Difference between revisions of "Heart valves"

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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.
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*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.
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**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>
*Not seen in acute disease and healed endocarditis.<ref name=pmid6190729/>


DDx of aortic stenosis:
Hypothesis:
#Calcific aortic stenosis.
*Amyloid deposition in the aortic valve is the precursor lesion of [[calcific aortic stenosis]].<ref name=pmid7223424/>
#[[Bicuspid aortic valve]] with calcific aortic stenosis.
#[[Rheumatic heart disease]].
 
Clinical (mnemonic ''SAD''):
*Syncope.
*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.


==Heart valve amyloidosis==
DDx:
*[[AKA]] ''amyloidosis of heart valve''.
*Systemic [[amyloidosis]], e.g. [[senile systemic amyloidosis]], AL-amyloidosis.
{{Main|Amyloidosis}}
===General===
*Common:<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>
**~80% of valves with calcific degeneration, i.e. [[calcific aortic stenosis]].
**~50% of valves with post-rheumatic sclerosis.
*Not seen in acute disease, healed endocarditis.<ref name=pmid6190729/>
 
===Microscopic===
Features:
*Pink amorphous material - '''key feature'''.
**Commonly around the calicific foci.<ref name=pmid6190729/>


===Stains===
===Stains===
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==Myxomatous degeneration==
==Myxomatous degeneration==
===General===
{{Main|Myxomatous degeneration}}
*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===
=Infective conditions=
Features:<ref name=Ref_PBoD591>{{Ref PBoD|591}}</ref>
==Rheumatic heart disease==
*No commissural fusion.
{{Main|Rheumatic heart disease}}
**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===
==Infective endocarditis==
*Thinning of ''fibrosa layer''.
{{Main|Infective endocarditis}}
*Thickening of ''spongiosa layer'' with mucoid (myxomatous) material. (key feature).
*+/-Secondary changes (due to valvular dysfunction): thrombi, fibrosis.


====Staining====
=Non-infective conditions=
*Movat stain.
==Mitral valve prolapse==
**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>
*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===
*Classically young women.
*Afflicts ~ 3% of population in the USA.<ref name=Ref_PCPBoD8_296>{{Ref PCPBoD8|296}}</ref>  


Interpretation of Movat stain:<ref name=penn_med/>
Clinical:
*Black = nuclei and elastic fibers.
*Pansystolic murmur.
*Yellow = collagen and reticular fibers.
*+/-Left ventricular hypertrophy - secondary to MVP.
*Blue = mucin, ground substance.
*Red (intense) = fibrin.
*Red = muscle.


Image:
Complications:<ref name=Ref_PCPBoD8_296>{{Ref PCPBoD8|296}}</ref>
*[http://commons.wikimedia.org/wiki/File:Myxomatous_aortic_valve.jpg Myxomatous valve (WC)].
*[[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.


==Rheumatic heart disease==
===Gross===
===General===
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>
*Classically leads to mitral valve stenosis.
# "Intrachordal hooding" = ballooning/bulging of leaflet between chordal attachments.
**Rheumatic fever accounts for 99% of mitral stenosis.<ref name=Ref_PBoD594>{{Ref PBoD|594}}</ref>
# Hooding or doming of the body of the leftlet into the left atrium.
*Disease less frequent today - as streptococcal pharynigits is treated.
#* Extreme concavity of the valve when seen from the left ventricle.
# Elongated leaflets/large valve area.
# Dilated valve annulus.
*Thickening of the valve.
*+/-Left ventricular hypertrophy.


===Gross===
Note - location:
*"Fish-mouth appearance".
*Posterior leaflet pathology more common than anterior leaflet pathology.<ref name=pmid3049284/>
**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.
**Image: [http://www.principia-eng.com/services/construction/IMG_3098.jpg Fish-mouth appearance - pipe (principia-eng.com)].
*Significant valvular thickening.
*Thickening of the cordae tendinae.


*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>


==Tumours==
===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.
 
=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]]

Latest revision as of 01:14, 26 July 2016

Aortic valve - drawing. (WC/Gray's Anatomy)

Heart valves are the domain of the cardiac surgeon and their bread & butter.

Clinical

General

  • Insufficiency (regurgitant flow) - murmur in diastole.
  • Stenosis (decreased flow area) - murmur in systole.

Pathology

Which valves cause the most trouble?

  • Mostly those on the left side (subjected to higher pressures), i.e. mitral valve (or left atrioventricular v.) and aortic valve.

Aortic stenosis - cause?

  • Mostly "calcific aortic stenosis".

Quick approach to valves

Gross

  • Calcification?
    • Consider calcific stenosis.
  • Vegetations?
    • Consider infective endocarditis.
  • Thin (see-through)?
    • Consider myxomatous change.

