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Abnormal atrioventricular valve physiology

MedGen UID:
1647733
•Concept ID:
C4703462
•
Pathologic Function
HPO: HP:0031650

Definition

Any functional defect of the mitral or tricuspid valve. [from HPO]

Professional guidelines

PubMed

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
Management of the adult Fontan patient.
Rodriguez FH, Book WM
Heart 2020 Jan;106(2):105-110. Epub 2019 Oct 29 doi: 10.1136/heartjnl-2019-314937. PMID: 31662332
Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections.
Day TG, Woodgate T, Knee O, Zidere V, Vigneswaran T, Charakida M, Miller O, Sharland G, Simpson J
Pediatr Cardiol 2019 Oct;40(7):1509-1515. Epub 2019 Jul 25 doi: 10.1007/s00246-019-02176-2. PMID: 31342118

Recent clinical studies

Etiology

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
Hot topics in tetralogy of Fallot.
Villafañe J, Feinstein JA, Jenkins KJ, Vincent RN, Walsh EP, Dubin AM, Geva T, Towbin JA, Cohen MS, Fraser C, Dearani J, Rosenthal D, Kaufman B, Graham TP Jr; Adult Congenital and Pediatric Cardiology Section, American College of Cardiology
J Am Coll Cardiol 2013 Dec 10;62(23):2155-66. Epub 2013 Sep 27 doi: 10.1016/j.jacc.2013.07.100. PMID: 24076489

Diagnosis

Congenital heart defects in the recurrent 2q13 deletion syndrome.
Digilio MC, Dentici ML, Loddo S, Laino L, Calcagni G, Genovese S, Capolino R, Bottillo I, Calvieri G, Dallapiccola B, Marino B, Novelli A, Versacci P
Eur J Med Genet 2022 Jan;65(1):104381. Epub 2021 Nov 8 doi: 10.1016/j.ejmg.2021.104381. PMID: 34763108
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
Hot topics in tetralogy of Fallot.
Villafañe J, Feinstein JA, Jenkins KJ, Vincent RN, Walsh EP, Dubin AM, Geva T, Towbin JA, Cohen MS, Fraser C, Dearani J, Rosenthal D, Kaufman B, Graham TP Jr; Adult Congenital and Pediatric Cardiology Section, American College of Cardiology
J Am Coll Cardiol 2013 Dec 10;62(23):2155-66. Epub 2013 Sep 27 doi: 10.1016/j.jacc.2013.07.100. PMID: 24076489
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
The aging heart.
Klausner SC, Schwartz AB
Clin Geriatr Med 1985 Feb;1(1):119-41. PMID: 3913496

Therapy

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
Arrhythmias in Adults With Congenital Heart Disease: What the Practicing Cardiologist Needs to Know.
Khairy P
Can J Cardiol 2019 Dec;35(12):1698-1707. Epub 2019 Jul 16 doi: 10.1016/j.cjca.2019.07.009. PMID: 31703824
Perioperative and Anesthetic Considerations in Atrioventricular Septal Defect.
Ross FJ, Nasr VG, Joffe D, Latham GJ
Semin Cardiothorac Vasc Anesth 2017 Sep;21(3):221-228. Epub 2017 Jun 7 doi: 10.1177/1089253217706166. PMID: 28592182
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

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
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
Atrioventricular valve repair in patients with single-ventricle physiology: mechanisms, techniques of repair, and clinical outcomes.
Honjo O, Mertens L, Van Arsdell GS
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2011;14(1):75-84. doi: 10.1053/j.pcsu.2011.02.001. PMID: 21444052
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

Ex Vivo Modeling of Atrioventricular Valve Mechanics in Single Ventricle Physiology.
Moye SC, Kidambi S, Lee JY, Cowles TH, Gilligan-Steinberg SD, Bryan AY, Wilkerson R, Woo YJ, Ma MR
Ann Biomed Eng 2023 Aug;51(8):1738-1746. Epub 2023 Mar 25 doi: 10.1007/s10439-023-03178-1. PMID: 36966247
Biomarkers for heart failure and prognostic prediction in patients with Fontan circulation.
Inai K
Pediatr Int 2022 Jan;64(1):e14983. doi: 10.1111/ped.14983. PMID: 34480813
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
Atrioventricular Valve Repair in Single Ventricle Physiology: Timing Matters.
Miana LA, Manuel V, Turquetto AL, Issa HN, Guerreiro GP, Caneo LF, Jatene FB, Jatene MB
World J Pediatr Congenit Heart Surg 2020 Jan;11(1):22-28. doi: 10.1177/2150135119884916. PMID: 31835992
Arrhythmias in Adults With Congenital Heart Disease: What the Practicing Cardiologist Needs to Know.
Khairy P
Can J Cardiol 2019 Dec;35(12):1698-1707. Epub 2019 Jul 16 doi: 10.1016/j.cjca.2019.07.009. PMID: 31703824

Recent systematic reviews

Risk factors for death or heart transplantation in single-ventricle physiology (tricuspid atresia, pulmonary atresia, and heterotaxy): A systematic review and meta-analysis.
Kulkarni A, Patel N, Singh TP, Mossialos E, Mehra MR
J Heart Lung Transplant 2019 Jul;38(7):739-747. Epub 2019 Apr 4 doi: 10.1016/j.healun.2019.04.001. PMID: 31006521
Predicting long-term mortality after Fontan procedures: A risk score based on 6707 patients from 28 studies.
Alsaied T, Bokma JP, Engel ME, Kuijpers JM, Hanke SP, Zuhlke L, Zhang B, Veldtman GR
Congenit Heart Dis 2017 Jul;12(4):393-398. Epub 2017 May 8 doi: 10.1111/chd.12468. PMID: 28480627

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