Difference between revisions of "Heart valves"

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Heart valves (view source)

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[[Image:Gray497.png|thumb|right|Aortic valve - drawing. (WC/Gray's Anatomy)]]
'''[[Heart]] valves''' are the domain of the cardiac surgeon and their bread & butter.
'''[[Heart]] valves''' are the domain of the cardiac surgeon and their bread & butter.


==Clinical==  
=Clinical=
===General===
===General===
*Insufficiency (regurgitant flow) - murmur in diastole.
*Insufficiency (regurgitant flow) - murmur in diastole.
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*Mostly "calcific aortic stenosis".
*Mostly "calcific aortic stenosis".


==Quick approach to valves==
=Quick approach to valves=
===Gross===
===Gross===
*Calcification?
*Calcification?
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**Myxomatous change?
**Myxomatous change?


==Normal histology==
=Normal morphology=
===Aortic valve===
==Aortic valve==
General:
===General===
*Covered by endothelium.
*Covered by endothelium.
*Mostly avascular (nutrients supplied by diffusion).
*Mostly avascular (nutrients supplied by diffusion).


===Gross===
Terminology:
Terminology:
*Base - closest to the aortic wall.
*Base - closest to the aortic wall.
*Free edge - closest to the centre of the valve/interacts with other valve cusps.
*Free edge - closest to the centre of the valve/interacts with other valve cusps.


Cusps:
*Left - has LMCA ostium.
*Right - has RCA ostium.
*Posterior (non-coronary cusp).
Note:
*The [[cut-up]] is described in ''[[grossing aortic valves]]''.
===Microscopic===
Three layers (from proximal (ventricular side) to distal (valsalva side)):<ref name=Ref_PBoD558>{{Ref PBoD|558}}</ref>
Three layers (from proximal (ventricular side) to distal (valsalva side)):<ref name=Ref_PBoD558>{{Ref PBoD|558}}</ref>
#Ventricularis.
#Ventricularis.
Line 58: Line 68:
*The loading of the ventricular aspect is tensile and the valsalva side compressive. Thus, it makes sense that the tissue on the ventricular aspect is good in tensile loading and the tissue on the valsalva side good in compression.  The elastic tissue can be thought of as [http://en.wikipedia.org/wiki/Rebar rebar]... the collagen as concrete.
*The loading of the ventricular aspect is tensile and the valsalva side compressive. Thus, it makes sense that the tissue on the ventricular aspect is good in tensile loading and the tissue on the valsalva side good in compression.  The elastic tissue can be thought of as [http://en.wikipedia.org/wiki/Rebar rebar]... the collagen as concrete.


===Mitral valve===
Image:
*[http://www.e-heart.org/Photos/01_Cardiac_Structure_Photos/%C2%A9Mitral%20Valve%20Normal%20Chordae%20Tendinea%20640%20x%20419.jpg Normal mitral valve (e-heart.org)].<ref>URL: [http://www.e-heart.org/pages/01_cardiac_structure/01_Cardiac_Structure_MV_003.htm http://www.e-heart.org/pages/01_cardiac_structure/01_Cardiac_Structure_MV_003.htm]. Accessed on: 20 December 2012.</ref>
 
==Mitral valve==
===Gross===
===Gross===
*Cordae tendinae.
*Cordae tendinae.
Line 64: Line 77:
**No fusion.  
**No fusion.  


===Histology===
Note:
*Similar to the aortic valve.
*The [[cut-up]] is described in ''[[grossing mitral valves]]''.
===Microscopic===
Similar to the aortic valve - layers:
#Atrialis.<ref name=pmid6821906 >{{Cite journal  | last1 = Chesler | first1 = E. | last2 = King | first2 = RA. | last3 = Edwards | first3 = JE. | title = The myxomatous mitral valve and sudden death. | journal = Circulation | volume = 67 | issue = 3 | pages = 632-9 | month = Mar | year = 1983 | doi =  | PMID = 6821906 | URL = http://circ.ahajournals.org/content/67/3/632.full.pdf }}</ref>
#Spongiosa.
#Fibrosa.


