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Coronary artery atherosclerosis

MedGen UID:
3623
Concept ID:
C0010054
Disease or Syndrome
Synonym: Coronary atherosclerosis
SNOMED CT: Arteriosclerotic heart disease (53741008); Coronary sclerosis (53741008); Coronary atherosclerosis (443502000); Atherosclerosis of coronary artery (443502000); Atherosclerosis of native coronary artery (443502000); Coronary arteriosclerosis (53741008)
 
HPO: HP:0001677
Monarch Initiative: MONDO:0021661

Definition

Reduction of the diameter of the coronary arteries as the result of an accumulation of atheromatous plaques within the walls of the coronary arteries, which increases the risk of myocardial ischemia. [from HPO]

Conditions with this feature

Tangier disease
MedGen UID:
52644
Concept ID:
C0039292
Disease or Syndrome
Tangier disease is characterized by severe deficiency or absence of high-density lipoprotein (HDL) in the circulation resulting in tissue accumulation of cholesteryl esters throughout the body, particularly in the reticuloendothelial system. The major clinical signs of Tangier disease include hyperplastic yellow-orange tonsils, hepatosplenomegaly, and peripheral neuropathy, which may be either relapsing-remitting or chronic progressive in nature. Rarer complications may include corneal opacities that typically do not affect vision, premature atherosclerotic coronary artery disease occurring in the sixth and seventh decades of life (not usually before age 40 years), and mild hematologic manifestations, such as mild thrombocytopenia, reticulocytosis, stomatocytosis, or hemolytic anemia. The clinical expression of Tangier disease is variable, with some affected individuals only showing biochemical perturbations.
Hypercholesterolemia, familial, 1
MedGen UID:
152875
Concept ID:
C0745103
Disease or Syndrome
Familial hypercholesterolemia (FH) is characterized by significantly elevated low-density lipoprotein cholesterol (LDL-C) that leads to atherosclerotic plaque deposition in the coronary arteries and proximal aorta at an early age and increases the risk of premature cardiovascular events such as angina and myocardial infarction; stroke occurs more rarely. Xanthomas (cholesterol deposits in tendons) may be visible in the Achilles tendons or tendons of the hands and worsen with age as a result of extremely high cholesterol levels. Xanthelasmas (yellowish, waxy deposits) can occur around the eyelids. Individuals with FH may develop corneal arcus (white, gray, or blue opaque ring in the corneal margin as a result of cholesterol deposition) at a younger age than those without FH. Individuals with a more severe phenotype, often as a result of biallelic variants, can present with very significant elevations in LDL-C (>500 mg/dL), early-onset coronary artery disease (CAD; presenting as early as childhood in some), and calcific aortic valve disease.
Autosomal recessive inherited pseudoxanthoma elasticum
MedGen UID:
698415
Concept ID:
C1275116
Disease or Syndrome
Pseudoxanthoma elasticum (PXE) is a systemic disorder that affects the elastic tissue of the skin, the eye, and vascular system. Individuals most commonly present with angioid streaks of the retina found on routine eye examination or associated with retinal hemorrhage and/or characteristic papules in the skin. The most frequent cause of morbidity and disability in PXE is reduced vision due to complications of subretinal neovascularizations and macular atrophy. Other manifestations include premature gastrointestinal angina and/or bleeding, intermittent claudication of arm and leg muscles, stroke, renovascular hypertension, and cardiovascular complications (angina/myocardial infarction). Most affected individuals live a normal life span.
Hypercholesterolemia, autosomal dominant, type B
MedGen UID:
309962
Concept ID:
C1704417
Disease or Syndrome
Familial hypercholesterolemia (FH) is characterized by significantly elevated low-density lipoprotein cholesterol (LDL-C) that leads to atherosclerotic plaque deposition in the coronary arteries and proximal aorta at an early age and increases the risk of premature cardiovascular events such as angina and myocardial infarction; stroke occurs more rarely. Xanthomas (cholesterol deposits in tendons) may be visible in the Achilles tendons or tendons of the hands and worsen with age as a result of extremely high cholesterol levels. Xanthelasmas (yellowish, waxy deposits) can occur around the eyelids. Individuals with FH may develop corneal arcus (white, gray, or blue opaque ring in the corneal margin as a result of cholesterol deposition) at a younger age than those without FH. Individuals with a more severe phenotype, often as a result of biallelic variants, can present with very significant elevations in LDL-C (>500 mg/dL), early-onset coronary artery disease (CAD; presenting as early as childhood in some), and calcific aortic valve disease.
