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Proximal aortic arch hypoplasia

MedGen UID:
1814378
•Concept ID:
C5676814
•
Congenital Abnormality
HPO: HP:0034228

Definition

A type of aortic arch hypoplasia whereby the proximal aortic arch (arch proximal to the origin of the left subclavian artery) has a diameter of less than 60% of the diameter of the ascending aorta. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProximal aortic arch hypoplasia

Professional guidelines

PubMed

Study on the views and methods of ultrasonic screening and diagnosis for abnormal aortic arch in infants.
He X, Chen J, Li G
Cardiovasc Ultrasound 2021 Jan 14;19(1):8. doi: 10.1186/s12947-021-00237-2. PMID: 33446185Free PMC Article
Diagnosis and treatment of proximal carotid lesions.
Clair DG, Greenberg RK
Semin Vasc Surg 2000 Jun;13(2):103-8. PMID: 10879550
Echocardiographic study of the morphology and growth of the aortic arch in the human fetus. Observations related to the prenatal diagnosis of coarctation.
Hornberger LK, Weintraub RG, Pesonen E, Murillo-Olivas A, Simpson IA, Sahn C, Hagen-Ansert S, Sahn DJ
Circulation 1992 Sep;86(3):741-7. doi: 10.1161/01.cir.86.3.741. PMID: 1516185

Recent clinical studies

Etiology

Growth of unrepaired hypoplastic proximal aortic arch and reintervention rate after aortic coarctation repair.
Huuskonen A, Hui L, Runeckles K, Hui W, Barron DJ, Friedberg MK, Honjo O
J Thorac Cardiovasc Surg 2023 May;165(5):1631-1640.e1. Epub 2022 Sep 8 doi: 10.1016/j.jtcvs.2022.08.030. PMID: 36202666
Regional Vascular Changes and Aortic Dilatation in Pediatric Patients With Bicuspid Aortic Valve.
Rumman RK, Slorach C, Hui W, Lopez C, Larios G, Fan S, Franco-Cereceda A, Loeys B, Mohamed SA, Dietz H, Mital S, Andelfinger G, Mertens L, Grattan M; Mechanistic Interrogation of Bicuspid Aortic Valve-associated Aortopathy (MIBAVA) Leducq Consortium
Can J Cardiol 2022 May;38(5):688-694. Epub 2022 Jan 29 doi: 10.1016/j.cjca.2022.01.020. PMID: 35093466
Morphometry of the aortic arch and its branches. A computed tomography angiography-based study.
Tapia-Nañez M, Landeros-Garcia GA, Sada-Treviño MA, Pinales-Razo R, Quiroga-Garza A, Fernandez-Rodarte BA, Elizondo-Omaña RE, Guzman-Lopez S
Folia Morphol (Warsz) 2021;80(3):575-582. Epub 2020 Aug 26 doi: 10.5603/FM.a2020.0098. PMID: 32844389
Surgical management of the aorta in BAV patients.
Etz CD, Haunschild J, Girdauskas E, Della Corte A, Fedak PWM, Schäfers HJ, Sundt TM, Borger MA
Prog Cardiovasc Dis 2020 Jul-Aug;63(4):475-481. Epub 2020 Jul 5 doi: 10.1016/j.pcad.2020.06.013. PMID: 32640281
Multimodality imaging in bicuspid aortic valve.
Galian-Gay L, Rodríguez-Palomares J, Guala A, Michelena HI, Evangelista A
Prog Cardiovasc Dis 2020 Jul-Aug;63(4):442-451. Epub 2020 Jun 9 doi: 10.1016/j.pcad.2020.06.003. PMID: 32531300

