Aortic regurgitation causes

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.

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.

Causes

Common Causes

• Worldwide the most common cause of aortic insufficiency is the 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.
• In general, aortic insufficiency is due to abnormalities of the aortic valve itself or the aortic root.

Aortic Valve Diseases

• Rheumatic fever causes retraction of the cusps with resultant mixed aortic stenosis and aortic insufficiency with involvement of mitral valve disease.
• Infective endocarditis
• Traumatic aortic rupture following blunt chest trauma or deceleration injury.
• Congenital bicuspid aortic valve associated with increased risk of aortic dissection which then leads to aortic insufficiency.
• Dopamine agonists

Aortic Root Diseases

• Hypertension
• Aortic dissection
• Marfan syndrome
• HLA-B27 positive conditions: psoriatic arthritis, ankylosing spondylitis, Reiter's syndrome
• Giant cell arteritis
• Syphilitic aortitis

Causes by Organ System

Cardiovascular	After aortic valvuloplasty, Aortic dissection, Arteriosclerosis, Bacterial endocarditis, Bicuspid aortic valve, Prosthetic valve dysfunction, 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
Chemical / poisoning	No underlying causes
Dermatologic	Psoriatic arthritis
Drug Side Effect	Fenfluramine and Dexfenfluramine: These drugs have been associated with degeneration of the aortic valve.
Ear Nose Throat	No underlying causes
Endocrine	No underlying causes
Environmental	No underlying causes
Gastroenterologic	Ulcerative colitis, Whipple disease
Genetic	Ankylosing spondylitis, Ehlers-Danlos syndrome, Marfan syndrome, Osteogenesis imperfecta, Pseudoxanthoma elasticum, Reiter's syndrome, Rheumatoid arthritis, Systemic lupus erythematosus, Takayasu arteritis, Turner's syndrome
Hematologic	No underlying causes
Iatrogenic	After aortic valvuloplasty, Prosthetic valve dysfunction
Infectious Disease	Bacterial endocarditis, Rheumatic fever, Syphilis
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	No underlying causes
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	Ankylosing spondylitis, 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, Spondyloarthropathy, Systemic lupus erythematosus, Takayasu arteritis, Ulcerative colitis, Whipple disease
Sexual	Syphilis
Trauma	Blunt chest trauma disrupting the supporting apparatus of the aortic valve.
Urologic	No underlying causes
Miscellaneous	Myxomatous aortic valve

Causes in Alphabetical Order

Causes of Acute Aortic Insufficiency

• 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

Causes of Chronic Aortic Insufficiency

• 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.