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BICUSPID AORTIC VALVE DISEASE

. . BICUSPID AORTIC VALVE DISEASE. . . . FREQUENCY OF BAV. -BAV is the most common congenital heart defect with a frequency of between 0.9-2% in the general population-There is a male predominance of approximately 3:1. . . GENETICS. -Numerous studies have documented familial clustering of BAV with a prevalence in first-degree relatives of 9-21%-The genetics appear to complex with BAV of multiple types due to mutations of different genes with variable patterns of inheritance. .

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BICUSPID AORTIC VALVE DISEASE

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    2. BICUSPID AORTIC VALVE DISEASE

    3. FREQUENCY OF BAV -BAV is the most common congenital heart defect with a frequency of between 0.9-2% in the general population -There is a male predominance of approximately 3:1

    4. GENETICS -Numerous studies have documented familial clustering of BAV with a prevalence in first-degree relatives of 9-21% -The genetics appear to complex with BAV of multiple types due to mutations of different genes with variable patterns of inheritance.

    5. STRUCTURAL ABNORMALITY OF BAV -The BAV is made up of two unequal sized leaflets -The larger leaflet has a central raphe that results from fusion of one of the commissures. -This commisural fusion results in two leaflets oriented anterior posterior or right and left. Rarely there is no raphe with two leaflets.

    6. Normal tricuspid valve

    7. Bicuspid valve

    8. BICUSPID AORTIC VALVE

    9. ASSOCIATED ABNORMALITES -Nonvalvular findings occur in 50% of patients -The most common associated abnormality is dilatation of the thoracic aorta (Aortopathy) -Other abnormalities include coarctation of the aorta (50% of patients with coarctation will have a BAV), ASD, VSD, PDA, supravavular stenosis.

    10. DIAGNOSIS OF BAV -Phys Exam: Basilar early systolic click caused by doming of the valve. Possible murmurs of AS (SEM loudest at the base) or AI (high frequency diastolic decrecendo murmur). Findings of Coarctation. -2D echo- usually confirms dx with sensitivity of 92% and specificity of 96%. If necessary 3D echo or MRI can be done if there are questions.

    11. CLINICAL COURSE -During childhood sx are infrequent and seen in only 1 of 50 children -The frequency of medical (CHF, SBE, CV sx, CVA) or surgical (AVR or aortic surgery) complications increase with age. This was 25% at age 44yo in one study and 30% at age 50yo in a second.

    12. CLINICAL COURSE -In a Mayo Clinic study 27% of adults with no significant disease at baseline required surgery in the next 20 years. -It is felt that eventually the majority will require surgery.

    13. LATE OUTCOMES BAV

    14. LATE OUTCOMES BAV

    15. AORTIC STENOSIS -process of endothelial dysfunction, inflammation, lipoprotein deposition, calcification and ossification of the aortic side for the leaflet. This is the same process as in trileaflet valves. -this process is accelerated by folding and creasing of the abnormal leaflets leading to earlier presentation in bicuspid valves. (25years earlier than trileaflet valves)

    16. AORTIC INSUFFICENCY -much less frequent than AS -isolated AI is uncommon especially as a cause for intervention - in one series the frequency of mod to severe AI (with or without AS) was 21%

    17. AORTOPATHY(AORTIC DILATATION) -Prevalence-variable depending on study and age of subjects. -Canadian study- 35% (avg age 35) -UCLA study- 53% (avg age 45) -degree of dilatation greater than AS patients with trileaflet valves

    18. AORTOPATHY -the etiology is unclear but probably includes degeneration of the extracellular matrix of the aorta. “Poststenotic dilatation” is no longer accepted as the etiology. -aortic dilatation can occur in the presence of a normally functioning BAV

    19. AORTOPATHY -the area of dilatation can be isolated to the root, tubular ascending aorta or transverse aorta or all three. In one series the most common finding was dilatation of all three (45%). -occurs in First Degree Relatives with a normal AV. UCLA series showed that 21% of FDR had aortic dilatation. Prior studies reported a much lower rate of 3%.

    20. AORTIC DISSECTION -the incidence of this is low (0.1% per pt year) -however it is 5-10x more frequent in patients with BAV than in patients with trileaflet valves -in patients presenting with aortic dissection, BAVs represent 7-15% of the cases. This is equal to or greater than the number of dissections due to Marfan’s Syndrome due to the prevalence of BAV

    21. SURVIVAL With close follow up and appropriate treatment of the valvular abnormalities and the aortopathy, the survival of adult patients with BAV is not different from the general population.

    22. FOLLOW-UP Annual echo for pts with significant valve disease or aortic diameter >4.0cm Echo every 2 years if no significant valve disease and aortic diameter<4.0cm Newer recommendations index the aortic diameter to BSA. Complete imaging of the aorta “periodically” in view of possible involvement of the aorta above that seen on most 2D echos. Need to keep in mind the radiation exposure from a CT scan.

    23. FOLLOW-UP In the future new biomarkers may be of help in follow up and risk assessment of pts with BAV 6) Current AHA recommendations includes screening of first degree relatives for both BAV and aortic dilatation.

    24. MEDICAL THERAPY -Beta blockers may slow the progression of the aortopathy. This is not a clear recommendation for BAV as it is for Marfan’s. It is II-A. -ARBs have been shown to slow aortic dilatation in animal studies but no studies have been published in humans. -vasodilators are recommended in patients with significant AI and concomitant hypertension.

    25. SURGICAL THERAPY -tends to occur at an earlier age than in patients with degenerative forms of valvular disease. (in one study 25yrs earlier) -indications for surgery are the same as for degenerative disease -30% will require concomitant aortic surgery. The indication for aortic surgery is a lower (4.5cm) aortic diameter if the primary indication for surgery is AS or AI.

    26. SURGICAL THERAPY -aortic surgery is indicated if the aorta is >5cm or is enlarging at >0.5cm/year. Newer recommendations will index the aortic diameter to BSA. A lower threshold for surgery in BAV pts is recommended by some -the Ross procedure is not recommended due to frequent concomitant involvement of the media of the pulmonary artery

    27. TAKE HOME POINTS BAV is the most common congenital heart defect 2) Complications include AS, AI, IE and Aortic dissection/aneurysm 3) Over their lifetime >50% of patients will require an intervention

    28. TAKE HOME POINTS 4) Complications increase age so continued follow up is required 5) Aortopathy can occur with or without severe valve disease and must be evaluated. 6) First degree relatives should be screened for aortopathy and BAV with a 2D echo

    29. Thank-you, are there any questions/

    30. References Siu SC, Silversides CK. Bicuspid Aortic Valve Disease. JACC 2010;55;2789-2800. Available at http://content.onlinejacc.org/cgi/content Biner S et al. Aortopathy is Prevalent in Relatives of Bicuspid Aortic Valve Patients. JACC 2009;53;2288-2295. Available at http://content.onlinejacc.org/cgi/content

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