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Anomalous origin of left coronary artery from the pulmonary artery

MedGen UID:
760471
Concept ID:
C1735886
Disease or Syndrome
Synonym: Anomalous left coronary artery from the pulmonary artery
SNOMED CT: Anomalous origin of left coronary artery from pulmonary artery (450301003)
 
HPO: HP:0011638
Monarch Initiative: MONDO:0000811

Definition

Left main coronary artery begins (branches off from) the pulmonary artery rather than as normal from the root of the aorta, above the left cusp of the aortic valve. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAnomalous origin of left coronary artery from the pulmonary artery

Conditions with this feature

Vertebral, cardiac, renal, and limb defects syndrome 3
MedGen UID:
1709064
Concept ID:
C5394250
Disease or Syndrome
Vertebral, cardiac, renal, and limb defects syndrome-3 (VCRL3) is an autosomal recessive disorder characterized by severe cardiac and renal anomalies that are lethal in infancy, including hypoplastic or absent left ventricle, transposition of the great arteries, absent pulmonary trunk, and hypoplastic or absent kidneys. Patients also exhibit vertebral segmentation defects and shortening of the proximal long bones or micromelia (Szot et al., 2020). For a discussion of genetic heterogeneity of VCRL, see VCRL1 (617660).

Professional guidelines

PubMed

Bonnemains L, Lambert V, Moulin-Zinch A, Youssef D, Serraf A
Arch Cardiovasc Dis 2010 Nov-Dec;103(11-12):579-84. Epub 2010 Nov 25 doi: 10.1016/j.acvd.2010.10.005. PMID: 21147442
Birk E, Stamler A, Katz J, Berant M, Dagan O, Matitiau A, Erez E, Blieden LC, Vidne BA
Isr Med Assoc J 2000 Feb;2(2):111-4. PMID: 10804930
Chiariello L, Meyer J, Reul GJ Jr, Hallman GL, Cooley DA
Ann Thorac Surg 1975 Apr;19(4):443-50. doi: 10.1016/s0003-4975(10)64046-5. PMID: 123735

Recent clinical studies

Therapy

Ojala T, Salminen J, Happonen JM, Pihkala J, Jokinen E, Sairanen H
Interact Cardiovasc Thorac Surg 2010 Jan;10(1):70-5. Epub 2009 Oct 6 doi: 10.1510/icvts.2009.209627. PMID: 19808708
Guo HW, Xu JP, Song YH, Shen XD, Li SJ, Hu SS
Asian Cardiovasc Thorac Ann 2007 Jun;15(3):240-2. doi: 10.1177/021849230701500314. PMID: 17540996

Prognosis

Suzuki T, Saito K, Yoshikawa T, Okada N, Yasuda K, Murayama H
Pediatr Cardiol 2022 Feb;43(2):443-448. Epub 2021 Sep 29 doi: 10.1007/s00246-021-02741-8. PMID: 34586456
Bhalgat P, Naik A, Salvi P, Bhadane N, Shah K, Paunipagar B, Joshi S
Indian Heart J 2018 Mar-Apr;70(2):303-307. Epub 2017 Aug 16 doi: 10.1016/j.ihj.2017.08.004. PMID: 29716711Free PMC Article
Naimo PS, Fricke TA, d'Udekem Y, Cochrane AD, Bullock A, Robertson T, Brizard CP, Konstantinov IE
Ann Thorac Surg 2016 May;101(5):1842-8. Epub 2016 Feb 18 doi: 10.1016/j.athoracsur.2015.11.020. PMID: 26897320
Zhu Y, Tang L, Xu Y
J Thorac Cardiovasc Surg 2014 May;147(5):1706-7. Epub 2013 Nov 20 doi: 10.1016/j.jtcvs.2013.10.017. PMID: 24267778
Guo HW, Xu JP, Song YH, Shen XD, Li SJ, Hu SS
Asian Cardiovasc Thorac Ann 2007 Jun;15(3):240-2. doi: 10.1177/021849230701500314. PMID: 17540996

Clinical prediction guides

Suzuki T, Saito K, Yoshikawa T, Okada N, Yasuda K, Murayama H
Pediatr Cardiol 2022 Feb;43(2):443-448. Epub 2021 Sep 29 doi: 10.1007/s00246-021-02741-8. PMID: 34586456
Vizzuso A, Righi R, Zerbini M, Gamanji S, Cucchi P, Gallo F, Giganti M, Benea G, Carnevale A
J Med Case Rep 2018 Oct 22;12(1):308. doi: 10.1186/s13256-018-1851-4. PMID: 30343670Free PMC Article
Bhalgat P, Naik A, Salvi P, Bhadane N, Shah K, Paunipagar B, Joshi S
Indian Heart J 2018 Mar-Apr;70(2):303-307. Epub 2017 Aug 16 doi: 10.1016/j.ihj.2017.08.004. PMID: 29716711Free PMC Article

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