Aortic stenosis overview: Difference between revisions

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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}}; {{AOEIC}} [[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}}; {{AOEIC}} [[User:Mohammed Sbeih|Mohammed A. Sbeih, M.D.]] [mailto:msbeih@wikidoc.org]; {{LG}}; {{USAMA}} '''Assistant Editor-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]


==Overview==
==Overview==
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==Classification==
==Classification==
Aortic stenosis can be classified broadly into two main categories: acquired and congenital. Further classification can be applied based on the origin of the stenosis such as [[Rheumatic fever|acquired rheumatic]], [[bicuspid aortic valve|congenital bicuspid]], [[Subaortic stenosis|congenital subaortic]], [[Subaortic stenosis|congenital subvalvular]], and [[Supravalvular aortic stenosis|congenital supravalvular]] aortic stenosis.
Aortic stenosis can be classified broadly into two main categories: acquired and congenital. Further classification can be applied based on the origin of the stenosis such as [[Rheumatic fever|acquired rheumatic]], [[bicuspid aortic valve|congenital bicuspid]], [[Subaortic stenosis|congenital subaortic]], [[Subaortic stenosis|congenital subvalvular]], and [[Supravalvular aortic stenosis|congenital supravalvular]] aortic stenosis.<ref name="pmid15468729">{{cite journal| author=Nasonova VA, Kuz'mina NN, Belov BS| title=[Present-day classification and nomenclature of rheumatic fever]. | journal=Klin Med (Mosk) | year= 2004 | volume= 82 | issue= 8 | pages= 61-6 | pmid=15468729 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15468729  }} </ref><ref name="pmid27989035">{{cite journal| author=Girdauskas E, Rouman M, Disha K, Dubslaff G, Fey B, Theis B et al.| title=Aortopathy in Bicuspid Aortic Valve Stenosis with Fusion of Right-Left versus Right-Non-Coronary Cusps: Are These Different Diseases? | journal=J Heart Valve Dis | year= 2016 | volume= 25 | issue= 3 | pages= 262-269 | pmid=27989035 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27989035  }} </ref>


==Pathophysiology==
==Pathophysiology==
[[Aortic stenosis]] is the progressive narrowing of the [[aortic valve]]. Calcific aortic stenosis, in particular, is an active atherosclerotic pathology where inflammation, fibrosis and calcification are involved in the progressive narrowing of the effective aortic valve area in the absence of any commissural fusion. In contrast, rheumatic aortic stenosis is due to fusion of the commissures with valvular scarring and calcification<ref name="pmid18820172">{{cite journal| author=Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD et al.| title=2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=Circulation | year= 2008 | volume= 118 | issue= 15 | pages= e523-661 | pmid=18820172 | doi=10.1161/CIRCULATIONAHA.108.190748 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18820172  }} </ref>. [[Aortic stenosis]] causes an impedance to the antegrade blood flow not only at the level of the aortic valve itself, but also at the [[subvalvular aortic stenosis|subvalvular]] ([[subvalvular aortic stenosis|below the aortic valve]]) or [[Supravalvular aortic stenosis|supravalvular]] ([[Supravalvular aortic stenosis|above the aortic valve]]) levels. As a result, chronic pressure overload develops in the [[left ventricle]].  The [[left ventricle]] undergoes hypertrophy as an initial adaptive mechanism to overcome the increased afterload. This compensatory mechanism ends up being maladpative by causing apoptosis of the hypertrophied [[myocyte]]s and subsequent [[heart failure]]. Hence, [[aortic stenosis]] is a progressive valvular disease which progression depends mainly on the degree of the narrowing of the aortic valve as well as on the maladaptive ventricular wall response.<ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </ref>
[[Aortic stenosis]] is the progressive narrowing of the [[aortic valve]]. Calcific aortic stenosis, in particular, is an active atherosclerotic pathology where [[Inflammation|inflammation,]] fibrosis and calcification are involved in the progressive narrowing of the effective aortic valve area in the absence of any commissural fusion. In contrast, rheumatic aortic stenosis is due to fusion of the commissures with valvular scarring and calcification.<ref name="pmid18820172">{{cite journal| author=Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD et al.| title=2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=Circulation | year= 2008 | volume= 118 | issue= 15 | pages= e523-661 | pmid=18820172 | doi=10.1161/CIRCULATIONAHA.108.190748 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18820172  }} </ref> [[Aortic stenosis]] causes an impedance to the antegrade blood flow not only at the level of the aortic valve itself, but also at the [[subvalvular aortic stenosis|subvalvular]] ([[subvalvular aortic stenosis|below the aortic valve]]) or [[Supravalvular aortic stenosis|supravalvular]] ([[Supravalvular aortic stenosis|above the aortic valve]]) levels. As a result, chronic pressure overload develops in the [[left ventricle]].  The [[left ventricle]] undergoes hypertrophy as an initial adaptive mechanism to overcome the increased afterload. This compensatory mechanism ends up being maladpative by causing apoptosis of the hypertrophied [[myocyte]]s and subsequent [[heart failure]]. <ref>{{Cite journal
| author = [[C. M. Otto]], [[I. G. Burwash]], [[M. E. Legget]], [[B. I. Munt]], [[M. Fujioka]], [[N. L. Healy]], [[C. D. Kraft]], [[C. Y. Miyake-Hull]] & [[R. G. Schwaegler]]
| title = Prospective study of asymptomatic valvular aortic stenosis. Clinical, echocardiographic, and exercise predictors of outcome
| journal = [[Circulation]]
| volume = 95
| issue = 9
| pages = 2262–2270
| year = 1997
| month = May
| pmid = 9142003
}}</ref>
Hence, [[aortic stenosis]] is a progressive valvular disease which progression depends mainly on the degree of the narrowing of the aortic valve as well as on the maladaptive ventricular wall response.<ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </ref>
 
==Causes==
The frequency of causes of [[aortic stenosis]] varies with the age of the patient. [[Bicuspid valve]] is the most common cause of aortic stenosis in people below 50 years of age whereas calcified aortic valve is more common in older patients.<ref name="pmid27884484">{{cite journal| author=Valdis M, DeRose G, Guo L, Chu MW| title=Ross, Hybrid Arch, and Frozen Elephant Trunk Reconstruction for Late Complications of Bicuspid Aortic Valve and Aortopathy. | journal=Can J Cardiol | year= 2016 | volume= 32 | issue= 12 | pages= 1576.e11-1576.e14 | pmid=27884484 | doi=10.1016/j.cjca.2016.07.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27884484  }} </ref>
 
==Differentiating Aortic Stenosis from other Disorders==
Aortic stenosis must be differentiated from other cardiac or pulmonary causes of [[dyspnea]], [[weakness]], and [[dizziness]].  Furthermore, if there is [[left ventricular outflow tract obstruction]], it is critical to identify whether the obstruction is [[subvalvular aortic stenosis|subvalvular]], valvular or [[supravalvular aortic stenosis|supravalvular]] or due to [[hypertrophic cardiomyopathy]] ([[HOCM]]).