=Degenerative conditions=
==Calcific aortic stenosis==
==Calcific aortic stenosis==
*Abbreviated ''CAS''.
{{Main|Calcific aortic stenosis}}
==Localized dystrophic heart valve amyloidosis==
===General===
===General===
*Somewhat similar to [[atherosclerosis]]; however, considered a separate entity.<ref>{{cite journal |author=Otto CM |title=Calcific aortic stenosis--time to look more closely at the valve |journal=N. Engl. J. Med. |volume=359 |issue=13 |pages=1395-8 |year=2008 |month=September |pmid=18815402 |doi=10.1056/NEJMe0807001 |url=}}</ref>
*Common:
*Mitral valve is usually normal.
**~75<ref>{{Cite journal | last1 = Kristen | first1 = AV. | last2 = Schnabel | first2 = PA. | last3 = Winter | first3 = B. | last4 = Helmke | first4 = BM. | last5 = Longerich | first5 = T. | last6 = Hardt | first6 = S. | last7 = Koch | first7 = A. | last8 = Sack | first8 = FU. | last9 = Katus | first9 = HA. | title = High prevalence of amyloid in 150 surgically removed heart valves--a comparison of histological and clinical data reveals a correlation to atheroinflammatory conditions. | journal = Cardiovasc Pathol | volume = 19 | issue = 4 | pages = 228-35 | month = | year = | doi = 10.1016/j.carpath.2009.04.005 | PMID = 19502085 }}</ref>-100%<ref name=pmid7223424>{{Cite journal  | last1 = Falk | first1 = E. | last2 = Ladefoged | first2 = C. | last3 = Christensen | first3 = HE. | title = Amyloid deposits in calcified aortic valves. | journal = Acta Pathol Microbiol Scand A | volume = 89 | issue = 1 | pages = 23-6 | month = Jan | year = 1981 | doi =  | PMID = 7223424 }}
*Most common cause of aortic stenosis.
</ref> of valves with calcific degeneration, i.e. [[calcific aortic stenosis]].
 
**~50% of valves with post-rheumatic sclerosis.<ref name=pmid6190729>{{Cite journal  | last1 = Cooper | first1 = JH. | title = Localized dystrophic amyloidosis of heart valves. | journal = Hum Pathol | volume = 14 | issue = 7 | pages = 649-53 | month = Jul | year = 1983 | doi =  | PMID = 6190729 }}</ref>
DDx of aortic stenosis:
*Not seen in acute disease and healed endocarditis.<ref name=pmid6190729/>
#Calcific aortic stenosis.  
#[[Bicuspid aortic valve]] with calcific aortic stenosis.
#[[Rheumatic heart disease]].


Clinical (mnemonic ''SAD''):
Hypothesis:
*Syncope.
*Amyloid deposition in the aortic valve is the precursor lesion of [[calcific aortic stenosis]].<ref name=pmid7223424/>
*Angina.
*Dyspnea (shortness of breath) - first symptom.


===Microscopic===
===Microscopic===
Features:<ref name=Ref_PBoD590>{{Ref PBoD|590}}</ref>
Features:<ref name=pmid6190729/>
*Affects the valsalva side of the valve.
*Pink amorphous material - '''key feature'''.
**It affects the fibrosa.
**Usually around the calcific foci.
*Primarily at the base of the valve, i.e. there is relative sparing the free edge.


==Myxomatous degeneration==
DDx:
===General===
*Systemic [[amyloidosis]], e.g. [[senile systemic amyloidosis]], AL-amyloidosis.
*Usually affects the mitral valve.
*Female > male,<ref>URL: [http://emedicine.medscape.com/article/759004-overview http://emedicine.medscape.com/article/759004-overview]. Accessed on: 8 June 2010.</ref> disputed by Toronto data.<ref name=leong>{{cite journal |author=Leong SW, Soor GS, Butany J, Henry J, Thangaroopan M, Leask RL |title=Morphological findings in 192 surgically excised native mitral valves |journal=Can J Cardiol |volume=22 |issue=12 |pages=1055-61 |year=2006 |month=October |pmid=17036100 |doi= |url=}}</ref>
*Associated with [[Marfan's syndrome]] and [[Turner syndrome]] (Monosomy X).<ref>{{cite journal |author=Wigle ED, Rakowski H, Ranganathan N, Silver MC |title=Mitral valve prolapse |journal=Annu. Rev. Med. |volume=27 |issue= |pages=165–80 |year=1976 |pmid=779595 |doi=10.1146/annurev.me.27.020176.001121 |url=}}</ref>