Familial partial lipodystrophy, Kobberling type
MedGen UID:
318591
Concept ID:
C1720859
Disease or Syndrome
Familial partial lipodystrophy type 1 (FPLD1), or Kobberling-type lipodystrophy, is characterized by loss of adipose tissue confined to the extremities, with normal or increased distribution of fat on the face, neck, and trunk (Kobberling and Dunnigan, 1986). For a general description and a discussion of genetic heterogeneity of familial partial lipodystrophy (FPLD), see 151660.
Aortic aneurysm, familial thoracic 4
MedGen UID:
338704
Concept ID:
C1851504
Disease or Syndrome
Any familial thoracic aortic aneurysm and aortic dissection in which the cause of the disease is a mutation in the MYH11 gene.
Atherosclerosis-deafness-diabetes-epilepsy-nephropathy syndrome
MedGen UID:
349198
Concept ID:
C1859596
Disease or Syndrome
A rare, severe, circulatory system disease characterized by premature, diffuse, severe atherosclerosis (including the aorta and renal, coronary, and cerebral arteries), sensorineural deafness, diabetes mellitus, progressive neurological deterioration with cerebellar symptoms and photomyoclonic seizures, and progressive nephropathy. Partial deficiency of mitochondrial complexes III and IV in the kidney and fibroblasts (but not in muscle) may be associated. There have been no further descriptions in the literature since 1994.
Sitosterolemia 1
MedGen UID:
440869
Concept ID:
C2749759
Disease or Syndrome
Sitosterolemia is characterized by: Hypercholesterolemia (especially in children) which (1) shows an unexpected significant lowering of plasma cholesterol level in response to low-fat diet modification or to bile acid sequestrant therapy; or (2) does not respond to statin therapy; Tendon xanthomas or tuberous (i.e., planar) xanthomas that can occur in childhood and in unusual locations (heels, knees, elbows, and buttocks); Premature atherosclerosis, which can lead to angina, aortic valve involvement, myocardial infarction, and sudden death; Hemolytic anemia, abnormally shaped erythrocytes (stomatocytes), and large platelets (macrothrombocytopenia). On occasion, the abnormal hematologic findings may be the initial presentation or the only clinical feature of this disorder. Arthritis, arthralgias, and splenomegaly may sometimes be seen and one study has concluded that "idiopathic" liver disease could be undiagnosed sitosterolemia. The clinical spectrum of sitosterolemia is probably not fully appreciated due to underdiagnosis and the fact that the phenotype in infants is likely to be highly dependent on diet.
Paget disease of bone 6
MedGen UID:
908743
Concept ID:
C4085250
Disease or Syndrome
Paget disease of bone-6 is an autosomal dominant disorder characterized by adult onset of bone pain associated with polyostotic bone lesions primarily affecting the axial skeleton. A subset of patients can develop coronary artery disease and/or malignant giant cell tumor (GCT) of the bone, which arises within the Paget bone lesions (summary by Divisato et al., 2016). For a general phenotypic description and a discussion of genetic heterogeneity of Paget disease of bone, see 167250.
Aortic aneurysm, familial thoracic 10
MedGen UID:
924785
Concept ID:
C4284414
Disease or Syndrome
Any familial thoracic aortic aneurysm and aortic dissection in which the cause of the disease is a mutation in the LOX gene.
Abdominal obesity-metabolic syndrome 4
MedGen UID:
1704861
Concept ID:
C5231430
Disease or Syndrome
Abdominal obesity-metabolic syndrome-4 (AOMS4) is characterized by obesity, hypertension, and early-onset coronary artery disease. Most affected individuals meet the criteria for metabolic syndrome, including elevated triglyceride and low high-density lipoprotein levels, and type 2 diabetes (Esteghamat et al., 2019). For a discussion of the genetic heterogeneity of abdominal obesity-metabolic syndrome, see AOMS1 (605552).

