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| [[File:Siren.gif|30px|link=Aortic stenosis resident survival guide]]|| <br> || <br>
| [[Aortic stenosis resident survival guide|'''Resident'''<br>'''Survival'''<br>'''Guide''']]
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{{Aortic stenosis}}
{{Aortic stenosis}}
{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[User:Mohammed Sbeih|Mohammed A. Sbeih, M.D.]] [mailto:msbeih@wikidoc.org]; {{LG}} '''Assistant Editor-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]
{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[User:Mohammed Sbeih|Mohammed A. Sbeih, M.D.]] [mailto:msbeih@wikidoc.org]; {{LG}}; {{VR}}; {{USAMA}} {{MC}}  '''Assistant Editor-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]


==Overview==
==Overview==
Aortic stenosis can be classified as either '''acquired''' or '''congenital'''. The frequency of causes of [[aortic stenosis]] varies depending on the age of the patient: whereas bicuspid valve is the most common cause of [[aortic stenosis]] in people below 50 years of age, calcified tricuspid valve is more common in older patients.
Aortic stenosis can be either congenital or acquired, and its common causes vary between different age groups and between different populations. [[Rheumatic heart disease]] remains the most common cause of aortic stenosis worldwide.  Calcification of the [[aortic valve]] is the most common cause of aortic stenosis in North America and Europe. While calcification of [[Bicuspid aortic stenosis|congenital bicuspid aortic valve]] is the most common cause of aortic stenosis in people less than 70 years of age, acquired degenerative calcific aortic stenosis is the most common cause in older patients above the age of 70 years.


==Etiology of Aortic Stenosis==
==Causes==
===Valvular Aortic Stenosis:===
===Life Threatening Causes===
====Acquired <ref name="pmid12633546">{{cite journal |author=Cleland JG, Swedberg K, Follath F, Komajda M, Cohen-Solal A, Aguilar JC, Dietz R, Gavazzi A, Hobbs R, Korewicki J, Madeira HC, Moiseyev VS, Preda I, van Gilst WH, Widimsky J, Freemantle N, Eastaugh J, Mason J |title=The EuroHeart Failure survey programme-- a survey on the quality of care among patients with heart failure in Europe. Part 1: patient characteristics and diagnosis |journal=[[European Heart Journal]] |volume=24 |issue=5 |pages=442–63 |year=2003 |month=March |pmid=12633546 |doi= |url=http://eurheartj.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=12633546 |accessdate=2012-04-11}}</ref>====
Life-threatening causes include conditions which may result in death or permanent [[disability]] within 24 hours if left untreated. Aortic stenosis is a progressive disease and does not have a life threatening cause. However, [[complications]] resulting from untreated disease name is common.
*[[Calcific aortic valve disease|Calcific degeneration]]
*[[Rheumatic fever]]
*[[Infective endocarditis]]
*[[SLE|Systemic lupus erythromatosis]]
*[[Fabry's disease]]
*[[Aortic sclerosis]]
*[[Alkaptonuria]]<ref name="pmid22100375">{{cite journal| author=Hannoush H, Introne WJ, Chen MY, Lee SJ, O'Brien K, Suwannarat P et al.| title=Aortic stenosis and vascular calcifications in alkaptonuria. | journal=Mol Genet Metab | year= 2012 | volume= 105 | issue= 2 | pages= 198-202 | pmid=22100375 | doi=10.1016/j.ymgme.2011.10.017 | pmc=PMC3276068 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22100375  }} </ref>
*[[Paget's disease]]<ref>Strickberger SA, Schulman SP, Hutchins GM. Association of Paget’s disease of bone with calcific aortic valve disease. Am J Med 1987;82:953-6.</ref>


====Congenital <ref name="pmid12633546">{{cite journal |author=Cleland JG, Swedberg K, Follath F, Komajda M, Cohen-Solal A, Aguilar JC, Dietz R, Gavazzi A, Hobbs R, Korewicki J, Madeira HC, Moiseyev VS, Preda I, van Gilst WH, Widimsky J, Freemantle N, Eastaugh J, Mason J |title=The EuroHeart Failure survey programme-- a survey on the quality of care among patients with heart failure in Europe. Part 1: patient characteristics and diagnosis |journal=[[European Heart Journal]] |volume=24 |issue=5 |pages=442–63 |year=2003 |month=March |pmid=12633546 |doi= |url=http://eurheartj.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=12633546 |accessdate=2012-04-11}}</ref>====
===Common Causes===
*[[Bicuspid aortic valve]]
Common causes of aortic stenosis may include:<ref name="pmid16709942">{{cite journal |author=O'Brien KD |title=Pathogenesis of calcific aortic valve disease: a disease process comes of age (and a good deal more) |journal=[[Arteriosclerosis, Thrombosis, and Vascular Biology]] |volume=26 |issue=8 |pages=1721–8 |year=2006 |month=August |pmid=16709942 |doi=10.1161/01.ATV.0000227513.13697.ac |url=http://atvb.ahajournals.org/cgi/pmidlookup?view=long&pmid=16709942 |accessdate=2012-04-11}}</ref><ref name="pmid22685681">{{cite journal| author=Mordi I, Tzemos N| title=Bicuspid aortic valve disease: a comprehensive review. | journal=Cardiol Res Pract | year= 2012 | volume= 2012 | issue=  | pages= 196037 | pmid=22685681 | doi=10.1155/2012/196037 | pmc=3368178 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22685681  }} </ref><ref name="Lugiano-2013">{{Cite journal | last1 = Lugiano | first1 = CA. | title = Aortic stenosis. | journal = JAAPA | volume = 26 | issue = 11 | pages = 46-7 | month = Nov | year = 2013 | doi = 10.1097/01.JAA.0000436518.69169.8e | PMID = 24153092 }}</ref><ref name="uas">VOC=VITIUM ORGANICUM CORDIS, a compendium of the Department of Cardiology at Uppsala Academic Hospital. By Per Kvidal September 1999, with revision by Erik Björklund May 2008</ref>
*Hypoplastic annulus
*[[Calcific aortic valve disease]]
*Three cuspid valve with commissural fusion
*A congenitally abnormal aortic valve with superimposed calcification (unicuspid or [[Bicuspid Aortic Valve|bicuspid]]).
*[[Rheumatic fever|Rheumatic heart disease]]


