Aortic stenosis causes
Aortic Stenosis Microchapters |
Diagnosis |
---|
Treatment |
Percutaneous Aortic Balloon Valvotomy (PABV) or Aortic Valvuloplasty |
Transcatheter Aortic Valve Replacement (TAVR) |
Case Studies |
Aortic stenosis causes On the Web |
American Roentgen Ray Society Images of Aortic stenosis causes |
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 70 years of age, acquired degenerative calcific aortic stenosis is the most common cause in older patients above 70 years of age. 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
Valvular Aortic Stenosis
- Acquired [2]
- Congenital [2]
- 3-Methylglutaconic aciduria type 4
- Bardet-Biedl syndrome
- Bicuspid aortic valve
- Chromosome 15q deletion syndromes
- 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
Supravalvular Aortic Stenosis
- Familial
- Fibromembranous aortic lesion
- Homozygous familial hypercholesterolemia[7]
- Hourglass constriction of aorta
- Hypoplasia of aorta
- Post surgical
- Sequelae of aortitis
- Sporadic
- Williams syndrome[8]
Frequency of Underlying Causes of Aortic Stenosis
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% [9]
- 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 AS 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. [9]
Cause of Aortic Stenosis as a Function of Age
The cause of aortic stenosis will vary with age:[10][11]
- 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
Causes by Organ System
Cardiovascular | 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. |
Chemical / poisoning | No underlying causes |
Dermatologic | No underlying causes |
Drug Side Effect | No underlying causes |
Ear Nose Throat | No underlying causes |
Endocrine | No underlying causes |
Environmental | Radiation. |
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). |
Hematologic | No underlying causes. |
Iatrogenic | Radiation treatment to the chest. |
Infectious Disease | Bacterial endocarditis in which the vegetations may favor increase risk of stenosis. |
Musculoskeletal / Ortho | No underlying causes |
Neurologic | No underlying causes |
Nutritional / Metabolic | Fabry disease, Homozygous type II hypercholesterolemia, Ochronosis, 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). |
Sexual | No underlying causes |
Trauma | No underlying causes |
Urologic | No underlying causes |
Miscellaneous | No underlying causes |
Causes in Alphabetic Order
- Age-induced calcification of normal tricuspid aortic valve with degenerative 'wear and tear'
- Congenital bicuspid aortic valve
- Endocarditis
- Fabry disease
- Fryns-van den Berghe syndrome
- Homozygous type II hypercholesterolemia
- Ochronosis
- Paget disease
- Prosthetic aortic valve
- Radiation treatment to the chest
- Rheumatic fever (slowly progressive stenosis)
- Singleton-Merten syndrome
- Subvalvular aortic stenosis
- Supravalvular aortic stenosis
- Williams syndrome, autosomal dominant trait is associated with supravalvular aortic stenosis
References
- ↑ Lugiano, CA. (2013). "Aortic stenosis". JAAPA. 26 (11): 46–7. doi:10.1097/01.JAA.0000436518.69169.8e. PMID 24153092. Unknown parameter
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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
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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
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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
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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
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ignored (help) - ↑ 9.0 9.1 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
- ↑ 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