Professional guidelines

PubMed

Choi KA, Kim JH, Ryu K, Kaushik N
Int J Mol Sci 2022 Oct 17;23(20) doi: 10.3390/ijms232012397. PMID: 36293254Free PMC Article
Ramos-Leví AM, Marazuela M
Endocrine 2017 Feb;55(2):346-359. Epub 2017 Jan 2 doi: 10.1007/s12020-016-1191-3. PMID: 28042644
McVeigh C, Passmore P
Clin Interv Aging 2006;1(3):229-35. doi: 10.2147/ciia.2006.1.3.229. PMID: 18046875Free PMC Article

Recent clinical studies

Etiology

Gaba P, Gersh BJ, Muller J, Narula J, Stone GW
Nat Rev Cardiol 2023 Mar;20(3):181-196. Epub 2022 Sep 23 doi: 10.1038/s41569-022-00769-8. PMID: 36151312
Bergström G, Persson M, Adiels M, Björnson E, Bonander C, Ahlström H, Alfredsson J, Angerås O, Berglund G, Blomberg A, Brandberg J, Börjesson M, Cederlund K, de Faire U, Duvernoy O, Ekblom Ö, Engström G, Engvall JE, Fagman E, Eriksson M, Erlinge D, Fagerberg B, Flinck A, Gonçalves I, Hagström E, Hjelmgren O, Lind L, Lindberg E, Lindqvist P, Ljungberg J, Magnusson M, Mannila M, Markstad H, Mohammad MA, Nystrom FH, Ostenfeld E, Persson A, Rosengren A, Sandström A, Själander A, Sköld MC, Sundström J, Swahn E, Söderberg S, Torén K, Östgren CJ, Jernberg T
Circulation 2021 Sep 21;144(12):916-929. Epub 2021 Sep 20 doi: 10.1161/CIRCULATIONAHA.121.055340. PMID: 34543072Free PMC Article
Amarenco P, Kim JS, Labreuche J, Charles H, Abtan J, Béjot Y, Cabrejo L, Cha JK, Ducrocq G, Giroud M, Guidoux C, Hobeanu C, Kim YJ, Lapergue B, Lavallée PC, Lee BC, Lee KB, Leys D, Mahagne MH, Meseguer E, Nighoghossian N, Pico F, Samson Y, Sibon I, Steg PG, Sung SM, Touboul PJ, Touzé E, Varenne O, Vicaut É, Yelles N, Bruckert E; Treat Stroke to Target Investigators
N Engl J Med 2020 Jan 2;382(1):9. Epub 2019 Nov 18 doi: 10.1056/NEJMoa1910355. PMID: 31738483
Katz D, Gavin MC
Ann Intern Med 2019 Aug 6;171(3):ITC17-ITC32. doi: 10.7326/AITC201908060. PMID: 31382288
Lee SE, Chang HJ, Sung JM, Park HB, Heo R, Rizvi A, Lin FY, Kumar A, Hadamitzky M, Kim YJ, Conte E, Andreini D, Pontone G, Budoff MJ, Gottlieb I, Lee BK, Chun EJ, Cademartiri F, Maffei E, Marques H, Leipsic JA, Shin S, Choi JH, Chinnaiyan K, Raff G, Virmani R, Samady H, Stone PH, Berman DS, Narula J, Shaw LJ, Bax JJ, Min JK
JACC Cardiovasc Imaging 2018 Oct;11(10):1475-1484. Epub 2018 Jun 13 doi: 10.1016/j.jcmg.2018.04.015. PMID: 29909109