===Gross===
===Stains===
Features:<ref name=Ref_PBoD591>{{Ref PBoD|591}}</ref>
*Congo red +ve.
*No commissural fusion.
**Commissural fusion typical of rheumatic heart disease.
*Thickened.
*Rubbery consistency.
*Reactive/secondary changes.
**Fibrosis due to prolapse/abnormal contact of valve with other structures.
**Clots/organized thrombus - due to stasis.


===Microscopic===
Dystrophic amyloid:<ref name=pmid6190729/>
*Thinning of ''fibrosa layer''.
*Alcian blue -ve.
*Thickening of ''spongiosa layer'' with mucoid (myxomatous) material. (key feature).
*Periodic acid-Schiff -ve.
*+/-Secondary changes (due to valvular dysfunction): thrombi, fibrosis.


====Staining====
==Myxomatous degeneration==
*Movat stain.
{{Main|Myxomatous degeneration}}
**Acid fuchsin, alcian blue, crocein scarlet, elastic hematoxylin, pathology consultation, and saffron.<ref>URL: [http://www.mayomedicallaboratories.com/test-catalog/Overview/9832 http://www.mayomedicallaboratories.com/test-catalog/Overview/9832]. Accessed on: 8 June 2010.</ref><ref name=penn_med>Modified Movat's Pentachrome Stain. University Penn Medicine. URL: [http://www.med.upenn.edu/mcrc/histology_core/movat.shtml http://www.med.upenn.edu/mcrc/histology_core/movat.shtml]. Accessed on: January 29, 2009.</ref>


Interpretation of Movat stain:<ref name=penn_med/>
=Infective conditions=
*Black = nuclei and elastic fibers.
==Rheumatic heart disease==
*Yellow = collagen and reticular fibers.
{{Main|Rheumatic heart disease}}
*Blue = mucin, ground substance.
*Red (intense) = fibrin.
*Red = muscle.


Image:
==Infective endocarditis==
*[http://commons.wikimedia.org/wiki/File:Myxomatous_aortic_valve.jpg Myxomatous valve (WC)].
{{Main|Infective endocarditis}}


==Rheumatic heart disease==
=Non-infective conditions=
==Mitral valve prolapse==
*Abbreviated ''MVP''.
*[[AKA]] ''floppy mitral valve''.<ref name=pmid11433820>{{Cite journal  | last1 = Boudoulas | first1 = H. | last2 = Wooley | first2 = CF. | title = Floppy mitral valve/mitral valve prolapse/mitral valvular regurgitation: effects on the circulation. | journal = J Cardiol | volume = 37 Suppl 1 | issue =  | pages = 15-20 | month =  | year = 2001 | doi =  | PMID = 11433820 }}</ref>
===General===
===General===
*Classically leads to mitral valve stenosis.
*Classically young women.
**Rheumatic fever accounts for 99% of mitral stenosis.<ref name=Ref_PBoD594>{{Ref PBoD|594}}</ref>
*Afflicts ~ 3% of population in the USA.<ref name=Ref_PCPBoD8_296>{{Ref PCPBoD8|296}}</ref>  
*Disease less frequent today - as streptococcal pharynigits is treated.
 
Clinical:
*Pansystolic murmur.
*+/-Left ventricular hypertrophy - secondary to MVP.
 
Complications:<ref name=Ref_PCPBoD8_296>{{Ref PCPBoD8|296}}</ref>
*[[Infective endocarditis]].
*Congestive heart failure.
*[[Sudden cardiac death]].<ref name=pmid21133272>{{Cite journal  | last1 = Franchitto | first1 = N. | last2 = Bounes | first2 = V. | last3 = Telmon | first3 = N. | last4 = Rougé | first4 = D. | title = Mitral valve prolapse and out-of-hospital sudden death: a case report and literature review. | journal = Med Sci Law | volume = 50 | issue = 3 | pages = 164-7 | month = Jul | year = 2010 | doi =  | PMID = 21133272 }}</ref>
*Thromboembolism.


