Aortic insufficiency causes


 * Associate Editor-In-Chief: ; Varun Kumar, M.B.B.S.; Lakshmi Gopalakrishnan, M.B.B.S.

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

 * Worldwide the most common cause of aortic insufficiency is rheumatic heart disease.
 * In the United States, senile degenerative calcific aortic valve disease and bicuspid aortic valve disease are the most common causes.
 * Aortic regurgitation secondary to dilation of the ascending aorta has overtaken valvular aortic disease as the most common cause of aortic regurgitation.

Differential Diagnosis of the Causes of Acute Aortic Insufficiency
In alphabetical order:
 * After aortic valvuloplasty
 * Aortic dissection: A type A dissection may disrupt the integrity of the valve leaflets causing malcoaptation of the aortic leaflets.
 * Bacterial Endocarditis: The infection can erode the valve, can cause a leaflet to become flail, or a vegetation can prevent coaptation of the leaflets.
 * Blunt chest trauma: Trauma can disrupt the supporting apparatus of the aortic valve.
 * Myxomatous aortic valve: The valvular structure can degenerate
 * Prosthetic valve dysfunction
 * Rheumatic Fever

Differential Diagnosis of the Causes of Chronic Aortic Insufficiency
In alphabetical order:
 * Ankylosing Spondylitis: Ankylosing spondylitis can be associated with inflammation of the aorta (aortitis). The inflammatory process can also involve the septum and cause conduction disease.
 * Aortic Dissection: A type A dissection may disrupt the integrity of the valve leaflets causing malcoaptation of the aortic leaflets.
 * Arteriosclerosis
 * Bechterew's Disease: Ankylosing spondylitis can be associated with inflammation of the aorta (aortitis).  The inflammatory process can also involve the septum and cause conduction disease.
 * 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.
 * 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.
 * 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.
 * 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.
 * 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. SLE can also be associated with the development of sterile verrucous vegetations on the valves called Libman-Sacks endocarditis.
 * Takayasu arteritis: Takayasu arteritis can involve the aorta in an aortitis, the aortic valve itself, and the coronary arteries.
 * 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.

Differential Diagnosis Based Upon Aortic Valvular Causes

 * Bicuspid aortic valve
 * Collagen vascular disease
 * Senile or degenerative calcific aortic valve disease
 * Endocarditis
 * Myxomatous aortic valve
 * Rheumatic fever
 * Rheumatoid arthritis
 * Sinus of Valsalva Aneurysm
 * Systemic Lupus Erythematosus
 * Trauma
 * Turner's Syndrome
 * 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
 * Hypertension
 * Idiopathic dilation of the ascending aorta
 * Marfan syndrome
 * Osteogenesis imperfecta
 * Polymyalgia Rheumatica
 * Pseudoxanthoma Elasticum
 * Psoriatic arthritis
 * Reiter's syndrome
 * Relapsing polychondritis
 * Senile aortic ectasia and dilation
 * Spondyloarthropathy
 * Syphilitic aortitis
 * Takayasu arteritis
 * Trauma
 * Ulcerative colitis
 * Whipple's disease