Aortic regurgitation causes: Difference between revisions

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__NOTOC__
{{Aortic insufficiency}}
{{Aortic insufficiency}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}; [[Varun Kumar]], M.B.B.S.; [[Lakshmi Gopalakrishnan]], M.B.B.S.; {{VR}}; {{USAMA}} 
==Overview==
Aortic insufficiency can be an acute illness or a chronic illness and the causes differ depending upon the acuity of the disease. In general, aortic insufficiency is due to abnormalities of the aortic valve itself or the aortic root. Aortic regurgitation secondary to dilation of the ascending aorta has overtaken the valvular aortic disease as the most common cause of aortic regurgitation.It can also occur after surgical valve placement.


{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}; [[Varun Kumar]], M.B.B.S.; [[Lakshmi Gopalakrishnan]], M.B.B.S.
==Aortic regurgitation causes==


==Overview==
Aortic insufficiency can be an acute illness or a chronic illness and the causes differ depending upon the acuity of the disease.  In general, aortic insufficiency is due to abnormalities of the aortic valve itself or the aortic root.  Aortic regurgitation secondary to dilation of the ascending aorta has overtaken valvular aortic disease as the most common cause of aortic regurgitation.


==Common Causes==
===Life Threatening Causes===
*Worldwide the most common cause of aortic insufficiency is [[rheumatic heart disease]].
Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.
*In the United States, senile degenerative calcific aortic valve disease and bicuspid aortic valve disease are the most common causes.
*[[Aortic dissection]]<ref name="pmid15470217">{{cite journal| author=Enriquez-Sarano M, Tajik AJ| title=Clinical practice. Aortic regurgitation. | journal=N Engl J Med | year= 2004 | volume= 351 | issue= 15 | pages= 1539-46 | pmid=15470217 | doi=10.1056/NEJMcp030912 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15470217  }} </ref>
*Aortic regurgitation secondary to dilation of the ascending aorta has overtaken valvular aortic disease as the most common cause of aortic regurgitation.
*[[Bacterial endocarditis]]<ref name="pmid24603191">{{cite journal| author=Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA et al.| title=2014 AHA/ACC guideline 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. | journal=J Am Coll Cardiol | year= 2014 | volume= 63 | issue= 22 | pages= e57-185 | pmid=24603191 | doi=10.1016/j.jacc.2014.02.536 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24603191  }} </ref>
 
===Common Causes===
*[[Bicuspid aortic valve]]<ref name="Nishimura-2002">{{Cite journal  | last1 = Nishimura | first1 = RA. | title = Cardiology patient pages. Aortic valve disease. | journal = Circulation | volume = 106 | issue = 7 | pages = 770-2 | month = Aug | year = 2002 | doi =  | PMID = 12176943 }}</ref>
*[[Calcific aortic valve disease|Senile or degenerative calcific aortic valve disease]]<ref name="Nishimura-2002">{{Cite journal  | last1 = Nishimura | first1 = RA. | title = Cardiology patient pages. Aortic valve disease. | journal = Circulation | volume = 106 | issue = 7 | pages = 770-2 | month = Aug | year = 2002 | doi =  | PMID = 12176943 }}</ref>
*[[Rheumatic heart disease]]<ref name="Bekeredjian-2005">{{Cite journal  | last1 = Bekeredjian | first1 = R. | last2 = Grayburn | first2 = PA. | title = Valvular heart disease: aortic regurgitation. | journal = Circulation | volume = 112 | issue = 1 | pages = 125-34 | month = Jul | year = 2005 | doi = 10.1161/CIRCULATIONAHA.104.488825 | PMID = 15998697 }}</ref>
*After Valvular Surgery<ref name="pmid27491900">{{cite journal| author=Ribeiro HB, Orwat S, Hayek SS, Larose É, Babaliaros V, Dahou A et al.| title=Cardiovascular Magnetic Resonance to Evaluate Aortic Regurgitation After Transcatheter Aortic Valve Replacement. | journal=J Am Coll Cardiol | year= 2016 | volume= 68 | issue= 6 | pages= 577-85 | pmid=27491900 | doi=10.1016/j.jacc.2016.05.059 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27491900  }} </ref>
 
===Causes by Location of the Abnormality===
 
The cause of Aortic Regurgitation by location can be classified as following:<ref name="pmid15470217">{{cite journal| author=Enriquez-Sarano M, Tajik AJ| title=Clinical practice. Aortic regurgitation. | journal=N Engl J Med | year= 2004 | volume= 351 | issue= 15 | pages= 1539-46 | pmid=15470217 | doi=10.1056/NEJMcp030912 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15470217  }} </ref>
 
====Causes Based Upon Abnormalities in the Aortic Valve====
*[[Bicuspid aortic valve]]<ref name="Nishimura-2002">{{Cite journal  | last1 = Nishimura | first1 = RA. | title = Cardiology patient pages. Aortic valve disease. | journal = Circulation | volume = 106 | issue = 7 | pages = 770-2 | month = Aug | year = 2002 | doi =  | PMID = 12176943 }}</ref>
*[[Collagen vascular disease]]
*[[Senile]] or degenerative [[calcific aortic valve disease]]
*[[Endocarditis]]
* [[Myxomatous]] aortic valve
*[[Rheumatic fever]]<ref name="Spagnuolo-1971">{{Cite journal  | last1 = Spagnuolo | first1 = M. | last2 = Kloth | first2 = H. | last3 = Taranta | first3 = A. | last4 = Doyle | first4 = E. | last5 = Pasternack | first5 = B. | title = Natural history of rheumatic aortic regurgitation. Criteria predictive of death, congestive heart failure, and angina in young patients. | journal = Circulation | volume = 44 | issue = 3 | pages = 368-80 | month = Sep | year = 1971 | doi =  | PMID = 4255488 }}</ref>
*[[Rheumatoid arthritis]] <ref name="pmid17519398">{{cite journal| author=Pierpont ME, Basson CT, Benson DW, Gelb BD, Giglia TM, Goldmuntz E et al.| title=Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. | journal=Circulation | year= 2007 | volume= 115 | issue= 23 | pages= 3015-38 | pmid=17519398 | doi=10.1161/CIRCULATIONAHA.106.183056 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17519398  }} </ref>
*[[Sinus of Valsalva Aneurysm]]
*[[Systemic Lupus Erythematosus]]
*[[Trauma]]<ref name="pmid8267458">{{cite journal |author=Prêtre R, Faidutti B |title=Surgical management of aortic valve injury after nonpenetrating trauma |journal=[[The Annals of Thoracic Surgery]] |volume=56 |issue=6 |pages=1426–31 |year=1993 |month=December |pmid=8267458 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/0003-4975(93)90708-P |accessdate=2011-03-28}}</ref><ref name="pmid15172299">{{cite journal |author=Onorati F, De Santo LS, Carozza A, De Feo M, Renzulli A, Cotrufo M |title=Marfan syndrome as a predisposing factor for traumatic aortic insufficiency |journal=[[The Annals of Thoracic Surgery]] |volume=77 |issue=6 |pages=2192–4 |year=2004 |month=June |pmid=15172299 |doi=10.1016/S0003-4975(03)01409-7 |url=http://linkinghub.elsevier.com/retrieve/pii/S0003497503014097 |accessdate=2011-03-28}}</ref>
*[[Turner's Syndrome]]
*[[Ventricular Septal Defect]]
*[[Weight loss]] medications
*Post Aortic Valve Surgery<ref name="pmid2016464">{{cite journal| author=Isner JM| title=Acute catastrophic complications of balloon aortic valvuloplasty. The Mansfield Scientific Aortic Valvuloplasty Registry Investigators. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 6 | pages= 1436-44 | pmid=2016464 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2016464  }} </ref>
*Quadricuspid Aortic Valve <ref name="pmid27195176">{{cite journal| author=Vasudev R, Shah P, Bikkina M, Shamoon F| title=Quadricuspid Aortic Valve: A Rare Congenital Cause of Aortic Insufficiency. | journal=J Clin Imaging Sci | year= 2016 | volume= 6 | issue=  | pages= 10 | pmid=27195176 | doi=10.4103/2156-7514.179417 | pmc=4860453 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27195176  }} </ref>