===Gross===
===Gross===
*"Fish-mouth appearance".
Features - any of the following:<ref name=pmid3049284>{{Cite journal  | last1 = Virmani | first1 = R. | last2 = Atkinson | first2 = JB. | last3 = Forman | first3 = MB. | title = The pathology of mitral valve prolapse. | journal = Herz | volume = 13 | issue = 4 | pages = 215-26 | month = Aug | year = 1988 | doi =  | PMID = 3049284 }}</ref>
**Slit-like morphology; elliptical cross-sectional flow area (mitral valve) has an abnormally small semi-minor axis<ref>URL: [http://en.wikipedia.org/wiki/Ellipse http://en.wikipedia.org/wiki/Ellipse]. Accessed on: 13 November 2010.</ref> axis due to valve thickening.
# "Intrachordal hooding" = ballooning/bulging of leaflet between chordal attachments.
**Image: [http://www.principia-eng.com/services/construction/IMG_3098.jpg Fish-mouth appearance - pipe (principia-eng.com)].
# Hooding or doming of the body of the leftlet into the left atrium.
*Significant valvular thickening.
#* Extreme concavity of the valve when seen from the left ventricle.
*Thickening of the cordae tendinae.
# Elongated leaflets/large valve area.
# Dilated valve annulus.  
*Thickening of the valve.
*+/-Left ventricular hypertrophy.
 
Note - location:
*Posterior leaflet pathology more common than anterior leaflet pathology.<ref name=pmid3049284/>


*Images:  
Image:
** [http://en.wikipedia.org/wiki/File:Rheumatic_heart_disease,_gross_pathology_20G0013_lores.jpg Rheumatic heart disease at autopsy - showing valvular thickening and thickening of the cordae tendinae (WP)].
*[http://www.e-heart.org/Pages/06_Valvular_Disease/06_Valvular_Disease_MV_Acquired_MVP_001.htm MVP - intrachordal hooding (e-heart.org)].
**[http://commons.wikimedia.org/wiki/File:Aortic_stenosis_rheumatic,_gross_pathology_20G0014_lores.jpg Rheumatic heart disease - showing valvular thickening - aortic valve (WP)].


===Microscopic===
===Microscopic===
Features:<ref name=Ref_PBoD593>{{Ref PBoD|593}}</ref>
Features:<ref name=pmid3049284/><ref name=Ref_PCPBoD8_296>{{Ref PCPBoD8|296}}</ref>
*Caterpillar cell ([[AKA]] Anitschkow cells)
*Increased thickness of spongiosa layer. ‡
**Abundant eosinophilic cytoplasm.
*Thinning of the fibrosa layer.
**Moderately-poorly defined cell border.
*+/-Fibrin deposition - atrial aspect.
**Well-defined central ovoid nucleus with a prominent wavy ribbon-like chromatin -- looks vaguely like a caterpillar with some imagination.
**Pathognomonic for rheumatic fever.


*Aschoff bodies:
Notes:
**Usually in the heart itself,
*‡ The Tthicking may be due to superimposed fibrosis, instead of spongiosa layer thickening.<ref name=pmid24316086>{{Cite journal  | last1 = Roberts | first1 = WC. | last2 = Vowels | first2 = TJ. | last3 = Ko | first3 = JM. | last4 = Hebeler | first4 = RF. | title = Gross and histological features of excised portions of posterior mitral leaflet in patients having operative repair of mitral valve prolapse and comments on the concept of missing (= ruptured) chordae tendineae. | journal = J Am Coll Cardiol | volume = 63 | issue = 16 | pages = 1667-74 | month = Apr | year = 2014 | doi = 10.1016/j.jacc.2013.11.017 | PMID = 24316086 }}</ref>
**Jumbled collagen, eosinophilic, and
**Surrounded by lymphocytes (T cells) +/- plasma cells.


