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Complete left sided absence of pericardium

MedGen UID:
868846
•Concept ID:
C4023256
•
Congenital Abnormality
Synonym: Absent lining around of left side of heart
 
HPO: HP:0011633

Definition

A congenital anomaly with complete lack of the pericardium on the lefthand side of the heart. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVComplete left sided absence of pericardium

Recent clinical studies

Etiology

Complete absence of precordial R waves due to absence of left-sided pericardium.
Steinberg C, Pelletier MJ, Champagne J
Ann Noninvasive Electrocardiol 2012 Oct;17(4):401-4. Epub 2012 Aug 13 doi: 10.1111/j.1542-474X.2012.00507.x. PMID: 23094888Free PMC Article
Video-assisted thoracoscopic pericardial window for penetrating cardiac trauma.
Navsaria PH, Nicol AJ
S Afr J Surg 2006 Feb;44(1):18-20. PMID: 16619986
Bilateral congenital diaphragmatic hernia with absent pleura and pericardium.
St Peter SD, Shah SR, Little DC, Calkins CM, Sharp RJ, Ostlie DJ
Birth Defects Res A Clin Mol Teratol 2005 Sep;73(9):624-7. doi: 10.1002/bdra.20173. PMID: 16001446
Magnetic resonance imaging differentiated partial from complete absence of the left pericardium in a case of leftward displacement of the heart.
Yamano T, Sawada T, Sakamoto K, Nakamura T, Azuma A, Nakagawa M
Circ J 2004 Apr;68(4):385-8. doi: 10.1253/circj.68.385. PMID: 15056840

Diagnosis

Partial Congenital Absence of The Pericardium: A Case Report.
Benameur N, Arous Y, Landolsi M, Chenik S, Abdallah NB, Kraiem T
Braz J Cardiovasc Surg 2019 Dec 1;34(5):633-636. doi: 10.21470/1678-9741-2018-0357. PMID: 31165613Free PMC Article
Congenital defects of the pericardium: a review.
Shah AB, Kronzon I
Eur Heart J Cardiovasc Imaging 2015 Aug;16(8):821-7. Epub 2015 May 23 doi: 10.1093/ehjci/jev119. PMID: 26003149
Congenital absence of the pericardium and its mimics.
Verde F, Johnson PT, Jha S, Fishman EK, Zimmerman SL
J Cardiovasc Comput Tomogr 2013 Jan-Feb;7(1):11-7. Epub 2013 Jan 19 doi: 10.1016/j.jcct.2013.01.003. PMID: 23452995
Complete absence of precordial R waves due to absence of left-sided pericardium.
Steinberg C, Pelletier MJ, Champagne J
Ann Noninvasive Electrocardiol 2012 Oct;17(4):401-4. Epub 2012 Aug 13 doi: 10.1111/j.1542-474X.2012.00507.x. PMID: 23094888Free PMC Article
Congenital complete absence of the left pericardium: a rare cause of chest pain or pseudo-right heart overload.
Garnier F, Eicher JC, Philip JL, Lalande A, Bieber H, Voute MF, Brenot R, Brunotte F, Wolf JE
Clin Cardiol 2010 Feb;33(2):E52-7. doi: 10.1002/clc.20607. PMID: 20043342Free PMC Article

Therapy

Complete absence of precordial R waves due to absence of left-sided pericardium.
Steinberg C, Pelletier MJ, Champagne J
Ann Noninvasive Electrocardiol 2012 Oct;17(4):401-4. Epub 2012 Aug 13 doi: 10.1111/j.1542-474X.2012.00507.x. PMID: 23094888Free PMC Article

Prognosis

Complete absence of precordial R waves due to absence of left-sided pericardium.
Steinberg C, Pelletier MJ, Champagne J
Ann Noninvasive Electrocardiol 2012 Oct;17(4):401-4. Epub 2012 Aug 13 doi: 10.1111/j.1542-474X.2012.00507.x. PMID: 23094888Free PMC Article
Bilateral congenital diaphragmatic hernia with absent pleura and pericardium.
St Peter SD, Shah SR, Little DC, Calkins CM, Sharp RJ, Ostlie DJ
Birth Defects Res A Clin Mol Teratol 2005 Sep;73(9):624-7. doi: 10.1002/bdra.20173. PMID: 16001446

Clinical prediction guides

Video-assisted thoracoscopic pericardial window for penetrating cardiac trauma.
Navsaria PH, Nicol AJ
S Afr J Surg 2006 Feb;44(1):18-20. PMID: 16619986

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