==Differential Diagnosis of the Causes of Aortic Insufficiency==
====Causes Based Upon Abnormalities in the Ascending Aorta====
===By Organ System===
*[[Ankylosing spondylitis]] <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Raffle A, Gray J, MacDonald HR, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Gatzy JT |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1136/bmj.1.6001.93-a |url=}}</ref>
*[[Aortic dissection]] <ref name="pmid7925504">{{cite journal| author=Lindroos M, Kupari M, Valvanne J, Strandberg T, Heikkilä J, Tilvis R| title=Factors associated with calcific aortic valve degeneration in the elderly. | journal=Eur Heart J | year= 1994 | volume= 15 | issue= 7 | pages= 865-70 | pmid=7925504 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7925504  }} </ref>
*[[Arteriosclerosis]] <ref name="pmid11359741">{{cite journal| author=Ortlepp JR, Hoffmann R, Ohme F, Lauscher J, Bleckmann F, Hanrath P| title=The vitamin D receptor genotype predisposes to the development of calcific aortic valve stenosis. | journal=Heart | year= 2001 | volume= 85 | issue= 6 | pages= 635-8 | pmid=11359741 | doi= | pmc=PMC1729782 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11359741  }} </ref>
* [[Bechterew's Disease]] <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Raffle A, Gray J, MacDonald HR, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Gatzy JT |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1136/bmj.1.6001.93-a |url=}}</ref>
*[[Behcet's syndrome]]
*[[Cystic medial necrosis]] of the [[aorta]]
*[[Ehlers-Danlos Syndrome]]
*[[Giant cell arteritis]]
*[[Hypertension]]<ref name="Kim-1996">{{Cite journal  | last1 = Kim | first1 = M. | last2 = Roman | first2 = MJ. | last3 = Cavallini | first3 = MC. | last4 = Schwartz | first4 = JE. | last5 = Pickering | first5 = TG. | last6 = Devereux | first6 = RB. | title = Effect of hypertension on aortic root size and prevalence of aortic regurgitation. | journal = Hypertension | volume = 28 | issue = 1 | pages = 47-52 | month = Jul | year = 1996 | doi =  | PMID = 8675263 }}</ref>
*[[Idiopathic]] dilation of the [[ascending aorta]]
*[[Marfan syndrome]]<ref name="pmid15172299">{{cite journal |author=Onorati F, De Santo LS, Carozza A, De Feo M, Renzulli A, Cotrufo M |title=Marfan syndrome as a predisposing factor for traumatic aortic insufficiency |journal=[[The Annals of Thoracic Surgery]] |volume=77 |issue=6 |pages=2192–4 |year=2004 |month=June |pmid=15172299 |doi=10.1016/S0003-4975(03)01409-7 |url=http://linkinghub.elsevier.com/retrieve/pii/S0003497503014097 |accessdate=2011-03-28}}</ref>
*[[Osteogenesis imperfecta]]
*[[Polymyalgia Rheumatica]]
*[[Pseudoxanthoma Elasticum]]
*[[Psoriatic arthritis]]
*[[Reiter's syndrome]]
*[[Relapsing polychondritis]]
*[[Senile]] aortic ectasia and dilation
*[[Spondyloarthropathy]]
*[[Syphilitic aortitis]]
*[[Takayasu arteritis]]<ref name="pmid27267341">{{cite journal| author=Ferreira TF, Freire M, Teodoro RB| title=Difficulties in the differential diagnosis between Takayasu arteritis and rheumatic fever: case report. | journal=Rev Bras Reumatol Engl Ed | year= 2016 | volume= 56 | issue= 1 | pages= 90-2 | pmid=27267341 | doi=10.1016/j.rbre.2015.07.001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27267341  }} </ref>
*[[Trauma]]
*[[Ulcerative colitis]]
*[[Whipple's disease]]


