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Total absence of the pericardium

MedGen UID:
576786
•Concept ID:
C0345140
•
Congenital Abnormality
Synonym: Congenital absence of the pericardium
SNOMED CT: Totally absent pericardium (253732001); Congenital absence of pericardium (253732001)
 
HPO: HP:0011629

Definition

No pericardium around the heart, occurring as a congenital defect, not the result of a surgical pericardectomy. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTotal absence of the pericardium

Conditions with this feature

Pancreatic hypoplasia-diabetes-congenital heart disease syndrome
MedGen UID:
860891
•Concept ID:
C4012454
•
Congenital Abnormality
A rare, syndromic diabetes mellitus characterized by partial pancreatic agenesis, diabetes mellitus, and heart anomalies (including transposition of the great vessels, ventricular or atrial septal defects, pulmonary stenosis, or patent ductus arteriosis).
See: Condition Record
Pancreatic hypoplasia-diabetes-congenital heart disease syndrome

Recent clinical studies

Etiology

Association of Pericardiac Adipose Tissue With Coronary Artery Disease.
Li M, Qi L, Li Y, Zhang S, Lin L, Zhou L, Han W, Qu X, Cai J, Ye M, Shi K
Front Endocrinol (Lausanne) 2021;12:724859. Epub 2021 Sep 6 doi: 10.3389/fendo.2021.724859. PMID: 34552562Free PMC Article
Ablation of Inappropriate Sinus Tachycardia: A Systematic Review of the Literature.
Rodríguez-Mañero M, Kreidieh B, Al Rifai M, Ibarra-Cortez S, Schurmann P, Álvarez PA, Fernández-López XA, García-Seara J, Martínez-Sande L, González-Juanatey JR, Valderrábano M
JACC Clin Electrophysiol 2017 Mar;3(3):253-265. Epub 2016 Dec 21 doi: 10.1016/j.jacep.2016.09.014. PMID: 29759520Free PMC Article
Echocardiographic diagnosis of total or left congenital pericardial absence with positional change.
Kim MJ, Kim HK, Jung JH, Yoon YE, Kim HL, Park JB, Lee SP, Kim YJ, Cho GY, Sohn DW, Oh JK
Heart 2017 Aug;103(15):1203-1209. Epub 2017 Mar 9 doi: 10.1136/heartjnl-2016-310870. PMID: 28280147
Constrictive pericarditis in 26 patients with histologically normal pericardial thickness.
Talreja DR, Edwards WD, Danielson GK, Schaff HV, Tajik AJ, Tazelaar HD, Breen JF, Oh JK
Circulation 2003 Oct 14;108(15):1852-7. Epub 2003 Sep 29 doi: 10.1161/01.CIR.0000087606.18453.FD. PMID: 14517161
Bilateral congenital diaphragmatic hernia.
Neville HL, Jaksic T, Wilson JM, Lally PA, Hardin WD Jr, Hirschl RB, Lally KP; Congenital Diaphragmatic Hernia Study Group
J Pediatr Surg 2003 Mar;38(3):522-4. doi: 10.1053/jpsu.2003.50092. PMID: 12632380

