more common in men, dilated aortas, dysfunctional BAV with interval AVR By operating AA, we are saving dissections? Answer = genetics?

27 Limitations Retrospective 80% >18 year-old---not pediatric True Incidence of Aneurysms could be underestimated since 20% did not have follow-up echocardiogram