Images:  
DDx:
*[http://commons.wikimedia.org/wiki/File:Aschoff_Body_in_Rheumatic_Myocarditis.jpg Aschoff body (WC)].
*[[Myxomatous degeneration]].{{fact}}
*[http://en.wikipedia.org/wiki/File:Anitschkow_Myocytes_in_an_Aschoff_Body,_Rheumatic_Myocarditis.jpg Anitschkow myocytes (WC)].


==Infective endocarditis==
==Nonbacterial thrombotic endocarditis==
*Abbreviated ''NBTE''.
*[[AKA]] ''marantic endocarditis''.<ref name=pmid15919332>{{Cite journal  | last1 = Borowski | first1 = A. | last2 = Ghodsizad | first2 = A. | last3 = Cohnen | first3 = M. | last4 = Gams | first4 = E. | title = Recurrent embolism in the course of marantic endocarditis. | journal = Ann Thorac Surg | volume = 79 | issue = 6 | pages = 2145-7 | month = Jun | year = 2005 | doi = 10.1016/j.athoracsur.2003.12.024 | PMID = 15919332 }}</ref>
===General===
===General===
*Infection of the endocardium - often involves the valves (which are covered by endocardium).
Associations:
*Before the time of antibiotics -- 100% fatal.
*Cardiac catheterization.<ref name=pmid7303049>{{Cite journal  | last1 = Young | first1 = RS. | last2 = Zalneraitis | first2 = EL. | title = Marantic endocarditis in children and young adults: clinical and pathological findings. | journal = Stroke | volume = 12 | issue = 5 | pages = 635-9 | month =  | year =  | doi =  | PMID = 7303049 }}</ref>
*Embolization.<ref name=pmid15919332/>
*Malignancy - leading cause, usu. adenocarcinoma.<ref name=pmid2012496>{{Cite journal  | last1 = Dickens | first1 = P. | last2 = Chan | first2 = AC. | title = Nonbacterial thrombotic endocarditis in Hong Kong Chinese. | journal = Arch Pathol Lab Med | volume = 115 | issue = 4 | pages = 359-61 | month = Apr | year = 1991 | doi =  | PMID = 2012496 }}</ref>


====Clinical====
Note:
*Diagnosed (clinically) using the ''Duke criteria''.<ref>[http://www.medcalc.com/endocarditis.html http://www.medcalc.com/endocarditis.html]</ref><ref>{{cite journal |author=Durack DT, Lukes AS, Bright DK |title=New criteria for diagnosis of infective endocarditis: utilization of specific echocardiographic findings. Duke Endocarditis Service |journal=Am. J. Med. |volume=96 |issue=3 |pages=200-9 |year=1994 |month=March |pmid=8154507 |doi= |url=}}</ref>
*''Marantic'' = wasting away.
**Positive blood cultures.
**Cardiac involvement - vegetation.
**+/-Febrile.


===Microscopic===
===Gross===
*Inflammatory infiltrate (key feature @ low power):
*Round non-destructive vegetations, usually at the line of closure.<ref name=Ref_PBoD8_567>{{Ref PBoD8|567}}</ref>
**+/-Plasma cells.
**+/-Neutrophils.
*Microorganisms - '''key feature''' (diagnostic).
**Hard to see (even at high power).
 
===Stains===
*[[GMS stain]] (Gomori Methenamine-silver stain).
**Look for [[fungi]].
*[[Gram stain]].
**Look for bacteria.
 
==Nonbacterial thrombotic endocarditis==
===General===
*Abbreviated ''NBTE''.
*May be associated with catheterization.


===Microscopic===
===Microscopic===
Features:
Features:
*No inflammation.
*Vegetation without inflammation and microorganisms.
*No organisms.