Diagnosis

Gaba P, Gersh BJ, Muller J, Narula J, Stone GW
Nat Rev Cardiol 2023 Mar;20(3):181-196. Epub 2022 Sep 23 doi: 10.1038/s41569-022-00769-8. PMID: 36151312
Aziz MU, Singh S
J Med Imaging Radiat Sci 2021 Nov;52(3S):S19-S39. Epub 2021 Sep 1 doi: 10.1016/j.jmir.2021.08.007. PMID: 34479831
West HW, Antoniades C
Antioxid Redox Signal 2021 May 20;34(15):1217-1243. Epub 2020 Jun 18 doi: 10.1089/ars.2020.8128. PMID: 32458744
Amarenco P, Kim JS, Labreuche J, Charles H, Abtan J, Béjot Y, Cabrejo L, Cha JK, Ducrocq G, Giroud M, Guidoux C, Hobeanu C, Kim YJ, Lapergue B, Lavallée PC, Lee BC, Lee KB, Leys D, Mahagne MH, Meseguer E, Nighoghossian N, Pico F, Samson Y, Sibon I, Steg PG, Sung SM, Touboul PJ, Touzé E, Varenne O, Vicaut É, Yelles N, Bruckert E; Treat Stroke to Target Investigators
N Engl J Med 2020 Jan 2;382(1):9. Epub 2019 Nov 18 doi: 10.1056/NEJMoa1910355. PMID: 31738483
Katz D, Gavin MC
Ann Intern Med 2019 Aug 6;171(3):ITC17-ITC32. doi: 10.7326/AITC201908060. PMID: 31382288

Therapy

Bergström G, Persson M, Adiels M, Björnson E, Bonander C, Ahlström H, Alfredsson J, Angerås O, Berglund G, Blomberg A, Brandberg J, Börjesson M, Cederlund K, de Faire U, Duvernoy O, Ekblom Ö, Engström G, Engvall JE, Fagman E, Eriksson M, Erlinge D, Fagerberg B, Flinck A, Gonçalves I, Hagström E, Hjelmgren O, Lind L, Lindberg E, Lindqvist P, Ljungberg J, Magnusson M, Mannila M, Markstad H, Mohammad MA, Nystrom FH, Ostenfeld E, Persson A, Rosengren A, Sandström A, Själander A, Sköld MC, Sundström J, Swahn E, Söderberg S, Torén K, Östgren CJ, Jernberg T
Circulation 2021 Sep 21;144(12):916-929. Epub 2021 Sep 20 doi: 10.1161/CIRCULATIONAHA.121.055340. PMID: 34543072Free PMC Article
West HW, Antoniades C
Antioxid Redox Signal 2021 May 20;34(15):1217-1243. Epub 2020 Jun 18 doi: 10.1089/ars.2020.8128. PMID: 32458744
Amarenco P, Kim JS, Labreuche J, Charles H, Abtan J, Béjot Y, Cabrejo L, Cha JK, Ducrocq G, Giroud M, Guidoux C, Hobeanu C, Kim YJ, Lapergue B, Lavallée PC, Lee BC, Lee KB, Leys D, Mahagne MH, Meseguer E, Nighoghossian N, Pico F, Samson Y, Sibon I, Steg PG, Sung SM, Touboul PJ, Touzé E, Varenne O, Vicaut É, Yelles N, Bruckert E; Treat Stroke to Target Investigators
N Engl J Med 2020 Jan 2;382(1):9. Epub 2019 Nov 18 doi: 10.1056/NEJMoa1910355. PMID: 31738483
Katz D, Gavin MC
Ann Intern Med 2019 Aug 6;171(3):ITC17-ITC32. doi: 10.7326/AITC201908060. PMID: 31382288
Lee SE, Chang HJ, Sung JM, Park HB, Heo R, Rizvi A, Lin FY, Kumar A, Hadamitzky M, Kim YJ, Conte E, Andreini D, Pontone G, Budoff MJ, Gottlieb I, Lee BK, Chun EJ, Cademartiri F, Maffei E, Marques H, Leipsic JA, Shin S, Choi JH, Chinnaiyan K, Raff G, Virmani R, Samady H, Stone PH, Berman DS, Narula J, Shaw LJ, Bax JJ, Min JK
JACC Cardiovasc Imaging 2018 Oct;11(10):1475-1484. Epub 2018 Jun 13 doi: 10.1016/j.jcmg.2018.04.015. PMID: 29909109