===Subvalvular Aortic Stenosis:===
===Causes by Location of Stenosis===
*Membranous diaphragm
====Valvular Aortic Stenosis====
*[[Hypertrophic cardiomyopathy]]
*Acquired
**[[Alkaptonuria]]<ref name="pmid22100375">{{cite journal| author=Hannoush H, Introne WJ, Chen MY, Lee SJ, O'Brien K, Suwannarat P et al.| title=Aortic stenosis and vascular calcifications in alkaptonuria. | journal=Mol Genet Metab | year= 2012 | volume= 105 | issue= 2 | pages= 198-202 | pmid=22100375 | doi=10.1016/j.ymgme.2011.10.017 | pmc=PMC3276068 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22100375 }} </ref>
**[[Aortic sclerosis]]<ref name="Aronow-2013">{{Cite journal | last1 = Aronow | first1 = WS. | title = A review of the pathophysiology, diagnosis, and treatment of aortic valve stenosis in elderly patients. | journal = Hosp Pract (1995) | volume = 41 | issue = 4 | pages = 66-77 | month = Oct | year = 2013 | doi = 10.3810/hp.2013.10.1082 | PMID = 24145591 }}</ref>
**[[Hyperuricemia]]<ref name="pmid17921746">{{cite journal| author=Moura LM, Maganti K, Puthumana JJ, Rocha-Gonçalves F, Rajamannan NM| title=New understanding about calcific aortic stenosis and opportunities for pharmacologic intervention. | journal=Curr Opin Cardiol | year= 2007 | volume= 22 | issue= 6 | pages= 572-7 | pmid=17921746 | doi=10.1097/HCO.0b013e3282f0dae6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17921746  }} </ref>
**[[Paget's disease]]<ref>Strickberger SA, Schulman SP, Hutchins GM. Association of Paget’s disease of bone with calcific aortic valve disease. Am J Med 1987;82:953-6.</ref>
**[[Radiation]] <ref name="pmid24597417">{{cite journal| author=Ong DS, Aertker RA, Clark AN, Kiefer T, Hughes GC, Harrison JK et al.| title=Radiation-associated valvular heart disease. | journal=J Heart Valve Dis | year= 2013 | volume= 22 | issue= 6 | pages= 883-92 | pmid=24597417 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24597417  }} </ref>
**[[Rheumatic fever]]<ref name="uas">VOC=VITIUM ORGANICUM CORDIS, a compendium of the Department of Cardiology at Uppsala Academic Hospital. By Per Kvidal September 1999, with revision by Erik Björklund May 2008</ref>
**[[Systemic lupus erythematosus]]<ref name="pmid23884983">{{cite journal| author=Bert JS, Abdullah M, Dahle TG, Gertner E| title=Transcatheter aortic valve replacement for advanced valvular disease in active SLE and APS. | journal=Lupus | year= 2013 | volume= 22 | issue= 10 | pages= 1046-9 | pmid=23884983 | doi=10.1177/0961203313498797 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23884983  }} </ref>


===Supravalvular Aortic Stenosis:===
*Congenital
*Hypoplasia of aorta
**[[Bardet-Biedl syndrome]]<ref name="pmid10874630">{{cite journal| author=Beales PL, Elcioglu N, Woolf AS, Parker D, Flinter FA| title=New criteria for improved diagnosis of Bardet-Biedl syndrome: results of a population survey. | journal=J Med Genet | year= 1999 | volume= 36 | issue= 6 | pages= 437-46 | pmid=10874630 | doi= | pmc=1734378 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10874630  }} </ref>
*Hourglass constriction of aorta
**[[Bicuspid aortic valve]]<ref name="pmid22685681">{{cite journal| author=Mordi I, Tzemos N| title=Bicuspid aortic valve disease: a comprehensive review. | journal=Cardiol Res Pract | year= 2012 | volume= 2012 | issue=  | pages= 196037 | pmid=22685681 | doi=10.1155/2012/196037 | pmc=3368178 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22685681  }} </ref>
*Fibromembranous aortic lesion
**[[Fabry's disease]]<ref name="pmid7596372">{{cite journal| author=Nakao S, Takenaka T, Maeda M, Kodama C, Tanaka A, Tahara M et al.| title=An atypical variant of Fabry's disease in men with left ventricular hypertrophy. | journal=N Engl J Med | year= 1995 | volume= 333 | issue= 5 | pages= 288-93 | pmid=7596372 | doi=10.1056/NEJM199508033330504 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7596372  }} </ref>
**[[Familial hypercholesterolemia]]<ref name="pmid6331147">{{cite journal| author=Sprecher DL, Schaefer EJ, Kent KM, Gregg RE, Zech LA, Hoeg JM et al.| title=Cardiovascular features of homozygous familial hypercholesterolemia: analysis of 16 patients. | journal=Am J Cardiol | year= 1984 | volume= 54 | issue= 1 | pages= 20-30 | pmid=6331147 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6331147  }} </ref>
**[[Familial thoracic aortic aneurysm|Familial thoracic aortic aneurysm syndrome]]<ref name="pmid17676603">{{cite journal| author=Loscalzo ML, Goh DL, Loeys B, Kent KC, Spevak PJ, Dietz HC| title=Familial thoracic aortic dilation and bicommissural aortic valve: a prospective analysis of natural history and inheritance. | journal=Am J Med Genet A | year= 2007 | volume= 143A | issue= 17 | pages= 1960-7 | pmid=17676603 | doi=10.1002/ajmg.a.31872 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17676603  }} </ref>
**[[Aortic valve#Disease of the aortic valve|Hypoplastic aortic annulus]]<ref name="REEVE-1964">{{Cite journal | last1 = REEVE | first1 = R. | last2 = ROBINSON | first2 = SJ. | title = HYPOPLASTIC ANNULUS--AN UNUSUAL TYPE OF AORTIC STENOSIS: A REPORT OF THREE CASES IN CHILDREN. | journal = Dis Chest | volume = 45 | issue = | pages = 99-102 | month = Jan | year = 1964 | doi = | PMID = 14114644 }}</ref>
**Subaortic stenosis short stature syndrome (Onat syndrome) <ref name="urlSubaortic stenosis short stature syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program">{{cite web |url=https://rarediseases.info.nih.gov/diseases/405/subaortic-stenosis-short-stature-syndrome |title=Subaortic stenosis short stature syndrome &#124; Genetic and Rare Diseases Information Center (GARD) – an NCATS Program |format= |work= |accessdate=}}</ref>
**[[Quadricuspid aortic valve]]<ref name="pmid27278482">{{cite journal| author=Yamagishi Y, Yuda S, Tsuchihashi K, Saitoh S, Miura T, Ura N et al.| title=Quadricuspid aortic valve associated with aortic stenosis and regurgitation: report of a case and a review of the literature. | journal=J Med Ultrason (2001) | year= 2007 | volume= 34 | issue= 4 | pages= 197-200 | pmid=27278482 | doi=10.1007/s10396-007-0148-9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27278482  }} </ref>
**[[Singleton-Merten syndrome]]<ref name="pmid23322711">{{cite journal| author=Feigenbaum A, Müller C, Yale C, Kleinheinz J, Jezewski P, Kehl HG et al.| title=Singleton-Merten syndrome: an autosomal dominant disorder with variable expression. | journal=Am J Med Genet A | year= 2013 | volume= 161A | issue= 2 | pages= 360-70 | pmid=23322711 | doi=10.1002/ajmg.a.35732 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23322711  }} </ref>
**[[Sweet syndrome]]<ref name="pmid14521689">{{cite journal| author=Cohen PR, Kurzrock R| title=Sweet's syndrome revisited: a review of disease concepts. | journal=Int J Dermatol | year= 2003 | volume= 42 | issue= 10 | pages= 761-78 | pmid=14521689 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14521689  }} </ref>
**[[Aortic valve#Disease of the aortic valve|Tricuspid aortic valve with commissural fushion]]<ref name="pmid10928509">{{cite journal| author=Bedynek JL, Galioto FM, Leon DF| title=Tricuspid aortic valve with partial commissural fusion: prelminary report of an apparently common cause of aortic ejection sounds with potential for serious sequelae, illustrated by two cases. | journal=Tex Heart Inst J | year= 2000 | volume= 27 | issue= 2 | pages= 201-5 | pmid=10928509 | doi= | pmc=101056 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10928509  }} </ref>
**[[Turner syndrome]]<ref name="pmid4386970">{{cite journal| author=Noonan JA| title=Hypertelorism with Turner phenotype. A new syndrome with associated congenital heart disease. | journal=Am J Dis Child | year= 1968 | volume= 116 | issue= 4 | pages= 373-80 | pmid=4386970 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4386970  }} </ref>
**[[Aortic valve#Disease of the aortic valve|Unicuspid aortic valve]]<ref name="pmid27721957">{{cite journal| author=Kwon HJ, Park JH, Kim SS, Sun BJ, Jin SA, Kim JH et al.| title=Severe Aortic Stenosis Associated with Unicommissural Unicuspid Aortic Valve in a Middle Aged Male. | journal=J Cardiovasc Ultrasound | year= 2016 | volume= 24 | issue= 3 | pages= 247-250 | pmid=27721957 | doi=10.4250/jcu.2016.24.3.247 | pmc=5050315 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27721957  }} </ref>