{|height:100px" border="1"
===Causes by Organ System===
{|style="width:80%; height:100px" border="1"
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | After [[aortic valvuloplasty]]<ref name="pmid2016464">{{cite journal |author=Isner JM |title=Acute catastrophic complications of balloon aortic valvuloplasty. The Mansfield Scientific Aortic Valvuloplasty Registry Investigators |journal=[[Journal of the American College of Cardiology]] |volume=17 |issue=6 |pages=1436–44 |year=1991 |month=May |pmid=2016464 |doi= |url= |accessdate=2011-03-28}}</ref>, [[Aortic dissection]], [[Arteriosclerosis]], [[Bacterial Endocarditis]], [[Bicuspid aortic valve]], [[Prosthetic valve]] dysfunction<ref>Friedman T, Mani A, Elefteriades JA. Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity. Expert Rev Cardiovasc Ther. Feb 2008;6(2):235-48.</ref>, [[Hypertension]], [[Myxomatous]] aortic valve, [[Rheumatic Fever]], Senile aortic ectasia and dilation, Senile or degenerative calcific aortic valve disease, [[Sinus of Valsalva Aneurysm]], [[Ventricular Septal Defect]]
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | [[Annuloaortic ectasia]], [[aortic valvuloplasty]], [[aortic dissection]], [[arterial tortuosity syndrome]], [[bacterial endocarditis]], [[Aortic stenosis valvuloplasty#Complications|balloon valvotomy]], [[bicuspid aortic valve]], [[Aortic valve#Disease of the aortic valve|congenitally fenestrated cusp rupture]], [[cystic medial necrosis]], [[hypertension]], [[marantic endocarditis]], [[Prosthetic valve|prosthetic valve dysfunction]], [[Annuloaortic ectasia|senile aortic ectasia and dilation]], [[Calcific aortic valve disease|senile or degenerative calcific aortic valve disease]], [[Aneurysm of sinus of valsalva|sinus of valsalva aneurysm]], [[subaortic stenosis]], [[syphilitic aortitis]], [[Taussig-Bing syndrome|subpulmonic ventricular septal defect]], [[tetralogy of Fallot]], [[thoracic aortic aneurysm]], [[Aortic valve#Disease of the aortic valve|unicuspid aortic valve]], [[ventricular septal defect]]  
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Drug Side Effect'''
| '''Drug Side Effect'''
|bgcolor="Beige"| [[Fenfluramine]] and [[Dexfenfluramine]]: These drugs have been associated with degeneration of the aortic valve.
|bgcolor="Beige"| [[Cabergoline]], [[dexfenfluramine]], [[dopamine agonists]], [[fenfluramine]], [[methysergide]], [[pergolide]], [[phentermine]]  
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Endocrine'''
| '''Endocrine'''
|bgcolor="Beige"| No underlying causes
|bgcolor="Beige"| [[Acromegaly]]
|-  
|-  
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Gastroenterologic'''
| '''Gastroenterologic'''
|bgcolor="Beige"| [[Ulcerative colitis]], [[Whipple disease]]<ref>Jeserich M, Ihling C, Holubarsch C. Aortic valve endocarditis with Whipple disease. Ann Intern Med. Jun 1 1997;126(11):920.</ref>
|bgcolor="Beige"| [[Inflammatory bowel disease]], [[ulcerative colitis]], [[Whipple disease]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Genetic'''
| '''Genetic'''
|bgcolor="Beige"| [[Ankylosing Spondylitis]]<ref>Palazzi C, D' Angelo S, Lubrano E, Olivieri I. Aortic involvement in ankylosing spondylitis. Clin Exp Rheumatol. May-Jun 2008;26(3 Suppl 49):S131-4.</ref>, [[Ehlers-Danlos Syndrome]], [[Marfan Syndrome]], [[Osteogenesis imperfecta]], [[Pseudoxanthoma Elasticum]], [[Reiter's Syndrome]], [[Rheumatoid Arthritis]]<ref>Chand EM, Freant LJ, Rubin JW. Aortic valve rheumatoid nodules producing clinical aortic regurgitation and a review of the literature. Cardiovasc Pathol. Nov-Dec 1999;8(6):333-8.</ref>, [[Systemic Lupus Erythematosus]], [[Takayasu arteritis]], [[Turner's Syndrome]]
|bgcolor="Beige"| [[Ankylosing spondylitis]], [[autosomal dominant polycystic kidney disease]], [[bicuspid aortic valve]], [[Down syndrome]], [[Ehlers-Danlos syndrome]], [[Hurler syndrome]], [[Marfan syndrome]], [[Maroteaux-Lamy syndrome]], [[I-cell disease|mucolipidosis II alpha/beta]], [[Pseudo-Hurler polydystrophy|mucolipidosis III gamma]], [[mucopolysaccharidosis]], [[mucopolysaccharidosis type I Hurler/Scheie syndrome]], [[osteogenesis imperfecta]], [[pseudoxanthoma elasticum]], [[rheumatoid arthritis]], [[Scheie syndrome]], [[Shprintzen-Goldberg syndrome]], [[truncus arteriosus]], [[Turner's syndrome]], [[Aortic valve#Disease of the aortic valve|unicuspid aortic valve]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Iatrogenic'''
| '''Iatrogenic'''
|bgcolor="Beige"| After [[aortic valvuloplasty]]<ref name="pmid2016464">{{cite journal |author=Isner JM |title=Acute catastrophic complications of balloon aortic valvuloplasty. The Mansfield Scientific Aortic Valvuloplasty Registry Investigators |journal=[[Journal of the American College of Cardiology]] |volume=17 |issue=6 |pages=1436–44 |year=1991 |month=May |pmid=2016464 |doi= |url= |accessdate=2011-03-28}}</ref>, [[Prosthetic valve]] dysfunction
|bgcolor="Beige"| [[Aortic valvuloplasty]], [[Aortic stenosis valvuloplasty#Complications|balloon valvotomy]], [[Transposition of the great vessels corrective surgery|double switch operation]], [[Prosthetic valve|prosthetic valve dysfunction]], [[radiation]], [[Transposition of the great vessels corrective surgery#Rastelli operation|Senning-Rastelli procedure]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Infectious Disease'''
| '''Infectious Disease'''
|bgcolor="Beige"| [[Bacterial Endocarditis]], [[Rheumatic Fever]], [[Syphilis]]
|bgcolor="Beige"| [[Bacterial endocarditis]], [[Endocarditis|perivalvular abscess]], [[Q fever]], [[rheumatic fever]], [[syphilis]], [[syphilitic aortitis]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Musculoskeletal / Ortho'''
| '''Musculoskeletal / Ortho'''
|bgcolor="Beige"| [[Ankylosing Spondylitis]], [[Collagen vascular disease]], [[Ehlers-Danlos Syndrome]], [[Osteogenesis imperfecta]], [[Psoriatic arthritis]], [[Reiter's Syndrome]], [[Relapsing polychondritis]], [[Rheumatoid Arthritis]]<ref>Chand EM, Freant LJ, Rubin JW. Aortic valve rheumatoid nodules producing clinical aortic regurgitation and a review of the literature. Cardiovasc Pathol. Nov-Dec 1999;8(6):333-8.</ref>, [[Spondyloarthropathy]], [[Systemic Lupus Erythematosus]]
|bgcolor="Beige"| [[Ankylosing spondylitis]], [[collagen vascular disease]], [[Ehlers-Danlos syndrome]], [[osteogenesis imperfecta]], [[psoriatic arthritis]], [[Reiter's Syndrome]], [[relapsing polychondritis]], [[rheumatoid arthritis]], [[spondyloarthropathy]], [[systemic lupus erythematosus]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Nutritional / Metabolic'''
| '''Nutritional / Metabolic'''
|bgcolor="Beige"| No underlying causes
|bgcolor="Beige"| [[I-cell disease|Mucolipidosis II alpha/beta]], [[Pseudo-Hurler polydystrophy|mucolipidosis III gamma]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Renal / Electrolyte'''
| '''Renal / Electrolyte'''
|bgcolor="Beige"| No underlying causes
|bgcolor="Beige"| [[Autosomal dominant polycystic kidney disease]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Rheum / Immune / Allergy'''
| '''Rheum / Immune / Allergy'''
|bgcolor="Beige"| [[Ankylosing Spondylitis]]<ref>Palazzi C, D' Angelo S, Lubrano E, Olivieri I. Aortic involvement in ankylosing spondylitis. Clin Exp Rheumatol. May-Jun 2008;26(3 Suppl 49):S131-4.</ref>, [[Bechterew's Disease]], [[Behcet disease]], [[Collagen vascular disease]], [[Cystic medial necrosis]], [[Ehlers-Danlos Syndrome]], [[Giant cell arteritis]], [[Marfan Syndrome]], [[Myxomatous]] aortic valve: The valvular structure can degenerate, [[Osteogenesis imperfecta]], [[Polymyalgia Rheumatica]], [[Pseudoxanthoma Elasticum]], [[Psoriatic arthritis]], [[Reiter's Syndrome]], [[Rheumatic Fever]], [[Rheumatoid Arthritis]]<ref>Chand EM, Freant LJ, Rubin JW. Aortic valve rheumatoid nodules producing clinical aortic regurgitation and a review of the literature. Cardiovasc Pathol. Nov-Dec 1999;8(6):333-8.</ref>, [[Spondyloarthropathy]], [[Systemic Lupus Erythematosus]], [[Takayasu arteritis]], [[Ulcerative colitis]], [[Whipple disease]]<ref>Jeserich M, Ihling C, Holubarsch C. Aortic valve endocarditis with Whipple disease. Ann Intern Med. Jun 1 1997;126(11):920.</ref>
|bgcolor="Beige"| [[Ankylosing spondylitis]], [[antiphospholipid syndrome]], [[Bechterew's Disease|Bechterew's disease]], [[Behcet disease]], [[collagen vascular disease]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[inflammatory bowel disease]], [[Marfan syndrome]], [[Myxomatous degeneration|myxomatous aortic valve]], [[osteogenesis imperfecta]], [[polychondritis]], [[polymyalgia rheumatica]], [[pseudoxanthoma elasticum]], [[psoriatic arthritis]], [[reactive arthritis]], [[Reiter's syndrome]], [[relapsing polychondritis]], [[rheumatic fever]], [[rheumatoid arthritis]], [[sarcoidosis]], [[spondyloarthropathy]], [[systemic lupus erythematosus]], [[Takayasu arteritis]], [[ulcerative colitis]], [[Whipple disease]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Sexual'''
| '''Sexual'''
|bgcolor="Beige"| [[Syphilis]]
|bgcolor="Beige"| No underlying causes
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Trauma'''
| '''Trauma'''
|bgcolor="Beige"| Blunt [[chest trauma]] disrupting the supporting apparatus of the aortic valve.
|bgcolor="Beige"| [[Chest trauma]], [[traumatic aortic rupture]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Miscellaneous'''
| '''Miscellaneous'''
|bgcolor="Beige"| [[Myxomatous]] aortic valve
|bgcolor="Beige"| No underlying causes
|-
|-
|}
|}