Microscopic

  • Inflammation?
    • Consider endocarditis.
  • Anitschkow's cells (caterpillar cells)?
    • Rheumatic heart disease.
  • Aschoff bodies?
    • Rheumatic heart disease.
  • Thickening of spongiosa (layer)?
    • Myxomatous change?

Normal morphology

Aortic valve

General

  • Covered by endothelium.
  • Mostly avascular (nutrients supplied by diffusion).

Gross

Terminology:

  • Base - closest to the aortic wall.
  • 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:

Microscopic

Three layers (from proximal (ventricular side) to distal (valsalva side)):[1]

  1. Ventricularis.
    • Elastic tissue.
  2. Spongiosa.
    • Loose connective tissue.
  3. Fibrosa.
    • Mostly collagen, thickest part in a normal valve.

Notes:

  • 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 rebar... the collagen as concrete.

Image:

Mitral valve

Gross

  • Cordae tendinae.
    • Should be thin.
    • No fusion.

Note:

Microscopic

Similar to the aortic valve - layers:

  1. Atrialis.[3]
  2. Spongiosa.
  3. Fibrosa.

Degenerative conditions

Calcific aortic stenosis

  • Abbreviated CAS.
Main article: Calcific aortic stenosis

Localized dystrophic heart valve amyloidosis

General

  • Common:
  • Not seen in acute disease and healed endocarditis.[6]

Hypothesis:

Microscopic

Features:[6]

  • Pink amorphous material - key feature.
    • Usually around the calcific foci.

DDx:

Stains

  • Congo red +ve.

Dystrophic amyloid:[6]

  • Alcian blue -ve.
  • Periodic acid-Schiff -ve.

Myxomatous degeneration

Main article: Myxomatous degeneration

Infective conditions

Rheumatic heart disease

Main article: Rheumatic heart disease

Infective endocarditis

Main article: Infective endocarditis

Non-infective conditions

Mitral valve prolapse

  • Abbreviated MVP.
  • AKA floppy mitral valve.[7]

General

  • Classically young women.
  • Afflicts ~ 3% of population in the USA.[8]

Clinical:

  • Pansystolic murmur.
  • +/-Left ventricular hypertrophy - secondary to MVP.

Complications:[8]

Gross

Features - any of the following:[10]

  1. "Intrachordal hooding" = ballooning/bulging of leaflet between chordal attachments.
  2. Hooding or doming of the body of the leftlet into the left atrium.
    • Extreme concavity of the valve when seen from the left ventricle.
  3. Elongated leaflets/large valve area.
  4. Dilated valve annulus.
  • Thickening of the valve.
  • +/-Left ventricular hypertrophy.

Note - location:

  • Posterior leaflet pathology more common than anterior leaflet pathology.[10]

Image:

Microscopic

Features:[10][8]

  • Increased thickness of spongiosa layer. ‡
  • Thinning of the fibrosa layer.
  • +/-Fibrin deposition - atrial aspect.

Notes:

  • ‡ The Tthicking may be due to superimposed fibrosis, instead of spongiosa layer thickening.[11]

DDx:

Nonbacterial thrombotic endocarditis

  • Abbreviated NBTE.
  • AKA marantic endocarditis.[12]

General

Associations:

  • Cardiac catheterization.[13]
  • Embolization.[12]
  • Malignancy - leading cause, usu. adenocarcinoma.[14]

Note:

  • Marantic = wasting away.

Gross

  • Round non-destructive vegetations, usually at the line of closure.[15]

Microscopic

Features:

  • Vegetation without inflammation and microorganisms.

Libman-Sacks endocarditis

General

  • Associated with systemic lupus erythematosus.
    • Seen in approximately in 1/10 SLE cases by echocardiography.[16]
  • Affects the mitral and aortic valves.[17]
    • Mitral valve most commonly affected.[18]
  • It has been suggested that it may be a manifestation of APLA syndrome.[17][18]

Clinical:

  • Usually regurgitation.

Gross

  • Vegetations anywhere on the valve surface[16] - often seen on both sides (flow surface & non-flow surface).[19]
  • Flat, pale brown/tan, usually small.[20]

Images:

Microscopic

Features:[citation needed]

  • Fibrin.
  • No microorganisms.
  • No inflammation.

