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Aorto-ventricular tunnel

MedGen UID:
868852
•Concept ID:
C4023262
•
Congenital Abnormality
Synonyms: Aortic Ventricular Tunnel; Aortic-Ventricular Tunnel; Aortic-Ventricular Tunnels; Aortico Ventricular Tunnel; Aortico-Ventricular Tunnel; Aortico-Ventricular Tunnels; Aorticoventricular Tunnel; Aorticoventricular Tunnels; Aorto Ventricular Tunnel; Aorto-Ventricular Tunnel; Aorto-Ventricular Tunnels; Aortoventricular Tunnel; Aortoventricular Tunnels; Tunnel, Aorticoventricular
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0011627
Monarch Initiative: MONDO:0018082
Orphanet: ORPHA3400

Definition

Aorto-ventricular tunnel is a congenital, extracardiac channel which connects the ascending aorta above the sinotubular junction to the cavity of the left, or (less commonly) right ventricle. [from HPO]

Professional guidelines

PubMed

Left aorto-ventricular tunnel: A differential diagnosis to aortic regurgitation.
Wong AR, Amelia A, Mohd Zain MR, Sayuti KA
Med J Malaysia 2022 Jan;77(1):101-103. PMID: 35087005

Recent clinical studies

Etiology

Congenital Aorto-Cardiac Connections (CACC) Revisited: Introduction of a Novel Anatomic-therapeutic Classification.
Rad EM, Hijazi ZM, Pouraliakbar H, Mirzaaghayan MR, Zamani H
Pediatr Cardiol 2021 Oct;42(7):1459-1477. Epub 2021 Jul 29 doi: 10.1007/s00246-021-02671-5. PMID: 34327543
Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.
Protopapas EM, Anderson RH, Backer CL, Fragata J, Hakim N, Vida VL, Sarris GE; European Congenital Heart Surgeons Association-World Society for Pediatric and Congenital Heart Surgery (ECHSA-WSPCHS) Study Group, Barron DJ, Berggren H, Hazekamp MG, Ilyin V, Kornoukhov OJ, Kostolny M, Lazarov S, Lo Rito M, Monge MC, Mykychak Y, Nosal M, Prêtre R, Polimenakos AC, Sojak V, Stellin G, Veshti A, Yemets I
Semin Thorac Cardiovasc Surg 2020 Summer;32(2):271-279. Epub 2020 Feb 10 doi: 10.1053/j.semtcvs.2020.01.011. PMID: 32057970

Diagnosis

Left aorto-ventricular tunnel: A differential diagnosis to aortic regurgitation.
Wong AR, Amelia A, Mohd Zain MR, Sayuti KA
Med J Malaysia 2022 Jan;77(1):101-103. PMID: 35087005
Congenital Aorto-Cardiac Connections (CACC) Revisited: Introduction of a Novel Anatomic-therapeutic Classification.
Rad EM, Hijazi ZM, Pouraliakbar H, Mirzaaghayan MR, Zamani H
Pediatr Cardiol 2021 Oct;42(7):1459-1477. Epub 2021 Jul 29 doi: 10.1007/s00246-021-02671-5. PMID: 34327543
A Review of the Surgical Management of Aorto-ventricular Tunnels.
Chowdhury UK, Anderson RH, George N, Singh S, Sankhyan LK, Pradeep D, Chauhan A, Sengupta S, Vaswani P
World J Pediatr Congenit Heart Surg 2021 Jan;12(1):103-115. doi: 10.1177/2150135120954809. PMID: 33407031
Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.
Protopapas EM, Anderson RH, Backer CL, Fragata J, Hakim N, Vida VL, Sarris GE; European Congenital Heart Surgeons Association-World Society for Pediatric and Congenital Heart Surgery (ECHSA-WSPCHS) Study Group, Barron DJ, Berggren H, Hazekamp MG, Ilyin V, Kornoukhov OJ, Kostolny M, Lazarov S, Lo Rito M, Monge MC, Mykychak Y, Nosal M, Prêtre R, Polimenakos AC, Sojak V, Stellin G, Veshti A, Yemets I
Semin Thorac Cardiovasc Surg 2020 Summer;32(2):271-279. Epub 2020 Feb 10 doi: 10.1053/j.semtcvs.2020.01.011. PMID: 32057970
Aorto-ventricular tunnel.
McKay R
Orphanet J Rare Dis 2007 Oct 8;2:41. doi: 10.1186/1750-1172-2-41. PMID: 17922908Free PMC Article

Therapy

Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.
Protopapas EM, Anderson RH, Backer CL, Fragata J, Hakim N, Vida VL, Sarris GE; European Congenital Heart Surgeons Association-World Society for Pediatric and Congenital Heart Surgery (ECHSA-WSPCHS) Study Group, Barron DJ, Berggren H, Hazekamp MG, Ilyin V, Kornoukhov OJ, Kostolny M, Lazarov S, Lo Rito M, Monge MC, Mykychak Y, Nosal M, Prêtre R, Polimenakos AC, Sojak V, Stellin G, Veshti A, Yemets I
Semin Thorac Cardiovasc Surg 2020 Summer;32(2):271-279. Epub 2020 Feb 10 doi: 10.1053/j.semtcvs.2020.01.011. PMID: 32057970

Prognosis

Congenital Aorto-Cardiac Connections (CACC) Revisited: Introduction of a Novel Anatomic-therapeutic Classification.
Rad EM, Hijazi ZM, Pouraliakbar H, Mirzaaghayan MR, Zamani H
Pediatr Cardiol 2021 Oct;42(7):1459-1477. Epub 2021 Jul 29 doi: 10.1007/s00246-021-02671-5. PMID: 34327543

Clinical prediction guides

Aorto-ventricular tunnel with three orifices: a unique case report diagnosed by transthoracic echocardiography.
Yang C, Yang J, Huang X, Wang J
Cardiovasc Ultrasound 2023 Apr 1;21(1):5. doi: 10.1186/s12947-023-00303-x. PMID: 37004030Free PMC Article
Aorto-ventricular tunnel.
McKay R
Orphanet J Rare Dis 2007 Oct 8;2:41. doi: 10.1186/1750-1172-2-41. PMID: 17922908Free PMC Article

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