Diagnosis

Echocardiographic diagnosis of total or left congenital pericardial absence with positional change.
Kim MJ, Kim HK, Jung JH, Yoon YE, Kim HL, Park JB, Lee SP, Kim YJ, Cho GY, Sohn DW, Oh JK
Heart 2017 Aug;103(15):1203-1209. Epub 2017 Mar 9 doi: 10.1136/heartjnl-2016-310870. PMID: 28280147
Total absence of the pericardium incidentally found during surgery for spontaneous pneumothorax.
Sugiyama A, Izumi Y, Inoue Y, Aoki K, Fukuda H, Gika M, Nakayama M
Gen Thorac Cardiovasc Surg 2016 May;64(5):286-9. Epub 2014 Oct 4 doi: 10.1007/s11748-014-0482-y. PMID: 25281370
Advances in malignant peritoneal mesothelioma.
Cao S, Jin S, Cao J, Shen J, Hu J, Che D, Pan B, Zhang J, He X, Ding D, Gu F, Yu Y
Int J Colorectal Dis 2015 Jan;30(1):1-10. Epub 2014 Oct 21 doi: 10.1007/s00384-014-2029-1. PMID: 25331029
Congenital defects of pericardium: case reports and review of literature.
Cuccuini M, Lisi F, Consoli A, Mancini S, Bellino V, Galanti G, Capaccioli L
Ital J Anat Embryol 2013;118(1):136-50. PMID: 23898583
Bilateral congenital diaphragmatic hernia.
Neville HL, Jaksic T, Wilson JM, Lally PA, Hardin WD Jr, Hirschl RB, Lally KP; Congenital Diaphragmatic Hernia Study Group
J Pediatr Surg 2003 Mar;38(3):522-4. doi: 10.1053/jpsu.2003.50092. PMID: 12632380

Therapy

Effusive-Constrictive Pericarditis After Pericardiocentesis: Incidence, Associated Findings, and Natural History.
Kim KH, Miranda WR, Sinak LJ, Syed FF, Melduni RM, Espinosa RE, Kane GC, Oh JK
JACC Cardiovasc Imaging 2018 Apr;11(4):534-541. Epub 2017 Oct 5 doi: 10.1016/j.jcmg.2017.06.017. PMID: 28917680Free PMC Article
Outcomes of a New-Generation Stentless Aortic Valve: A Single-Center Experience with 251 Consecutive Implants.
Stefanelli G, Pirro F, Danniballe G, Olaru A, Meli M, Weltert L
J Heart Valve Dis 2017 Sep;26(5):518-527. PMID: 29762920
Ablation of Inappropriate Sinus Tachycardia: A Systematic Review of the Literature.
Rodríguez-Mañero M, Kreidieh B, Al Rifai M, Ibarra-Cortez S, Schurmann P, Álvarez PA, Fernández-López XA, García-Seara J, Martínez-Sande L, González-Juanatey JR, Valderrábano M
JACC Clin Electrophysiol 2017 Mar;3(3):253-265. Epub 2016 Dec 21 doi: 10.1016/j.jacep.2016.09.014. PMID: 29759520Free PMC Article
Percutaneous epicardial ablation of ventricular arrhythmias arising from the left ventricular summit: outcomes and electrocardiogram correlates of success.
Santangeli P, Marchlinski FE, Zado ES, Benhayon D, Hutchinson MD, Lin D, Frankel DS, Riley MP, Supple GE, Garcia FC, Bala R, Desjardins B, Callans DJ, Dixit S
Circ Arrhythm Electrophysiol 2015 Apr;8(2):337-43. Epub 2015 Jan 30 doi: 10.1161/CIRCEP.114.002377. PMID: 25637596
Thiamine responsive megaloblastic anemia: the puzzling phenotype.
Beshlawi I, Al Zadjali S, Bashir W, Elshinawy M, Alrawas A, Wali Y
Pediatr Blood Cancer 2014 Mar;61(3):528-31. Epub 2013 Nov 19 doi: 10.1002/pbc.24849. PMID: 24249281

