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Sinus venosus atrial septal defect

MedGen UID:
138011
•Concept ID:
C0344730
•
Congenital Abnormality
Synonyms: Sinus venosus ASD; Sinus venosus atrial septal defects
SNOMED CT: Sinus venosus atrial septal defect (95268002); Sinus venosus defect (95268002)
 
HPO: HP:0011567
Monarch Initiative: MONDO:0020436
Orphanet: ORPHA99105

Definition

An interatrial communication caused by a deficiency of the common wall between the superior vena cava (SVC) and the right-sided pulmonary veins. SVASD is commonly associated with anomalous pulmonary venous connection (APVC) of some or all of the pulmonary veins, which produces additional left-to-right shunting. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSinus venosus atrial septal defect

Professional guidelines

PubMed

Transcatheter versus surgical treatment for isolated superior sinus venosus atrial septal defect.
Brancato F, Stephenson N, Rosenthal E, Hansen JH, Jones MI, Qureshi S, Austin C, Speggiorin S, Caner S, Butera G
Catheter Cardiovasc Interv 2023 May;101(6):1098-1107. Epub 2023 Apr 1 doi: 10.1002/ccd.30650. PMID: 37002948
Surgical treatment of adult atrial septal defect: early and long-term results.
Horvath KA, Burke RP, Collins JJ Jr, Cohn LH
J Am Coll Cardiol 1992 Nov 1;20(5):1156-9. doi: 10.1016/0735-1097(92)90372-t. PMID: 1401616

Recent clinical studies

Etiology

Novel technique for transcatheter closure of sinus venosus atrial septal defect: The temporary suture-holding technique.
Hejazi Y, Hijazi ZM, Saloos HA, Ibrahim H, Mann GS, Boudjemline Y
Catheter Cardiovasc Interv 2022 Nov;100(6):1068-1077. Epub 2022 Oct 2 doi: 10.1002/ccd.30415. PMID: 36183408
Evolution and Current Results of a Unified Strategy for Sinus Venosus Surgery.
Stephens EH, Mongé MC, Eltayeb O, Patel A, Webster G, Cornicelli MD, Kennedy C, Popescu AR, Rigsby CK, Backer CL
Ann Thorac Surg 2021 Mar;111(3):980-986. Epub 2020 May 11 doi: 10.1016/j.athoracsur.2020.03.113. PMID: 32437676
Repair of Superior Sinus Venosus Atrial Septal Defect Using a Modified Two-Patch Technique.
Elzein C, Abdulkarim M, Abbas U, Vricella L, Ilbawi M
Ann Thorac Surg 2020 Feb;109(2):583-587. Epub 2019 Aug 28 doi: 10.1016/j.athoracsur.2019.07.023. PMID: 31472131
Dasatinib-Induced Pulmonary Arterial Hypertension.
Orlikow E, Weatherald J, Hirani N
Can J Cardiol 2019 Nov;35(11):1604.e1-1604.e3. Epub 2019 Aug 8 doi: 10.1016/j.cjca.2019.08.002. PMID: 31590985
An adult with a sinus venosus atrial septal defect and dilated cardiomyopathy.
Oakley L, Foley S, Cox J, Seidensticker D
BMJ Case Rep 2014 May 22;2014 doi: 10.1136/bcr-2013-201306. PMID: 24855073Free PMC Article

Diagnosis

Evolution and Current Results of a Unified Strategy for Sinus Venosus Surgery.
Stephens EH, Mongé MC, Eltayeb O, Patel A, Webster G, Cornicelli MD, Kennedy C, Popescu AR, Rigsby CK, Backer CL
Ann Thorac Surg 2021 Mar;111(3):980-986. Epub 2020 May 11 doi: 10.1016/j.athoracsur.2020.03.113. PMID: 32437676
Dasatinib-Induced Pulmonary Arterial Hypertension.
Orlikow E, Weatherald J, Hirani N
Can J Cardiol 2019 Nov;35(11):1604.e1-1604.e3. Epub 2019 Aug 8 doi: 10.1016/j.cjca.2019.08.002. PMID: 31590985
An adult with a sinus venosus atrial septal defect and dilated cardiomyopathy.
Oakley L, Foley S, Cox J, Seidensticker D
BMJ Case Rep 2014 May 22;2014 doi: 10.1136/bcr-2013-201306. PMID: 24855073Free PMC Article
Sinus venosus atrial septal defect in adults: utility of cardiovascular MRI in resolving this diagnostic dilemma.
Gulati GS, Hoey ET, Gopalan D, Agrawal BS, Screaton NJ
Heart Lung Circ 2010 Oct;19(10):615-9. Epub 2010 Jul 22 doi: 10.1016/j.hlc.2010.06.666. PMID: 20655279
Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients.
Attenhofer Jost CH, Connolly HM, Danielson GK, Bailey KR, Schaff HV, Shen WK, Warnes CA, Seward JB, Puga FJ, Tajik AJ
Circulation 2005 Sep 27;112(13):1953-8. Epub 2005 Sep 19 doi: 10.1161/CIRCULATIONAHA.104.493775. PMID: 16172274