Biscupid aortic valve

General

  • Aortic valve usually tricuspid.
  • Most common congenital heart defect.[21]
    • 1-2% of general population.[22]
      • Male:female ~ 2:1.[23]
  • Inherited in autosomal dominant pattern.
    • NOTCH1 gene - implicated.[24]

Significance:

Gross

Features - either:

  1. Raphe does not reach the free margin of the cusp.[26]
  2. No raphe - uncommon (~7% of cases).[27]

Note:

  • Raphe ~ suture or seam.[28]

Images:

Microscopic

Features - section through raphe:

  • "No evidence of fusion."[26]
  • Elastic fibres through-out (not interrupted by fibrous tissue). (???)

Note:

  • The clinical impression and gross pathologic impression of bicuspid valve should concur.

DDx:

Sign out

AORTIC VALVE, REPLACEMENT:
- BICUSPID AORTIC VALVE WITH CALCIFIC STENOSIS.

Micro

The sections show valve tissue with marked calcification of the fibrosa layer. No neutrophils are identified. No microorganisms are identified with routine stains.

Heart valve tumours

Main article: Cardiac tumours

Papillary fibroelastomas are the most common tumour of the valve.

Other

Subvalvular membrane

Subvalvular aortic membrane redirects here.

General

Clinical:

  • Symptoms of aortic stenosis.
  • No ejection sound (as in aortic valvular stenosis).[31]

Microscopic

Features:

Note:

  • Similar to valvular tissue.

Stains

Sign out

SUBVALVULAR MEMBRANE, AORTA, EXCISION:
- BENIGN PAUCICELLULAR FIBROUS TISSUE CONSISTENT WITH SUBVALVULAR MEMBRANE.