Diagnosis

Regional Vascular Changes and Aortic Dilatation in Pediatric Patients With Bicuspid Aortic Valve.
Rumman RK, Slorach C, Hui W, Lopez C, Larios G, Fan S, Franco-Cereceda A, Loeys B, Mohamed SA, Dietz H, Mital S, Andelfinger G, Mertens L, Grattan M; Mechanistic Interrogation of Bicuspid Aortic Valve-associated Aortopathy (MIBAVA) Leducq Consortium
Can J Cardiol 2022 May;38(5):688-694. Epub 2022 Jan 29 doi: 10.1016/j.cjca.2022.01.020. PMID: 35093466
Imaging spectrum of extracranial arterial vascular pathology: pearls for the radiologist.
Culleton S, Wiggins R, McNally JS
Clin Radiol 2022 Mar;77(3):167-178. Epub 2021 Nov 17 doi: 10.1016/j.crad.2021.10.011. PMID: 34799048
Surgical management of the aorta in BAV patients.
Etz CD, Haunschild J, Girdauskas E, Della Corte A, Fedak PWM, Schäfers HJ, Sundt TM, Borger MA
Prog Cardiovasc Dis 2020 Jul-Aug;63(4):475-481. Epub 2020 Jul 5 doi: 10.1016/j.pcad.2020.06.013. PMID: 32640281
Multimodality imaging in bicuspid aortic valve.
Galian-Gay L, Rodríguez-Palomares J, Guala A, Michelena HI, Evangelista A
Prog Cardiovasc Dis 2020 Jul-Aug;63(4):442-451. Epub 2020 Jun 9 doi: 10.1016/j.pcad.2020.06.003. PMID: 32531300
Persistent fifth aortic arch: the "great pretender" in clinical practice.
Lloyd DFA, Ho SY, Pushparajah K, Chakraborty S, Nasser M, Uemura H, Franklin R, Magee AG
Cardiol Young 2018 Feb;28(2):175-181. Epub 2017 Aug 30 doi: 10.1017/S1047951117001342. PMID: 28851468

Therapy

Technical Aspects of Branched Thoracic Arch Graft Implantation for Aortic Arch Pathologies.
Spanos K, Panuccio G, Rohlffs F, Heidemann F, Tsilimparis N, Kölbel T
J Endovasc Ther 2020 Oct;27(5):792-800. Epub 2020 May 20 doi: 10.1177/1526602820925443. PMID: 32431247
Total arch replacement and frozen elephant trunk for aortic dissection in aberrant right subclavian artery.
Li JR, Ma WG, Chen Y, Zhu JM, Zheng J, Xu SD, Liu YM, Sun LZ
Eur J Cardiothorac Surg 2020 Jul 1;58(1):104-111. doi: 10.1093/ejcts/ezaa029. PMID: 32057078
Arch Augmentation via Median Sternotomy for Coarctation of Aorta With Proximal Arch Hypoplasia.
Gray WH, Wells WJ, Starnes VA, Kumar SR
Ann Thorac Surg 2018 Oct;106(4):1214-1219. Epub 2018 May 16 doi: 10.1016/j.athoracsur.2018.04.025. PMID: 29753817
Hybrid aortic arch and frozen elephant trunk reconstruction: bridging the gap between conventional and total endovascular arch repair.
Bozso SJ, White A, Nagendran J, Moon MC, Chu MWA
Expert Rev Cardiovasc Ther 2018 Mar;16(3):209-217. Epub 2018 Jan 29 doi: 10.1080/14779072.2018.1429913. PMID: 29343137
Distal aortic arch aneurysm associated with persistent fifth aortic arch.
Inoue T, Morita K, Tanaka K, Yoshitake M, Naruse H, Nakao M, Hashimoto K
Ann Thorac Cardiovasc Surg 2014;20 Suppl:878-81. Epub 2013 Jun 18 doi: 10.5761/atcs.cr.13-00013. PMID: 23774614