==Frequency of Underlying Causes of Aortic Stenosis==
====Subvalvular Aortic Stenosis====
According to the '''''Euro Heart Survey'''''<ref name="pmid12633546">{{cite journal |author=Cleland JG, Swedberg K, Follath F, Komajda M, Cohen-Solal A, Aguilar JC, Dietz R, Gavazzi A, Hobbs R, Korewicki J, Madeira HC, Moiseyev VS, Preda I, van Gilst WH, Widimsky J, Freemantle N, Eastaugh J, Mason J |title=The EuroHeart Failure survey programme-- a survey on the quality of care among patients with heart failure in Europe. Part 1: patient characteristics and diagnosis |journal=[[European Heart Journal]] |volume=24 |issue=5 |pages=442–63 |year=2003 |month=March |pmid=12633546 |doi= |url=http://eurheartj.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=12633546 |accessdate=2012-04-11}}</ref> on [[valvular heart disease]], the frequencies of the different underlying causes of [[aortic stenosis]] were the following:
*[[Subvalvular aortic stenosis|Familial subvalvular aortic stenosis]]<ref name="Stern-2012">{{Cite journal | last1 = Stern | first1 = JA. | last2 = Meurs | first2 = KM. | last3 = Nelson | first3 = OL. | last4 = Lahmers | first4 = SM. | last5 = Lehmkuhl | first5 = LB. | title = Familial subvalvular aortic stenosis in golden retrievers: inheritance and echocardiographic findings. | journal = J Small Anim Pract | volume = 53 | issue = 4 | pages = 213-6 | month = Apr | year = 2012 | doi = 10.1111/j.1748-5827.2011.01187.x | PMID = 22417094 }}</ref>
#[[Calcific aortic valve disease|Calcific degeneration]]- 81.9%
*[[Hypertrophic cardiomyopathy]]<ref name="pmid24732610">{{cite journal| author=Varma PK, Neema PK| title=Hypertrophic cardiomyopathy: part 1 - introduction, pathology and pathophysiology. | journal=Ann Card Anaesth | year= 2014 | volume= 17 | issue= 2 | pages= 118-24 | pmid=24732610 | doi=10.4103/0971-9784.129841 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24732610  }} </ref>
#[[Rheumatic fever]]- 11.2%  <ref name=uas>VOC=VITIUM ORGANICUM CORDIS, a compendium of the Department of Cardiology at Uppsala Academic Hospital. By Per Kvidal September 1999, with revision by Erik Björklund May 2008</ref>.
*[[Idiopathic hypertrophic subaortic stenosis]]<ref name="pmid4737963">{{cite journal| author=Clark CE, Henry WL, Epstein SE| title=Familial prevalence and genetic transmission of idiopathic hypertrophic subaortic stenosis. | journal=N Engl J Med | year= 1973 | volume= 289 | issue= 14 | pages= 709-14 | pmid=4737963 | doi=10.1056/NEJM197310042891402 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4737963  }} </ref>
#[[Congenital]]- 5.6%
#[[endocarditis|Post-endocarditis]]- 1.3%