==Differential Diagnosis of the Causes of Acute Aortic Insufficiency==
===Causes in Alphabetical Order===
In alphabetical order:
{{col-begin|width=80%}}
* After [[aortic valvuloplasty]]<ref name="pmid2016464">{{cite journal |author=Isner JM |title=Acute catastrophic complications of balloon aortic valvuloplasty. The Mansfield Scientific Aortic Valvuloplasty Registry Investigators |journal=[[Journal of the American College of Cardiology]] |volume=17 |issue=6 |pages=1436–44 |year=1991 |month=May |pmid=2016464 |doi= |url= |accessdate=2011-03-28}}</ref>
{{col-break|width=33%}}
* [[Aortic dissection]]: A type A dissection may disrupt the integrity of the valve leaflets causing malcoaptation of the aortic leaflets.
*[[Acromegaly]] <ref name="Pereira-2004">{{Cite journal | last1 = Pereira | first1 = AM. | last2 = van Thiel | first2 = SW. | last3 = Lindner | first3 = JR. | last4 = Roelfsema | first4 = F. | last5 = van der Wall | first5 = EE. | last6 = Morreau | first6 = H. | last7 = Smit | first7 = JW. | last8 = Romijn | first8 = JA. | last9 = Bax | first9 = JJ. | title = Increased prevalence of regurgitant valvular heart disease in acromegaly. | journal = J Clin Endocrinol Metab | volume = 89 | issue = 1 | pages = 71-5 | month = Jan | year = 2004 | doi = | PMID = 14715829 }}</ref>
* [[Bacterial Endocarditis]]: The infection can erode the valve, can cause a leaflet to become flail, or a vegetation can prevent coaptation of the leaflets.
*[[Ankylosing spondylitis]]<ref>Palazzi C, D' Angelo S, Lubrano E, Olivieri I. Aortic involvement in ankylosing spondylitis. Clin Exp Rheumatol. May-Jun 2008;26(3 Suppl 49):S131-4.</ref>
* Blunt [[chest trauma]]: Trauma can disrupt the supporting apparatus of the aortic valve.
*[[Annuloaortic ectasia]]
* [[Myxomatous]] aortic valve: The valvular structure can degenerate
*[[Antiphospholipid syndrome]]
* [[Prosthetic valve]] dysfunction
*[[Aortic dissection]]
* [[Rheumatic Fever]]
*[[Aortic valvuloplasty]]
 
*[[Arterial tortuosity syndrome]]
==Differential Diagnosis of the Causes of Chronic Aortic Insufficiency==
*[[Autosomal dominant polycystic kidney disease]]
In alphabetical order:
*[[Bacterial endocarditis]] <ref name="Stiles-1980">{{Cite journal  | last1 = Stiles | first1 = GL. | last2 = Friesinger | first2 = GC. | title = Bacterial endocarditis with aortic regurgitation: implications of embolism. | journal = South Med J | volume = 73 | issue = 5 | pages = 582-6 | month = May | year = 1980 | doi =  | PMID = 7375973 }}</ref>
* [[Ankylosing Spondylitis]]: Ankylosing spondylitis can be associated with inflammation of the aorta ([[aortitis]]).<ref>Palazzi C, D' Angelo S, Lubrano E, Olivieri I. Aortic involvement in ankylosing spondylitis. Clin Exp Rheumatol. May-Jun 2008;26(3 Suppl 49):S131-4.</ref> The inflammatory process can also involve the septum and cause [[conduction disease]].
*[[Aortic stenosis valvuloplasty#Complications|Balloon valvotomy]]
* [[Aortic Dissection]]: A type A dissection may disrupt the integrity of the valve leaflets causing malcoaptation of the aortic leaflets.
*[[Bechterew's Disease|Bechterew's disease]]
* [[Arteriosclerosis]]
*[[Behcet disease]]
* [[Bechterew's Disease]]:  Ankylosing spondylitis can be associated with inflammation of the aorta ([[aortitis]]).<ref>Palazzi C, D' Angelo S, Lubrano E, Olivieri I. Aortic involvement in ankylosing spondylitis. Clin Exp Rheumatol. May-Jun 2008;26(3 Suppl 49):S131-4.</ref>  The inflammatory process can also involve the septum and cause [[conduction disease]].
*[[Bicuspid aortic valve]]<ref name="Roberts-2012">{{Cite journal  | last1 = Roberts | first1 = WC. | last2 = Vowels | first2 = TJ. | last3 = Ko | first3 = JM. | title = Natural history of adults with congenitally malformed aortic valves (unicuspid or bicuspid). | journal = Medicine (Baltimore) | volume = 91 | issue = 6 | pages = 287-308 | month = Nov | year = 2012 | doi = 10.1097/MD.0b013e3182764b84 | PMID = 23117850 }}</ref>
* [[Behcet disease]]: Rarely, in 5% of Behcet's cases there is an [[aortitis]] that involves the ascending aorta and in some cases the [[coronary arteries]].
*[[Cabergoline]]
* [[Bicuspid aortic valve]]: This is the most common congenital abnormality of the ehart.  It is the most common cause of isolated aortic insufficiency requiring surgical repair.  Bicuspid aortic valve is also associated with inflammation of the aorta (an aortopathy) which may lead to dilation of the aorta and or aortic dissection that can worsen the aortic insufficiency.<ref>Friedman T, Mani A, Elefteriades JA. Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity. Expert Rev Cardiovasc Ther. Feb 2008;6(2):235-48.</ref>
* [[Cystic medial necrosis]] of the aorta
* [[Ehlers-Danlos Syndrome]]
* [[Giant cell arteritis]]: [[GCA]] may also be associated with an [[aortitis]] as well as [[coronary artery disease]].<ref>Eberhardt RT, Dhadly M. Giant cell arteritis: diagnosis, management, and cardiovascular implications. Cardiol Rev. Mar-Apr 2007;15(2):55-61.</ref>
* [[Hypertension]]
* [[Marfan Syndrome]]
* [[Myxomatous]] aortic valve
* [[Polymyalgia Rheumatica]]
* [[Pseudoxanthoma Elasticum]]
* [[Reiter's Syndrome]]
* [[Rheumatic Fever]]: Rheumatic fever remains a cause of AS in the Middle East, Northern Africa and Asia as well as some immigrant populations in the United States.  It less prevalent in the US than it was in the 20th century. AR occurs as a result of fibrosis of the valve that is associated with thickening and retraction of the leaflets of the aortic valve.  This retraction results in regurgitation in the center of the valve.  At the same time, there may also be fusion of the aortic leaflets which results in concurrent aortic stenosis.  It should be noted that rheumatic disease of the mitral valve is usually present in the same patient.
* [[Rheumatoid Arthritis]]: [[RA]] rarely causes symptomatic AR, but can as a result of [[granulomatous]] nodules that may form on the aortic leaflets.<ref>Chand EM, Freant LJ, Rubin JW. Aortic valve rheumatoid nodules producing clinical aortic regurgitation and a review of the literature. Cardiovasc Pathol. Nov-Dec 1999;8(6):333-8.</ref>
* [[Sinus of Valsalva Aneurysm]]
* [[Syphilis]]
* [[Systemic Lupus Erythematosus]]: There are tow mechanisms by which [[SLE]] can cause AR. SLE can cause inflammation and fibrosis of the aortic leaflets leading to their damage and retraction.<ref>Jain D, Halushka MK. Cardiac pathology of systemic lupus erythematosus. J Clin Pathol. Jul 2009;62(7):584-92.</ref> SLE can also be associated with the development of sterile verrucous vegetations on the valves called [[Libman-Sacks endocarditis]].<ref>Moyssakis I, Tektonidou MG, Vasilliou VA, Samarkos M, Votteas V, Moutsopoulos HM. Libman-Sacks endocarditis in systemic lupus erythematosus: prevalence, associations, and evolution. Am J Med. Jul 2007;120(7):636-42.</ref><ref>Lee JL, Naguwa SM, Cheema GS, Gershwin ME. Revisiting Libman-Sacks endocarditis: a historical review and update. Clin Rev Allergy Immunol. Jun 2009;36(2-3):126-30.</ref>
* [[Takayasu arteritis]]: [[Takayasu arteritis]] can involve the aorta in an [[aortitis]], the aortic valve itself, and the [[coronary arteries]].<ref>Adachi O, Saiki Y, Akasaka J, Oda K, Iguchi A, Tabayashi K. Surgical management of aortic regurgitation associated with takayasu arteritis and other forms of aortitis. Ann Thorac Surg. Dec 2007;84(6):1950-3.</ref>
* [[Turner's Syndrome]]
* [[Ventricular Septal Defect]]
* [[Weight loss]] medications including [[fenfluramine]] and [[dexfenfluramine]] (a.k.a. [[Phen-Fen]]). These drugs have been associated with degeneration of the aortic valve.
* [[Whipple disease]]: Can involve the aortic valve.<ref>Jeserich M, Ihling C, Holubarsch C. Aortic valve endocarditis with Whipple disease. Ann Intern Med. Jun 1 1997;126(11):920.</ref>
 
