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Abnormal atrioventricular valve morphology

MedGen UID:
870547
•Concept ID:
C4024995
•
Anatomical Abnormality
Synonym: Abnormality of the atrioventricular valves
 
HPO: HP:0006705

Definition

An abnormality of an atrioventricular valve. [from HPO]

Professional guidelines

PubMed

Neonatal Marfan syndrome: a case report of a novel fibrillin 1 mutation, with genotype-phenotype correlation and brief review of the literature.
Pugnaloni F, De Rose DU, Digilio MC, Magliozzi M, Braguglia A, Valfrè L, Toscano A, Dotta A, Di Pede A
Ital J Pediatr 2024 Sep 18;50(1):183. doi: 10.1186/s13052-024-01756-0. PMID: 39294662Free PMC Article
Prenatal Diagnosis and Management of Single-Ventricle Heart Disease.
Freud LR, Seed M
Can J Cardiol 2022 Jul;38(7):897-908. Epub 2022 Apr 13 doi: 10.1016/j.cjca.2022.04.003. PMID: 35429589
Current options and outcomes for the management of atrioventricular septal defect.
Shuhaiber JH, Ho SY, Rigby M, Sethia B
Eur J Cardiothorac Surg 2009 May;35(5):891-900. Epub 2009 Feb 24 doi: 10.1016/j.ejcts.2009.01.009. PMID: 19243971

Recent clinical studies

Etiology

Atrioventricular valve surgery in patients with univentricular heart and two separate atrioventricular valves.
Mayr B, Osawa T, Staehler H, Schaeffer T, Röhlig C, Cleuziou J, Hager A, Ewert P, Hörer J, Lange R, Ono M
Cardiol Young 2024 Jul;34(7):1554-1562. Epub 2024 Apr 3 doi: 10.1017/S104795112400012X. PMID: 38567959
Atrioventricular valve surgery: Restoration of the fibrous skeleton of the heart.
Buratto E, Konstantinov IE
J Thorac Cardiovasc Surg 2021 Aug;162(2):360-365. Epub 2021 May 7 doi: 10.1016/j.jtcvs.2021.03.128. PMID: 34059335
Atrioventricular Valve Regurgitation in Single Ventricle Heart Disease: A Common Problem Associated With Progressive Deterioration and Mortality.
Tseng SY, Siddiqui S, Di Maria MV, Hill GD, Lubert AM, Kutty S, Opotowsky AR, Possner M, Morales DLS, Quintessenza JA, Alsaied T
J Am Heart Assoc 2020 Jun 2;9(11):e015737. Epub 2020 May 16 doi: 10.1161/JAHA.119.015737. PMID: 32419552Free PMC Article
Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326
Cor triatriatum sinister.
Kadner A, Meszaros K, Mueller C, Schoenhoff F, Hutter D, Carrel T
Multimed Man Cardiothorac Surg 2014;2014 Epub 2014 May 30 doi: 10.1093/mmcts/mmu005. PMID: 24878580

Diagnosis

Atrioventricular valve surgery in patients with univentricular heart and two separate atrioventricular valves.
Mayr B, Osawa T, Staehler H, Schaeffer T, Röhlig C, Cleuziou J, Hager A, Ewert P, Hörer J, Lange R, Ono M
Cardiol Young 2024 Jul;34(7):1554-1562. Epub 2024 Apr 3 doi: 10.1017/S104795112400012X. PMID: 38567959
Atrioventricular Septal Defects: Pathology, Imaging, and Treatment Options.
Taqatqa AS, Vettukattil JJ
Curr Cardiol Rep 2021 Jul 1;23(8):93. doi: 10.1007/s11886-021-01523-1. PMID: 34196822
Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326
Congenitally corrected transposition.
Wallis GA, Debich-Spicer D, Anderson RH
Orphanet J Rare Dis 2011 May 14;6:22. doi: 10.1186/1750-1172-6-22. PMID: 21569592Free PMC Article
Complete atrioventricular canal.
Calabrò R, Limongelli G
Orphanet J Rare Dis 2006 Apr 5;1:8. doi: 10.1186/1750-1172-1-8. PMID: 16722604Free PMC Article