====Causes of Aortic Valve Calcification====
====Supravalvular Aortic Stenosis====
*Aortic stenosis is most commonly caused by age-related progressive [[calcific aortic valve disease|calcification]] of the normal tricuspid aortic valve (>50% of cases).
*[[Aortitis]]<ref name="pmid9271649">{{cite journal| author=Sharma S, Bahl VK, Rajani M| title=Stent treatment of obstructing dissection after percutaneous transluminal angioplasty of aortic stenosis caused by nonspecific aortitis. | journal=Cardiovasc Intervent Radiol | year= 1997 | volume= 20 | issue= 5 | pages= 377-9 | pmid=9271649 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9271649  }} </ref>
*Other causes include:
*[[Supravalvular aortic stenosis|Familial supravalvular aortic stenosis]]<ref name="Chiarella-1989">{{Cite journal | last1 = Chiarella | first1 = F. | last2 = Bricarelli | first2 = FD. | last3 = Lupi | first3 = G. | last4 = Bellotti | first4 = P. | last5 = Domenicucci | first5 = S. | last6 = Vecchio | first6 = C. | title = Familial supravalvular aortic stenosis: a genetic study. | journal = J Med Genet | volume = 26 | issue = 2 | pages = 86-92 | month = Feb | year = 1989 | doi = | PMID = 2918546 }}</ref>
:*Calcification of a congenital [[bicuspid aortic valve]] (30-40% of cases of calcification)
*[[Homozygous familial hypercholesterolemia]]<ref name="Giannakoulas-">{{Cite journal | last1 = Giannakoulas | first1 = G. | last2 = Mouratoglou | first2 = SA. | last3 = Kelekis | first3 = A. | last4 = Hatzimiltiades | first4 = S. | last5 = Karvounis | first5 = H. | title = Supravalvular aortic stenosis in homozygous familial hypercholesterolemia. | journal = Hellenic J Cardiol | volume = 54 | issue = 5 | pages = 392-3 | month = | year = | doi = | PMID = 24100183 }}</ref>
:*[[Acute rheumatic fever]] (less than 10% of cases of calcification)
*[[Hypoplastic left heart syndrome]]<ref name="pmid6168869">{{cite journal| author=Norwood WI, Lang P, Casteneda AR, Campbell DN| title=Experience with operations for hypoplastic left heart syndrome. | journal=J Thorac Cardiovasc Surg | year= 1981 | volume= 82 | issue= 4 | pages= 511-9 | pmid=6168869 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6168869  }} </ref>
*[[Williams syndrome]]<ref name="Deo-2013">{{Cite journal | last1 = Deo | first1 = SV. | last2 = Burkhart | first2 = HM. | last3 = Dearani | first3 = JA. | last4 = Schaff | first4 = HV. | title = Supravalvar aortic stenosis: current surgical approaches and outcomes. | journal = Expert Rev Cardiovasc Ther | volume = 11 | issue = 7 | pages = 879-90 | month = Jul | year = 2013 | doi = 10.1586/14779072.2013.811967 | PMID = 23895031 }}</ref><ref name="pmid2456379">{{cite journal| author=Morris CA, Demsey SA, Leonard CO, Dilts C, Blackburn BL| title=Natural history of Williams syndrome: physical characteristics. | journal=J Pediatr | year= 1988 | volume= 113 | issue= 2 | pages= 318-26 | pmid=2456379 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2456379  }} </ref>


====Causes of More Rapid AS Progression====
===Causes by Age Groups===
*Normal valves have three leaflets (tricuspid), but some valves have only two (bicuspid). Aortic stenosis due to calcification of a [[bicuspid valve]] typically becomes symptomatic in the 40s and 50s whereas that due to calcification of a normal valve manifests later in the 70s and 80s. [[Hypertension]], [[diabetes mellitus]], [[hyperlipoproteinemia]] and [[uremia]] may speed up the process <ref name=uas/>.
====Under Age 70====
*[[Bicuspid aortic valve]]<ref name="pmid20579534">{{cite journal| author=Siu SC, Silversides CK| title=Bicuspid aortic valve disease. | journal=J Am Coll Cardiol | year= 2010 | volume= 55 | issue= 25 | pages= 2789-800 | pmid=20579534 | doi=10.1016/j.jacc.2009.12.068 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20579534  }} </ref>
*[[Rheumatic heart disease]]<ref name="pmid23703332">{{cite journal| author=Kumar RK, Tandon R| title=Rheumatic fever & rheumatic heart disease: the last 50 years. | journal=Indian J Med Res | year= 2013 | volume= 137 | issue= 4 | pages= 643-58 | pmid=23703332 | doi= | pmc=3724245 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23703332  }} </ref>
*[[Calcification|Degenerative calcification]]<ref name="pmid26227196">{{cite journal| author=Pawade TA, Newby DE, Dweck MR| title=Calcification in Aortic Stenosis: The Skeleton Key. | journal=J Am Coll Cardiol | year= 2015 | volume= 66 | issue= 5 | pages= 561-77 | pmid=26227196 | doi=10.1016/j.jacc.2015.05.066 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26227196  }} </ref>
*Uni-commissural aortic valve<ref name="pmid27721957">{{cite journal| author=Kwon HJ, Park JH, Kim SS, Sun BJ, Jin SA, Kim JH et al.| title=Severe Aortic Stenosis Associated with Unicommissural Unicuspid Aortic Valve in a Middle Aged Male. | journal=J Cardiovasc Ultrasound | year= 2016 | volume= 24 | issue= 3 | pages= 247-250 | pmid=27721957 | doi=10.4250/jcu.2016.24.3.247 | pmc=5050315 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27721957  }} </ref>
*[[Hypoplasia]] of the annulus<ref name="pmid25914355">{{cite journal| author=Philip F, Faza NN, Schoenhagen P, Desai MY, Tuzcu EM, Svensson LG et al.| title=Aortic annulus and root characteristics in severe aortic stenosis due to bicuspid aortic valve and tricuspid aortic valves: implications for transcatheter aortic valve therapies. | journal=Catheter Cardiovasc Interv | year= 2015 | volume= 86 | issue= 2 | pages= E88-98 | pmid=25914355 | doi=10.1002/ccd.25948 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25914355  }} </ref>