==Epidemiology and Demographics==
==Epidemiology and Demographics==
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The most common risk factor for the subsequent development of aortic stenosis is congenital [[bicuspid aortic valve]]. [[Rheumatic fever]] is another risk factor for the subsequent development of aortic stenosis (rheumatic heart disease).  Risk factors that may speed up the progression of degenerative calcific aortic stenosis include: [[hypertension]], [[diabetes mellitus]], [[hyperlipoproteinemia]], [[uremia]] and [[smoking]].
The most common risk factor for the subsequent development of aortic stenosis is congenital [[bicuspid aortic valve]]. [[Rheumatic fever]] is another risk factor for the subsequent development of aortic stenosis (rheumatic heart disease).  Risk factors that may speed up the progression of degenerative calcific aortic stenosis include: [[hypertension]], [[diabetes mellitus]], [[hyperlipoproteinemia]], [[uremia]] and [[smoking]].


==Causes==
==Natural History, Complications and Prognosis==
The frequency of causes of [[aortic stenosis]] varies depending on the age of the patient. [[Bicuspid valve]] is the most common cause of aortic stenosis in people below 50 years of age whereas calcified tricuspid valve is more common in older patients.
Left untreated, aortic valve stenosis can lead to [[angina]], [[syncope]], [[congestive heart failure]], [[atrial fibrillation]], [[endocarditis]], and [[sudden cardiac death]]. Surgical treatment of aortic stenosis also carries risks and potential complications that include vascular complications and [[mitral valve]] injury.
 
==Differential Diagnosis==
Aortic stenosis must be differentiated from other cardiac or pulmonary causes of [[dyspnea]], [[weakness]], and [[dizziness]].  Furthermore, if there is [[left ventricular outflow tract obstruction]], it is critical to identify whether the obstruction is [[subvalvular aortic stenosis|subvalvular]], valvular or [[supravalvular aortic stenosis|supravalvular]] or due to [[hypertrophic cardiomyopathy]] ([[HOCM]]).
 
==Natural History, Complications & Prognosis==
===Degenerative Calcific Aortic Stenosis===
When aortic stenosis is due to the degeneration of a calcified aortic valve, there is a prolonged latent period during which symptoms may be minimal or even lacking <ref name="pmid8701905">{{cite journal| author=Faggiano P, Aurigemma GP, Rusconi C, Gaasch WH| title=Progression of valvular aortic stenosis in adults: literature review and clinical implications. | journal=Am Heart J | year= 1996 | volume= 132 | issue= 2 Pt 1 | pages= 408-17 | pmid=8701905 | doi= | pmc= | url= }} </ref>. This form of aortic stenosis presents later in life, usually after the age of 75 <ref>Townsend CM, et al. Sabiston Textbook of Surgery. 18th ed. Saunders; 2008:1841-1844.</ref>. The average rate of progression in valvular aortic stenosis, once moderate stenosis is present and symptomatic, is a decrease in valve area of 0.1 cm<sup>2</sup> per year <ref name="pmid12835667">{{cite journal| author=Zoghbi WA, Enriquez-Sarano M, Foster E, Grayburn PA, Kraft CD, Levine RA et al.| title=Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography. | journal=J Am Soc Echocardiogr | year= 2003 | volume= 16 | issue= 7 | pages= 777-802 | pmid=12835667 | doi=10.1016/S0894-7317(03)00335-3 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12835667  }} </ref><ref name="pmid8701905">{{cite journal| author=Faggiano P, Aurigemma GP, Rusconi C, Gaasch WH| title=Progression of valvular aortic stenosis in adults: literature review and clinical implications. | journal=Am Heart J | year= 1996 | volume= 132 | issue= 2 Pt 1 | pages= 408-17 | pmid=8701905 | doi= | pmc= | url= }} </ref>. Also on average, there is an increase in the jet velocity of 0.3 m / second per year and an increase in the mean pressure gradient of 7 mm Hg per year <ref name="pmid495418">{{cite journal| author=Cheitlin MD, Gertz EW, Brundage BH, Carlson CJ, Quash JA, Bode RS| title=Rate of progression of severity of valvular aortic stenosis in the adult. | journal=Am Heart J | year= 1979 | volume= 98 | issue= 6 | pages= 689-700 | pmid=495418 | doi= | pmc= | url= }} </ref> <ref name="pmid6829320">{{cite journal| author=Jonasson R, Jonsson B, Nordlander R, Orinius E, Szamosi A| title=Rate of progression of severity of valvular aortic stenosis. | journal=Acta Med Scand | year= 1983 | volume= 213 | issue= 1 | pages= 51-4 | pmid=6829320 | doi= | pmc= | url= }} </ref><ref name="pmid8404089">{{cite journal| author=Peter M, Hoffmann A, Parker C, Lüscher T, Burckhardt D| title=Progression of aortic stenosis. Role of age and concomitant coronary artery disease. | journal=Chest | year= 1993 | volume= 103 | issue= 6 | pages= 1715-9 | pmid=8404089 | doi= | pmc= | url= }} </ref>. There is tremendous individual variability in the rate of progression of aortic stenosis. Risk factors for [[atherosclerosis]] (such as age, smoking, [[hypertension]], obesity and [[diabetes]], lipid abnormalities, chronic [[renal failure]] and dialysis) and atherosclerotic disease itself (as in the case of a concomitant [[coronary artery disease]]) are associated with more rapid rates of aortic stenosis progression.
 
===Aortic Stenosis Due to Rheumatic Heart Disease===
These patients generally become symptomatic after the sixth decade.
 
===Bicuspid Aortic Valve Disease===
[[Bicuspid aortic valve stenosis]] presents one to two decades earlier than regular aortic valve stenosis.  The rate of progression of degenerative aortic stenosis can be faster than in those with [[congenital]] or [[rheumatic]] disease <ref name="pmid10965007">{{cite journal| author=Rosenhek R, Binder T, Porenta G, Lang I, Christ G, Schemper M et al.| title=Predictors of outcome in severe, asymptomatic aortic stenosis. | journal=N Engl J Med | year= 2000 | volume= 343 | issue= 9 | pages= 611-7 | pmid=10965007 | doi=10.1056/NEJM200008313430903 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10965007  }} </ref>.
Bicuspid aortic valve functions without any significant pressure gradient during childhood. However, the thickening and calcification of the valves may be detectable pathologically and on echocardiography in the second decade<ref name="pmid8427176">{{cite journal |author=Beppu S, Suzuki S, Matsuda H, Ohmori F, Nagata S, Miyatake K |title=Rapidity of progression of aortic stenosis in patients with congenital bicuspid aortic valves |journal=[[The American Journal of Cardiology]] |volume=71 |issue=4 |pages=322–7 |year=1993 |month=February |pmid=8427176 |doi= |url= |accessdate=2012-04-09}}</ref>. Bicuspid aortic valve progresses to aortic stenosis requiring operative correction in approximately 75% of cases.<ref name="pmid835475">{{cite journal |author=Fenoglio JJ, McAllister HA, DeCastro CM, Davia JE, Cheitlin MD |title=Congenital bicuspid aortic valve after age 20 |journal=[[The American Journal of Cardiology]] |volume=39 |issue=2 |pages=164–9 |year=1977 |month=February |pmid=835475 |doi= |url= |accessdate=2012-04-10}}</ref><ref name="pmid15723989">{{cite journal |author=Lewin MB, Otto CM |title=The bicuspid aortic valve: adverse outcomes from infancy to old age |journal=[[Circulation]] |volume=111 |issue=7 |pages=832–4 |year=2005 |month=February |pmid=15723989 |doi=10.1161/01.CIR.0000157137.59691.0B |url=http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=15723989 |accessdate=2012-04-10}}</ref>
 