==Differential Diagnosis Based Upon Aortic Valvular Causes==
*[[Bicuspid aortic valve]]
*[[Collagen vascular disease]]
*[[Collagen vascular disease]]
*Senile or degenerative calcific aortic valve disease
*[[Aortic valve#Disease of the aortic valve|Congenitally fenestrated cusp rupture]]
*[[Endocarditis]]
*[[Cystic medial necrosis]]
* [[Myxomatous]] aortic valve
*[[Dexfenfluramine]]
*[[Rheumatic fever]]
*[[Dissecting aortic aneurysm]]
*[[Rheumatoid arthritis]]
*[[Dopamine agonists]]<ref name="pmid17202453">{{cite journal |author=Schade R, Andersohn F, Suissa S, Haverkamp W, Garbe E |title=Dopamine agonists and the risk of cardiac-valve regurgitation |journal=[[The New England Journal of Medicine]] |volume=356 |issue=1 |pages=29–38 |year=2007 |month=January |pmid=17202453 |doi=10.1056/NEJMoa062222 |url=http://dx.doi.org/10.1056/NEJMoa062222 |accessdate=2011-03-28}}</ref><ref name="pmid11104741">{{cite journal |author=Rothman RB, Baumann MH, Savage JE, Rauser L, McBride A, Hufeisen SJ, Roth BL |title=Evidence for possible involvement of 5-HT(2B) receptors in the cardiac valvulopathy associated with fenfluramine and other serotonergic medications |journal=[[Circulation]] |volume=102 |issue=23 |pages=2836–41 |year=2000 |month=December |pmid=11104741 |doi= |url=http://circ.ahajourals.org/cgi/pmidlookup?view=long&pmid=11104741 |accessdate=2011-03-28}}</ref><ref name="pmid16092580">{{cite journal |author=Waller EA, Kaplan J, Heckman MG |title=Valvular heart disease in patients taking pergolide |journal=[[Mayo Clinic Proceedings. Mayo Clinic]] |volume=80 |issue=8 |pages=1016–20 |year=2005 |month=August |pmid=16092580 |doi= |url=http://www.mayoclinicproceedings.com/cgi/pmidlookup?view=long&pmid=16092580 |accessdate=2011-03-28}}</ref>
*[[Sinus of Valsalva Aneurysm]]
*[[Transposition of the great vessels corrective surgery|Double switch operation]]
*[[Systemic Lupus Erythematosus]]
*[[Down syndrome]]
*[[Trauma]]
*[[Ehlers-Danlos syndrome]] <ref name="Wunderlich-2005">{{Cite journal  | last1 = Wunderlich | first1 = C. | last2 = Schulze | first2 = MR. | last3 = Strasser | first3 = RH. | title = Severe aortic regurgitation in Ehlers-Danlos syndrome type IV. | journal = Heart | volume = 91 | issue = 1 | pages = 126 | month = Jan | year = 2005 | doi = 10.1136/hrt.2004.035097 | PMID = 15604357 }}</ref>
*[[Turner's Syndrome]]
*[[Fenfluramine]] <ref name="Jollis-2000">{{Cite journal  | last1 = Jollis | first1 = JG. | last2 = Landolfo | first2 = CK. | last3 = Kisslo | first3 = J. | last4 = Constantine | first4 = GD. | last5 = Davis | first5 = KD. | last6 = Ryan | first6 = T. | title = Fenfluramine and phentermine and cardiovascular findings: effect of treatment duration on prevalence of valve abnormalities. | journal = Circulation | volume = 101 | issue = 17 | pages = 2071-7 | month = May | year = 2000 | doi =  | PMID = 10790349 }}</ref>
*[[Ventricular Septal Defect]]
*[[Weight loss]] medications
 