==Cause of Aortic Stenosis as a Function of Age==
The cause of aortic stenosis will vary with age <ref>Roberts WC, Vowels TJ, Ko JM. Comparison of interpretations of valve structure between cardiac surgeon and cardiac pathologist among adults having isolated aortic valve replacement for aortic valve stenosis (+/- aortic regurgitation). Am J Cardiol. Apr 15 2009;103(8):1139-45.</ref><ref>http://emedicine.medscape.com/article/150638-overview#aw2aab6b2b3aa</ref>.
===Under Age 70===
*Bicuspid aortic valve: 50%
*Postinflammatory: 25%
*Degenerative calcific: 18%
*Unicommissural 3%
*Hypoplastic: 2%
*Indeterminant:2%
====Over Age 70====
====Over Age 70====
*Degenerative calcific: 48%
*[[Calcification|Degenerative calcification]]<ref name="pmid26227196">{{cite journal| author=Pawade TA, Newby DE, Dweck MR| title=Calcification in Aortic Stenosis: The Skeleton Key. | journal=J Am Coll Cardiol | year= 2015 | volume= 66 | issue= 5 | pages= 561-77 | pmid=26227196 | doi=10.1016/j.jacc.2015.05.066 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26227196  }} </ref>
*Bicuspid: 27%
*[[Bicuspid aortic valve]]<ref name="pmid22685681">{{cite journal| author=Mordi I, Tzemos N| title=Bicuspid aortic valve disease: a comprehensive review. | journal=Cardiol Res Pract | year= 2012 | volume= 2012 | issue=  | pages= 196037 | pmid=22685681 | doi=10.1155/2012/196037 | pmc=3368178 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22685681  }} </ref>
*Postinflammatory: 23%
*[[Rheumatic heart disease]]<ref name="pmid22405798">{{cite journal| author=Marijon E, Mirabel M, Celermajer DS, Jouven X| title=Rheumatic heart disease. | journal=Lancet | year= 2012 | volume= 379 | issue= 9819 | pages= 953-64 | pmid=22405798 | doi=10.1016/S0140-6736(11)61171-9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22405798  }} </ref>
*Hypoplastic: 2%
*[[Hypoplasia]] of the annulus<ref name="pmid25914355">{{cite journal| author=Philip F, Faza NN, Schoenhagen P, Desai MY, Tuzcu EM, Svensson LG et al.| title=Aortic annulus and root characteristics in severe aortic stenosis due to bicuspid aortic valve and tricuspid aortic valves: implications for transcatheter aortic valve therapies. | journal=Catheter Cardiovasc Interv | year= 2015 | volume= 86 | issue= 2 | pages= E88-98 | pmid=25914355 | doi=10.1002/ccd.25948 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25914355  }} </ref>


==Complete Differential Diagnosis for the Causes of Aortic Stenosis==
===Causes by Organ System===
 
{|style="width:80%; height:100px" border="1"
====Organized by Organ System====
{|style="width:70%; height:100px" border="1"
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | Age-induced calcification of normal tricuspid aortic valve 'wear and tear' (around the 7th or 8th decade of life), [[atherosclerosis]] (normal tricuspid valve becomes rigid with age, usually stenosis develops over age 70 and it is rarely severe), [[congenital]] [[bicuspid aortic valve]] (it is twice as common in men, there is slow increase in stenosis -progressive sclerosis- and as individuals age, calcification of the aortic valve may occur and result in stenosis, this occurs in the 40s and 50s in case of bicuspid valve), prosthetic aortic valve, [[rheumatic fever]] (slowly progressive stenosis), subacute bacterial [[endocarditis]].
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | Age-induced calcification, [[aortic sclerosis]], [[aortitis]], [[bicuspid aortic valve]] or [[Bicuspid aortic stenosis|bicommissural aortic valve]], prosthetic aortic valve, [[hypertrophic cardiomyopathy]], [[Aortic valve#Disease of the aortic valve|hypoplastic aortic annulus]], [[hypoplastic left heart syndrome]], [[idiopathic hypertrophic subaortic stenosis]], [[quadricuspid aortic valve]], [[rheumatic fever]], [[Aortic valve#Disease of the aortic valve|tricuspid aortic valve with commissural fushion]]  
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
Line 76: Line 84:
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Dermatologic'''
| '''Dermatologic'''
|bgcolor="Beige"| No underlying causes
|bgcolor="Beige"| [[Sweet syndrome]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
Line 92: Line 100:
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Environmental'''
| '''Environmental'''
|bgcolor="Beige"| [[Radiation]].
|bgcolor="Beige"| No underlying causes
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
Line 100: Line 108:
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Genetic'''
| '''Genetic'''
|bgcolor="Beige"| 1/3rd of supravalvular aortic stenosis cases are transmitted as an [[autosomal dominant]] trait as 60% of patients with supravalvular obstruction have [[williams syndrome]] (supravalvular obstruction, intellectual impairment and facial abnormalities).
|bgcolor="Beige"| [[Williams syndrome]], [[3-Methylglutaconic aciduria|3-methylglutaconic aciduria type 4]], [[alkaptonuria]], [[Bardet-Biedl syndrome]], [[Chromosome 15q partial deletion|chromosome 15q deletion syndromes]], [[Fabry's disease]], [[Familial thoracic aortic aneurysm|familial thoracic aortic aneurysm syndrome]], [[Fryns-Van den Berghe syndrome]], [[homozygous type II hypercholesterolemia]], [[Loeys-Dietz syndrome]], [[Pseudo-Hurler polydystrophy|mucolipidosis III gamma]], [[Onat syndrome]], [[Paget's disease]], [[Singleton-Merten syndrome]], [[Sweet syndrome]], [[Turner syndrome]], [[Aortic valve#Disease of the aortic valve|unicommissural aortic valve]] or [[Aortic valve#Disease of the aortic valve|unicuspid aortic valve]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Hematologic'''
| '''Hematologic'''
|bgcolor="Beige"| No underlying causes.
|bgcolor="Beige"| No underlying causes
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Iatrogenic'''
| '''Iatrogenic'''
|bgcolor="Beige"| [[Radiation treatment]] to the chest.
|bgcolor="Beige"| [[Aortic valve#Aortic Valve Replacement|Prosthetic aortic valve]], [[radiation]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Infectious Disease'''
| '''Infectious Disease'''
|bgcolor="Beige"| Bacterial [[endocarditis]] in which the vegetations may favor increase risk of stenosis.
|bgcolor="Beige"| [[rheumatic fever]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Musculoskeletal / Ortho'''
| '''Musculoskeletal / Ortho'''
|bgcolor="Beige"| No underlying causes
|bgcolor="Beige"| [[Paget's disease]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
Line 124: Line 132:
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Nutritional / Metabolic'''
| '''Nutritional / Metabolic'''
|bgcolor="Beige"| [[Fabry disease]], [[Homozygous type II hypercholesterolemia]], [[Ochronosis]], [[Paget's disease]]
|bgcolor="Beige"| [[3-Methylglutaconic aciduria|3-Methylglutaconic aciduria type 4]], [[alkaptonuria]], [[Fabry's disease]], [[Fryns-Van den Berghe syndrome]], [[homozygous type II hypercholesterolemia]], [[hyperuricemia]], [[Pseudo-Hurler polydystrophy|mucolipidosis III gamma]], [[ochronosis]], [[Onat syndrome]], [[Paget's disease]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
Line 132: Line 140:
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Oncologic'''
| '''Oncologic'''
|bgcolor="Beige"| No underlying causes.
|bgcolor="Beige"| No underlying causes
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
Line 156: Line 164:
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Rheum / Immune / Allergy'''
| '''Rheum / Immune / Allergy'''
|bgcolor="Beige"| [[rheumatic fever]] (slowly progressive stenosis).
|bgcolor="Beige"| [[Rheumatic fever]], [[systemic lupus erythematosus]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
Line 176: Line 184:
|}
|}