Bicuspid aortic stenosis progressively leads to [[heart failure]], [[arrythmias]], [[angina]] and other symptoms. The manifestations of aortic stenosis occur between 40 to 60 years of age which is relatively younger than the age at which the symptoms of tricuspid aortic stenosis manifests.<ref name="pmid835475">{{cite journal |author=Fenoglio JJ, McAllister HA, DeCastro CM, Davia JE, Cheitlin MD |title=Congenital bicuspid aortic valve after age 20 |journal=[[The American Journal of Cardiology]] |volume=39 |issue=2 |pages=164–9 |year=1977 |month=February |pmid=835475 |doi= |url= |accessdate=2012-04-10}}</ref> However, children who develop early pathologic changes in bicuspid aortic valve are more likely to develop aortic insufficiency than stenosis.
 
===Aortic Sclerosis===
[[Aortic sclerosis]] (defined as [[aortic valve]] thickening without obstruction to ventricular outflow) may progress to narrowing of the aortic valve or [[aortic stenosis]].  If the pulse pressure or upstroke of the pulse diminishes in the patient with aortic sclerosis, this can be a sign of progression to aortic stenosis.
 
==Complications==
===Degenerative Calcific Aortic Stenosis===
If left untreated, aortic stenosis may lead to complications such as [[angina]], [[syncope]], or [[heart failure]].
A complete list of complications of aortic stenosis includes the following:
 
*[[Angina]]
*[[Arrhythmias]]
*[[Atrial fibrillation]]
*Bleeding: Impaired platelet function and coagulation abnormalities, as decreased levels of [[Von Willebrand factor]], can be seen in most patients with severe AS. This resolves after valve replacement procedure. 20% of patients have clinical bleeding, most often [[epistaxis]] or [[ecchymoses]] <ref name="pmid12878741">{{cite journal| author=Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F et al.| title=Acquired von Willebrand syndrome in aortic stenosis. | journal=N Engl J Med | year= 2003 | volume= 349 | issue= 4 | pages= 343-9 | pmid=12878741 | doi=10.1056/NEJMoa022831 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12878741  }} </ref>[[Aortic stenosis]] may result in a form of [[von Willebrand disease]] due to an increased turbulence around the stenosed aortic valve which subsequently triggers a break down of [[coagulation]] [[factor VIII]]-associated antigen, ''(also called [[von Willebrand factor]])'' and results in a variant of [[von Willebrand disease]].
*[[Congestive heart failure]], particularly left-sided [[heart failure]] or [[systolic dysfunction]]
*[[Endocarditis]]
*[[Fainting]] or [[syncope]]: Since the stenosed aortic valve may limit the heart's output, people with aortic stenosis are at risk of [[syncope]] and dangerously low blood pressure with the use of some common medications. Ironically, these same medicines are used to treat a variety of cardiovascular diseases, many of which may co-exist with aortic stenosis. Examples include [[nitroglycerin]], [[nitrates]], [[ACE inhibitor]]s, [[terazosin]] (Hytrin), and [[hydralazine]]. Note that all of these substances lead to peripheral [[vasodilation]]. Normally, however, in the absence of aortic stenosis, the heart is able to increase its output and thereby offset the effect of the dilated blood vessels. However, some cases of aortic stenosis can be associated with outflow blood obstruction which can prevent an increase of the [[cardiac output]]. Hence, low blood pressure or [[syncope]] may ensue.
*[[Left ventricular hypertrophy]]
*[[Myocardial infarction]]
 
===Bicuspid Aortic Valve Disease===
Bicuspid aortic valve disease is associated with the following complications:
*Aortic stenosis in the majority of patients (75%).<ref name="pmid835475">{{cite journal |author=Fenoglio JJ, McAllister HA, DeCastro CM, Davia JE, Cheitlin MD |title=Congenital bicuspid aortic valve after age 20 |journal=[[The American Journal of Cardiology]] |volume=39 |issue=2 |pages=164–9 |year=1977 |month=February |pmid=835475 |doi= |url= |accessdate=2012-04-10}}</ref>
*[[Aortic insufficiency]]<ref name="pmid11082359">{{cite journal |author=Keane MG, Wiegers SE, Plappert T, Pochettino A, Bavaria JE, Sutton MG |title=Bicuspid aortic valves are associated with aortic dilatation out of proportion to coexistent valvular lesions |journal=[[Circulation]] |volume=102 |issue=19 Suppl 3 |pages=III35–9 |year=2000 |month=November |pmid=11082359 |doi= |url=http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=11082359 |accessdate=2012-04-10}}</ref><ref name="pmid7468467">{{cite journal |author=Roberts WC, Morrow AG, McIntosh CL, Jones M, Epstein SE |title=Congenitally bicuspid aortic valve causing severe, pure aortic regurgitation without superimposed infective endocarditis. Analysis of 13 patients requiring aortic valve replacement |journal=[[The American Journal of Cardiology]] |volume=47 |issue=2 |pages=206–9 |year=1981 |month=February |pmid=7468467 |doi= |url= |accessdate=2012-04-10}}</ref>
*[[Endocarditis]]<ref name="pmid8425318">{{cite journal |author=Gersony WM, Hayes CJ, Driscoll DJ, Keane JF, Kidd L, O'Fallon WM, Pieroni DR, Wolfe RR, Weidman WH |title=Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect |journal=[[Circulation]] |volume=87 |issue=2 Suppl |pages=I121–6 |year=1993 |month=February |pmid=8425318 |doi= |url= |accessdate=2012-04-10}}</ref>
*[[Aortic aneurysm]]<ref name="pmid15723989">{{cite journal |author=Lewin MB, Otto CM |title=The bicuspid aortic valve: adverse outcomes from infancy to old age |journal=[[Circulation]] |volume=111 |issue=7 |pages=832–4 |year=2005 |month=February |pmid=15723989 |doi=10.1161/01.CIR.0000157137.59691.0B |url=http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=15723989 |accessdate=2012-04-10}}</ref>
*[[Aortic dissection]]<ref name="pmid15723989">{{cite journal |author=Lewin MB, Otto CM |title=The bicuspid aortic valve: adverse outcomes from infancy to old age |journal=[[Circulation]] |volume=111 |issue=7 |pages=832–4 |year=2005 |month=February |pmid=15723989 |doi=10.1161/01.CIR.0000157137.59691.0B |url=http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=15723989 |accessdate=2012-04-10}}</ref>
*[[Sudden death]] can occur in children during and immediately after exertion especially among those with pressure gradient > 50 mmHg across the aortic valve.<ref name="pmid8425319">{{cite journal |author=Keane JF, Driscoll DJ, Gersony WM, Hayes CJ, Kidd L, O'Fallon WM, Pieroni DR, Wolfe RR, Weidman WH |title=Second natural history study of congenital heart defects. Results of treatment of patients with aortic valvar stenosis |journal=[[Circulation]] |volume=87 |issue=2 Suppl |pages=I16–27 |year=1993 |month=February |pmid=8425319 |doi= |url= |accessdate=2012-04-10}}</ref>
 
==Prognosis==
===Asymptomatic Patients===
The prognosis of patients with aortic stenosis who do not have symptoms is quite good <ref>Lancellotti P, Magne J, Donal E, et al. Clinical outcome in asymptomatic severe aortic stenosis insights from the new proposed aortic stenosis grading classification. J Am Coll Cardiol. Jan 17 2012;59(3):235-43.</ref>. The annual mortality rate is < 1% per year in asymptomatic patients.  Only 4% of [[sudden cardiac death]]s that occur in patients with aortic stenosis occur in those patients who are asymptomatic.
 
