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Ostium primum defect

MedGen UID:
45825
•Concept ID:
C0031192
•
Congenital Abnormality
Synonyms: Atrial Septal Defect Ostium Primum; Ostium Primum Atrial Septal Defect; Ostium Primum, Persistent; Persistent Ostium Primum; Primum, Persistent Ostium
SNOMED CT: Ostium primum defect (17718000); Persistent ostium primum (17718000); Atrial septum primum defect (17718000)

Definition

A congenital defect in the atrial septum at the level of the atrioventricular valves, resulting in abnormalities in the mitral and/or tricuspid valve; this defect is most commonly seen in those with Trisomy 21. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOstium primum defect

Professional guidelines

PubMed

Diagnosis and Management of Cardioembolic Stroke.
Yaghi S
Continuum (Minneap Minn) 2023 Apr 1;29(2):462-485. doi: 10.1212/CON.0000000000001217. PMID: 37039405
Advances in the management of cardioembolic stroke associated with patent foramen ovale.
Mac Grory B, Ohman EM, Feng W, Xian Y, Yaghi S, Kamel H, Reznik ME
BMJ 2022 Feb 9;376:e063161. doi: 10.1136/bmj-2020-063161. PMID: 35140114
European position paper on the management of patients with patent foramen ovale. General approach and left circulation thromboembolism.
Pristipino C, Sievert H, D'Ascenzo F, Louis Mas J, Meier B, Scacciatella P, Hildick-Smith D, Gaita F, Toni D, Kyrle P, Thomson J, Derumeaux G, Onorato E, Sibbing D, Germonpré P, Berti S, Chessa M, Bedogni F, Dudek D, Hornung M, Zamorano J; Evidence Synthesis Team; Eapci Scientific Documents and Initiatives Committee; International Experts
Eur Heart J 2019 Oct 7;40(38):3182-3195. doi: 10.1093/eurheartj/ehy649. PMID: 30358849

Recent clinical studies

Etiology

Antiplatelet and Antithrombotic Therapy After Patent Foramen Oval and Atrial Septal Defect Closure.
Drakopoulou M, Soulaidopoulos S, Stathogiannis K, Oikonomou G, Papanikolaou A, Toutouzas K, Tousoulis D
Curr Pharm Des 2020;26(23):2769-2779. doi: 10.2174/1385272824999200427083838. PMID: 32338207
Echocardiographic evaluation and surgical implications of common atrioventricular canal defects with absent or diminutive ostium primum defect.
Kaur A, Srivastava S, Lytrivi ID, Nguyen K, Lai WW, Parness IA
Am J Cardiol 2008 Jun 1;101(11):1648-51. Epub 2008 Apr 8 doi: 10.1016/j.amjcard.2008.01.049. PMID: 18489945
Direct closure of ostium primum defect in the repair of atrioventricular septal defect.
Chikada M, Sekiguchi A, Miyamoto T, Matsuzaki M, Ishida R, Ishizawa A
Ann Thorac Surg 2001 Aug;72(2):430-2; discussion 432-3. doi: 10.1016/s0003-4975(01)02809-0. PMID: 11515878
Congenital Heart Surgery Nomenclature and Database Project: atrioventricular canal defect.
Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C
Ann Thorac Surg 2000 Apr;69(4 Suppl):S36-43. doi: 10.1016/s0003-4975(99)01235-7. PMID: 10798414
Genetics of atrial septal defect.
Sánchez Cascos A
Arch Dis Child 1972 Aug;47(254):581-8. doi: 10.1136/adc.47.254.581. PMID: 4261647Free PMC Article