==Differential Diagnosis Based Upon Abnormalities of the Ascending Aorta==
*[[Ankylosing spondylitis]]
*[[Aortic dissection]]
*[[Arteriosclerosis]]
* [[Bechterew's Disease]]
*[[Behcet's syndrome]]
*[[Cystic medial necrosis]] of the [[aorta]]
*[[Ehlers-Danlos Syndrome]]
*[[Giant cell arteritis]]
*[[Giant cell arteritis]]
*[[Hypertension]]
*[[Hurler syndrome]]
*Idiopathic dilation of the [[ascending aorta]]
{{col-break|width=33%}}
*[[Marfan syndrome]]
*[[Hypertension]]<ref name="Kim-1996">{{Cite journal  | last1 = Kim | first1 = M. | last2 = Roman | first2 = MJ. | last3 = Cavallini | first3 = MC. | last4 = Schwartz | first4 = JE. | last5 = Pickering | first5 = TG. | last6 = Devereux | first6 = RB. | title = Effect of hypertension on aortic root size and prevalence of aortic regurgitation. | journal = Hypertension | volume = 28 | issue = 1 | pages = 47-52 | month = Jul | year = 1996 | doi =  | PMID = 8675263 }}</ref>
*[[Osteogenesis imperfecta]]
*[[Inflammatory bowel disease]]
*[[Polymyalgia Rheumatica]]
*[[Marantic endocarditis]]
*[[Pseudoxanthoma Elasticum]]
*[[Marfan syndrome]] <ref name="Brown-1975">{{Cite journal  | last1 = Brown | first1 = OR. | last2 = DeMots | first2 = H. | last3 = Kloster | first3 = FE. | last4 = Roberts | first4 = A. | last5 = Menashe | first5 = VD. | last6 = Beals | first6 = RK. | title = Aortic root dilatation and mitral valve prolapse in Marfan's syndrome: an ECHOCARDIOgraphic study. | journal = Circulation | volume = 52 | issue = 4 | pages = 651-7 | month = Oct | year = 1975 | doi =  | PMID = 1157278 }}</ref>
*[[Maroteaux-Lamy syndrome]]
*[[Methysergide]]
*[[I-cell disease|Mucolipidosis II alpha/beta]]
*[[Pseudo-Hurler polydystrophy|Mucolipidosis III gamma]]
*[[Mucopolysaccharidosis]]
*[[Mucopolysaccharidosis type I Hurler/Scheie syndrome]]
*[[Myxomatous degeneration|Myxomatous aortic valve]]
*[[Osteogenesis imperfecta]] <ref name="Lamanna-2013">{{Cite journal  | last1 = Lamanna | first1 = A. | last2 = Fayers | first2 = T. | last3 = Clarke | first3 = S. | last4 = Parsonage | first4 = W. | title = Valvular and aortic diseases in osteogenesis imperfecta. | journal = Heart Lung Circ | volume = 22 | issue = 10 | pages = 801-10 | month = Oct | year = 2013 | doi = 10.1016/j.hlc.2013.05.640 | PMID = 23791715 }}</ref>
*[[Pergolide]]
*[[Endocarditis|Perivalvular abscess]]
*[[Phentermine]] <ref name="Jollis-2000">{{Cite journal  | last1 = Jollis | first1 = JG. | last2 = Landolfo | first2 = CK. | last3 = Kisslo | first3 = J. | last4 = Constantine | first4 = GD. | last5 = Davis | first5 = KD. | last6 = Ryan | first6 = T. | title = Fenfluramine and phentermine and cardiovascular findings: effect of treatment duration on prevalence of valve abnormalities. | journal = Circulation | volume = 101 | issue = 17 | pages = 2071-7 | month = May | year = 2000 | doi =  | PMID = 10790349 }}</ref>
*[[Polychondritis]]
*[[Polymyalgia rheumatica]]
*[[Prosthetic valve|Prosthetic valve dysfunction]]<ref name="Mullenix-2008">{{Cite journal  | last1 = Mullenix | first1 = PS. | last2 = Parsa | first2 = CJ. | last3 = Mackensen | first3 = GB. | last4 = Jollis | first4 = JG. | last5 = Harrison | first5 = JK. | last6 = Hughes | first6 = GC. | title = Pannus-related prosthetic valve dysfunction and life-threatening aortic regurgitation. | journal = J Heart Valve Dis | volume = 17 | issue = 6 | pages = 666-9 | month = Nov | year = 2008 | doi =  | PMID = 19137799 }}</ref>
*[[Pseudoxanthoma elasticum]] <ref name="COFFMAN-1959">{{Cite journal  | last1 = COFFMAN | first1 = JD. | last2 = SOMMERS | first2 = SC. | title = Familial pseudoxanthoma elasticum and valvular heart disease. | journal = Circulation | volume = 19 | issue = 2 | pages = 242-50 | month = Feb | year = 1959 | doi =  | PMID = 13629785 }}</ref>
*[[Psoriatic arthritis]]
*[[Psoriatic arthritis]]
*[[Q fever]]
*[[Radiation]]
*[[Reactive arthritis]]
*[[Reiter's syndrome]]
*[[Reiter's syndrome]]
*[[Relapsing polychondritis]]
*[[Relapsing polychondritis]]
*Senile aortic ectasia and dilation
*[[Rheumatic fever]] <ref name="Spagnuolo-1971">{{Cite journal  | last1 = Spagnuolo | first1 = M. | last2 = Kloth | first2 = H. | last3 = Taranta | first3 = A. | last4 = Doyle | first4 = E. | last5 = Pasternack | first5 = B. | title = Natural history of rheumatic aortic regurgitation. Criteria predictive of death, congestive heart failure, and angina in young patients. | journal = Circulation | volume = 44 | issue = 3 | pages = 368-80 | month = Sep | year = 1971 | doi =  | PMID = 4255488 }}</ref>
{{col-break|width=33%}}
*[[Rheumatoid arthritis]] <ref name="Levine-1999">{{Cite journal  | last1 = Levine | first1 = AJ. | last2 = Dimitri | first2 = WR. | last3 = Bonser | first3 = RS. | title = Aortic regurgitation in rheumatoid arthritis necessitating aortic valve replacement. | journal = Eur J Cardiothorac Surg | volume = 15 | issue = 2 | pages = 213-4 | month = Feb | year = 1999 | doi =  | PMID = 10219558 }}</ref>
*[[Sarcoidosis]]
*[[Scheie syndrome]]
*[[Annuloaortic ectasia|Senile aortic ectasia and dilation]]
*[[Calcific aortic valve disease|Senile or degenerative calcific aortic valve disease]]
*[[Transposition of the great vessels corrective surgery#Rastelli operation|Senning-Rastelli procedure]]
*[[Shprintzen-Goldberg syndrome]]
*[[Aneurysm of sinus of valsalva|Sinus of valsalva aneurysm]]
*[[Spondyloarthropathy]]
*[[Spondyloarthropathy]]
*[[Syphilitic]] aortitis
*[[Subaortic stenosis]]
*[[Taussig-Bing syndrome|Subpulmonic ventricular septal defect]]
*[[Syphilis]]
*[[Syphilitic aortitis]] <ref name="Aizawa-1998">{{Cite journal  | last1 = Aizawa | first1 = H. | last2 = Hasegawa | first2 = A. | last3 = Arai | first3 = M. | last4 = Naganuma | first4 = F. | last5 = Hatori | first5 = M. | last6 = Kanda | first6 = T. | last7 = Suzuki | first7 = T. | last8 = Murata | first8 = K. | last9 = Satoh | first9 = Y. | title = Bilateral coronary ostial stenosis and aortic regurgitation due to syphilitic aortitis. | journal = Intern Med | volume = 37 | issue = 1 | pages = 56-9 | month = Jan | year = 1998 | doi =  | PMID = 9510401 }}</ref>
*[[Systemic lupus erythematosus]] <ref name="Miyashita-2010">{{Cite journal  | last1 = Miyashita | first1 = T. | last2 = Abe | first2 = Y. | last3 = Kato | first3 = Y. | last4 = Nakagawa | first4 = E. | last5 = Komatsu | first5 = R. | last6 = Hattori | first6 = K. | last7 = Shibata | first7 = T. | last8 = Yoshioka | first8 = K. | last9 = Naruko | first9 = T. | title = Aortic aneurysm with severe aortic regurgitation in a patient with systemic lupus erythematosus. | journal = Intern Med | volume = 49 | issue = 20 | pages = 2263-6 | month =  | year = 2010 | doi =  | PMID = 20962447 }}</ref>
*[[Takayasu arteritis]]
*[[Takayasu arteritis]]
*[[Trauma]]
*[[Tetralogy of Fallot]]
*[[Thoracic aortic aneurysm]]
*[[Traumatic aortic rupture]]
*[[Chest trauma|Traumatic rupture of valve leaflets]]
*[[Truncus arteriosus]]
*[[Turner's syndrome]]
*[[Ulcerative colitis]]
*[[Ulcerative colitis]]
*[[Whipple's disease]]
*[[Aortic valve#Disease of the aortic valve|Unicuspid aortic valve]]
*[[Ventricular septal defect]]
*[[Whipple disease]]
{{col-end}}
 
===Causes of Aortic Insufficiency as of Type of Onset===
====Causes of Acute Aortic Insufficiency====
The causes of Acute Aortic Insufficiency  include the following:<ref name="pmid19564568">{{cite journal| author=Stout KK, Verrier ED| title=Acute valvular regurgitation. | journal=Circulation | year= 2009 | volume= 119 | issue= 25 | pages= 3232-41 | pmid=19564568 | doi=10.1161/CIRCULATIONAHA.108.782292 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19564568  }} </ref><ref name="pmid21423463">{{cite journal| author=Mokadam NA, Stout KK, Verrier ED| title=Management of acute regurgitation in left-sided cardiac valves. | journal=Tex Heart Inst J | year= 2011 | volume= 38 | issue= 1 | pages= 9-19 | pmid=21423463 | doi= | pmc=3060740 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21423463  }} </ref><ref name="pmid10724525">{{cite journal| author=Blaszyk H, Witkiewicz AJ, Edwards WD| title=Acute aortic regurgitation due to spontaneous rupture of a fenestrated cusp: report in a 65-year-old man and review of seven additional cases. | journal=Cardiovasc Pathol | year= 1999 | volume= 8 | issue= 4 | pages= 213-6 | pmid=10724525 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10724525  }} </ref><ref name="pmid2016464">{{cite journal| author=Isner JM| title=Acute catastrophic complications of balloon aortic valvuloplasty. The Mansfield Scientific Aortic Valvuloplasty Registry Investigators. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 6 | pages= 1436-44 | pmid=2016464 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2016464  }} </ref>
 