See also

References

  1. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 558. ISBN 0-7216-0187-1.
  2. ↑ URL: http://www.e-heart.org/pages/01_cardiac_structure/01_Cardiac_Structure_MV_003.htm. Accessed on: 20 December 2012.
  3. ↑ Chesler, E.; King, RA.; Edwards, JE. (Mar 1983). "The myxomatous mitral valve and sudden death.". Circulation 67 (3): 632-9. PMID 6821906.
  4. ↑ Kristen, AV.; Schnabel, PA.; Winter, B.; Helmke, BM.; Longerich, T.; Hardt, S.; Koch, A.; Sack, FU. et al. "High prevalence of amyloid in 150 surgically removed heart valves--a comparison of histological and clinical data reveals a correlation to atheroinflammatory conditions.". Cardiovasc Pathol 19 (4): 228-35. doi:10.1016/j.carpath.2009.04.005. PMID 19502085.
  5. ↑ 5.0 5.1 Falk, E.; Ladefoged, C.; Christensen, HE. (Jan 1981). "Amyloid deposits in calcified aortic valves.". Acta Pathol Microbiol Scand A 89 (1): 23-6. PMID 7223424.
  6. ↑ 6.0 6.1 6.2 6.3 Cooper, JH. (Jul 1983). "Localized dystrophic amyloidosis of heart valves.". Hum Pathol 14 (7): 649-53. PMID 6190729.
  7. ↑ Boudoulas, H.; Wooley, CF. (2001). "Floppy mitral valve/mitral valve prolapse/mitral valvular regurgitation: effects on the circulation.". J Cardiol 37 Suppl 1: 15-20. PMID 11433820.
  8. ↑ 8.0 8.1 8.2 Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 296. ISBN 978-1416054542.
  9. ↑ Franchitto, N.; Bounes, V.; Telmon, N.; Rougé, D. (Jul 2010). "Mitral valve prolapse and out-of-hospital sudden death: a case report and literature review.". Med Sci Law 50 (3): 164-7. PMID 21133272.
  10. ↑ 10.0 10.1 10.2 Virmani, R.; Atkinson, JB.; Forman, MB. (Aug 1988). "The pathology of mitral valve prolapse.". Herz 13 (4): 215-26. PMID 3049284.
  11. ↑ Roberts, WC.; Vowels, TJ.; Ko, JM.; Hebeler, RF. (Apr 2014). "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.". J Am Coll Cardiol 63 (16): 1667-74. doi:10.1016/j.jacc.2013.11.017. PMID 24316086.
  12. ↑ 12.0 12.1 Borowski, A.; Ghodsizad, A.; Cohnen, M.; Gams, E. (Jun 2005). "Recurrent embolism in the course of marantic endocarditis.". Ann Thorac Surg 79 (6): 2145-7. doi:10.1016/j.athoracsur.2003.12.024. PMID 15919332.
  13. ↑ Young, RS.; Zalneraitis, EL.. "Marantic endocarditis in children and young adults: clinical and pathological findings.". Stroke 12 (5): 635-9. PMID 7303049.
  14. ↑ Dickens, P.; Chan, AC. (Apr 1991). "Nonbacterial thrombotic endocarditis in Hong Kong Chinese.". Arch Pathol Lab Med 115 (4): 359-61. PMID 2012496.
  15. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 567. ISBN 978-1416031215.
  16. ↑ 16.0 16.1 Plastiras, SC.; Pamboucas, CA.; Tektonidou, M.; Toumanidis, ST. (Mar 2010). "Real-time three-dimensional echocardiography in evaluating Libman-Sacks vegetations.". Eur J Echocardiogr 11 (2): 184-5. doi:10.1093/ejechocard/jep172. PMID 19946116.
  17. ↑ 17.0 17.1 Lee, JL.; Naguwa, SM.; Cheema, GS.; Gershwin, ME. (Jun 2009). "Revisiting Libman-Sacks endocarditis: a historical review and update.". Clin Rev Allergy Immunol 36 (2-3): 126-30. doi:10.1007/s12016-008-8113-y. PMID 19089657.
  18. ↑ 18.0 18.1 Hojnik, M.; George, J.; Ziporen, L.; Shoenfeld, Y. (Apr 1996). "Heart valve involvement (Libman-Sacks endocarditis) in the antiphospholipid syndrome.". Circulation 93 (8): 1579-87. PMID 8608627.
  19. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 567. ISBN 978-1416031215.
  20. ↑ URL: http://library.med.utah.edu/WebPath/CVHTML/CV122.html. Accessed on: 6 March 2013.
  21. ↑ Siu SC, Silversides CK (June 2010). "Bicuspid aortic valve disease". J. Am. Coll. Cardiol. 55 (25): 2789–800. doi:10.1016/j.jacc.2009.12.068. PMID 20579534.
  22. ↑ 22.0 22.1 22.2 Vallely MP, Semsarian C, Bannon PG (October 2008). "Management of the ascending aorta in patients with bicuspid aortic valve disease". Heart Lung Circ 17 (5): 357-63. doi:10.1016/j.hlc.2008.01.007. PMID 18514024.
  23. ↑ Tzemos, N.; Therrien, J.; Yip, J.; Thanassoulis, G.; Tremblay, S.; Jamorski, MT.; Webb, GD.; Siu, SC. (Sep 2008). "Outcomes in adults with bicuspid aortic valves.". JAMA 300 (11): 1317-25. doi:10.1001/jama.300.11.1317. PMID 18799444.
  24. ↑ Garg, V.; Muth, AN.; Ransom, JF.; Schluterman, MK.; Barnes, R.; King, IN.; Grossfeld, PD.; Srivastava, D. (Sep 2005). "Mutations in NOTCH1 cause aortic valve disease.". Nature 437 (7056): 270-4. doi:10.1038/nature03940. PMID 16025100.
  25. ↑ Braverman, AC.; Güven, H.; Beardslee, MA.; Makan, M.; Kates, AM.; Moon, MR. (Sep 2005). "The bicuspid aortic valve.". Curr Probl Cardiol 30 (9): 470-522. doi:10.1016/j.cpcardiol.2005.06.002. PMID 16129122.
  26. ↑ 26.0 26.1 Rose, Alan G. (2008). Atlas of Gross Pathology with Histologic Correlation (1st ed.). Cambridge University Press. pp. 2. ISBN 978-0521868792.
  27. ↑ Sabet, HY.; Edwards, WD.; Tazelaar, HD.; Daly, RC. (Jan 1999). "Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases.". Mayo Clin Proc 74 (1): 14-26. doi:10.4065/74.1.14. PMID 9987528.
  28. ↑ URL: http://dictionary.reference.com/browse/raphe. Accessed on: 25 February 2012.
  29. ↑ Fedak, PW.; Verma, S.; David, TE.; Leask, RL.; Weisel, RD.; Butany, J. (Aug 2002). "Clinical and pathophysiological implications of a bicuspid aortic valve.". Circulation 106 (8): 900-4. PMID 12186790.
  30. ↑ 30.0 30.1 Turan, AA.; Guven, T.; Karayel, F.; Pakis, I.; Gurpinar, K.; Ozaslan, A. (Mar 2006). "Subvalvular aortic stenosis as a cause of sudden death: two case reports.". Am J Forensic Med Pathol 27 (1): 90-2. doi:10.1097/01.paf.0000203301.96511.f7. PMID 16501359.
  31. ↑ van der Schaar, PJ.; Roos, JP.; Rohmer, J. (May 1969). "Subvalvular membranous aortic stenosis. Results of surgical therapy.". Thorax 24 (3): 276-82. PMID 5817839.
  32. ↑ Dearani, JA.; Croti, UA.; Price, TN.; Braile, DM.. "Subvalvular aortic membrane resection.". Rev Bras Cir Cardiovasc 26 (1): 135-6. PMID 21881724.
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