===Causes in Alphabetic Order===
===Causes in Alphabetical Order===
*[[Calcific aortic valve disease|Age-induced calcification]] of normal tricuspid aortic valve with [[Degeneration|degenerative 'wear and tear']].
{{col-begin|width=80%}}
*[[Congenital]] [[bicuspid aortic valve]]
{{col-break|width=33%}}
*[[Endocarditis]]
*[[Alkaptonuria]]
*[[Fabry disease]]
*[[Aortic sclerosis]]
*[[Fryns-van den Berghe syndrome]]
*[[Aortitis]]
*[[Homozygous type II hypercholesterolemia]]
*[[Bardet-Biedl syndrome]]
*[[Williams syndrome|Beuren-Williams syndrome]]
*[[Bicuspid aortic stenosis|Bicommissural aortic valve]]
*[[Bicuspid aortic valve]]
*[[Fabry's disease]]
*[[Subvalvular aortic stenosis|Familial subvalvular aortic stenosis]]
*[[Supravalvular aortic stenosis|Familial supravalvular aortic stenosis]]
*[[Familial thoracic aortic aneurysm|Familial thoracic aortic aneurysm syndrome]]
{{col-break|width=33%}}
*[[Fryns-Van den Berghe syndrome]]
*[[Homozygous familial hypercholesterolemia]]
*[[Hypertrophic cardiomyopathy]]
*[[Hyperuricemia]]
*[[Aortic valve#Disease of the aortic valve|Hypoplastic aortic annulus]]
*[[Hypoplastic left heart syndrome]]
*[[Idiopathic hypertrophic subaortic stenosis]]
*[[Loeys-Dietz syndrome]]
*[[Pseudo-Hurler polydystrophy|Mucolipidosis III gamma]]
*[[Ochronosis]]
*[[Ochronosis]]
*[[Paget disease]]
*[[Onat syndrome]]
*[[Prosthetic aortic valve]]
*[[Paget's disease]]
*[[Radiation treatment]] to the chest
{{col-break|width=33%}}
*[[Rheumatic fever]] (slowly progressive stenosis)
*[[Aortic valve#Aortic Valve Replacement|Prosthetic aortic valve]]
*[[Quadricuspid aortic valve]]
*[[Radiation]]
*[[Rheumatic fever]]
*[[Senile calcific aortic stenosis]]
*[[Singleton-Merten syndrome]]
*[[Singleton-Merten syndrome]]
*[[Subvalvular aortic stenosis]]
*[[Sweet syndrome]]
*[[Supravalvular aortic stenosis]]
*[[Systemic lupus erythematosus]]
*[[Williams syndrome]], [[autosomal dominant]] trait is associated with [[supravalvular aortic stenosis]]
*[[Aortic valve#Disease of the aortic valve|Tricuspid aortic valve with commissural fushion]]
*[[Turner syndrome]]
*[[Aortic valve#Disease of the aortic valve|Unicommissural aortic valve]]
*[[Aortic valve#Disease of the aortic valve|Unicuspid aortic valve]]
*[[Williams syndrome]]
{{col-end}}


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


{{WH}}
{{WH}}
{{WS}}
{{WS}}
[[CME Category::Cardiology]]
[[Category:Disease]]
[[Category:Disease]]
[[Category:Valvular heart disease]]
[[Category:Valvular heart disease]]

Latest revision as of 17:08, 3 March 2020
Resident
Survival
Guide

Aortic Stenosis Microchapters

Home

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Overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Mohammed A. Sbeih, M.D. [2]; Lakshmi Gopalakrishnan, M.B.B.S. [3]; Vendhan Ramanujam M.B.B.S [4]; Usama Talib, BSc, MD [5] Mandana Chitsazan, M.D. [6] Assistant Editor-In-Chief: Kristin Feeney, B.S. [7]

Overview

Aortic stenosis can be either congenital or acquired, and its common causes vary between different age groups and between different populations. Rheumatic heart disease remains the most common cause of aortic stenosis worldwide. Calcification of the aortic valve is the most common cause of aortic stenosis in North America and Europe. While calcification of congenital bicuspid aortic valve is the most common cause of aortic stenosis in people less than 70 years of age, acquired degenerative calcific aortic stenosis is the most common cause in older patients above the age of 70 years.

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated. Aortic stenosis is a progressive disease and does not have a life threatening cause. However, complications resulting from untreated disease name is common.

