Aortic stenosis can be either congenital or acquired, and its common causes vary between different age groups and between different populations. Rheumatic heart disease remains the most common cause of aortic stenosis worldwide. Calcification of the aortic valve is the most common cause of aortic stenosis in North America and Europe. While calcification of congenital bicuspid aortic valve is the most common cause of aortic stenosis in people less than 70 years of age, acquired degenerative calcific aortic stenosis is the most common cause in older patients above the age of 70 years.

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated. Aortic stenosis is a progressive disease and does not have a life threatening cause. However, complications resulting from untreated disease name is common.

Common causes of aortic stenosis may include:^[1][2][3][4]

• Calcific aortic valve disease
• A congenitally abnormal aortic valve with superimposed calcification (unicuspid or bicuspid).
• Rheumatic heart disease

• Acquired
  • Alkaptonuria^[5]
  • Aortic sclerosis^[6]
  • Hyperuricemia^[7]
  • Paget’s disease^[8]
  • Radiation ^[9]
  • Rheumatic fever^[4]
  • Systemic lupus erythematosus^[10]

• Congenital
  • Bardet-Biedl syndrome^[11]
  • Bicuspid aortic valve^[2]
  • Fabry’s disease^[12]
  • Familial hypercholesterolemia^[13]
  • Familial thoracic aortic aneurysm syndrome^[14]
  • Hypoplastic aortic annulus^[15]
  • Subaortic stenosis short stature syndrome (Onat syndrome) ^{_Genetic_and_Rare_Diseases_Information_Center_(GARD)_–_an_NCATS_Program-16|[16]}
  • Quadricuspid aortic valve^[17]
  • Singleton-Merten syndrome^[18]
  • Sweet syndrome^[19]
  • Tricuspid aortic valve with commissural fushion^[20]
  • Turner syndrome^[21]
  • Unicuspid aortic valve^[22]

• Familial subvalvular aortic stenosis^[23]
• Hypertrophic cardiomyopathy^[24]
• Idiopathic hypertrophic subaortic stenosis^[25]

• Aortitis^[26]
• Familial supravalvular aortic stenosis^[27]
• Homozygous familial hypercholesterolemia^[28]
• Hypoplastic left heart syndrome^[29]
• Williams syndrome^[30][31]

• Bicuspid aortic valve^[32]
• Rheumatic heart disease^[33]
• Degenerative calcification^[34]
• Uni-commissural aortic valve^[22]
• Hypoplasia of the annulus^[35]

• Degenerative calcification^[34]
• Bicuspid aortic valve^[2]
• Rheumatic heart disease^[36]
• Hypoplasia of the annulus^[35]

Cardiology