==Libman-Sacks endocarditis==
==Libman-Sacks endocarditis==
===General===
===General===
*Associated with [[systemic lupus erythematosus]].<ref>URL: [http://dictionary.reference.com/browse/libman-sacks+endocarditis http://dictionary.reference.com/browse/libman-sacks+endocarditis]. Accessed on: 24 May 2011.</ref>
*Associated with [[systemic lupus erythematosus]].
**Seen in approximately in 1/10 SLE cases by echocardiography.<ref name=pmid19946116/>
*Affects the mitral and aortic valves.<ref name=pmid19089657>{{Cite journal  | last1 = Lee | first1 = JL. | last2 = Naguwa | first2 = SM. | last3 = Cheema | first3 = GS. | last4 = Gershwin | first4 = ME. | title = Revisiting Libman-Sacks endocarditis: a historical review and update. | journal = Clin Rev Allergy Immunol | volume = 36 | issue = 2-3 | pages = 126-30 | month = Jun | year = 2009 | doi = 10.1007/s12016-008-8113-y | PMID = 19089657 }}</ref>
**Mitral valve most commonly affected.<ref name=pmid8608627>{{Cite journal  | last1 = Hojnik | first1 = M. | last2 = George | first2 = J. | last3 = Ziporen | first3 = L. | last4 = Shoenfeld | first4 = Y. | title = Heart valve involvement (Libman-Sacks endocarditis) in the antiphospholipid syndrome. | journal = Circulation | volume = 93 | issue = 8 | pages = 1579-87 | month = Apr | year = 1996 | doi =  | PMID = 8608627 }}</ref>
*It has been suggested that it may be a manifestation of APLA syndrome.<ref name=pmid19089657/><ref name=pmid8608627/>
 
Clinical:
*Usually regurgitation.


===Gross===
===Gross===
*Vegetation. (???)
*Vegetations anywhere on the valve surface<ref name=pmid19946116>{{Cite journal  | last1 = Plastiras | first1 = SC. | last2 = Pamboucas | first2 = CA. | last3 = Tektonidou | first3 = M. | last4 = Toumanidis | first4 = ST. | title = Real-time three-dimensional echocardiography in evaluating Libman-Sacks vegetations. | journal = Eur J Echocardiogr | volume = 11 | issue = 2 | pages = 184-5 | month = Mar | year = 2010 | doi = 10.1093/ejechocard/jep172 | PMID = 19946116 }}</ref> - often seen on both sides (flow surface & non-flow surface).<ref>{{Ref PBoD8|567}}</ref>
*Flat, pale brown/tan, usually small.<ref>URL: [http://library.med.utah.edu/WebPath/CVHTML/CV122.html http://library.med.utah.edu/WebPath/CVHTML/CV122.html]. Accessed on: 6 March 2013.</ref>
 
Images:
*[http://media.photobucket.com/image/nonbacterial%20thrombotic%20endocarditis/cat_at_uw/Osler%20-%20Cardiac/libman-sacks.jpg?t=1269402236 Libman-Sacks endocarditis (photobucket.com)].
*[http://library.med.utah.edu/WebPath/CVHTML/CV122.html Libman-Sacks endocarditis (utah.edu)].


===Microscopic===
===Microscopic===
Features:
Features:{{fact}}
*Hematoxylin bodies. (???)
*Fibrin.
*No microorganisms.
*No inflammation.


==Biscupid aortic valve==
==Biscupid aortic valve==
===General===
===General===
*Aortic valve usually tricuspid.
*Aortic valve usually tricuspid.
*1-2% of general population.<ref name=pmid18514024>{{cite journal |author=Vallely MP, Semsarian C, Bannon PG |title=Management of the ascending aorta in patients with bicuspid aortic valve disease |journal=Heart Lung Circ |volume=17 |issue=5 |pages=357-63 |year=2008 |month=October |pmid=18514024 |doi=10.1016/j.hlc.2008.01.007 |url=}}</ref>
 