Diagnosis

Atrial Septal Defect.
Bradley EA, Zaidi AN
Cardiol Clin 2020 Aug;38(3):317-324. Epub 2020 Jun 6 doi: 10.1016/j.ccl.2020.04.001. PMID: 32622487
Diagnosis of ostium primum defect at multidetector CT in an adult.
Rajiah P, Renapurkar R, Kanne J
J Thorac Imaging 2009 Aug;24(3):234-6. doi: 10.1097/RTI.0b013e3181a6d0d2. PMID: 19704330
Echocardiographic evaluation and surgical implications of common atrioventricular canal defects with absent or diminutive ostium primum defect.
Kaur A, Srivastava S, Lytrivi ID, Nguyen K, Lai WW, Parness IA
Am J Cardiol 2008 Jun 1;101(11):1648-51. Epub 2008 Apr 8 doi: 10.1016/j.amjcard.2008.01.049. PMID: 18489945
Cleft mitral valve without ostium primum defect: anatomic data and surgical considerations based on 41 cases.
Van Praagh S, Porras D, Oppido G, Geva T, Van Praagh R
Ann Thorac Surg 2003 Jun;75(6):1752-62. doi: 10.1016/s0003-4975(03)00167-x. PMID: 12822611
Congenital Heart Surgery Nomenclature and Database Project: atrioventricular canal defect.
Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C
Ann Thorac Surg 2000 Apr;69(4 Suppl):S36-43. doi: 10.1016/s0003-4975(99)01235-7. PMID: 10798414

Therapy

Left atrioventricular valve regurgitation after repair of incomplete atrioventricular septal defect.
Murashita T, Kubota T, Oba J, Aoki T, Matano J, Yasuda K
Ann Thorac Surg 2004 Jun;77(6):2157-62. doi: 10.1016/j.athoracsur.2003.12.019. PMID: 15172287

Prognosis

Port-access minimally invasive surgery for atrial septal defects: a 10-year single-center experience in 166 patients.
Vistarini N, Aiello M, Mattiucci G, Alloni A, Cattadori B, Tinelli C, Pellegrini C, D'Armini AM, Viganò M
J Thorac Cardiovasc Surg 2010 Jan;139(1):139-45. Epub 2009 Aug 25 doi: 10.1016/j.jtcvs.2009.07.022. PMID: 19709683
Echocardiographic evaluation and surgical implications of common atrioventricular canal defects with absent or diminutive ostium primum defect.
Kaur A, Srivastava S, Lytrivi ID, Nguyen K, Lai WW, Parness IA
Am J Cardiol 2008 Jun 1;101(11):1648-51. Epub 2008 Apr 8 doi: 10.1016/j.amjcard.2008.01.049. PMID: 18489945
Direct closure of ostium primum defect in the repair of atrioventricular septal defect.
Chikada M, Sekiguchi A, Miyamoto T, Matsuzaki M, Ishida R, Ishizawa A
Ann Thorac Surg 2001 Aug;72(2):430-2; discussion 432-3. doi: 10.1016/s0003-4975(01)02809-0. PMID: 11515878
Long-term follow-up after surgical repair of ostium primum atrial septal defects in adults.
Burke RP, Horvath K, Landzberg M, Hyde P, Collins JJ Jr, Cohn LH
J Am Coll Cardiol 1996 Mar 1;27(3):696-9. doi: 10.1016/0735-1097(95)00501-3. PMID: 8606284
Ostium primum defect in the adult: postoperative follow-up studies.
Goodman DJ, Harrison DC, Schroeder JS
Chest 1975 Feb;67(2):185-9. doi: 10.1378/chest.67.2.185. PMID: 123190

Clinical prediction guides

Closure of atrial septal defects via limited right anterolateral thoracotomy as a minimal invasive approach in female patients.
Däbritz S, Sachweh J, Walter M, Messmer BJ
Eur J Cardiothorac Surg 1999 Jan;15(1):18-23. doi: 10.1016/s1010-7940(98)00267-x. PMID: 10077368
Long-term follow-up after surgical repair of ostium primum atrial septal defects in adults.
Burke RP, Horvath K, Landzberg M, Hyde P, Collins JJ Jr, Cohn LH
J Am Coll Cardiol 1996 Mar 1;27(3):696-9. doi: 10.1016/0735-1097(95)00501-3. PMID: 8606284
Echocardiographic diagnosis of endocardial cushion defects.
Yoshikawa J, Owaki T, Kato H, Tomita Y, Baba K
Jpn Heart J 1975 Jan;16(1):1-10. doi: 10.1536/ihj.16.1. PMID: 1113447

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