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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

Patent Foramen Ovale Management for Secondary Stroke Prevention: State-of-the-Art Appraisal of Current Evidence.
Sposato LA, Albin CSW, Elkind MSV, Kamel H, Saver JL
Stroke 2024 Jan;55(1):236-247. Epub 2023 Nov 21 doi: 10.1161/STROKEAHA.123.040546. PMID: 38134261
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
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

Revisiting Atrioventricular Septal Defects: Exploring Chromosomal Abnormalities, Cardiac and Extracardiac Anomalies in a Contemporary Prenatal Cohort.
Ayhan I, Demirci O, Odacılar AŞ, Yücel İK, Karaman A
Pediatr Cardiol 2024 Jun;45(5):1036-1047. Epub 2024 Apr 3 doi: 10.1007/s00246-024-03477-x. PMID: 38570366
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
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

Revisiting Atrioventricular Septal Defects: Exploring Chromosomal Abnormalities, Cardiac and Extracardiac Anomalies in a Contemporary Prenatal Cohort.
Ayhan I, Demirci O, Odacılar AŞ, Yücel İK, Karaman A
Pediatr Cardiol 2024 Jun;45(5):1036-1047. Epub 2024 Apr 3 doi: 10.1007/s00246-024-03477-x. PMID: 38570366
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
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

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
Double mitral valve orifice in atrioventricular defects.
Warnes C, Somerville J
Br Heart J 1983 Jan;49(1):59-64. doi: 10.1136/hrt.49.1.59. PMID: 6821612Free PMC Article
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
OSTIUM PRIMUM DEFECT: FACTORS CAUSING DETERIORATION IN THE NATURAL HISTORY.
SOMERVILLE J
Br Heart J 1965 May;27(3):413-9. doi: 10.1136/hrt.27.3.413. PMID: 14284359Free PMC Article

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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