Common Causes

Common causes of aortic stenosis may include:[1][2][3][4]

Causes by Location of Stenosis

Valvular Aortic Stenosis

Subvalvular Aortic Stenosis

Supravalvular Aortic Stenosis

Causes by Age Groups

Under Age 70

Over Age 70

Causes by Organ System

Cardiovascular Age-induced calcification, aortic sclerosis, aortitis, bicuspid aortic valve or bicommissural aortic valve, prosthetic aortic valve, hypertrophic cardiomyopathy, hypoplastic aortic annulus, hypoplastic left heart syndrome, idiopathic hypertrophic subaortic stenosis, quadricuspid aortic valve, rheumatic fever, tricuspid aortic valve with commissural fushion
Chemical / poisoning No underlying causes
Dermatologic Sweet syndrome
Drug Side Effect No underlying causes
Ear Nose Throat No underlying causes
Endocrine No underlying causes
Environmental No underlying causes
Gastroenterologic No underlying causes
Genetic Williams syndrome, 3-methylglutaconic aciduria type 4, alkaptonuria, Bardet-Biedl syndrome, chromosome 15q deletion syndromes, Fabry's disease, familial thoracic aortic aneurysm syndrome, Fryns-Van den Berghe syndrome, homozygous type II hypercholesterolemia, Loeys-Dietz syndrome, mucolipidosis III gamma, Onat syndrome, Paget's disease, Singleton-Merten syndrome, Sweet syndrome, Turner syndrome, unicommissural aortic valve or unicuspid aortic valve
Hematologic No underlying causes
Iatrogenic Prosthetic aortic valve, radiation
Infectious Disease rheumatic fever
Musculoskeletal / Ortho Paget's disease
Neurologic No underlying causes
Nutritional / Metabolic 3-Methylglutaconic aciduria type 4, alkaptonuria, Fabry's disease, Fryns-Van den Berghe syndrome, homozygous type II hypercholesterolemia, hyperuricemia, mucolipidosis III gamma, ochronosis, Onat syndrome, Paget's disease
Obstetric/Gynecologic No underlying causes
Oncologic No underlying causes
Opthalmologic No underlying causes
Overdose / Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary No underlying causes
Renal / Electrolyte No underlying causes
Rheum / Immune / Allergy Rheumatic fever, systemic lupus erythematosus
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Miscellaneous No underlying causes