*[[Aortic valvuloplasty]]<ref name="pmid2016464">{{cite journal |author=Isner JM |title=Acute catastrophic complications of balloon aortic valvuloplasty. The Mansfield Scientific Aortic Valvuloplasty Registry Investigators |journal=[[Journal of the American College of Cardiology]] |volume=17 |issue=6 |pages=1436–44 |year=1991 |month=May |pmid=2016464 |doi= |url= |accessdate=2011-03-28}}</ref>
*A [[Aortic dissection classification#Stanford Classification System|type A aortic dissection]] may disrupt the integrity of the valve leaflets causing malcoaptation of the aortic leaflets.<ref name="Lai-2003">{{Cite journal  | last1 = Lai | first1 = DT. | last2 = Miller | first2 = DC. | last3 = Mitchell | first3 = RS. | last4 = Oyer | first4 = PE. | last5 = Moore | first5 = KA. | last6 = Robbins | first6 = RC. | last7 = Shumway | first7 = NE. | last8 = Reitz | first8 = BA. | title = Acute type A aortic dissection complicated by aortic regurgitation: composite valve graft versus separate valve graft versus conservative valve repair. | journal = J Thorac Cardiovasc Surg | volume = 126 | issue = 6 | pages = 1978-86 | month = Dec | year = 2003 | doi = 10.1016/S0022 | PMID = 14688716 }}</ref>
*In [[bacterial endocarditis]] the [[infection]] can erode the valve and cause a leaflet to become flail, or a vegetation can prevent coaptation of the leaflets.
*Blunt [[chest trauma]] can disrupt the supporting apparatus of the [[aortic valve]].
*[[Myxomatous degeneration|Myxomatous aortic valve]] can degenerate, leading to insufficiency.
*[[Prosthetic valve|Prosthetic valve dysfunction]]<ref>Friedman T, Mani A, Elefteriades JA. Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity. Expert Rev Cardiovasc Ther. Feb 2008;6(2):235-48.</ref>
*[[Rheumatic fever]]<ref name="Spagnuolo-1971">{{Cite journal  | last1 = Spagnuolo | first1 = M. | last2 = Kloth | first2 = H. | last3 = Taranta | first3 = A. | last4 = Doyle | first4 = E. | last5 = Pasternack | first5 = B. | title = Natural history of rheumatic aortic regurgitation. Criteria predictive of death, congestive heart failure, and angina in young patients. | journal = Circulation | volume = 44 | issue = 3 | pages = 368-80 | month = Sep | year = 1971 | doi =  | PMID = 4255488 }}</ref>
*Rupture of a [[Aortic valve#Disease of the aortic valve|congenitally fenestrated cusp]]<ref name="Stout-2009">{{Cite journal  | last1 = Stout | first1 = KK. | last2 = Verrier | first2 = ED. | title = Acute valvular regurgitation. | journal = Circulation | volume = 119 | issue = 25 | pages = 3232-41 | month = Jun | year = 2009 | doi = 10.1161/CIRCULATIONAHA.108.782292 | PMID = 19564568 }}</ref>
 
====Causes of Chronic Aortic Insufficiency====
The cause of Chronic Aortic Insufficiency include the following:<ref name="pmid15470217">{{cite journal| author=Enriquez-Sarano M, Tajik AJ| title=Clinical practice. Aortic regurgitation. | journal=N Engl J Med | year= 2004 | volume= 351 | issue= 15 | pages= 1539-46 | pmid=15470217 | doi=10.1056/NEJMcp030912 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15470217  }} </ref><ref name="pmid10051296">{{cite journal| author=Devlin WH, Petrusha J, Briesmiester K, Montgomery D, Starling MR| title=Impact of vascular adaptation to chronic aortic regurgitation on left ventricular performance. | journal=Circulation | year= 1999 | volume= 99 | issue= 8 | pages= 1027-33 | pmid=10051296 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10051296  }} </ref>
*[[Ankylosing spondylitis]] can be associated with inflammation of the [[aorta]] ([[aortitis]]).<ref>Palazzi C, D' Angelo S, Lubrano E, Olivieri I. Aortic involvement in ankylosing spondylitis. Clin Exp Rheumatol. May-Jun 2008;26(3 Suppl 49):S131-4.</ref>  The inflammatory process can also involve the septum and cause [[conduction disease]].
*A [[Aortic dissection classification#Stanford Classification System|type A aortic dissection]] may disrupt the integrity of the valve leaflets causing malcoaptation of the aortic leaflets.
*[[Bechterew's Disease|Bechterew's disease]]
*Rarely, in 5% of cases with [[Behcet disease]] there is an [[aortitis]] that involves the [[ascending aorta]] and in some cases the [[coronary arteries]] are involved.
*[[Bicuspid aortic valve]] is the most common congenital abnormality of the [[heart]].  It is the most common cause of isolated aortic insufficiency requiring surgical repair.  Bicuspid aortic valve is also associated with [[inflammation]] of the [[aorta]] (an aortopathy) which may lead to dilation of the [[aorta]] and/or [[aortic dissection]] that can worsen the aortic insufficiency.<ref>Friedman T, Mani A, Elefteriades JA. Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity. Expert Rev Cardiovasc Ther. Feb 2008;6(2):235-48.</ref>
*[[Cystic medial necrosis]] of the aorta
*[[Ehlers-Danlos syndrome]]
*[[Giant cell arteritis]] may also be associated with an [[aortitis]] as well as [[coronary artery disease]].<ref>Eberhardt RT, Dhadly M. Giant cell arteritis: diagnosis, management, and cardiovascular implications. Cardiol Rev. Mar-Apr 2007;15(2):55-61.</ref>
*[[Hypertension]]
*[[Marfan Syndrome]]
*[[Myxomatous degeneration|Myxomatous aortic valve]]
*[[Polymyalgia rheumatica]]
*[[Pseudoxanthoma elasticum]]
*[[Reiter's syndrome]]
*[[Rheumatic fever]] remains a cause of [[AS]] in the Middle East, Northern Africa and Asia as well as some immigrant populations in the United States.  It is less prevalent in the US than it was in the 20th century.  AR occurs as a result of [[fibrosis]] of the valve that is associated with thickening and retraction of the leaflets of the [[aortic valve]].  This retraction results in regurgitation in the center of the valve.  At the same time, there may also be fusion of the aortic leaflets which results in concurrent [[aortic stenosis]].  It should be noted that rheumatic disease of the [[mitral valve]] is usually present in the same patient.
*[[Rheumatoid arthritis]] rarely causes symptomatic AR, but it can cause [[granulomatous]] nodules on the aortic leaflets which leads to clinical aortic regurgitation.<ref>Chand EM, Freant LJ, Rubin JW. Aortic valve rheumatoid nodules producing clinical aortic regurgitation and a review of the literature. Cardiovasc Pathol. Nov-Dec 1999;8(6):333-8.</ref>
*[[Aneurysm of sinus of valsalva|Sinus of valsalva aneurysm]]
*[[Syphilis]]
*[[Systemic lupus erythematosus]] can cause AR by tow mechanisms.  SLE can cause [[inflammation]] and [[fibrosis]] of the aortic leaflets leading to their damage and retraction.<ref>Jain D, Halushka MK. Cardiac pathology of systemic lupus erythematosus. J Clin Pathol. Jul 2009;62(7):584-92.</ref>  SLE can also be associated with the development of sterile verrucous vegetations on the valves called [[Libman-Sacks endocarditis]].<ref>Moyssakis I, Tektonidou MG, Vasilliou VA, Samarkos M, Votteas V, Moutsopoulos HM. Libman-Sacks endocarditis in systemic lupus erythematosus: prevalence, associations, and evolution. Am J Med. Jul 2007;120(7):636-42.</ref><ref>Lee JL, Naguwa SM, Cheema GS, Gershwin ME. Revisiting Libman-Sacks endocarditis: a historical review and update. Clin Rev Allergy Immunol. Jun 2009;36(2-3):126-30.</ref>
*[[Takayasu arteritis]] can involve the [[aorta]] in an [[aortitis]], the [[aortic valve]] itself, and the [[coronary arteries]].<ref>Adachi O, Saiki Y, Akasaka J, Oda K, Iguchi A, Tabayashi K. Surgical management of aortic regurgitation associated with takayasu arteritis and other forms of aortitis. Ann Thorac Surg. Dec 2007;84(6):1950-3.</ref>
*[[Turner's syndrome]]
*[[Ventricular septal defect]]<ref name="Schmaltz-2004">{{Cite journal  | last1 = Schmaltz | first1 = AA. | last2 = Schaefer | first2 = M. | last3 = Hentrich | first3 = F. | last4 = Neudorf | first4 = U. | last5 = Brecher | first5 = AM. | last6 = Asfour | first6 = B. | last7 = Urban | first7 = AE. | title = [Ventricular septal defect and aortic regurgitation-pathophysiological aspects and therapeutic consequences]. | journal = Z Kardiol | volume = 93 | issue = 3 | pages = 194-200 | month = Mar | year = 2004 | doi = 10.1007/s00392-004-0015-2 | PMID = 15024586 }}</ref>
*[[Weight loss]] medications including [[fenfluramine]] and [[dexfenfluramine]] (a.k.a. [[Phen-Fen]]).  These drugs have been associated with degeneration of the aortic valve.
* [[Whipple disease]] can involve the aortic valve.<ref>Jeserich M, Ihling C, Holubarsch C. Aortic valve endocarditis with Whipple disease. Ann Intern Med. Jun 1 1997;126(11):920.</ref>


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


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[[CME Category::Cardiology]]
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[[Category:Valvular heart disease]]
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[[Category:Mature chapter]]
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Latest revision as of 04:33, 7 July 2020


Aortic Regurgitation Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Varun Kumar, M.B.B.S.; Lakshmi Gopalakrishnan, M.B.B.S.; Vendhan Ramanujam M.B.B.S [3]; Usama Talib, BSc, MD [4]

Overview

Aortic insufficiency can be an acute illness or a chronic illness and the causes differ depending upon the acuity of the disease. In general, aortic insufficiency is due to abnormalities of the aortic valve itself or the aortic root. Aortic regurgitation secondary to dilation of the ascending aorta has overtaken the valvular aortic disease as the most common cause of aortic regurgitation.It can also occur after surgical valve placement.

