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Complete Atrioventricular Septal Defect with Tetralogy of Fallot

MedGen UID:
768726
•Concept ID:
C3640086
•
Disease or Syndrome

Definition

A rare congenital heart anomaly in which there is coexistence of tetralogy of Fallot and complete atrioventricular septal defect. The latter is characterized by defects in the atrial and ventricular septa and a common atrioventricular valve. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVComplete Atrioventricular Septal Defect with Tetralogy of Fallot

Professional guidelines

PubMed

Influence of staged repair and primary repair on outcomes in patients with complete atrioventricular septal defect and tetralogy of Fallot: a systematic review and meta-analysis.
Lenko E, Kulyabin Y, Zubritskiy A, Gorbatykh Y, Naberukhin Y, Nichay N, Bogachev-Prokophiev A, Karaskov A
Interact Cardiovasc Thorac Surg 2018 Jan 1;26(1):98-105. doi: 10.1093/icvts/ivx267. PMID: 29049707
Complete atrioventricular septal defect with tetralogy of Fallot: diagnosis and management.
Gatzoulis MA, Shore D, Yacoub M, Shinebourne EA
Br Heart J 1994 Jun;71(6):579-83. doi: 10.1136/hrt.71.6.579. PMID: 8043343Free PMC Article

Recent clinical studies

Etiology

Contemporary surgical management of complete atrioventricular septal defect with tetralogy of Fallot in Japan.
Sugimoto A, Tachimori H, Hirata Y, Sakamoto K, Ota N, Shiraishi S, Tsuchida M, Motomura N
Gen Thorac Cardiovasc Surg 2022 Oct;70(10):835-841. Epub 2022 Mar 25 doi: 10.1007/s11748-022-01809-3. PMID: 35332445
Influence of staged repair and primary repair on outcomes in patients with complete atrioventricular septal defect and tetralogy of Fallot: a systematic review and meta-analysis.
Lenko E, Kulyabin Y, Zubritskiy A, Gorbatykh Y, Naberukhin Y, Nichay N, Bogachev-Prokophiev A, Karaskov A
Interact Cardiovasc Thorac Surg 2018 Jan 1;26(1):98-105. doi: 10.1093/icvts/ivx267. PMID: 29049707
Repair of complete atrioventricular septal defect with tetralogy of fallot: our experience and literature review.
Prifti E, Bonacchi M, Bernabei M, Leacche M, Bartolozzi F, Murzi B, Battaglia F, Nadia NS, Vanini V
J Card Surg 2004 Mar-Apr;19(2):175-83. doi: 10.1111/j.0886-0440.2004.04031.x. PMID: 15016061
Complete atrioventricular septal defect associated with tetralogy of fallot: surgical indications and results.
Okada Y, Tatsuno K, Kikuchi T, Takahashi Y, Shimokawa T
Jpn Circ J 1999 Nov;63(11):889-92. doi: 10.1253/jcj.63.889. PMID: 10598897
Complete atrioventricular septal defect with tetralogy of Fallot: diagnosis and management.
Gatzoulis MA, Shore D, Yacoub M, Shinebourne EA
Br Heart J 1994 Jun;71(6):579-83. doi: 10.1136/hrt.71.6.579. PMID: 8043343Free PMC Article

