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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).
See: Condition Record
Vertebral, cardiac, renal, and limb defects syndrome 3

Professional guidelines

PubMed

Very early correction of anomalous left coronary artery from the pulmonary artery improves intensive care management.
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
Anomalous origin of the left coronary artery from the pulmonary trunk: its clinical spectrum and current surgical management.
Choh JH, Levinsky L, Srinivasan V, Idbeis B, Subramanian S
Thorac Cardiovasc Surg 1980 Aug;28(4):239-42. doi: 10.1055/s-2007-1022086. PMID: 6158124
Surgical treatment for anomalous origin of left coronary artery from pulmonary artery.
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

Etiology

Coronary Artery Z Score is Associated with Postoperative Outcomes in Patients with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery.
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
Cardiac magnetic resonance imaging, myocardial scar and coronary flow pattern in anomalous origin of left coronary artery from the pulmonary artery.
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
Surgical Intervention for Anomalous Origin of Left Coronary Artery From the Pulmonary Artery in Children: A Long-Term Follow-Up.
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
Excellent functional result in children after correction of anomalous origin of left coronary artery from the pulmonary artery--a population-based complete follow-up study.
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
Repair of anomalous origin of left coronary artery from the pulmonary artery.
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

Diagnosis

Lung Ultrasound in Congenital Cardiac Abnormality: ALCAPA.
De Rose C, Valentini P, Curatola A, Morello R, Musolino AM, Piastra M, Buonsenso D
Indian J Pediatr 2021 Feb;88(2):161-164. Epub 2020 Feb 11 doi: 10.1007/s12098-020-03208-z. PMID: 32048221
Image Diagnosis: An Anomalous Origin of Left Coronary Artery from the Pulmonary Artery.
Bu H, Zhao T
Arq Bras Cardiol 2020 Apr;114(4 Suppl 1):4-7. Epub 2020 May 18 doi: 10.36660/abc.20180207. PMID: 32428105Free PMC Article
Myocardial Function Following Repair of Anomalous Origin of Left Coronary Artery from the Pulmonary Artery in Children.
Naqvi N, Babu-Narayan SV, Krupickova S, Muthialu N, Maiya S, Chandershekar P, Cheang MH, Kostolny M, Tsang V, Marek J
J Am Soc Echocardiogr 2020 May;33(5):622-630. Epub 2020 Feb 29 doi: 10.1016/j.echo.2019.12.014. PMID: 32122741
Cardiac magnetic resonance imaging, myocardial scar and coronary flow pattern in anomalous origin of left coronary artery from the pulmonary artery.
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
Anomalous origin of left coronary artery from the pulmonary artery in a 60-year-old woman.
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

Therapy

Excellent functional result in children after correction of anomalous origin of left coronary artery from the pulmonary artery--a population-based complete follow-up study.
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
Repair of anomalous origin of left coronary artery from the pulmonary artery.
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

Coronary Artery Z Score is Associated with Postoperative Outcomes in Patients with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery.
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
Cardiac magnetic resonance imaging, myocardial scar and coronary flow pattern in anomalous origin of left coronary artery from the pulmonary artery.
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
Surgical Intervention for Anomalous Origin of Left Coronary Artery From the Pulmonary Artery in Children: A Long-Term Follow-Up.
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
Anomalous origin of left coronary artery from the pulmonary artery in a 60-year-old woman.
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
Repair of anomalous origin of left coronary artery from the pulmonary artery.
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

Coronary Artery Z Score is Associated with Postoperative Outcomes in Patients with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery.
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
An unusual presentation of anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome in a 70-year-old man: a case report.
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
Cardiac magnetic resonance imaging, myocardial scar and coronary flow pattern in anomalous origin of left coronary artery from the pulmonary artery.
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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