Aortic regurgitation causes

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 Location of the Abnormality

The cause of Aortic Regurgitation by location can be classified as following:[1]

Causes Based Upon Abnormalities in the Aortic Valve

Causes Based Upon Abnormalities in the Ascending Aorta

Causes by Organ System

Cardiovascular Annuloaortic ectasia, aortic valvuloplasty, aortic dissection, arterial tortuosity syndrome, bacterial endocarditis, balloon valvotomy, bicuspid aortic valve, congenitally fenestrated cusp rupture, cystic medial necrosis, hypertension, marantic endocarditis, prosthetic valve dysfunction, senile aortic ectasia and dilation, senile or degenerative calcific aortic valve disease, sinus of valsalva aneurysm, subaortic stenosis, syphilitic aortitis, subpulmonic ventricular septal defect, tetralogy of Fallot, thoracic aortic aneurysm, unicuspid aortic valve, ventricular septal defect
Chemical / poisoning No underlying causes
Dermatologic Psoriatic arthritis
Drug Side Effect Cabergoline, dexfenfluramine, dopamine agonists, fenfluramine, methysergide, pergolide, phentermine
Ear Nose Throat No underlying causes
Endocrine Acromegaly
Environmental No underlying causes
Gastroenterologic Inflammatory bowel disease, ulcerative colitis, Whipple disease
Genetic Ankylosing spondylitis, autosomal dominant polycystic kidney disease, bicuspid aortic valve, Down syndrome, Ehlers-Danlos syndrome, Hurler syndrome, Marfan syndrome, Maroteaux-Lamy syndrome, mucolipidosis II alpha/beta, mucolipidosis III gamma, mucopolysaccharidosis, mucopolysaccharidosis type I Hurler/Scheie syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum, rheumatoid arthritis, Scheie syndrome, Shprintzen-Goldberg syndrome, truncus arteriosus, Turner's syndrome, unicuspid aortic valve
Hematologic No underlying causes
Iatrogenic Aortic valvuloplasty, balloon valvotomy, double switch operation, prosthetic valve dysfunction, radiation, Senning-Rastelli procedure
Infectious Disease Bacterial endocarditis, perivalvular abscess, Q fever, rheumatic fever, syphilis, syphilitic aortitis
Musculoskeletal / Ortho Ankylosing spondylitis, collagen vascular disease, Ehlers-Danlos syndrome, osteogenesis imperfecta, psoriatic arthritis, Reiter's Syndrome, relapsing polychondritis, rheumatoid arthritis, spondyloarthropathy, systemic lupus erythematosus
Neurologic No underlying causes
Nutritional / Metabolic Mucolipidosis II alpha/beta, mucolipidosis III gamma
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 Autosomal dominant polycystic kidney disease
Rheum / Immune / Allergy Ankylosing spondylitis, antiphospholipid syndrome, Bechterew's disease, Behcet disease, collagen vascular disease, cystic medial necrosis, Ehlers-Danlos syndrome, giant cell arteritis, inflammatory bowel disease, Marfan syndrome, myxomatous aortic valve, osteogenesis imperfecta, polychondritis, polymyalgia rheumatica, pseudoxanthoma elasticum, psoriatic arthritis, reactive arthritis, Reiter's syndrome, relapsing polychondritis, rheumatic fever, rheumatoid arthritis, sarcoidosis, spondyloarthropathy, systemic lupus erythematosus, Takayasu arteritis, ulcerative colitis, Whipple disease
Sexual No underlying causes
Trauma Chest trauma, traumatic aortic rupture
Urologic No underlying causes
Miscellaneous No underlying causes

Causes in Alphabetical Order

Causes of Aortic Insufficiency as of Type of Onset

Causes of Acute Aortic Insufficiency

The causes of Acute Aortic Insufficiency include the following:[33][34][35][10]

Causes of Chronic Aortic Insufficiency

The cause of Chronic Aortic Insufficiency include the following:[1][39]

References

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  23. Waller EA, Kaplan J, Heckman MG (2005). "Valvular heart disease in patients taking pergolide". Mayo Clinic Proceedings. Mayo Clinic. 80 (8): 1016–20. PMID 16092580. Retrieved 2011-03-28. Unknown parameter |month= ignored (help)
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  37. Friedman T, Mani A, Elefteriades JA. Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity. Expert Rev Cardiovasc Ther. Feb 2008;6(2):235-48.
  38. Stout, KK.; Verrier, ED. (2009). "Acute valvular regurgitation". Circulation. 119 (25): 3232–41. doi:10.1161/CIRCULATIONAHA.108.782292. PMID 19564568. Unknown parameter |month= ignored (help)
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  40. Palazzi C, D' Angelo S, Lubrano E, Olivieri I. Aortic involvement in ankylosing spondylitis. Clin Exp Rheumatol. May-Jun 2008;26(3 Suppl 49):S131-4.
  41. Friedman T, Mani A, Elefteriades JA. Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity. Expert Rev Cardiovasc Ther. Feb 2008;6(2):235-48.
  42. Eberhardt RT, Dhadly M. Giant cell arteritis: diagnosis, management, and cardiovascular implications. Cardiol Rev. Mar-Apr 2007;15(2):55-61.
  43. Chand EM, Freant LJ, Rubin JW. Aortic valve rheumatoid nodules producing clinical aortic regurgitation and a review of the literature. Cardiovasc Pathol. Nov-Dec 1999;8(6):333-8.
  44. Jain D, Halushka MK. Cardiac pathology of systemic lupus erythematosus. J Clin Pathol. Jul 2009;62(7):584-92.
  45. Moyssakis I, Tektonidou MG, Vasilliou VA, Samarkos M, Votteas V, Moutsopoulos HM. Libman-Sacks endocarditis in systemic lupus erythematosus: prevalence, associations, and evolution. Am J Med. Jul 2007;120(7):636-42.
  46. Lee JL, Naguwa SM, Cheema GS, Gershwin ME. Revisiting Libman-Sacks endocarditis: a historical review and update. Clin Rev Allergy Immunol. Jun 2009;36(2-3):126-30.
  47. Adachi O, Saiki Y, Akasaka J, Oda K, Iguchi A, Tabayashi K. Surgical management of aortic regurgitation associated with takayasu arteritis and other forms of aortitis. Ann Thorac Surg. Dec 2007;84(6):1950-3.
  48. Schmaltz, AA.; Schaefer, M.; Hentrich, F.; Neudorf, U.; Brecher, AM.; Asfour, B.; Urban, AE. (2004). "[Ventricular septal defect and aortic regurgitation-pathophysiological aspects and therapeutic consequences]". Z Kardiol. 93 (3): 194–200. doi:10.1007/s00392-004-0015-2. PMID 15024586. Unknown parameter |month= ignored (help)
  49. Jeserich M, Ihling C, Holubarsch C. Aortic valve endocarditis with Whipple disease. Ann Intern Med. Jun 1 1997;126(11):920.

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