Diagnosis

Prenatal Diagnosis, Associations and Outcome for Fetuses with Congenital Absence of the Pulmonary Valve Syndrome.
Babaoğlu K, Doğan Y, Erdem S, Özbarlas N, Başar E, Uzun O
Anatol J Cardiol 2022 Sep;26(9):702-709. doi: 10.5152/AnatolJCardiol.2022.1461. PMID: 35943311Free PMC Article
Contemporary surgical management of complete atrioventricular septal defect with tetralogy of Fallot in Japan.
Sugimoto A, Tachimori H, Hirata Y, Sakamoto K, Ota N, Shiraishi S, Tsuchida M, Motomura N
Gen Thorac Cardiovasc Surg 2022 Oct;70(10):835-841. Epub 2022 Mar 25 doi: 10.1007/s11748-022-01809-3. PMID: 35332445
Cardiac Function After Tetralogy of Fallot/Complete Atrioventricular Canal Repair.
Stephens EH, Tingo J, Najjar M, Yilmaz B, Levasseur S, Dayton JD, Mosca RS, Chai P, Quaegebeur JM, Bacha EA
World J Pediatr Congenit Heart Surg 2017 Mar;8(2):189-195. doi: 10.1177/2150135116682719. PMID: 28329461
Surgical management of complete atrioventricular septal defect with tetralogy of fallot.
Hu R, Zhang H, Xu Z, Liu J, Su Z, Ding W
Ann Thorac Cardiovasc Surg 2014;20(5):341-6. Epub 2013 Oct 3 doi: 10.5761/atcs.oa.12-02198. PMID: 24088923
Complete atrioventricular septal defect with tetralogy of Fallot: diagnosis and management.
Gatzoulis MA, Shore D, Yacoub M, Shinebourne EA
Br Heart J 1994 Jun;71(6):579-83. doi: 10.1136/hrt.71.6.579. PMID: 8043343Free PMC Article

Therapy

Levosimendan Versus Milrinone and Release of Myocardial Biomarkers After Pediatric Cardiac Surgery: Post Hoc Analysis of Clinical Trial Data.
Thorlacius EM, Vistnes M, Ojala T, Keski-Nisula J, Molin M, Romlin BS, Synnergren M, Ricksten SE, Wåhlander H, Castellheim A
Pediatr Crit Care Med 2021 Jul 1;22(7):e402-e409. doi: 10.1097/PCC.0000000000002712. PMID: 33739957
Short to medium term outcomes of right ventricular outflow tract stenting as initial palliation for symptomatic infants with complete atrioventricular septal defect with associated tetralogy of Fallot.
Abumehdi M, Al Nasef M, Mehta C, Botha P, McMahon C, Oslizlok P, Walsh KP, McCrossan B, Kenny D, Stümper O
Catheter Cardiovasc Interv 2020 Dec;96(7):1445-1453. Epub 2020 Oct 6 doi: 10.1002/ccd.29306. PMID: 33022100
Surgical management of complete atrioventricular septal defect with tetralogy of fallot.
Hu R, Zhang H, Xu Z, Liu J, Su Z, Ding W
Ann Thorac Cardiovasc Surg 2014;20(5):341-6. Epub 2013 Oct 3 doi: 10.5761/atcs.oa.12-02198. PMID: 24088923
Outcome after repair of atrioventricular septal defect with tetralogy of Fallot.
Shuhaiber JH, Robinson B, Gauvreau K, Breitbart R, Mayer JE, Del Nido PJ, Pigula F
J Thorac Cardiovasc Surg 2012 Feb;143(2):338-43. Epub 2011 Sep 8 doi: 10.1016/j.jtcvs.2011.05.031. PMID: 21855095
Surgical treatment of complete atrioventricular septal defect with the two-patch technique: early-to-mid follow-up.
Monteiro AJ, Canale LS, Rangel I, Wetzel E, Pinto DF, Barbosa RC, Méier MA, Marcial ML
Interact Cardiovasc Thorac Surg 2007 Dec;6(6):737-40. Epub 2007 Aug 31 doi: 10.1510/icvts.2007.161778. PMID: 17766275