Therapy

The Secrets of the Frogs Heart.
Corno AF, Zhou Z, Uppu SC, Huang S, Marino B, Milewicz DM, Salazar JD
Pediatr Cardiol 2022 Oct;43(7):1471-1480. Epub 2022 Mar 15 doi: 10.1007/s00246-022-02870-8. PMID: 35290490
Atrioventricular valve surgery: Restoration of the fibrous skeleton of the heart.
Buratto E, Konstantinov IE
J Thorac Cardiovasc Surg 2021 Aug;162(2):360-365. Epub 2021 May 7 doi: 10.1016/j.jtcvs.2021.03.128. PMID: 34059335
Relationship of Ventricular Morphology and Atrioventricular Valve Function to Long-Term Outcomes Following Fontan Procedures.
Moon J, Shen L, Likosky DS, Sood V, Hobbs RD, Sassalos P, Romano JC, Ohye RG, Bove EL, Si MS
J Am Coll Cardiol 2020 Jul 28;76(4):419-431. doi: 10.1016/j.jacc.2020.05.059. PMID: 32703513Free PMC Article
Atrioventricular Valve Regurgitation in Single Ventricle Heart Disease: A Common Problem Associated With Progressive Deterioration and Mortality.
Tseng SY, Siddiqui S, Di Maria MV, Hill GD, Lubert AM, Kutty S, Opotowsky AR, Possner M, Morales DLS, Quintessenza JA, Alsaied T
J Am Heart Assoc 2020 Jun 2;9(11):e015737. Epub 2020 May 16 doi: 10.1161/JAHA.119.015737. PMID: 32419552Free PMC Article
Cor triatriatum sinister.
Kadner A, Meszaros K, Mueller C, Schoenhoff F, Hutter D, Carrel T
Multimed Man Cardiothorac Surg 2014;2014 Epub 2014 May 30 doi: 10.1093/mmcts/mmu005. PMID: 24878580

Prognosis

Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326
Early fetal echocardiography.
McBrien A, Hornberger LK
Birth Defects Res 2019 May 1;111(8):370-379. Epub 2018 Nov 14 doi: 10.1002/bdr2.1414. PMID: 30430770
Congenitally corrected transposition.
Wallis GA, Debich-Spicer D, Anderson RH
Orphanet J Rare Dis 2011 May 14;6:22. doi: 10.1186/1750-1172-6-22. PMID: 21569592Free PMC Article
Complex atrioventricular canal.
Mitchell ME, Litwin SB, Tweddell JS
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2007:32-41. doi: 10.1053/j.pcsu.2007.01.002. PMID: 17433989
Complete atrioventricular canal.
Calabrò R, Limongelli G
Orphanet J Rare Dis 2006 Apr 5;1:8. doi: 10.1186/1750-1172-1-8. PMID: 16722604Free PMC Article

Clinical prediction guides

Neonatal Marfan syndrome: a case report of a novel fibrillin 1 mutation, with genotype-phenotype correlation and brief review of the literature.
Pugnaloni F, De Rose DU, Digilio MC, Magliozzi M, Braguglia A, Valfrè L, Toscano A, Dotta A, Di Pede A
Ital J Pediatr 2024 Sep 18;50(1):183. doi: 10.1186/s13052-024-01756-0. PMID: 39294662Free PMC Article
Predictors of spontaneous pregnancy loss in single ventricle physiology.
Wazni Y, Sefton C, Sharew B, Ghandakly E, Blazevic P, Mehra N, Lappen JR, Dolin CD, Kern-Goldberger A, Bacak S, Fuchs M, Zahka K, McKenney A, Tereshchenko LG, Singh K, Aziz PF, Ghobrial J
Open Heart 2024 Sep 13;11(2) doi: 10.1136/openhrt-2024-002768. PMID: 39277186Free PMC Article
Morphology of the mural and commissural atrioventricular junction of the mitral valve.
Krawczyk-Ożóg A, Batko J, Zdzierak B, Dziewierz A, Tyrak K, Bolechała F, Kopacz P, Strona M, Gil K, Hołda J, Hołda MK
Heart 2024 Mar 12;110(7):517-522. doi: 10.1136/heartjnl-2023-322965. PMID: 37935571
Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326
Echocardiographic characteristics of the criss-cross heart.
Yang YL, Wang XF, Cheng TO, Xie MX, Lü Q, He L, Lu XF, Wang J, Li L, Anderson RH
Int J Cardiol 2010 Apr 15;140(2):133-7. Epub 2009 Sep 16 doi: 10.1016/j.ijcard.2009.08.039. PMID: 19762095

Recent systematic reviews

Surgical outcomes of double-orifice mitral valve repair in patients with atrioventricular canal defects: a systematic review and meta-analysis.
Ghosh S, Halder V, Mittal A, Mishra A, Haranal M, Aggarwal P, Singh H, Barwad P, Naganur S, Thingnam SKS
Cardiol Young 2023 Sep;33(9):1506-1516. Epub 2023 Jul 31 doi: 10.1017/S1047951123002664. PMID: 37518865
Ventricular morphology of single-ventricle hearts has a significant impact on outcomes after Fontan palliation: a meta-analysis.
Ponzoni M, Azzolina D, Vedovelli L, Gregori D, Di Salvo G, D'Udekem Y, Vida V, Padalino MA
Eur J Cardiothorac Surg 2022 Nov 3;62(6) doi: 10.1093/ejcts/ezac535. PMID: 36367236

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