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Premature coronary artery atherosclerosis

MedGen UID:
356830
Concept ID:
C1867743
Disease or Syndrome; Finding
Synonym: Coronary artery disease, premature
 
HPO: HP:0005181

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 before age of 45. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPremature coronary artery atherosclerosis

Conditions with this feature

Hutchinson-Gilford syndrome
MedGen UID:
46123
Concept ID:
C0033300
Disease or Syndrome
Hutchinson-Gilford progeria syndrome (HGPS) is characterized by clinical features that typically develop in childhood and resemble some features of accelerated aging. Children with HGPS usually appear normal at birth. Profound failure to thrive occurs during the first year. Characteristic facial features include head that is disproportionately large for the face, narrow nasal ridge, narrow nasal tip, thin vermilion of the upper and lower lips, small mouth, and retro- and micrognathia. Common features include loss of subcutaneous fat, delayed eruption and loss of primary teeth, abnormal skin with small outpouchings over the abdomen and upper thighs, alopecia, nail dystrophy, coxa valga, and progressive joint contractures. Later findings include low-frequency conductive hearing loss, dental crowding, and partial lack of secondary tooth eruption. Motor and mental development is normal. Death occurs as a result of complications of severe atherosclerosis, either cardiac disease (myocardial infarction or heart failure) or cerebrovascular disease (stroke), generally between ages six and 20 years. Average life span is approximately 14.5 years.
Coronary artery disease, autosomal dominant, 1
MedGen UID:
330802
Concept ID:
C1842247
Disease or Syndrome
Coronary artery disease (CAD) and its most important complication, acute myocardial infarction (MI), are leading causes of death and disability in the developed world. Multiple risk factors for CAD/MI have been identified, including family history, hypertension, hypercholesterolemia, obesity, smoking, and diabetes. Several genomewide scans of affected sib pairs have identified susceptibility loci for CAD, e.g., 607339 and 300464.
Coronary artery disease, autosomal dominant 2
MedGen UID:
370259
Concept ID:
C1970440
Disease or Syndrome
Any coronary artery disease in which the cause of the disease is a mutation in the LRP6 gene.
Aortic aneurysm, familial thoracic 6
MedGen UID:
435866
Concept ID:
C2673186
Disease or Syndrome
Any familial thoracic aortic aneurysm and aortic dissection in which the cause of the disease is a mutation in the ACTA2 gene.
Moyamoya angiopathy-short stature-facial dysmorphism-hypergonadotropic hypogonadism syndrome
MedGen UID:
463207
Concept ID:
C3151857
Disease or Syndrome
This multisystem disorder is characterized by moyamoya disease, short stature, hypergonadotropic hypogonadism, and facial dysmorphism. Other variable features include dilated cardiomyopathy, premature graying of the hair, and early-onset cataracts. Moyamoya disease is a progressive cerebrovascular disorder characterized by stenosis or occlusion of the internal carotid arteries and the main branches, leading to the development of small collateral vessels (moyamoya vessels) at the base of the brain. Affected individuals can develop acute neurologic events due to stroke-like episodes (summary by Miskinyte et al., 2011). For a general phenotypic description and a discussion of genetic heterogeneity of moyamoya disease, see MYMY1 (252350).
Obesity due to CEP19 deficiency
MedGen UID:
816654
Concept ID:
C3810324
Disease or Syndrome
A rare, genetic form of obesity characterized by morbid obesity, hypertension, type 2 diabetes mellitus and dyslipidemia leading to early coronary disease, myocardial infarction and congestive heart failure. Intellectual disability and decreased sperm counts or azoospermia have also been reported.
Hyperlipoproteinemia, type 1D
MedGen UID:
863204
Concept ID:
C4014767
Disease or Syndrome
Hyperlipoproteinemia type ID is a rare autosomal recessive disorder characterized by impaired clearance of triglyceride (TG)-rich lipoproteins in plasma, leading to severe hypertriglyceridemia (chylomicronemia). Clinical features include eruptive xanthomas, lipemia retinalis, hepatosplenomegaly, episodes of abdominal pain, and pancreatitis. Onset usually occurs in adulthood (summary by Brahm and Hegele, 2013). For a discussion of genetic heterogeneity of familial chylomicronemia, see 238600.
Sitosterolemia 2
MedGen UID:
1684715
Concept ID:
C5231453
Disease or Syndrome
Sitosterolemia, also known as phytosterolemia, is an autosomal recessive metabolic condition characterized by unrestricted intestinal absorption of both cholesterol and plant-derived cholesterol-like molecules, such as sitosterol. Patients with this disorder have very high levels of plant sterols in the plasma and develop tendon and tuberous xanthomas, accelerated atherosclerosis, and premature coronary artery disease (summary by Berge et al., 2000). For a general phenotypic description and a discussion of genetic heterogeneity of sitosterolemia, see 210250.
Hypoalphalipoproteinemia, primary, 1
MedGen UID:
1684828
Concept ID:
C5231558
Disease or Syndrome
Any ypoalphalipoproteinemia in which the cause of the disease is a mutation in the ABCA1 gene.
Hypoalphalipoproteinemia, primary, 2
MedGen UID:
1789263
Concept ID:
C5551172
Disease or Syndrome
Primary hypoalphalipoproteinemia-2 is an autosomal recessive disorder characterized by dysfunctional apoA-I production, resulting in undetectable levels of apoA-I in serum and in markedly low levels of serum high density lipoprotein cholesterol (HDL-C). The disorder is associated with extensive atherosclerosis, xanthomas, and corneal opacities (summary by Tanaka et al., 2018). For a discussion of genetic heterogeneity of primary hypoalphalipoproteinemia, see 604091.
Familial apolipoprotein gene cluster deletion syndrome
MedGen UID:
1824091
Concept ID:
C5774318
Disease or Syndrome
Apolipoprotein (apo) A-I is the major protein of HDL cholesterol, whereas apoC-III and apoA-IV are minor components. The genes coding for apoA-I, apoC-III, and apoA-IV are adjacent to one another on the long arm of chromosome 11. Familial apolipoprotein gene cluster deletion syndrome has been described in 1 family and found to be a homozygous deletion of the entire APOA1/C3/A4 gene complex. This results in a lack of expression of these plasma lipoproteins, with marked HDL-C deficiency in the homozygote and approximately half-normal levels of these apolipoproteins and HDL-C in the heterozygotes.