*Most common congenital heart defect.<ref name=pmid20579534>{{cite journal |author=Siu SC, Silversides CK |title=Bicuspid aortic valve disease |journal=J. Am. Coll. Cardiol. |volume=55 |issue=25 |pages=2789–800 |year=2010 |month=June |pmid=20579534 |doi=10.1016/j.jacc.2009.12.068 |url=}}</ref>
**1-2% of general population.<ref name=pmid18514024>{{cite journal |author=Vallely MP, Semsarian C, Bannon PG |title=Management of the ascending aorta in patients with bicuspid aortic valve disease |journal=Heart Lung Circ |volume=17 |issue=5 |pages=357-63 |year=2008 |month=October |pmid=18514024 |doi=10.1016/j.hlc.2008.01.007 |url=}}</ref>
***Male:female ~ 2:1.<ref name=pmid18799444>{{Cite journal  | last1 = Tzemos | first1 = N. | last2 = Therrien | first2 = J. | last3 = Yip | first3 = J. | last4 = Thanassoulis | first4 = G. | last5 = Tremblay | first5 = S. | last6 = Jamorski | first6 = MT. | last7 = Webb | first7 = GD. | last8 = Siu | first8 = SC. | title = Outcomes in adults with bicuspid aortic valves. | journal = JAMA | volume = 300 | issue = 11 | pages = 1317-25 | month = Sep | year = 2008 | doi = 10.1001/jama.300.11.1317 | PMID = 18799444 }}</ref>
*Inherited in autosomal dominant pattern.
*Inherited in autosomal dominant pattern.
*Most common congenital heart defect.<ref name=pmid20579534>{{cite journal |author=Siu SC, Silversides CK |title=Bicuspid aortic valve disease |journal=J. Am. Coll. Cardiol. |volume=55 |issue=25 |pages=2789–800 |year=2010 |month=June |pmid=20579534 |doi=10.1016/j.jacc.2009.12.068 |url=}}</ref>
**NOTCH1 gene - implicated.<ref name=pmid16025100>{{Cite journal | last1 = Garg | first1 = V. | last2 = Muth | first2 = AN. | last3 = Ransom | first3 = JF. | last4 = Schluterman | first4 = MK. | last5 = Barnes | first5 = R. | last6 = King | first6 = IN. | last7 = Grossfeld | first7 = PD. | last8 = Srivastava | first8 = D. | title = Mutations in NOTCH1 cause aortic valve disease. | journal = Nature | volume = 437 | issue = 7056 | pages = 270-4 | month = Sep | year = 2005 | doi = 10.1038/nature03940 | PMID = 16025100 }}</ref>


===Significance===
Significance:
*Associated with ascending [[aortic aneurysm]]s - x10 risk of [[aortic dissection]] vs. normal population.<ref name=pmid18514024/>  
*Associated with ascending [[aortic aneurysm]]s - x10 risk of [[aortic dissection]] vs. normal population.<ref name=pmid18514024/>  
*30% develop serious morbidity.<ref name=pmid18514024/>
*30% develop serious morbidity.<ref name=pmid18514024/>
*Associated with early development of [[calcific aortic stenosis]].
*Associated with early development of [[calcific aortic stenosis]] - often in 50s.
**Calcific disease in normal (tricuspid) aortic valves is typically seen in the 70s.
*[[Coarctation of the aorta]].<ref name=pmid16129122>{{Cite journal  | last1 = Braverman | first1 = AC. | last2 = Güven | first2 = H. | last3 = Beardslee | first3 = MA. | last4 = Makan | first4 = M. | last5 = Kates | first5 = AM. | last6 = Moon | first6 = MR. | title = The bicuspid aortic valve. | journal = Curr Probl Cardiol | volume = 30 | issue = 9 | pages = 470-522 | month = Sep | year = 2005 | doi = 10.1016/j.cpcardiol.2005.06.002 | PMID = 16129122 }}</ref>
 
===Gross===
Features - either:
#Raphe does not reach the free margin of the cusp.<ref name=Ref_AoGP>{{Ref AoGP|2}}</ref>
#No raphe - uncommon (~7% of cases).<ref name=pmid9987528>{{Cite journal  | last1 = Sabet | first1 = HY. | last2 = Edwards | first2 = WD. | last3 = Tazelaar | first3 = HD. | last4 = Daly | first4 = RC. | title = Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases. | journal = Mayo Clin Proc | volume = 74 | issue = 1 | pages = 14-26 | month = Jan | year = 1999 | doi = 10.4065/74.1.14 | PMID = 9987528 }}</ref>
 
Note:
*''Raphe'' ~ ''suture'' or ''seam''.<ref>URL: [http://dictionary.reference.com/browse/raphe http://dictionary.reference.com/browse/raphe]. Accessed on: 25 February 2012.</ref>
 