===Symptomatic Patients===
Medical treatment of newly diagnosed moderate to severe symptomatic aortic stenosis is associated with a 25% mortality at one year, and a 50% mortality at two years. Half of the deaths are due to [[sudden cardiac death]] <ref name="pmid4894151">{{cite journal| author=Ross J, Braunwald E| title=Aortic stenosis. | journal=Circulation | year= 1968 | volume= 38 | issue= 1 Suppl | pages= 61-7 | pmid=4894151 | doi= | pmc= | url= }} </ref> <ref name="pmid7189084">{{cite journal| author=Chizner MA, Pearle DL, deLeon AC| title=The natural history of aortic stenosis in adults. | journal=Am Heart J | year= 1980 | volume= 99 | issue= 4 | pages= 419-24 | pmid=7189084 | doi= | pmc= | url= }} </ref>.
 
When aortic stenosis is left untreated, the average survival is 5 years after the onset of angina, 3 years after the onset of syncope, and 1 year after the onset of congestive heart failure <ref name="pmid4894151">{{cite journal| author=Ross J, Braunwald E| title=Aortic stenosis. | journal=Circulation | year= 1968 | volume= 38 | issue= 1 Suppl | pages= 61-7 | pmid=4894151 | doi= | pmc= | url= }} </ref><ref name="pmid3337000">{{cite journal| author=Kelly TA, Rothbart RM, Cooper CM, Kaiser DL, Smucker ML, Gibson RS| title=Comparison of outcome of asymptomatic to symptomatic patients older than 20 years of age with valvular aortic stenosis. | journal=Am J Cardiol | year= 1988 | volume= 61 | issue= 1 | pages= 123-30 | pmid=3337000 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3337000  }} </ref><ref name="pmid8712130">{{cite journal| author=Iivanainen AM, Lindroos M, Tilvis R, Heikkilä J, Kupari M| title=Natural history of aortic valve stenosis of varying severity in the elderly. | journal=Am J Cardiol | year= 1996 | volume= 78 | issue= 1 | pages= 97-101 | pmid=8712130 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8712130  }} </ref>.


==Diagnosis==
==Diagnosis==
====History and Symptoms====
====History and Symptoms====
The main symptoms of aortic stenosis include [[angina]], [[syncope]] and [[congestive heart failure]].  Left untreated, the average survival is 5 years after the onset of angina, 3 years after the onset of syncope, and 1 year after the onset of congestive heart failure <ref name="pmid4894151">{{cite journal| author=Ross J, Braunwald E| title=Aortic stenosis. | journal=Circulation | year= 1968 | volume= 38 | issue= 1 Suppl | pages= 61-7 | pmid=4894151 | doi= | pmc= | url= }} </ref><ref name="pmid3337000">{{cite journal| author=Kelly TA, Rothbart RM, Cooper CM, Kaiser DL, Smucker ML, Gibson RS| title=Comparison of outcome of asymptomatic to symptomatic patients older than 20 years of age with valvular aortic stenosis. | journal=Am J Cardiol | year= 1988 | volume= 61 | issue= 1 | pages= 123-30 | pmid=3337000 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3337000  }} </ref><ref name="pmid8712130">{{cite journal| author=Iivanainen AM, Lindroos M, Tilvis R, Heikkilä J, Kupari M| title=Natural history of aortic valve stenosis of varying severity in the elderly. | journal=Am J Cardiol | year= 1996 | volume= 78 | issue= 1 | pages= 97-101 | pmid=8712130 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8712130  }} </ref>. Other symptoms include [[dyspnea on exertion]], [[orthopnea]] and [[paroxysmal nocturnal dyspnea]].
The main symptoms of aortic stenosis include [[angina]], [[syncope]] and [[congestive heart failure]].  Left untreated, the average survival is 5 years after the onset of angina, 3 years after the onset of syncope, and 1 year after the onset of congestive heart failure. <ref name="pmid4894151">{{cite journal| author=Ross J, Braunwald E| title=Aortic stenosis. | journal=Circulation | year= 1968 | volume= 38 | issue= 1 Suppl | pages= 61-7 | pmid=4894151 | doi= | pmc= | url= }} </ref><ref name="pmid3337000">{{cite journal| author=Kelly TA, Rothbart RM, Cooper CM, Kaiser DL, Smucker ML, Gibson RS| title=Comparison of outcome of asymptomatic to symptomatic patients older than 20 years of age with valvular aortic stenosis. | journal=Am J Cardiol | year= 1988 | volume= 61 | issue= 1 | pages= 123-30 | pmid=3337000 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3337000  }} </ref><ref name="pmid8712130">{{cite journal| author=Iivanainen AM, Lindroos M, Tilvis R, Heikkilä J, Kupari M| title=Natural history of aortic valve stenosis of varying severity in the elderly. | journal=Am J Cardiol | year= 1996 | volume= 78 | issue= 1 | pages= 97-101 | pmid=8712130 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8712130  }} </ref>  Other symptoms include [[dyspnea on exertion]], [[orthopnea]] and [[paroxysmal nocturnal dyspnea]].


====Physical Examination====
====Physical Examination====
Aortic stenosis is most often diagnosed when it is [[asymptomatic]] and can sometimes be detected during routine examination of the heart and circulatory system. The major signs include [[pulsus parvus et tardus]] ''(a slow-rising, small volume carotid pulse)'', a lag time between apical and carotid impulses, and a distinct [[systolic ejection murmur]].
Aortic stenosis is most often diagnosed when it is [[asymptomatic]] and can sometimes be detected during routine examination of the heart and circulatory system. The major signs include [[pulsus parvus et tardus]] (a slow-rising, small volume carotid pulse), a lag time between apical and carotid impulses, and a distinct [[systolic ejection murmur]].