Prognosis

Association of Pericardiac Adipose Tissue With Coronary Artery Disease.
Li M, Qi L, Li Y, Zhang S, Lin L, Zhou L, Han W, Qu X, Cai J, Ye M, Shi K
Front Endocrinol (Lausanne) 2021;12:724859. Epub 2021 Sep 6 doi: 10.3389/fendo.2021.724859. PMID: 34552562Free PMC Article
Ablation of Inappropriate Sinus Tachycardia: A Systematic Review of the Literature.
Rodríguez-Mañero M, Kreidieh B, Al Rifai M, Ibarra-Cortez S, Schurmann P, Álvarez PA, Fernández-López XA, García-Seara J, Martínez-Sande L, González-Juanatey JR, Valderrábano M
JACC Clin Electrophysiol 2017 Mar;3(3):253-265. Epub 2016 Dec 21 doi: 10.1016/j.jacep.2016.09.014. PMID: 29759520Free PMC Article
Echocardiographic diagnosis of total or left congenital pericardial absence with positional change.
Kim MJ, Kim HK, Jung JH, Yoon YE, Kim HL, Park JB, Lee SP, Kim YJ, Cho GY, Sohn DW, Oh JK
Heart 2017 Aug;103(15):1203-1209. Epub 2017 Mar 9 doi: 10.1136/heartjnl-2016-310870. PMID: 28280147
Advances in malignant peritoneal mesothelioma.
Cao S, Jin S, Cao J, Shen J, Hu J, Che D, Pan B, Zhang J, He X, Ding D, Gu F, Yu Y
Int J Colorectal Dis 2015 Jan;30(1):1-10. Epub 2014 Oct 21 doi: 10.1007/s00384-014-2029-1. PMID: 25331029
Bilateral congenital diaphragmatic hernia.
Neville HL, Jaksic T, Wilson JM, Lally PA, Hardin WD Jr, Hirschl RB, Lally KP; Congenital Diaphragmatic Hernia Study Group
J Pediatr Surg 2003 Mar;38(3):522-4. doi: 10.1053/jpsu.2003.50092. PMID: 12632380

Clinical prediction guides

Primary cardiac and pericardial lymphangiomas: clinical, radiologic, and pathologic characterization derived from an institutional series and review of the literature.
Pichler Sekulic S, Sekulic M
Virchows Arch 2022 Jun;480(6):1211-1221. Epub 2022 Jan 10 doi: 10.1007/s00428-022-03269-9. PMID: 35013774
Association of Pericardiac Adipose Tissue With Coronary Artery Disease.
Li M, Qi L, Li Y, Zhang S, Lin L, Zhou L, Han W, Qu X, Cai J, Ye M, Shi K
Front Endocrinol (Lausanne) 2021;12:724859. Epub 2021 Sep 6 doi: 10.3389/fendo.2021.724859. PMID: 34552562Free PMC Article
Association Between Primary Coronary Slow-Flow Phenomenon and Epicardial Fat Tissue.
Weferling M, Vietheer J, Keller T, Fischer-Rasokat U, Hamm CW, Liebetrau C
J Invasive Cardiol 2021 Jan;33(1):E59-E64. PMID: 33385988
Echocardiographic diagnosis of total or left congenital pericardial absence with positional change.
Kim MJ, Kim HK, Jung JH, Yoon YE, Kim HL, Park JB, Lee SP, Kim YJ, Cho GY, Sohn DW, Oh JK
Heart 2017 Aug;103(15):1203-1209. Epub 2017 Mar 9 doi: 10.1136/heartjnl-2016-310870. PMID: 28280147
Total absence of the pericardium incidentally found during surgery for spontaneous pneumothorax.
Sugiyama A, Izumi Y, Inoue Y, Aoki K, Fukuda H, Gika M, Nakayama M
Gen Thorac Cardiovasc Surg 2016 May;64(5):286-9. Epub 2014 Oct 4 doi: 10.1007/s11748-014-0482-y. PMID: 25281370

Recent systematic reviews

Ablation of Inappropriate Sinus Tachycardia: A Systematic Review of the Literature.
Rodríguez-Mañero M, Kreidieh B, Al Rifai M, Ibarra-Cortez S, Schurmann P, Álvarez PA, Fernández-López XA, García-Seara J, Martínez-Sande L, González-Juanatey JR, Valderrábano M
JACC Clin Electrophysiol 2017 Mar;3(3):253-265. Epub 2016 Dec 21 doi: 10.1016/j.jacep.2016.09.014. PMID: 29759520Free PMC Article

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