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Abnormal descending aorta morphology

MedGen UID:
1670974
•Concept ID:
C4732763
•
Anatomical Abnormality
HPO: HP:0031934

Definition

A structural abnormality of the part of the aorta that begins at the aortic arch and then descends through the chest and abdomen. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbnormal descending aorta morphology

Professional guidelines

PubMed

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.
Gaudino M, Di Franco A, Arbustini E, Bacha E, Bates ER, Cameron DE, Cao D, David TE, De Paulis R, El-Hamamsy I, Farooqi KM, Girardi LN, Gräni C, Kochav JD, Molossi S, Puskas JD, Rao SV, Sandner S, Tatoulis J, Truong QA, Weinsaft JW, Zimpfer D, Mery CM
J Am Coll Cardiol 2023 Nov 21;82(21):2034-2053. Epub 2023 Oct 16 doi: 10.1016/j.jacc.2023.08.012. PMID: 37855757
International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, Devereux RB, Fernández B, Asch FM, Barker AJ, Sierra-Galan LM, De Kerchove L, Fernandes SM, Fedak PWM, Girdauskas E, Delgado V, Abbara S, Lansac E, Prakash SK, Bissell MM, Popescu BA, Hope MD, Sitges M, Thourani VH, Pibarot P, Chandrasekaran K, Lancellotti P, Borger MA, Forrest JK, Webb J, Milewicz DM, Makkar R, Leon MB, Sanders SP, Markl M, Ferrari VA, Roberts WC, Song JK, Blanke P, White CS, Siu S, Svensson LG, Braverman AC, Bavaria J, Sundt TM, El Khoury G, De Paulis R, Enriquez-Sarano M, Bax JJ, Otto CM, Schäfers HJ
J Thorac Cardiovasc Surg 2021 Sep;162(3):e383-e414. Epub 2021 Jul 22 doi: 10.1016/j.jtcvs.2021.06.019. PMID: 34304896
Editor's Choice - Management of Descending Thoracic Aorta Diseases: Clinical Practice Guidelines of the European Society for Vascular Surgery (ESVS).
Riambau V, Böckler D, Brunkwall J, Cao P, Chiesa R, Coppi G, Czerny M, Fraedrich G, Haulon S, Jacobs MJ, Lachat ML, Moll FL, Setacci C, Taylor PR, Thompson M, Trimarchi S, Verhagen HJ, Verhoeven EL, Esvs Guidelines Committee, Kolh P, de Borst GJ, Chakfé N, Debus ES, Hinchliffe RJ, Kakkos S, Koncar I, Lindholt JS, Vega de Ceniga M, Vermassen F, Verzini F, Document Reviewers, Kolh P, Black JH 3rd, Busund R, Björck M, Dake M, Dick F, Eggebrecht H, Evangelista A, Grabenwöger M, Milner R, Naylor AR, Ricco JB, Rousseau H, Schmidli J
Eur J Vasc Endovasc Surg 2017 Jan;53(1):4-52. doi: 10.1016/j.ejvs.2016.06.005. PMID: 28081802

