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Coronary sinus atrial septal defect

MedGen UID:
488986
Concept ID:
C2063331
Congenital Abnormality
Synonyms: ASD coronary sinus; Coronary sinus atrial septal defects
 
HPO: HP:0011643
Monarch Initiative: MONDO:0020435
Orphanet: ORPHA99104

Definition

An atrial septal defect characterized by a deficiency in the tissue separating the coronary sinus from the left atrium (LA). This results in partial or complete unroofing of the coronary sinus leading to a predominantly left-to-right shunt through the coronary sinus (LA to coronary sinus to right atrium [RA]). The orifice of the ostium is frequently large because of the increased flow. From the RA side, the defect is located at the level of the coronary sinus ostium and may also include some deficiency in atrial tissue around the ostium. From the LA side, the size can be variable depending on the degree of unroofing of the coronary sinus. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCoronary sinus atrial septal defect

Professional guidelines

PubMed

Wang JK, Chen SJ, Hsu JY, Lin SM, Lin MT, Chiu SN, Chen CA, Wu MH
Catheter Cardiovasc Interv 2014 Feb;83(2):243-9. Epub 2013 Oct 30 doi: 10.1002/ccd.25185. PMID: 24038929
Xie MX, Yang YL, Cheng TO, Wang XF, Li K, Ren PP, Lü Q, Lin H, Li L
Int J Cardiol 2013 Sep 30;168(2):1258-63. Epub 2012 Dec 23 doi: 10.1016/j.ijcard.2012.11.113. PMID: 23266300
Ren JF, Schwartzman D, Callans DJ, Brode SE, Gottlieb CD, Marchlinski FE
Ultrasound Med Biol 1999 Sep;25(7):1077-86. doi: 10.1016/s0301-5629(99)00064-2. PMID: 10574340

Recent clinical studies

Etiology

Bozso SJ, Grant A, Iglesias I, Chu MWA
Ann Thorac Surg 2016 Sep;102(3):e223-e225. doi: 10.1016/j.athoracsur.2016.01.064. PMID: 27549549
Sandeep N, Slack MC
Cardiol Young 2014 Oct;24(5):797-806. Epub 2014 Mar 25 doi: 10.1017/S1047951114000353. PMID: 24666783
Quaegebeur J, Kirklin JW, Pacifico AD, Bargeron LM Jr
Ann Thorac Surg 1979 May;27(5):418-25. doi: 10.1016/s0003-4975(10)63339-5. PMID: 454016

Diagnosis

Yuge N, Manabe S, Hirayama D, Yamada R, Hori M, Saito T, Mochizuki N, Sugimura K, Shimokawa H
Int Heart J 2024;65(1):155-158. doi: 10.1536/ihj.23-401. PMID: 38296570
Sugimori H, Nakao T, Ikegaya Y, Iwahashi D, Tsuda S, Kume N, Onishi H, Nakamura S
J Cardiothorac Surg 2021 May 29;16(1):150. doi: 10.1186/s13019-021-01522-x. PMID: 34051799Free PMC Article
Karavelioglu Y, Cagli K, Yetim M, Bekar L, Golbasi Z
Echocardiography 2019 Mar;36(3):613-614. Epub 2019 Feb 8 doi: 10.1111/echo.14274. PMID: 30735270
De la Espriella-Juan R, Payá-Serrano R, Cubillos-Arango A, Bochard-Villanueva B, Sánchez-Álvarez S, Morell-Cabedo S
Rev Port Cardiol 2017 Jan;36(1):67-68. Epub 2016 Dec 12 doi: 10.1016/j.repc.2016.02.009. PMID: 27979322
Onan B, Aydin U, Basgoze S, Bakir I
Interact Cardiovasc Thorac Surg 2016 Oct;23(4):662-4. Epub 2016 Jun 26 doi: 10.1093/icvts/ivw200. PMID: 27354465

Prognosis

Yuge N, Manabe S, Hirayama D, Yamada R, Hori M, Saito T, Mochizuki N, Sugimura K, Shimokawa H
Int Heart J 2024;65(1):155-158. doi: 10.1536/ihj.23-401. PMID: 38296570
Sugimori H, Nakao T, Ikegaya Y, Iwahashi D, Tsuda S, Kume N, Onishi H, Nakamura S
J Cardiothorac Surg 2021 May 29;16(1):150. doi: 10.1186/s13019-021-01522-x. PMID: 34051799Free PMC Article
Bozso SJ, Grant A, Iglesias I, Chu MWA
Ann Thorac Surg 2016 Sep;102(3):e223-e225. doi: 10.1016/j.athoracsur.2016.01.064. PMID: 27549549
Sandeep N, Slack MC
Cardiol Young 2014 Oct;24(5):797-806. Epub 2014 Mar 25 doi: 10.1017/S1047951114000353. PMID: 24666783
Quaegebeur J, Kirklin JW, Pacifico AD, Bargeron LM Jr
Ann Thorac Surg 1979 May;27(5):418-25. doi: 10.1016/s0003-4975(10)63339-5. PMID: 454016

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