Causes in Alphabetical Order

References

  1. ↑ O'Brien KD (2006). "Pathogenesis of calcific aortic valve disease: a disease process comes of age (and a good deal more)". Arteriosclerosis, Thrombosis, and Vascular Biology. 26 (8): 1721–8. doi:10.1161/01.ATV.0000227513.13697.ac. PMID 16709942. Retrieved 2012-04-11. Unknown parameter |month= ignored (help)
  2. ↑ 2.0 2.1 2.2 Mordi I, Tzemos N (2012). "Bicuspid aortic valve disease: a comprehensive review". Cardiol Res Pract. 2012: 196037. doi:10.1155/2012/196037. PMC 3368178. PMID 22685681.
  3. ↑ Lugiano, CA. (2013). "Aortic stenosis". JAAPA. 26 (11): 46–7. doi:10.1097/01.JAA.0000436518.69169.8e. PMID 24153092. Unknown parameter |month= ignored (help)
  4. ↑ 4.0 4.1 VOC=VITIUM ORGANICUM CORDIS, a compendium of the Department of Cardiology at Uppsala Academic Hospital. By Per Kvidal September 1999, with revision by Erik Björklund May 2008
  5. ↑ Hannoush H, Introne WJ, Chen MY, Lee SJ, O'Brien K, Suwannarat P; et al. (2012). "Aortic stenosis and vascular calcifications in alkaptonuria". Mol Genet Metab. 105 (2): 198–202. doi:10.1016/j.ymgme.2011.10.017. PMC 3276068. PMID 22100375.
  6. ↑ Aronow, WS. (2013). "A review of the pathophysiology, diagnosis, and treatment of aortic valve stenosis in elderly patients". Hosp Pract (1995). 41 (4): 66–77. doi:10.3810/hp.2013.10.1082. PMID 24145591. Unknown parameter |month= ignored (help)
  7. ↑ Moura LM, Maganti K, Puthumana JJ, Rocha-Gonçalves F, Rajamannan NM (2007). "New understanding about calcific aortic stenosis and opportunities for pharmacologic intervention". Curr Opin Cardiol. 22 (6): 572–7. doi:10.1097/HCO.0b013e3282f0dae6. PMID 17921746.
  8. ↑ Strickberger SA, Schulman SP, Hutchins GM. Association of Paget’s disease of bone with calcific aortic valve disease. Am J Med 1987;82:953-6.
  9. ↑ Ong DS, Aertker RA, Clark AN, Kiefer T, Hughes GC, Harrison JK; et al. (2013). "Radiation-associated valvular heart disease". J Heart Valve Dis. 22 (6): 883–92. PMID 24597417.
  10. ↑ Bert JS, Abdullah M, Dahle TG, Gertner E (2013). "Transcatheter aortic valve replacement for advanced valvular disease in active SLE and APS". Lupus. 22 (10): 1046–9. doi:10.1177/0961203313498797. PMID 23884983.
  11. ↑ Beales PL, Elcioglu N, Woolf AS, Parker D, Flinter FA (1999). "New criteria for improved diagnosis of Bardet-Biedl syndrome: results of a population survey". J Med Genet. 36 (6): 437–46. PMC 1734378. PMID 10874630.
  12. ↑ Nakao S, Takenaka T, Maeda M, Kodama C, Tanaka A, Tahara M; et al. (1995). "An atypical variant of Fabry's disease in men with left ventricular hypertrophy". N Engl J Med. 333 (5): 288–93. doi:10.1056/NEJM199508033330504. PMID 7596372.
  13. ↑ Sprecher DL, Schaefer EJ, Kent KM, Gregg RE, Zech LA, Hoeg JM; et al. (1984). "Cardiovascular features of homozygous familial hypercholesterolemia: analysis of 16 patients". Am J Cardiol. 54 (1): 20–30. PMID 6331147.
  14. ↑ Loscalzo ML, Goh DL, Loeys B, Kent KC, Spevak PJ, Dietz HC (2007). "Familial thoracic aortic dilation and bicommissural aortic valve: a prospective analysis of natural history and inheritance". Am J Med Genet A. 143A (17): 1960–7. doi:10.1002/ajmg.a.31872. PMID 17676603.
  15. ↑ REEVE, R.; ROBINSON, SJ. (1964). "HYPOPLASTIC ANNULUS--AN UNUSUAL TYPE OF AORTIC STENOSIS: A REPORT OF THREE CASES IN CHILDREN". Dis Chest. 45: 99–102. PMID 14114644. Unknown parameter |month= ignored (help)
  16. ↑ "Subaortic stenosis short stature syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program".
  17. ↑ Yamagishi Y, Yuda S, Tsuchihashi K, Saitoh S, Miura T, Ura N; et al. (2007). "Quadricuspid aortic valve associated with aortic stenosis and regurgitation: report of a case and a review of the literature". J Med Ultrason (2001). 34 (4): 197–200. doi:10.1007/s10396-007-0148-9. PMID 27278482.
  18. ↑ Feigenbaum A, Müller C, Yale C, Kleinheinz J, Jezewski P, Kehl HG; et al. (2013). "Singleton-Merten syndrome: an autosomal dominant disorder with variable expression". Am J Med Genet A. 161A (2): 360–70. doi:10.1002/ajmg.a.35732. PMID 23322711.
  19. ↑ Cohen PR, Kurzrock R (2003). "Sweet's syndrome revisited: a review of disease concepts". Int J Dermatol. 42 (10): 761–78. PMID 14521689.
  20. ↑ Bedynek JL, Galioto FM, Leon DF (2000). "Tricuspid aortic valve with partial commissural fusion: prelminary report of an apparently common cause of aortic ejection sounds with potential for serious sequelae, illustrated by two cases". Tex Heart Inst J. 27 (2): 201–5. PMC 101056. PMID 10928509.
  21. ↑ Noonan JA (1968). "Hypertelorism with Turner phenotype. A new syndrome with associated congenital heart disease". Am J Dis Child. 116 (4): 373–80. PMID 4386970.
  22. ↑ 22.0 22.1 Kwon HJ, Park JH, Kim SS, Sun BJ, Jin SA, Kim JH; et al. (2016). "Severe Aortic Stenosis Associated with Unicommissural Unicuspid Aortic Valve in a Middle Aged Male". J Cardiovasc Ultrasound. 24 (3): 247–250. doi:10.4250/jcu.2016.24.3.247. PMC 5050315. PMID 27721957.
  23. ↑ Stern, JA.; Meurs, KM.; Nelson, OL.; Lahmers, SM.; Lehmkuhl, LB. (2012). "Familial subvalvular aortic stenosis in golden retrievers: inheritance and echocardiographic findings". J Small Anim Pract. 53 (4): 213–6. doi:10.1111/j.1748-5827.2011.01187.x. PMID 22417094. Unknown parameter |month= ignored (help)
  24. ↑ Varma PK, Neema PK (2014). "Hypertrophic cardiomyopathy: part 1 - introduction, pathology and pathophysiology". Ann Card Anaesth. 17 (2): 118–24. doi:10.4103/0971-9784.129841. PMID 24732610.
  25. ↑ Clark CE, Henry WL, Epstein SE (1973). "Familial prevalence and genetic transmission of idiopathic hypertrophic subaortic stenosis". N Engl J Med. 289 (14): 709–14. doi:10.1056/NEJM197310042891402. PMID 4737963.
  26. ↑ Sharma S, Bahl VK, Rajani M (1997). "Stent treatment of obstructing dissection after percutaneous transluminal angioplasty of aortic stenosis caused by nonspecific aortitis". Cardiovasc Intervent Radiol. 20 (5): 377–9. PMID 9271649.
  27. ↑ Chiarella, F.; Bricarelli, FD.; Lupi, G.; Bellotti, P.; Domenicucci, S.; Vecchio, C. (1989). "Familial supravalvular aortic stenosis: a genetic study". J Med Genet. 26 (2): 86–92. PMID 2918546. Unknown parameter |month= ignored (help)
  28. ↑ Giannakoulas, G.; Mouratoglou, SA.; Kelekis, A.; Hatzimiltiades, S.; Karvounis, H. "Supravalvular aortic stenosis in homozygous familial hypercholesterolemia". Hellenic J Cardiol. 54 (5): 392–3. PMID 24100183.
  29. ↑ Norwood WI, Lang P, Casteneda AR, Campbell DN (1981). "Experience with operations for hypoplastic left heart syndrome". J Thorac Cardiovasc Surg. 82 (4): 511–9. PMID 6168869.
  30. ↑ Deo, SV.; Burkhart, HM.; Dearani, JA.; Schaff, HV. (2013). "Supravalvar aortic stenosis: current surgical approaches and outcomes". Expert Rev Cardiovasc Ther. 11 (7): 879–90. doi:10.1586/14779072.2013.811967. PMID 23895031. Unknown parameter |month= ignored (help)
  31. ↑ Morris CA, Demsey SA, Leonard CO, Dilts C, Blackburn BL (1988). "Natural history of Williams syndrome: physical characteristics". J Pediatr. 113 (2): 318–26. PMID 2456379.
  32. ↑ Siu SC, Silversides CK (2010). "Bicuspid aortic valve disease". J Am Coll Cardiol. 55 (25): 2789–800. doi:10.1016/j.jacc.2009.12.068. PMID 20579534.
  33. ↑ Kumar RK, Tandon R (2013). "Rheumatic fever & rheumatic heart disease: the last 50 years". Indian J Med Res. 137 (4): 643–58. PMC 3724245. PMID 23703332.
  34. ↑ 34.0 34.1 Pawade TA, Newby DE, Dweck MR (2015). "Calcification in Aortic Stenosis: The Skeleton Key". J Am Coll Cardiol. 66 (5): 561–77. doi:10.1016/j.jacc.2015.05.066. PMID 26227196.
  35. ↑ 35.0 35.1 Philip F, Faza NN, Schoenhagen P, Desai MY, Tuzcu EM, Svensson LG; et al. (2015). "Aortic annulus and root characteristics in severe aortic stenosis due to bicuspid aortic valve and tricuspid aortic valves: implications for transcatheter aortic valve therapies". Catheter Cardiovasc Interv. 86 (2): E88–98. doi:10.1002/ccd.25948. PMID 25914355.
  36. ↑ Marijon E, Mirabel M, Celermajer DS, Jouven X (2012). "Rheumatic heart disease". Lancet. 379 (9819): 953–64. doi:10.1016/S0140-6736(11)61171-9. PMID 22405798.

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