Recent clinical studies

Etiology

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.
Gaudino M, Di Franco A, Arbustini E, Bacha E, Bates ER, Cameron DE, Cao D, David TE, De Paulis R, El-Hamamsy I, Farooqi KM, Girardi LN, Gräni C, Kochav JD, Molossi S, Puskas JD, Rao SV, Sandner S, Tatoulis J, Truong QA, Weinsaft JW, Zimpfer D, Mery CM
J Am Coll Cardiol 2023 Nov 21;82(21):2034-2053. Epub 2023 Oct 16 doi: 10.1016/j.jacc.2023.08.012. PMID: 37855757
Circumflex aorta: An uncharted territory.
Haranal M, Srimurugan B, Sivalingam S
Asian Cardiovasc Thorac Ann 2022 Feb;30(2):217-225. Epub 2021 May 6 doi: 10.1177/02184923211015092. PMID: 33957784
The aberrant subclavian artery: approach to management.
Karangelis D, Loggos S, Tzifa A, Mitropoulos FA
Curr Opin Cardiol 2020 Nov;35(6):636-642. doi: 10.1097/HCO.0000000000000793. PMID: 32852349
Congenital vascular ring.
Yoshimura N, Fukahara K, Yamashita A, Doi T, Yamashita S, Homma T, Yokoyama S, Aoki M, Higashida A, Shimada Y, Toritsuka D, Nagao K, Ikeno Y
Surg Today 2020 Oct;50(10):1151-1158. Epub 2019 Nov 1 doi: 10.1007/s00595-019-01907-5. PMID: 31676999
Preoperative Physiology, Imaging, and Management of Interrupted Aortic Arch.
Friedman K
Semin Cardiothorac Vasc Anesth 2018 Sep;22(3):265-269. Epub 2018 Apr 12 doi: 10.1177/1089253218770198. PMID: 29649938

Diagnosis

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.
Gaudino M, Di Franco A, Arbustini E, Bacha E, Bates ER, Cameron DE, Cao D, David TE, De Paulis R, El-Hamamsy I, Farooqi KM, Girardi LN, Gräni C, Kochav JD, Molossi S, Puskas JD, Rao SV, Sandner S, Tatoulis J, Truong QA, Weinsaft JW, Zimpfer D, Mery CM
J Am Coll Cardiol 2023 Nov 21;82(21):2034-2053. Epub 2023 Oct 16 doi: 10.1016/j.jacc.2023.08.012. PMID: 37855757
Congenital vascular ring.
Yoshimura N, Fukahara K, Yamashita A, Doi T, Yamashita S, Homma T, Yokoyama S, Aoki M, Higashida A, Shimada Y, Toritsuka D, Nagao K, Ikeno Y
Surg Today 2020 Oct;50(10):1151-1158. Epub 2019 Nov 1 doi: 10.1007/s00595-019-01907-5. PMID: 31676999
Editor's Choice - Management of Descending Thoracic Aorta Diseases: Clinical Practice Guidelines of the European Society for Vascular Surgery (ESVS).
Riambau V, Böckler D, Brunkwall J, Cao P, Chiesa R, Coppi G, Czerny M, Fraedrich G, Haulon S, Jacobs MJ, Lachat ML, Moll FL, Setacci C, Taylor PR, Thompson M, Trimarchi S, Verhagen HJ, Verhoeven EL, Esvs Guidelines Committee, Kolh P, de Borst GJ, Chakfé N, Debus ES, Hinchliffe RJ, Kakkos S, Koncar I, Lindholt JS, Vega de Ceniga M, Vermassen F, Verzini F, Document Reviewers, Kolh P, Black JH 3rd, Busund R, Björck M, Dake M, Dick F, Eggebrecht H, Evangelista A, Grabenwöger M, Milner R, Naylor AR, Ricco JB, Rousseau H, Schmidli J
Eur J Vasc Endovasc Surg 2017 Jan;53(1):4-52. doi: 10.1016/j.ejvs.2016.06.005. PMID: 28081802
Severe Arterial Tortuosity.
Cubero A, Ayala J, Hamzeh G, Cortes A, Udaondo J, Aramendi JI
World J Pediatr Congenit Heart Surg 2017 Mar;8(2):231-234. Epub 2016 Jun 23 doi: 10.1177/2150135116629396. PMID: 27095117
Aortic arch malformations.
Kellenberger CJ
Pediatr Radiol 2010 Jun;40(6):876-84. Epub 2010 Mar 31 doi: 10.1007/s00247-010-1607-9. PMID: 20354848