====Electrocardiogram====
====Electrocardiogram====
The electrocardiogram in the patient with moderate to severe [[aortic stenosis]] may reveal [[left ventricular hypertrophy]] and [[heart block]].
The electrocardiogram in the patient with moderate to severe [[aortic stenosis]] may reveal [[left ventricular hypertrophy]] and [[heart block]].
====Cardiac Stress Test====
[[Exercise test|Exercise testing]] should not be performed in symptomatic patients with [[AS]] but can be used to elicit exercise-induced symptoms and abnormal [[blood pressure]] responses in asymptomatic patients.<ref name="pmid18820172">{{cite journal |author=Bonow RO, Carabello BA, Chatterjee K, ''et al.'' |title=2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons |journal=Circulation |volume=118 |issue=15 |pages=e523–661 |year=2008 |month=October |pmid=18820172 |doi=10.1161/CIRCULATIONAHA.108.190748 |url=}}</ref>


====Chest X-ray====
====Chest X-ray====
Chest x ray may be used as a diagnostic tool in the evaluation of aortic stenosis. Findings associated with aortic stenosis include [[left ventricular hypertrophy]] and calcification of the aortic valve.  
[[Chest X-ray]] may be used as a diagnostic tool in the evaluation of aortic stenosis. Findings associated with aortic stenosis include [[left ventricular hypertrophy]] and calcification of the aortic valve.
 
====CT Scan====
[[Computed tomography]] can be helpful as a diagnostic tool in conditions where the [[echocardiographic]] findings are inconclusive.


====MRI====
====MRI====
[[Magnetic resonance imaging]] is rarely used in the diagnosis of aortic stenosis, except in rare cases where the echocardiographic findings are inconclusive. There is a signal void where the high velocity jet exits the aortic valve.
[[Magnetic resonance imaging]] is rarely used in the diagnosis of aortic stenosis, except in rare cases where the echocardiographic findings are inconclusive. There is a signal void where the high velocity jet exits the aortic valve.
====CT====
Computed tomography can be helpful as a diagnostic tool in conditions where the [[echocardiographic]] findings are inconclusive.


====Echocardiography====
====Echocardiography====
[[Echocardiography]] is the best non-invasive test to evaluate the aortic valve anatomy and function. Echocardiography can be used to estimate the gradient across the aortic valve using the modified Bernoulli equation (gradient = 4 X velocity<sup>2</sup>).  The flow must be constant, so as the velocity increases, the valve area decreases proportionally.  Echocardiography can also be used to assess the severity of [[left ventricular hypertrophy]].
[[Echocardiography]] is the best non-invasive test to evaluate the aortic valve anatomy and function. It is indicated in the case of presence of symptoms suggestive of valvular problems or in the case of detection of a systolic [[murmur]] with a grade greater than 3/6. Doppler echocardiography allows the measurement of the maximum jet velocity and can be used to estimate the effective orifice area of the [[aortic valve]] as well as the gradient across the aortic valve using the modified Bernoulli equation (gradient = 4 x velocity<sup>2</sup>).  The flow must be constant, so as the velocity increases, the valve area decreases proportionally. Attention to technical details is important as they may lead to underestimation of the severity of the [[aortic stenosis]].<ref name="pmid18820172">{{cite journal| author=Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD et al.| title=2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=Circulation | year= 2008 | volume= 118 | issue= 15 | pages= e523-661 | pmid=18820172 | doi=10.1161/CIRCULATIONAHA.108.190748 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18820172 }} </ref> Echocardiography can also be used to assess the severity of [[left ventricular hypertrophy]].


====Cardiac Catheterization====
====Cardiac Catheterization====
Left and right heart catheterization as well as angiography may be useful in the assessment of the patient prior to aortic valve replacement surgery.
Left and right heart [[catheterization]] as well as angiography may be useful in the assessment of the patient prior to aortic valve replacement surgery. In addition, asymptomatic patients with aortic stenosis should undergo cardiac catherization when echocardiographic findings are inconsistent with the clinical findings<ref name="pmid18820172">{{cite journal| author=Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD et al.| title=2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=Circulation | year= 2008 | volume= 118 | issue= 15 | pages= e523-661 | pmid=18820172 | doi=10.1161/CIRCULATIONAHA.108.190748 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18820172  }} </ref>.


====Aortic Valve Area====
====Aortic Valve Area====
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==Treatment==
==Treatment==
Once a patient becomes symptomatic with aortic stenosis, [[aortic valve replacement]] should be performed as long as the patient can tolerate surgery and has no co-morbidities. Patients undergoing [[aortic valve replacement]] and having co-existing multi-vessel [[coronary artery disease]] should have concomitantly [[CABG]].<ref name="pmid9870202">{{cite journal| author=Bonow RO, Carabello B, de Leon AC, Edmunds LH, Fedderly BJ, Freed MD et al.| title=ACC/AHA Guidelines for the Management of Patients With Valvular Heart Disease. Executive Summary. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Management of Patients With Valvular Heart Disease). | journal=J Heart Valve Dis | year= 1998 | volume= 7 | issue= 6 | pages= 672-707 | pmid=9870202 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9870202  }} </ref>If a patient has extensive co-morbidities, [[transcatheter aortic valve implantation]] can be considered.  [[Aortic valvuloplasty]] can be considered in those patients who are too sick for surgery or transcatheter aortic valve implantation.  If severe left ventricular dysfunction is present in the setting of aortic stenosis, it is of utmost importance to differentiate between true severe aortic stenosis and pseudo-severe aortic stenosis as these two entities have different pathophysiologies and different outcomes after aortic valve replacement.
===General Approach===
Medical therapy reduces symptoms but does not prolong life. Asymptomatic patients should be followed up yearly for any new onset of symptoms. No treatment has been proven to delay the progression of aortic stenosis. Follow up with an echocardiography should be done every 1, 3, 5 years in severe, moderate and mild aortic stenosis respectively.
Once a patient becomes symptomatic with aortic stenosis, [[aortic valve replacement]] should be performed as long as the patient can tolerate surgery and has no co-morbidities. If severe left ventricular dysfunction is present in the setting of aortic stenosis, it is of utmost importance to differentiate between true severe aortic stenosis and pseudo-severe aortic stenosis as these two entities have different pathophysiologies and different outcomes after aortic valve replacement.
 
Medical therapy reduces symptoms but does not prolong life. If a patient has extensive co-morbidities, [[transcatheter aortic valve implantation]] can be considered.  [[Aortic valvuloplasty]] can be considered in those patients who are too sick for surgery or transcatheter aortic valve implantation.
; Shown below is an algorithm showing the management of symptomatic and asymptomatic patients with aortic stenosis<ref name="pmid9870202">{{cite journal| author=Bonow RO, Carabello B, de Leon AC, Edmunds LH, Fedderly BJ, Freed MD et al.| title=ACC/AHA Guidelines for the Management of Patients With Valvular Heart Disease. Executive Summary. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Management of Patients With Valvular Heart Disease). | journal=J Heart Valve Dis | year= 1998 | volume= 7 | issue= 6 | pages= 672-707 | pmid=9870202 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9870202 }} </ref>.
[[Image:Management_of_Aortic_Stenosis.png|center|550px|Management of patients with aortic stenosis]]
 
; Shown below is an algorithm showing a more detailed approach to patients with low flow, low gradient aortic stenosis.
[[Image:Management_of_LGLFAS.png‎ |center|500px|Approach to patients with low flow, low gradient aortic stenosis]]


