Aortic stenosis causes: Difference between revisions
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==Overview== | ==Overview== | ||
Causes of aortic stenosis are either congenital or acquired, and its common causes vary between different age groups and between different human populations. While [[Bicuspid aortic stenosis|congenital bicuspid aortic valve]] is the most common cause of aortic stenosis in people below 70 years | Causes of aortic stenosis are either congenital or acquired, and its common causes vary between different age groups and between different human populations. While [[Bicuspid aortic stenosis|congenital bicuspid aortic valve]] is the most common cause of aortic stenosis in people below the age of 70 years, acquired degenerative calcific aortic stenosis is the most common cause in older patients above the age of 70 years. [[Rheumatic heart disease]] remains the most common cause of aortic stenosis worldwide and calcification, either degenerative calcification in older adults, or early calcification secondary to a congenital bicuspid aortic valve are the most common causes in North America.<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> | ||
==Causes== | ==Causes== | ||
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====Subvalvular Aortic Stenosis==== | ====Subvalvular Aortic Stenosis==== | ||
*[[Familial]] | *[[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> | ||
*[[Hypertrophic cardiomyopathy]] | *[[Hypertrophic cardiomyopathy]] | ||
*[[Idiopathic hypertrophic subaortic stenosis]] | *[[Idiopathic hypertrophic subaortic stenosis]] | ||
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====Supravalvular Aortic Stenosis==== | ====Supravalvular Aortic Stenosis==== | ||
*[[Aortitis]] | *[[Aortitis]] | ||
*[[Familial]] | *[[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> | ||
*[[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> | *[[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> | ||
*[[Hypoplastic left heart syndrome]] | *[[Hypoplastic left heart syndrome]] | ||
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:*[[Acute rheumatic fever]] (less than 10% of cases of calcification) | :*[[Acute rheumatic fever]] (less than 10% of cases of calcification) | ||
Causes of More Rapid | Causes of More Rapid Aortic Stenosis Progression: | ||
*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/> | *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/> | ||
==Causes of Aortic Stenosis as a Function of Age== | ==Causes of Aortic Stenosis as a Function of Age== | ||
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|-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"| 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), [[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]] | [[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]] | ||
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|-bgcolor="LightSteelBlue" | |-bgcolor="LightSteelBlue" | ||
| '''Nutritional / Metabolic''' | | '''Nutritional / Metabolic''' | ||
|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| | |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]] | ||
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|-bgcolor="LightSteelBlue" | |-bgcolor="LightSteelBlue" | ||
Revision as of 16:53, 18 November 2013
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Aortic Stenosis Microchapters |
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Diagnosis |
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Treatment |
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Percutaneous Aortic Balloon Valvotomy (PABV) or Aortic Valvuloplasty |
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Transcatheter Aortic Valve Replacement (TAVR) |
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Case Studies |
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Aortic stenosis causes On the Web |
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American Roentgen Ray Society Images of Aortic stenosis causes |
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Risk calculators and risk factors for Aortic stenosis causes |
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]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]
Overview
Causes of aortic stenosis are either congenital or acquired, and its common causes vary between different age groups and between different human populations. While congenital bicuspid aortic valve is the most common cause of aortic stenosis in people below the age of 70 years, acquired degenerative calcific aortic stenosis is the most common cause in older patients above the age of 70 years. Rheumatic heart disease remains the most common cause of aortic stenosis worldwide and calcification, either degenerative calcification in older adults, or early calcification secondary to a congenital bicuspid aortic valve are the most common causes in North America.[1]