Professional guidelines

PubMed

Zeppenfeld K, Tfelt-Hansen J, de Riva M, Winkel BG, Behr ER, Blom NA, Charron P, Corrado D, Dagres N, de Chillou C, Eckardt L, Friede T, Haugaa KH, Hocini M, Lambiase PD, Marijon E, Merino JL, Peichl P, Priori SG, Reichlin T, Schulz-Menger J, Sticherling C, Tzeis S, Verstrael A, Volterrani M; ESC Scientific Document Group
Eur Heart J 2022 Oct 21;43(40):3997-4126. doi: 10.1093/eurheartj/ehac262. PMID: 36017572
Flora GD, Nayak MK
Curr Pharm Des 2019;25(38):4063-4084. doi: 10.2174/1381612825666190925163827. PMID: 31553287
Grundy SM, Stone NJ, Bailey AL, Beam C, Birtcher KK, Blumenthal RS, Braun LT, de Ferranti S, Faiella-Tommasino J, Forman DE, Goldberg R, Heidenreich PA, Hlatky MA, Jones DW, Lloyd-Jones D, Lopez-Pajares N, Ndumele CE, Orringer CE, Peralta CA, Saseen JJ, Smith SC Jr, Sperling L, Virani SS, Yeboah J
Circulation 2019 Jun 18;139(25):e1082-e1143. Epub 2018 Nov 10 doi: 10.1161/CIR.0000000000000625. PMID: 30586774Free PMC Article

Recent clinical studies

Etiology

Kryczka KE, Płoski R, Księżycka E, Kruk M, Kostrzewa G, Kowalik I, Demkow M, Lubiszewska B
Pol Arch Intern Med 2020 Sep 30;130(9):748-756. Epub 2020 Jun 25 doi: 10.20452/pamw.15461. PMID: 32584014
Clarkson TB, Appt SE
Maturitas 2005 May 16;51(1):64-74. doi: 10.1016/j.maturitas.2005.02.016. PMID: 15883111
Asanuma Y, Oeser A, Shintani AK, Turner E, Olsen N, Fazio S, Linton MF, Raggi P, Stein CM
N Engl J Med 2003 Dec 18;349(25):2407-15. doi: 10.1056/NEJMoa035611. PMID: 14681506
Schaefer EJ, McNamara JR, Genest J Jr, Ordovas JM
Semin Thromb Hemost 1988 Apr;14(2):143-8. doi: 10.1055/s-2007-1002768. PMID: 3061000
Lamb AS, Johnson WM
Am J Med Genet 1987 Dec;28(4):873-80. doi: 10.1002/ajmg.1320280412. PMID: 3688025

Therapy

Clarkson TB, Appt SE
Maturitas 2005 May 16;51(1):64-74. doi: 10.1016/j.maturitas.2005.02.016. PMID: 15883111

Prognosis

Berger GM
S Afr Med J 1984 Mar 31;65(13):503-6. PMID: 6710253

Clinical prediction guides

Kryczka KE, Płoski R, Księżycka E, Kruk M, Kostrzewa G, Kowalik I, Demkow M, Lubiszewska B
Pol Arch Intern Med 2020 Sep 30;130(9):748-756. Epub 2020 Jun 25 doi: 10.20452/pamw.15461. PMID: 32584014
Lucena J, Blanco M, Jurado C, Rico A, Salguero M, Vazquez R, Thiene G, Basso C
Eur Heart J 2010 Feb;31(3):318-29. Epub 2010 Jan 12 doi: 10.1093/eurheartj/ehp557. PMID: 20071326
Clarkson TB, Appt SE
Maturitas 2005 May 16;51(1):64-74. doi: 10.1016/j.maturitas.2005.02.016. PMID: 15883111
Asanuma Y, Oeser A, Shintani AK, Turner E, Olsen N, Fazio S, Linton MF, Raggi P, Stein CM
N Engl J Med 2003 Dec 18;349(25):2407-15. doi: 10.1056/NEJMoa035611. PMID: 14681506

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