===Medical Therapy===
===Medical Therapy===
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===Surgery===
===Surgery===
Aortic stenosis requires aortic valve replacement once the patient becomes symptomatic.
Surgical intervention may be a necessary component of treatment for symptomatic severe aortic stenosis. Aortic valve replacement is the mainstay of treatment of symptomatic aortic stenosis, as it improves both the symptoms and [[life expectancy]] in aortic stenosis patients, in contrast to medical therapy alone which may improve the symptoms without prolonging [[life expectancy]].<ref name="pmid27884484">{{cite journal| author=Valdis M, DeRose G, Guo L, Chu MW| title=Ross, Hybrid Arch, and Frozen Elephant Trunk Reconstruction for Late Complications of Bicuspid Aortic Valve and Aortopathy. | journal=Can J Cardiol | year= 2016 | volume= 32 | issue= 12 | pages= 1576.e11-1576.e14 | pmid=27884484 | doi=10.1016/j.cjca.2016.07.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27884484  }} </ref>


===Percutaneous Aortic Balloon Valvotomy (PABV) or Aortic Valvuloplasty===
===Percutaneous Aortic Balloon Valvotomy (PABV) or Aortic Valvuloplasty===
Surgical [[aortic valve replacement]] is the mainstay of the treatment of aortic stenosis as it improves both symptoms and life expectancy. However, some patients may not be surgical candidates due to coexisting comorbidities. Hence, minimally invasive treatment such as percutaneous aortic balloon valvotomy (PABV) maybe an  alternative to surgery as a palliative strategy.  PABV is a procedure during which one or more balloons are placed across a stenotic valve and then inflated in order to cause a decrease the severity of aortic stenosis. This is to be distinguished from [[transcatheter aortic valve implantation]] ([[TAVI]]) which is a different method that involves replacement of the valve percutaneously.
Surgical [[aortic valve replacement]] is the mainstay of the treatment of aortic stenosis as it improves both symptoms and life expectancy. However, some patients may not be surgical candidates due to coexisting comorbidities. Hence, minimally invasive treatment such as [[Percutaneous aortic balloon valvotomy (PABV)|percutaneous aortic balloon valvotomy]] ([[PABV]]) maybe an  alternative to surgery as a palliative strategy.  [[PABV]] is a procedure during which one or more balloons are placed across a stenotic valve and then inflated in order to cause a decrease the severity of aortic stenosis. This is to be distinguished from [[transcatheter aortic valve implantation]] ([[TAVI]]) which is a different method that involves replacement of the valve percutaneously.


===Transcatheter Aortic Valve Implantation===
===Transcatheter Aortic Valve Implantation===
Until recently, [[aortic valve replacement]] ([[AVR]]) was the only effective treatment for severe symptomatic [[aortic stenosis]]. However, over the past decade [[percutaneous treatment]] of aortic valve disease with the implantation of a stent-based valve prosthesis has been introduced as a new treatment in patients considered inoperable because of [[severe co-morbidities]].<ref name="pmid17015786">{{cite journal |author=Grube E, Laborde JC, Gerckens U, Felderhoff T, Sauren B, Buellesfeld L, Mueller R, Menichelli M, Schmidt T, Zickmann B, Iversen S, Stone GW |title=Percutaneous implantation of the CoreValve self-expanding valve prosthesis in high-risk patients with aortic valve disease: the Siegburg first-in-man study |journal=[[Circulation]] |volume=114 |issue=15 |pages=1616–24 |year=2006 |month=October |pmid=17015786 |doi=10.1161/CIRCULATIONAHA.106.639450 |url=http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=17015786 |accessdate=2011-03-17}}</ref>
Until recently, [[aortic valve replacement]] ([[AVR]]) was the only effective treatment for severe symptomatic [[aortic stenosis]]. However, over the past decade percutaneous treatment of aortic valve disease with the implantation of a stent-based valve prosthesis has been introduced as a new treatment in patients considered inoperable because of severe co-morbidities.<ref name="pmid17015786">{{cite journal |author=Grube E, Laborde JC, Gerckens U, Felderhoff T, Sauren B, Buellesfeld L, Mueller R, Menichelli M, Schmidt T, Zickmann B, Iversen S, Stone GW |title=Percutaneous implantation of the CoreValve self-expanding valve prosthesis in high-risk patients with aortic valve disease: the Siegburg first-in-man study |journal=[[Circulation]] |volume=114 |issue=15 |pages=1616–24 |year=2006 |month=October |pmid=17015786 |doi=10.1161/CIRCULATIONAHA.106.639450 |url=http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=17015786 |accessdate=2011-03-17}}</ref>
In Transcatheter Aortic Valve Implantation (TAVI) also known as Percutaneous Aortic Valve Replacement (PAVR), a synthetic valve is advanced to the heart through a small hole made in the groin. This procedure is similar in its mechanism to the insertion of a [[stent]], or performing [[balloon angioplasty]] albeit with much larger equipment. Traditional [[aortic valve replacement]] is an invasive surgical procedure, with considerable mortality and [[morbidity]], especially in more fragile patients. In the newly developed TAVI procedure, the dysfunctional [[aortic valve]] is replaced [[percutaneously]], which obviates the need for [[open heart surgery]].
In Transcatheter Aortic Valve Implantation (TAVI) also known as Percutaneous Aortic Valve Replacement (PAVR), a synthetic valve is advanced to the heart through a small hole made in the groin. This procedure is similar in its mechanism to the insertion of a [[stent]], or performing [[balloon angioplasty]] albeit with much larger equipment. Traditional [[aortic valve replacement]] is an invasive surgical procedure, with considerable mortality and [[morbidity]], especially in more fragile patients. In the newly developed TAVI procedure, the dysfunctional [[aortic valve]] is replaced [[percutaneously]], which obviates the need for [[open heart surgery]].


==Prevention==
===Follow Up===
Aortic stenosis associated with rheumatic heart disease can be minimized with antibiotic therapy in patients with documented [[streptococcal pharyngitis]] ([[strep throat]]).  [[Bicuspid aortic valve disease]] is a congenital variant and cannot be prevented. Calcific degeneration of the valve can potentially be minimized by rosouvistatin and other measures targeting prevention of atherosclerosis.
Follow up is recommended for all patients with operated and unoperated [[aortic stenosis]]. Asymptomatic patients with [[aortic stenosis]] should undergo follow up since [[aortic stenosis]] is an ongoing disease that progresses with time. In fact, asymptomatic patients should undergo follow up every 1 year, 3 years and 5 years in case of severe, moderate and mild aortic stenosis respectively.<ref name="pmid9870202">{{cite journal| author=Bonow RO, Carabello B, de Leon AC, Edmunds LH, Fedderly BJ, Freed MD et al.| title=ACC/AHA Guidelines for the Management of Patients With Valvular Heart Disease. Executive Summary. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Management of Patients With Valvular Heart Disease). | journal=J Heart Valve Dis | year= 1998 | volume= 7 | issue= 6 | pages= 672-707 | pmid=9870202 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9870202  }} </ref>