Causes
Causes of Aortic Stenosis as of Type of Stenosis
Valvular Aortic Stenosis
- Acquired [2]
- Congenital [2]
- 3-Methylglutaconic aciduria type 4
- Bardet-Biedl syndrome
- Bicuspid aortic valve
- Chromosome 15q deletion syndromes
- Fabry's disease
- Familial hypercholesterolemia
- Familial thoracic aortic aneurysm syndrome
- Hypoplastic aortic annulus[6]
- Loeys-Dietz syndrome
- Mucolipidosis III gamma
- Onat syndrome
- Quadricuspid aortic valve
- Singleton-Merten syndrome
- Sweet syndrome
- Tricuspid aortic valve with commissural fushion
- Turner syndrome
- Unicuspid aortic valve
Subvalvular Aortic Stenosis
- Familial subvalvular aortic stenosis[7]
- Hypertrophic cardiomyopathy
- Idiopathic hypertrophic subaortic stenosis
Supravalvular Aortic Stenosis
- Aortitis
- Familial supravalvular aortic stenosis[8]
- Homozygous familial hypercholesterolemia[9]
- Hypoplastic left heart syndrome
- Williams syndrome[10]
Causes of Aortic Stenosis as of Frequency
According to the Euro Heart Survey[2] on valvular heart disease, the frequencies of the different underlying causes of aortic stenosis were the following:
- Calcific degeneration - 81.9%
- Rheumatic fever - 11.2% [11]
- Congenital - 5.6%
- Post-endocarditis - 1.3%
Causes of Aortic Valve Calcification:
- Aortic stenosis is most commonly caused by age-related progressive calcification of the normal tricuspid aortic valve (>50% of cases).
- Other causes include:
- Calcification of a congenital bicuspid aortic valve (30-40% of cases of calcification)
- Acute rheumatic fever (less than 10% of cases of calcification)
Causes of More Rapid Aortic Stenosis Progression:
- 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.[11]
Causes of Aortic Stenosis as a Function of Age
The cause of aortic stenosis will vary with age:[12][13]
- Under Age 70:
- Bicuspid aortic valve - 50%
- Postinflammatory - 25%
- Degenerative calcific - 18%
- Unicommissural - 3%
- Hypoplastic - 2%
- Indeterminant - 2%
- Over Age 70:
- Degenerative calcific - 48%
- Bicuspid - 27%
- Postinflammatory - 23%
- Hypoplastic - 2%
Complete Differential Diagnosis for the Causes of Aortic Stenosis
Life Threatening Causes
Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.
Common Causes
- Calcific degeneration
- Congenital bicuspid aortic valve
- Infective endocarditis
- Rheumatic fever[1][11]
Causes by Organ System
| Cardiovascular | Age-induced calcification of normal tricuspid aortic valve 'wear and tear' (around the 7th or 8th decade of life), aortic sclerosis (normal tricuspid valve becomes rigid with age, usually stenosis develops over age 70 and it is rarely severe), aortitis, congenital bicuspid aortic valve or bicommissural 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, hypertrophic cardiomyopathy, hypoplastic aortic annulus, hypoplastic left heart syndrome, idiopathic hypertrophic subaortic stenosis, infective endocarditis, quadricuspid aortic valve, rheumatic fever (slowly progressive stenosis), 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 | 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), 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 | Infective endocarditis in which the vegetations may favor increase risk of stenosis, 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 (slowly progressive stenosis), systemic lupus erythematosus |
| Sexual | No underlying causes |
| Trauma | No underlying causes |
| Urologic | No underlying causes |
| Miscellaneous | No underlying causes |
Causes in Alphabetical Order
References
- ↑ 1.0 1.1 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) - ↑ 2.0 2.1 2.2 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 (2003). "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". European Heart Journal. 24 (5): 442–63. PMID 12633546. Retrieved 2012-04-11. Unknown parameter
|month=ignored (help) - ↑ 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.
- ↑ 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) - ↑ 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.
- ↑ 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) - ↑ 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) - ↑ 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) - ↑ 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.
- ↑ 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) - ↑ 11.0 11.1 11.2 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
- ↑ 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.
- ↑ http://emedicine.medscape.com/article/150638-overview#aw2aab6b2b3aa