Therapy

Aortic disease in Marfan syndrome is caused by overactivation of sGC-PRKG signaling by NO.
de la Fuente-Alonso A, Toral M, Alfayate A, Ruiz-Rodríguez MJ, Bonzón-Kulichenko E, Teixido-Tura G, Martínez-Martínez S, Méndez-Olivares MJ, López-Maderuelo D, González-Valdés I, Garcia-Izquierdo E, Mingo S, Martín CE, Muiño-Mosquera L, De Backer J, Nistal JF, Forteza A, Evangelista A, Vázquez J, Campanero MR, Redondo JM
Nat Commun 2021 May 11;12(1):2628. doi: 10.1038/s41467-021-22933-3. PMID: 33976159Free PMC Article
The aberrant subclavian artery: approach to management.
Karangelis D, Loggos S, Tzifa A, Mitropoulos FA
Curr Opin Cardiol 2020 Nov;35(6):636-642. doi: 10.1097/HCO.0000000000000793. PMID: 32852349
Editor's Choice - Management of Descending Thoracic Aorta Diseases: Clinical Practice Guidelines of the European Society for Vascular Surgery (ESVS).
Riambau V, Böckler D, Brunkwall J, Cao P, Chiesa R, Coppi G, Czerny M, Fraedrich G, Haulon S, Jacobs MJ, Lachat ML, Moll FL, Setacci C, Taylor PR, Thompson M, Trimarchi S, Verhagen HJ, Verhoeven EL, Esvs Guidelines Committee, Kolh P, de Borst GJ, Chakfé N, Debus ES, Hinchliffe RJ, Kakkos S, Koncar I, Lindholt JS, Vega de Ceniga M, Vermassen F, Verzini F, Document Reviewers, Kolh P, Black JH 3rd, Busund R, Björck M, Dake M, Dick F, Eggebrecht H, Evangelista A, Grabenwöger M, Milner R, Naylor AR, Ricco JB, Rousseau H, Schmidli J
Eur J Vasc Endovasc Surg 2017 Jan;53(1):4-52. doi: 10.1016/j.ejvs.2016.06.005. PMID: 28081802
Post-stenotic aortic dilatation.
Wilton E, Jahangiri M
J Cardiothorac Surg 2006 Mar 3;1:7. doi: 10.1186/1749-8090-1-7. PMID: 16722611Free PMC Article
The "skinny aorta".
Israel G, Krinsky G, Lee V
Clin Imaging 2002 Mar-Apr;26(2):116-21. doi: 10.1016/s0899-7071(01)00368-0. PMID: 11852219

Prognosis

Outcomes of Repair of Kommerell Diverticulum.
Vinnakota A, Idrees JJ, Rosinski BF, Tucker NJ, Roselli EE, Pettersson GB, Vekstein AM, Stewart RD, Raja S, Svensson LG
Ann Thorac Surg 2019 Dec;108(6):1745-1750. Epub 2019 Jun 27 doi: 10.1016/j.athoracsur.2019.04.122. PMID: 31254511
Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification.
Li S, Wen H, Liang M, Luo D, Qin Y, Liao Y, Ouyang S, Bi J, Tian X, Norwitz ER, Luo G
Cardiovasc Pathol 2019 Mar-Apr;39:38-50. Epub 2018 Dec 6 doi: 10.1016/j.carpath.2018.11.004. PMID: 30623879
Introduction to anomalous aortic origin of a coronary artery.
Brothers JA
Congenit Heart Dis 2017 Sep;12(5):600-602. Epub 2017 Jun 27 doi: 10.1111/chd.12497. PMID: 28653465
Congenital Variants and Anomalies of the Aortic Arch.
Hanneman K, Newman B, Chan F
Radiographics 2017 Jan-Feb;37(1):32-51. Epub 2016 Nov 18 doi: 10.1148/rg.2017160033. PMID: 27860551
Imaging of congenital anomalies of the coronary arteries.
Raimondi F, Bonnet D
Diagn Interv Imaging 2016 May;97(5):561-9. Epub 2016 Apr 26 doi: 10.1016/j.diii.2016.03.009. PMID: 27132711