Prognosis

Predictors of reoperation and mortality after complete atrioventricular septal defect repair.
Fong LS, Betts K, Ayer J, Andrews D, Nicholson IA, Winlaw DS, Orr Y; Australian CAVSD study group
Eur J Cardiothorac Surg 2021 Dec 27;61(1):45-53. doi: 10.1093/ejcts/ezab221. PMID: 34002204
Surgical management of complete atrioventricular septal defect with tetralogy of fallot.
Hu R, Zhang H, Xu Z, Liu J, Su Z, Ding W
Ann Thorac Cardiovasc Surg 2014;20(5):341-6. Epub 2013 Oct 3 doi: 10.5761/atcs.oa.12-02198. PMID: 24088923
Repair of complete atrioventricular septal defect with tetralogy of fallot: our experience and literature review.
Prifti E, Bonacchi M, Bernabei M, Leacche M, Bartolozzi F, Murzi B, Battaglia F, Nadia NS, Vanini V
J Card Surg 2004 Mar-Apr;19(2):175-83. doi: 10.1111/j.0886-0440.2004.04031.x. PMID: 15016061
Complete atrioventricular septal defect associated with tetralogy of fallot: surgical indications and results.
Okada Y, Tatsuno K, Kikuchi T, Takahashi Y, Shimokawa T
Jpn Circ J 1999 Nov;63(11):889-92. doi: 10.1253/jcj.63.889. PMID: 10598897
Complete atrioventricular septal defect with tetralogy of Fallot: diagnosis and management.
Gatzoulis MA, Shore D, Yacoub M, Shinebourne EA
Br Heart J 1994 Jun;71(6):579-83. doi: 10.1136/hrt.71.6.579. PMID: 8043343Free PMC Article

Clinical prediction guides

Prenatal Diagnosis, Associations and Outcome for Fetuses with Congenital Absence of the Pulmonary Valve Syndrome.
Babaoğlu K, Doğan Y, Erdem S, Özbarlas N, Başar E, Uzun O
Anatol J Cardiol 2022 Sep;26(9):702-709. doi: 10.5152/AnatolJCardiol.2022.1461. PMID: 35943311Free PMC Article
Predictors of reoperation and mortality after complete atrioventricular septal defect repair.
Fong LS, Betts K, Ayer J, Andrews D, Nicholson IA, Winlaw DS, Orr Y; Australian CAVSD study group
Eur J Cardiothorac Surg 2021 Dec 27;61(1):45-53. doi: 10.1093/ejcts/ezab221. PMID: 34002204
Short to medium term outcomes of right ventricular outflow tract stenting as initial palliation for symptomatic infants with complete atrioventricular septal defect with associated tetralogy of Fallot.
Abumehdi M, Al Nasef M, Mehta C, Botha P, McMahon C, Oslizlok P, Walsh KP, McCrossan B, Kenny D, Stümper O
Catheter Cardiovasc Interv 2020 Dec;96(7):1445-1453. Epub 2020 Oct 6 doi: 10.1002/ccd.29306. PMID: 33022100
The role of intraoperative epicardial echocardiography in pediatric cardiac surgery.
Ozturk E, Cansaran Tanidir I, Ayyildiz P, Gokalp S, Candas Kafali H, Sahin M, Ergul Y, Haydin S, Guzeltas A
Echocardiography 2018 Jul;35(7):999-1004. Epub 2018 Mar 25 doi: 10.1111/echo.13874. PMID: 29577408
Interventricular communication in complete atrioventricular septal defect.
Suzuki K, Ho SY, Anderson RH, Becker AE, Neches WH, Tatsuno K, Mimori S
Ann Thorac Surg 1998 Oct;66(4):1389-93. doi: 10.1016/s0003-4975(98)00644-4. PMID: 9800838

Recent systematic reviews

Influence of staged repair and primary repair on outcomes in patients with complete atrioventricular septal defect and tetralogy of Fallot: a systematic review and meta-analysis.
Lenko E, Kulyabin Y, Zubritskiy A, Gorbatykh Y, Naberukhin Y, Nichay N, Bogachev-Prokophiev A, Karaskov A
Interact Cardiovasc Thorac Surg 2018 Jan 1;26(1):98-105. doi: 10.1093/icvts/ivx267. PMID: 29049707

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