==Precautions and Prophylaxis==
===Prevention===
Aortic stenosis associated with rheumatic heart disease can be minimized with antibiotic therapy in patients with documented [[streptococcal pharyngitis]] ([[strep throat]]).<ref name="pmid15468729">{{cite journal| author=Nasonova VA, Kuz'mina NN, Belov BS| title=[Present-day classification and nomenclature of rheumatic fever]. | journal=Klin Med (Mosk) | year= 2004 | volume= 82 | issue= 8 | pages= 61-6 | pmid=15468729 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15468729  }} </ref>[[Bicuspid aortic stenosis|Bicuspid aortic valve disease]] is a congenital variant and cannot be prevented. Calcific degeneration of the valve can potentially be minimized by rosouvistatin and other measures targeting prevention of atherosclerosis.<ref name="pmid27989035">{{cite journal| author=Girdauskas E, Rouman M, Disha K, Dubslaff G, Fey B, Theis B et al.| title=Aortopathy in Bicuspid Aortic Valve Stenosis with Fusion of Right-Left versus Right-Non-Coronary Cusps: Are These Different Diseases? | journal=J Heart Valve Dis | year= 2016 | volume= 25 | issue= 3 | pages= 262-269 | pmid=27989035 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27989035  }} </ref>
 
===Precautions and Prophylaxis===
People with aortic stenosis of any etiology are at risk for the development of infection of their stenosed valve, i.e. [[infective endocarditis]] and antibiotic prophylaxis should be considered.  Patients with severe aortic stenosis should avoid strenuous exercise and any exercise that greatly increases [[afterload]] such as weight lifting.
People with aortic stenosis of any etiology are at risk for the development of infection of their stenosed valve, i.e. [[infective endocarditis]] and antibiotic prophylaxis should be considered.  Patients with severe aortic stenosis should avoid strenuous exercise and any exercise that greatly increases [[afterload]] such as weight lifting.


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
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[[CME Category::Cardiology]]
[[Category:Disease]]
[[Category:Disease]]
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[[Category:Valvular heart disease]]
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[[Category:Cardiac surgery]]
[[Category:Cardiac surgery]]
[[Category:Surgery]]
[[Category:Surgery]]
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Latest revision as of 06:01, 22 August 2021



Resident
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Aortic Stenosis Microchapters

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Cardiac Stress Test

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Echocardiography

Cardiac Catheterization

Aortic Valve Area

Aortic Valve Area Calculation

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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]; Usama Talib, BSc, MD [4] Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]

Overview

The aortic valve ensures that the blood moves forward from the left ventricle into the aorta and that it does not leak backwards during diastole. When functioning appropriately, the aortic valve does not impede the flow of blood between the left ventricle and the aorta and it does not leak. Under some circumstances, the aortic valve becomes narrower than normal impeding the flow of blood. This is known as aortic valve stenosis, or aortic stenosis, often abbreviated as AS.

Classification

Aortic stenosis can be classified broadly into two main categories: acquired and congenital. Further classification can be applied based on the origin of the stenosis such as acquired rheumatic, congenital bicuspid, congenital subaortic, congenital subvalvular, and congenital supravalvular aortic stenosis.[1][2]

Pathophysiology

Aortic stenosis is the progressive narrowing of the aortic valve. Calcific aortic stenosis, in particular, is an active atherosclerotic pathology where inflammation, fibrosis and calcification are involved in the progressive narrowing of the effective aortic valve area in the absence of any commissural fusion. In contrast, rheumatic aortic stenosis is due to fusion of the commissures with valvular scarring and calcification.[3] Aortic stenosis causes an impedance to the antegrade blood flow not only at the level of the aortic valve itself, but also at the subvalvular (below the aortic valve) or supravalvular (above the aortic valve) levels. As a result, chronic pressure overload develops in the left ventricle. The left ventricle undergoes hypertrophy as an initial adaptive mechanism to overcome the increased afterload. This compensatory mechanism ends up being maladpative by causing apoptosis of the hypertrophied myocytes and subsequent heart failure. [4] Hence, aortic stenosis is a progressive valvular disease which progression depends mainly on the degree of the narrowing of the aortic valve as well as on the maladaptive ventricular wall response.[5]

Causes

The frequency of causes of aortic stenosis varies with the age of the patient. Bicuspid valve is the most common cause of aortic stenosis in people below 50 years of age whereas calcified aortic valve is more common in older patients.[6]

Differentiating Aortic Stenosis from other Disorders

Aortic stenosis must be differentiated from other cardiac or pulmonary causes of dyspnea, weakness, and dizziness. Furthermore, if there is left ventricular outflow tract obstruction, it is critical to identify whether the obstruction is subvalvular, valvular or supravalvular or due to hypertrophic cardiomyopathy (HOCM).

Epidemiology and Demographics

Aortic stenosis primarily affects older adults and the majority of cases are due to calcific degeneration. Aortic stenosis tends to affect approximately 2% of patients over the age of 65, 3% of patients over the age of 75, and 4% of patients over the age 85.[7]

Risk Factors

The most common risk factor for the subsequent development of aortic stenosis is congenital bicuspid aortic valve. Rheumatic fever is another risk factor for the subsequent development of aortic stenosis (rheumatic heart disease). Risk factors that may speed up the progression of degenerative calcific aortic stenosis include: hypertension, diabetes mellitus, hyperlipoproteinemia, uremia and smoking.

Natural History, Complications and Prognosis

Left untreated, aortic valve stenosis can lead to angina, syncope, congestive heart failure, atrial fibrillation, endocarditis, and sudden cardiac death. Surgical treatment of aortic stenosis also carries risks and potential complications that include vascular complications and mitral valve injury.

Diagnosis

History and Symptoms

The main symptoms of aortic stenosis include angina, syncope and congestive heart failure. Left untreated, the average survival is 5 years after the onset of angina, 3 years after the onset of syncope, and 1 year after the onset of congestive heart failure. [8][9][10] Other symptoms include dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea.

Physical Examination

Aortic stenosis is most often diagnosed when it is asymptomatic and can sometimes be detected during routine examination of the heart and circulatory system. The major signs include pulsus parvus et tardus (a slow-rising, small volume carotid pulse), a lag time between apical and carotid impulses, and a distinct systolic ejection murmur.

Electrocardiogram

The electrocardiogram in the patient with moderate to severe aortic stenosis may reveal left ventricular hypertrophy and heart block.

Cardiac Stress Test

Exercise testing should not be performed in symptomatic patients with AS but can be used to elicit exercise-induced symptoms and abnormal blood pressure responses in asymptomatic patients.[3]

Chest X-ray

Chest X-ray may be used as a diagnostic tool in the evaluation of aortic stenosis. Findings associated with aortic stenosis include left ventricular hypertrophy and calcification of the aortic valve.

CT Scan

Computed tomography can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive.

MRI

Magnetic resonance imaging is rarely used in the diagnosis of aortic stenosis, except in rare cases where the echocardiographic findings are inconclusive. There is a signal void where the high velocity jet exits the aortic valve.

Echocardiography

Echocardiography is the best non-invasive test to evaluate the aortic valve anatomy and function. It is indicated in the case of presence of symptoms suggestive of valvular problems or in the case of detection of a systolic murmur with a grade greater than 3/6. Doppler echocardiography allows the measurement of the maximum jet velocity and can be used to estimate the effective orifice area of the aortic valve as well as the gradient across the aortic valve using the modified Bernoulli equation (gradient = 4 x velocity2). The flow must be constant, so as the velocity increases, the valve area decreases proportionally. Attention to technical details is important as they may lead to underestimation of the severity of the aortic stenosis.[3] Echocardiography can also be used to assess the severity of left ventricular hypertrophy.