Clinical prediction guides

Growth of the Neo-Aortic Root and Prognosis of Transposition of the Great Arteries.
Sengupta A, Carreon CK, Gauvreau K, Lee JM, Sanders SP, Colan SD, Del Nido PJ, Mayer JE Jr, Nathan M
J Am Coll Cardiol 2024 Jan 30;83(4):516-527. Epub 2023 Nov 6 doi: 10.1016/j.jacc.2023.10.023. PMID: 37939977
Inherited Thoracic Aortic Disease: New Insights and Translational Targets.
Fletcher AJ, Syed MBJ, Aitman TJ, Newby DE, Walker NL
Circulation 2020 May 12;141(19):1570-1587. Epub 2020 May 11 doi: 10.1161/CIRCULATIONAHA.119.043756. PMID: 32392100Free PMC Article
Outcomes of Repair of Kommerell Diverticulum.
Vinnakota A, Idrees JJ, Rosinski BF, Tucker NJ, Roselli EE, Pettersson GB, Vekstein AM, Stewart RD, Raja S, Svensson LG
Ann Thorac Surg 2019 Dec;108(6):1745-1750. Epub 2019 Jun 27 doi: 10.1016/j.athoracsur.2019.04.122. PMID: 31254511
Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification.
Li S, Wen H, Liang M, Luo D, Qin Y, Liao Y, Ouyang S, Bi J, Tian X, Norwitz ER, Luo G
Cardiovasc Pathol 2019 Mar-Apr;39:38-50. Epub 2018 Dec 6 doi: 10.1016/j.carpath.2018.11.004. PMID: 30623879
Bicuspid aortic valve-associated aortopathy: update on biomarkers.
Maredia AK, Greenway SC, Verma S, Fedak PWM
Curr Opin Cardiol 2018 Mar;33(2):134-139. doi: 10.1097/HCO.0000000000000481. PMID: 29095713

Recent systematic reviews

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.
Gaudino M, Di Franco A, Arbustini E, Bacha E, Bates ER, Cameron DE, Cao D, David TE, De Paulis R, El-Hamamsy I, Farooqi KM, Girardi LN, Gräni C, Kochav JD, Molossi S, Puskas JD, Rao SV, Sandner S, Tatoulis J, Truong QA, Weinsaft JW, Zimpfer D, Mery CM
J Am Coll Cardiol 2023 Nov 21;82(21):2034-2053. Epub 2023 Oct 16 doi: 10.1016/j.jacc.2023.08.012. PMID: 37855757
Systematic Review and Meta-Analysis of Outcomes After Operative Treatment of Aberrant Subclavian Artery Pathologies and Suggested Reporting Items.
Konstantinou N, Antonopoulos CN, Tzanis K, Kölbel T, Peterß S, Pichlmaier M, Stana J, Tsilimparis N
Eur J Vasc Endovasc Surg 2022 May;63(5):759-767. Epub 2022 Feb 25 doi: 10.1016/j.ejvs.2022.02.027. PMID: 35459610
A systematic classification of the vertebral artery variable origin: clinical and surgical implications.
Lazaridis N, Piagkou M, Loukas M, Piperaki ET, Totlis T, Noussios G, Natsis K
Surg Radiol Anat 2018 Jul;40(7):779-797. Epub 2018 Feb 19 doi: 10.1007/s00276-018-1987-3. PMID: 29459992
A systematic review and meta-analysis of variations in branching patterns of the adult aortic arch.
Popieluszko P, Henry BM, Sanna B, Hsieh WC, Saganiak K, Pękala PA, Walocha JA, Tomaszewski KA
J Vasc Surg 2018 Jul;68(1):298-306.e10. Epub 2017 Aug 31 doi: 10.1016/j.jvs.2017.06.097. PMID: 28865978
Hypoplasia, pseudocoarctation and coarctation of the aorta - a systematic review.
Singh S, Hakim FA, Sharma A, Roy RR, Panse PM, Chandrasekaran K, Alegria JR, Mookadam F
Heart Lung Circ 2015 Feb;24(2):110-8. Epub 2014 Aug 23 doi: 10.1016/j.hlc.2014.08.006. PMID: 25442062

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