Prognosis

Regional Vascular Changes and Aortic Dilatation in Pediatric Patients With Bicuspid Aortic Valve.
Rumman RK, Slorach C, Hui W, Lopez C, Larios G, Fan S, Franco-Cereceda A, Loeys B, Mohamed SA, Dietz H, Mital S, Andelfinger G, Mertens L, Grattan M; Mechanistic Interrogation of Bicuspid Aortic Valve-associated Aortopathy (MIBAVA) Leducq Consortium
Can J Cardiol 2022 May;38(5):688-694. Epub 2022 Jan 29 doi: 10.1016/j.cjca.2022.01.020. PMID: 35093466
Multimodality imaging in bicuspid aortic valve.
Galian-Gay L, Rodríguez-Palomares J, Guala A, Michelena HI, Evangelista A
Prog Cardiovasc Dis 2020 Jul-Aug;63(4):442-451. Epub 2020 Jun 9 doi: 10.1016/j.pcad.2020.06.003. PMID: 32531300
Shear Stress and Aortic Strain Associations With Biomarkers of Ascending Thoracic Aortic Aneurysm.
Pasta S, Agnese V, Gallo A, Cosentino F, Di Giuseppe M, Gentile G, Raffa GM, Maalouf JF, Michelena HI, Bellavia D, Conaldi PG, Pilato M
Ann Thorac Surg 2020 Nov;110(5):1595-1604. Epub 2020 Apr 11 doi: 10.1016/j.athoracsur.2020.03.017. PMID: 32289298
The Value of Circulating Biomarkers in Bicuspid Aortic Valve-Associated Aortopathy.
Naito S, Hillebrand M, Bernhardt AMJ, Jagodzinski A, Conradi L, Detter C, Sydow K, Reichenspurner H, Kodolitsch YV, Girdauskas E
Thorac Cardiovasc Surg 2018 Jun;66(4):278-286. Epub 2016 May 5 doi: 10.1055/s-0036-1583525. PMID: 27148933
Ascending Aortic Slide for Interrupted Aortic Arch Repair.
Urencio M, Dodge-Khatami A, Greenleaf CE, Aru G, Salazar JD
World J Pediatr Congenit Heart Surg 2016 Sep;7(5):645-8. doi: 10.1177/2150135116655124. PMID: 27587504

Clinical prediction guides

Growth of unrepaired hypoplastic proximal aortic arch and reintervention rate after aortic coarctation repair.
Huuskonen A, Hui L, Runeckles K, Hui W, Barron DJ, Friedberg MK, Honjo O
J Thorac Cardiovasc Surg 2023 May;165(5):1631-1640.e1. Epub 2022 Sep 8 doi: 10.1016/j.jtcvs.2022.08.030. PMID: 36202666
Regional Vascular Changes and Aortic Dilatation in Pediatric Patients With Bicuspid Aortic Valve.
Rumman RK, Slorach C, Hui W, Lopez C, Larios G, Fan S, Franco-Cereceda A, Loeys B, Mohamed SA, Dietz H, Mital S, Andelfinger G, Mertens L, Grattan M; Mechanistic Interrogation of Bicuspid Aortic Valve-associated Aortopathy (MIBAVA) Leducq Consortium
Can J Cardiol 2022 May;38(5):688-694. Epub 2022 Jan 29 doi: 10.1016/j.cjca.2022.01.020. PMID: 35093466
Morphometry of the aortic arch and its branches. A computed tomography angiography-based study.
Tapia-Nañez M, Landeros-Garcia GA, Sada-Treviño MA, Pinales-Razo R, Quiroga-Garza A, Fernandez-Rodarte BA, Elizondo-Omaña RE, Guzman-Lopez S
Folia Morphol (Warsz) 2021;80(3):575-582. Epub 2020 Aug 26 doi: 10.5603/FM.a2020.0098. PMID: 32844389
Multimodality imaging in bicuspid aortic valve.
Galian-Gay L, Rodríguez-Palomares J, Guala A, Michelena HI, Evangelista A
Prog Cardiovasc Dis 2020 Jul-Aug;63(4):442-451. Epub 2020 Jun 9 doi: 10.1016/j.pcad.2020.06.003. PMID: 32531300
The Value of Circulating Biomarkers in Bicuspid Aortic Valve-Associated Aortopathy.
Naito S, Hillebrand M, Bernhardt AMJ, Jagodzinski A, Conradi L, Detter C, Sydow K, Reichenspurner H, Kodolitsch YV, Girdauskas E
Thorac Cardiovasc Surg 2018 Jun;66(4):278-286. Epub 2016 May 5 doi: 10.1055/s-0036-1583525. PMID: 27148933

Recent systematic reviews

Aortic aneurysms after correction of aortic coarctation: a systematic review.
von Kodolitsch Y, Aydin AM, Bernhardt AM, Habermann C, Treede H, Reichenspurner H, Meinertz T, Dodge-Khatami A
Vasa 2010 Feb;39(1):3-16. doi: 10.1024/0301-1526/a000001. PMID: 20186672

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