Aortic regurgitation causes

Resident Survival Guide

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]

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

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

• Aortic dissection
• Bacterial endocarditis

Common Causes

• Bicuspid aortic valve
• Senile or degenerative calcific aortic valve disease
• Rheumatic heart disease
• After Valvular Surgery

Causes by Location of the Abnormality

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

Causes Based Upon Abnormalities in the Aortic Valve

• 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

Causes Based Upon Abnormalities in 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

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

• Acromegaly
• Ankylosing spondylitis
• Annuloaortic ectasia
• Antiphospholipid syndrome
• Aortic dissection
• Aortic valvuloplasty
• Arterial tortuosity syndrome
• Autosomal dominant polycystic kidney disease
• Bacterial endocarditis
• Balloon valvotomy
• Bechterew's disease
• Behcet disease
• Bicuspid aortic valve
• Cabergoline
• Collagen vascular disease
• Congenitally fenestrated cusp rupture
• Cystic medial necrosis
• Dexfenfluramine
• Dissecting aortic aneurysm
• Dopamine agonists
• Double switch operation
• Down syndrome
• Ehlers-Danlos syndrome
• Fenfluramine
• Giant cell arteritis
• Hurler syndrome

• Hypertension
• Inflammatory bowel disease
• Marantic endocarditis
• Marfan syndrome
• Maroteaux-Lamy syndrome
• Methysergide
• Mucolipidosis II alpha/beta
• Mucolipidosis III gamma
• Mucopolysaccharidosis
• Mucopolysaccharidosis type I Hurler/Scheie syndrome
• Myxomatous aortic valve
• Osteogenesis imperfecta
• Pergolide
• Perivalvular abscess
• Phentermine
• Polychondritis
• Polymyalgia rheumatica
• Prosthetic valve dysfunction
• Pseudoxanthoma elasticum
• Psoriatic arthritis
• Q fever
• Radiation
• Reactive arthritis
• Reiter's syndrome
• Relapsing polychondritis
• Rheumatic fever

• Rheumatoid arthritis
• Sarcoidosis
• Scheie syndrome
• Senile aortic ectasia and dilation
• Senile or degenerative calcific aortic valve disease
• Senning-Rastelli procedure
• Shprintzen-Goldberg syndrome
• Sinus of valsalva aneurysm
• Spondyloarthropathy
• Subaortic stenosis
• Subpulmonic ventricular septal defect
• Syphilis
• Syphilitic aortitis
• Systemic lupus erythematosus
• Takayasu arteritis
• Tetralogy of Fallot
• Thoracic aortic aneurysm
• Traumatic aortic rupture
• Traumatic rupture of valve leaflets
• Truncus arteriosus
• Turner's syndrome
• Ulcerative colitis
• Unicuspid aortic valve
• Ventricular septal defect
• Whipple disease

Causes of Aortic Insufficiency as of Type of Onset

Causes of Acute Aortic Insufficiency

The causes of Acute Aortic Insufficiency include the following:

• Aortic valvuloplasty
• A type A aortic dissection may disrupt the integrity of the valve leaflets causing malcoaptation of the aortic leaflets.
• 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 aortic valve can degenerate, leading to insufficiency.
• Prosthetic valve dysfunction
• Rheumatic fever
• Rupture of a congenitally fenestrated cusp

Causes of Chronic Aortic Insufficiency

• Ankylosing spondylitis can be associated with inflammation of the aorta (aortitis). The inflammatory process can also involve the septum and cause conduction disease.
• A type A aortic dissection may disrupt the integrity of the valve leaflets causing malcoaptation of the aortic leaflets.
• 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.
• 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.
• Hypertension
• Marfan Syndrome
• 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.
• 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. SLE can also be associated with the development of sterile verrucous vegetations on the valves called Libman-Sacks endocarditis.
• 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.