Images:
*[http://circ.ahajournals.org/content/106/8/900.full.pdf Bicuspid valve (ahajournals.org)].<ref name=pmid12186790>{{Cite journal  | last1 = Fedak | first1 = PW. | last2 = Verma | first2 = S. | last3 = David | first3 = TE. | last4 = Leask | first4 = RL. | last5 = Weisel | first5 = RD. | last6 = Butany | first6 = J. | title = Clinical and pathophysiological implications of a bicuspid aortic valve. | journal = Circulation | volume = 106 | issue = 8 | pages = 900-4 | month = Aug | year = 2002 | doi =  | PMID = 12186790 | URL = http://circ.ahajournals.org/content/106/8/900.full.pdf }}</ref>
 
===Microscopic===
Features - section through raphe:
*"No evidence of fusion."<ref name=Ref_AoGP>{{Ref AoGP|2}}</ref>
*Elastic fibres through-out (not interrupted by fibrous tissue). (???)
 
Note:
*The clinical impression and gross pathologic impression of bicuspid valve should concur.
 
DDx:
*Senile [[calcific aortic stenosis]] with fusion of leaflets.
 
===Sign out===
<pre>
AORTIC VALVE, REPLACEMENT:
- BICUSPID AORTIC VALVE WITH CALCIFIC STENOSIS.
</pre>
 
====Micro====
The sections show valve tissue with marked calcification of the fibrosa layer. No neutrophils are identified. No microorganisms are identified with routine stains.


==Tumours==
=Heart valve tumours=
{{main|Cardiac tumours}}
{{main|Cardiac tumours}}
[[Papillary fibroelastoma]]s are the most common tumour of the valve.
[[Papillary fibroelastoma]]s are the most common tumour of the valve.


==See also==
=Other=
==Subvalvular membrane==
:''Subvalvular aortic membrane'' redirects here.
===General===
*Rare.
*May lead to [[sudden natural death]].<ref name=pmid16501359>{{Cite journal  | last1 = Turan | first1 = AA. | last2 = Guven | first2 = T. | last3 = Karayel | first3 = F. | last4 = Pakis | first4 = I. | last5 = Gurpinar | first5 = K. | last6 = Ozaslan | first6 = A. | title = Subvalvular aortic stenosis as a cause of sudden death: two case reports. | journal = Am J Forensic Med Pathol | volume = 27 | issue = 1 | pages = 90-2 | month = Mar | year = 2006 | doi = 10.1097/01.paf.0000203301.96511.f7 | PMID = 16501359 }}</ref>
 
Clinical:
*Symptoms of aortic stenosis.
*No ejection sound (as in aortic valvular stenosis).<ref name=pmid5817839>{{Cite journal  | last1 = van der Schaar | first1 = PJ. | last2 = Roos | first2 = JP. | last3 = Rohmer | first3 = J. | title = Subvalvular membranous aortic stenosis. Results of surgical therapy. | journal = Thorax | volume = 24 | issue = 3 | pages = 276-82 | month = May | year = 1969 | doi =  | PMID = 5817839 }}</ref>
 
===Microscopic===
Features:
*Fibrous tissue.<ref name=pmid16501359/><ref name=pmid21881724>{{Cite journal  | last1 = Dearani | first1 = JA. | last2 = Croti | first2 = UA. | last3 = Price | first3 = TN. | last4 = Braile | first4 = DM. | title = Subvalvular aortic membrane resection. | journal = Rev Bras Cir Cardiovasc | volume = 26 | issue = 1 | pages = 135-6 | month =  | year =  | doi =  | PMID = 21881724 }}</ref>
 
Note:
*Similar to valvular tissue.
 
===Stains===
*[[Movat stain]].
 
===Sign out===
<pre>
SUBVALVULAR MEMBRANE, AORTA, EXCISION:
- BENIGN PAUCICELLULAR FIBROUS TISSUE CONSISTENT WITH SUBVALVULAR MEMBRANE.
</pre>
 
=See also=
*[[Heart]].
*[[Heart]].
*[[Cardiomyopathy]].
*[[Cardiomyopathy]].
*[[Forensic pathology]].
*[[Forensic pathology]].


==References==
=References=
{{reflist|2}}
{{reflist|2}}


[[Category:Cardiovascular pathology]]
[[Category:Cardiovascular pathology]]
Michael
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