Prognosis

Gaba P, Gersh BJ, Muller J, Narula J, Stone GW
Nat Rev Cardiol 2023 Mar;20(3):181-196. Epub 2022 Sep 23 doi: 10.1038/s41569-022-00769-8. PMID: 36151312
Sun T, Zhan W, Wei L, Xu Z, Fan L, Zhuo Y, Wang C, Zhang J
Lipids Health Dis 2021 Nov 6;20(1):154. doi: 10.1186/s12944-021-01580-z. PMID: 34742313Free PMC Article
Aziz MU, Singh S
J Med Imaging Radiat Sci 2021 Nov;52(3S):S19-S39. Epub 2021 Sep 1 doi: 10.1016/j.jmir.2021.08.007. PMID: 34479831
Kim H, Kim JY, Min PK, Yoon YW, Lee BK, Hong BK, Rim SJ, Kwon HM, Choi EY
Int Heart J 2020 Nov 28;61(6):1142-1149. Epub 2020 Nov 13 doi: 10.1536/ihj.20-318. PMID: 33191349
Xie Y, Jin H, Zeng M, Li D
Curr Atheroscler Rep 2017 Sep;19(9):37. doi: 10.1007/s11883-017-0672-z. PMID: 28779432

Clinical prediction guides

Gaba P, Gersh BJ, Muller J, Narula J, Stone GW
Nat Rev Cardiol 2023 Mar;20(3):181-196. Epub 2022 Sep 23 doi: 10.1038/s41569-022-00769-8. PMID: 36151312
Bergström G, Persson M, Adiels M, Björnson E, Bonander C, Ahlström H, Alfredsson J, Angerås O, Berglund G, Blomberg A, Brandberg J, Börjesson M, Cederlund K, de Faire U, Duvernoy O, Ekblom Ö, Engström G, Engvall JE, Fagman E, Eriksson M, Erlinge D, Fagerberg B, Flinck A, Gonçalves I, Hagström E, Hjelmgren O, Lind L, Lindberg E, Lindqvist P, Ljungberg J, Magnusson M, Mannila M, Markstad H, Mohammad MA, Nystrom FH, Ostenfeld E, Persson A, Rosengren A, Sandström A, Själander A, Sköld MC, Sundström J, Swahn E, Söderberg S, Torén K, Östgren CJ, Jernberg T
Circulation 2021 Sep 21;144(12):916-929. Epub 2021 Sep 20 doi: 10.1161/CIRCULATIONAHA.121.055340. PMID: 34543072Free PMC Article
Aziz MU, Singh S
J Med Imaging Radiat Sci 2021 Nov;52(3S):S19-S39. Epub 2021 Sep 1 doi: 10.1016/j.jmir.2021.08.007. PMID: 34479831
Silva Andrade B, Siqueira S, de Assis Soares WR, de Souza Rangel F, Santos NO, Dos Santos Freitas A, Ribeiro da Silveira P, Tiwari S, Alzahrani KJ, Góes-Neto A, Azevedo V, Ghosh P, Barh D
Viruses 2021 Apr 18;13(4) doi: 10.3390/v13040700. PMID: 33919537Free PMC Article
Xie Y, Jin H, Zeng M, Li D
Curr Atheroscler Rep 2017 Sep;19(9):37. doi: 10.1007/s11883-017-0672-z. PMID: 28779432

Recent systematic reviews

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