Cardiac Catheterization

Left and right heart catheterization as well as angiography may be useful in the assessment of the patient prior to aortic valve replacement surgery. In addition, asymptomatic patients with aortic stenosis should undergo cardiac catherization when echocardiographic findings are inconsistent with the clinical findings[3].

Aortic Valve Area

The aortic valve area is the size of the orifice for blood to flow from the left ventricle to the aorta. The aortic valve area is reduced in aortic stenosis, and the aortic valve area is the metric that is used to gauge the need for aortic valve replacement surgery. The pressure gradient across a narrowed aortic valve cannot be used to gauge the need for valve replacement as the gradient may be low in patients with impaired left ventricular function.

Aortic Valve Area Calculation

The calculation of the aortic valve area is an indirect method used to determine the area of the aortic valve. The calculated aortic valve orifice area is currently one of the measures for evaluating the severity of aortic stenosis. An aortic valve having an area less than 0.8 cm² is considered to be severe aortic stenosis.[11][12]

There are many ways to calculate the aortic valve area. The most commonly used methods involve measurements taken during echocardiography. For interpretation of these values, the aortic valve area is generally divided by the body surface area.

Treatment

General Approach

Once a patient becomes symptomatic with aortic stenosis, aortic valve replacement should be performed as long as the patient can tolerate surgery and has no co-morbidities. If severe left ventricular dysfunction is present in the setting of aortic stenosis, it is of utmost importance to differentiate between true severe aortic stenosis and pseudo-severe aortic stenosis as these two entities have different pathophysiologies and different outcomes after aortic valve replacement. Medical therapy reduces symptoms but does not prolong life. If a patient has extensive co-morbidities, transcatheter aortic valve implantation can be considered. Aortic valvuloplasty can be considered in those patients who are too sick for surgery or transcatheter aortic valve implantation.

Medical Therapy

While medical therapy may improve the symptoms of patients with aortic stenosis, medical therapy does not prolong life expectancy. Aortic valve replacement remains the definitive treatment for symptomatic aortic stenosis and it improves both the symptoms and life expectancy of the patients. When pharmacological therapies are used, extreme caution must be taken in the administration of vasodilators as an excess in vasodilation may lead to hypotension, a reduction in perfusion pressure to the heart, a further decline in cardiac output and further hypotension. This vicious circle can be fatal and must be avoided at all costs.

Surgery

Surgical intervention may be a necessary component of treatment for symptomatic severe aortic stenosis. Aortic valve replacement is the mainstay of treatment of symptomatic aortic stenosis, as it improves both the symptoms and life expectancy in aortic stenosis patients, in contrast to medical therapy alone which may improve the symptoms without prolonging life expectancy.[6]

Percutaneous Aortic Balloon Valvotomy (PABV) or Aortic Valvuloplasty

Surgical aortic valve replacement is the mainstay of the treatment of aortic stenosis as it improves both symptoms and life expectancy. However, some patients may not be surgical candidates due to coexisting comorbidities. Hence, minimally invasive treatment such as percutaneous aortic balloon valvotomy (PABV) maybe an alternative to surgery as a palliative strategy. PABV is a procedure during which one or more balloons are placed across a stenotic valve and then inflated in order to cause a decrease the severity of aortic stenosis. This is to be distinguished from transcatheter aortic valve implantation (TAVI) which is a different method that involves replacement of the valve percutaneously.

Transcatheter Aortic Valve Implantation

Until recently, aortic valve replacement (AVR) was the only effective treatment for severe symptomatic aortic stenosis. However, over the past decade percutaneous treatment of aortic valve disease with the implantation of a stent-based valve prosthesis has been introduced as a new treatment in patients considered inoperable because of severe co-morbidities.[13] In Transcatheter Aortic Valve Implantation (TAVI) also known as Percutaneous Aortic Valve Replacement (PAVR), a synthetic valve is advanced to the heart through a small hole made in the groin. This procedure is similar in its mechanism to the insertion of a stent, or performing balloon angioplasty albeit with much larger equipment. Traditional aortic valve replacement is an invasive surgical procedure, with considerable mortality and morbidity, especially in more fragile patients. In the newly developed TAVI procedure, the dysfunctional aortic valve is replaced percutaneously, which obviates the need for open heart surgery.

Follow Up

Follow up is recommended for all patients with operated and unoperated aortic stenosis. Asymptomatic patients with aortic stenosis should undergo follow up since aortic stenosis is an ongoing disease that progresses with time. In fact, asymptomatic patients should undergo follow up every 1 year, 3 years and 5 years in case of severe, moderate and mild aortic stenosis respectively.[14]

Prevention

Aortic stenosis associated with rheumatic heart disease can be minimized with antibiotic therapy in patients with documented streptococcal pharyngitis (strep throat).[1]Bicuspid aortic valve disease is a congenital variant and cannot be prevented. Calcific degeneration of the valve can potentially be minimized by rosouvistatin and other measures targeting prevention of atherosclerosis.[2]

Precautions and Prophylaxis

People with aortic stenosis of any etiology are at risk for the development of infection of their stenosed valve, i.e. infective endocarditis and antibiotic prophylaxis should be considered. Patients with severe aortic stenosis should avoid strenuous exercise and any exercise that greatly increases afterload such as weight lifting.

References

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  3. 3.0 3.1 3.2 3.3 Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD; et al. (2008). "2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". Circulation. 118 (15): e523–661. doi:10.1161/CIRCULATIONAHA.108.190748. PMID 18820172.
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  5. Dweck MR, Boon NA, Newby DE (2012). "Calcific aortic stenosis: a disease of the valve and the myocardium". J Am Coll Cardiol. 60 (19): 1854–63. doi:10.1016/j.jacc.2012.02.093. PMID 23062541.
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  11. Charlson E, Legedza A, Hamel M (2006). "Decision-making and outcomes in severe symptomatic aortic stenosis". J Heart Valve Dis. 15 (3): 312–21. PMID 16784066.
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  13. Grube E, Laborde JC, Gerckens U, Felderhoff T, Sauren B, Buellesfeld L, Mueller R, Menichelli M, Schmidt T, Zickmann B, Iversen S, Stone GW (2006). "Percutaneous implantation of the CoreValve self-expanding valve prosthesis in high-risk patients with aortic valve disease: the Siegburg first-in-man study". Circulation. 114 (15): 1616–24. doi:10.1161/CIRCULATIONAHA.106.639450. PMID 17015786. Retrieved 2011-03-17. Unknown parameter |month= ignored (help)
  14. Bonow RO, Carabello B, de Leon AC, Edmunds LH, Fedderly BJ, Freed MD; et al. (1998). "ACC/AHA Guidelines for the Management of Patients With Valvular Heart Disease. Executive Summary. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Management of Patients With Valvular Heart Disease)". J Heart Valve Dis. 7 (6): 672–707. PMID 9870202.

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