Therapy

Novel technique for transcatheter closure of sinus venosus atrial septal defect: The temporary suture-holding technique.
Hejazi Y, Hijazi ZM, Saloos HA, Ibrahim H, Mann GS, Boudjemline Y
Catheter Cardiovasc Interv 2022 Nov;100(6):1068-1077. Epub 2022 Oct 2 doi: 10.1002/ccd.30415. PMID: 36183408
Dasatinib-Induced Pulmonary Arterial Hypertension.
Orlikow E, Weatherald J, Hirani N
Can J Cardiol 2019 Nov;35(11):1604.e1-1604.e3. Epub 2019 Aug 8 doi: 10.1016/j.cjca.2019.08.002. PMID: 31590985
An adult with a sinus venosus atrial septal defect and dilated cardiomyopathy.
Oakley L, Foley S, Cox J, Seidensticker D
BMJ Case Rep 2014 May 22;2014 doi: 10.1136/bcr-2013-201306. PMID: 24855073Free PMC Article
Is double or single patch for sinus venosus atrial septal defect repair the better option in prevention of postoperative venous obstruction?
Okonta KE, Tamatey M
Interact Cardiovasc Thorac Surg 2012 Nov;15(5):900-3. Epub 2012 Aug 22 doi: 10.1093/icvts/ivs369. PMID: 22914802Free PMC Article
Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients.
Attenhofer Jost CH, Connolly HM, Danielson GK, Bailey KR, Schaff HV, Shen WK, Warnes CA, Seward JB, Puga FJ, Tajik AJ
Circulation 2005 Sep 27;112(13):1953-8. Epub 2005 Sep 19 doi: 10.1161/CIRCULATIONAHA.104.493775. PMID: 16172274

Prognosis

Evolution and Current Results of a Unified Strategy for Sinus Venosus Surgery.
Stephens EH, Mongé MC, Eltayeb O, Patel A, Webster G, Cornicelli MD, Kennedy C, Popescu AR, Rigsby CK, Backer CL
Ann Thorac Surg 2021 Mar;111(3):980-986. Epub 2020 May 11 doi: 10.1016/j.athoracsur.2020.03.113. PMID: 32437676
Dasatinib-Induced Pulmonary Arterial Hypertension.
Orlikow E, Weatherald J, Hirani N
Can J Cardiol 2019 Nov;35(11):1604.e1-1604.e3. Epub 2019 Aug 8 doi: 10.1016/j.cjca.2019.08.002. PMID: 31590985
An adult with a sinus venosus atrial septal defect and dilated cardiomyopathy.
Oakley L, Foley S, Cox J, Seidensticker D
BMJ Case Rep 2014 May 22;2014 doi: 10.1136/bcr-2013-201306. PMID: 24855073Free PMC Article
Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients.
Attenhofer Jost CH, Connolly HM, Danielson GK, Bailey KR, Schaff HV, Shen WK, Warnes CA, Seward JB, Puga FJ, Tajik AJ
Circulation 2005 Sep 27;112(13):1953-8. Epub 2005 Sep 19 doi: 10.1161/CIRCULATIONAHA.104.493775. PMID: 16172274
Outcome following repair of sinus venosus atrial septal defects in children.
Russell JL, LeBlanc JG, Deagle ML, Potts JE
Asian Cardiovasc Thorac Ann 2002 Sep;10(3):231-4. doi: 10.1177/021849230201000309. PMID: 12213746

Clinical prediction guides

Repair of Superior Sinus Venosus Atrial Septal Defect Using a Modified Two-Patch Technique.
Elzein C, Abdulkarim M, Abbas U, Vricella L, Ilbawi M
Ann Thorac Surg 2020 Feb;109(2):583-587. Epub 2019 Aug 28 doi: 10.1016/j.athoracsur.2019.07.023. PMID: 31472131
Transcatheter closure of a sinus venosus atrial septal defect using 3D printing and image fusion guidance.
Thakkar AN, Chinnadurai P, Breinholt JP, Lin CH
Catheter Cardiovasc Interv 2018 Aug 1;92(2):353-357. Epub 2018 Jun 13 doi: 10.1002/ccd.27645. PMID: 29897667Free PMC Article
Warden repair for superior sinus venosus atrial septal defect and anomalous pulmonary venous drainage in children: Anesthesia and transesophageal echocardiography perspectives.
Aggarwal N, Gadhinglajkar S, Sreedhar R, Dharan BS, Chigurupati K, Babu S
Ann Card Anaesth 2016 Apr-Jun;19(2):293-9. doi: 10.4103/0971-9784.179631. PMID: 27052072Free PMC Article
Hypertensive pulmonary vascular disease in adults with secundum or sinus venosus atrial septal defect.
Sachweh JS, Daebritz SH, Hermanns B, Fausten B, Jockenhoevel S, Handt S, Messmer BJ
Ann Thorac Surg 2006 Jan;81(1):207-13. doi: 10.1016/j.athoracsur.2005.07.053. PMID: 16368366
Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients.
Attenhofer Jost CH, Connolly HM, Danielson GK, Bailey KR, Schaff HV, Shen WK, Warnes CA, Seward JB, Puga FJ, Tajik AJ
Circulation 2005 Sep 27;112(13):1953-8. Epub 2005 Sep 19 doi: 10.1161/CIRCULATIONAHA.104.493775. PMID: 16172274

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