U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

Valve-sparing aortic root replacement in a patient with retroaortic course of the left circumflex artery.
Akiel R, Guihaire J, Isorni MA, Deleuze P
J Card Surg 2020 Oct;35(10):2817-2820. Epub 2020 Aug 2 doi: 10.1111/jocs.14740. PMID: 32740986
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
Single-Stage Hybrid Repair of Right Aortic Arch With Kommerell's Diverticulum.
Rosu C, Dorval JF, Abraham CZ, Cartier R, Demers P
Ann Thorac Surg 2017 Apr;103(4):e381-e384. doi: 10.1016/j.athoracsur.2016.10.065. PMID: 28359506
Should aortas in patients with bicuspid aortic valve really be resected at an earlier stage than tricuspid? PRO.
Wald O, Korach A, Shapira OM
Cardiol Clin 2010 May;28(2):289-98. doi: 10.1016/j.ccl.2010.01.005. PMID: 20452544
The hemi-Fontan operation.
Jacobs ML, Pourmoghadam KK
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003;6:90-7. doi: 10.1053/pcsu.2003.50007. PMID: 12740775

Diagnosis

Unusual association of isolation of right pulmonary artery in absent pulmonary valve syndrome.
Sivakumar K, Mohakud AR, Agarwal R
Asian Cardiovasc Thorac Ann 2022 Mar;30(3):335-338. Epub 2021 Mar 31 doi: 10.1177/02184923211006309. PMID: 33789442
Reconstruction of the Mediastinum and Tracheopexy for Tracheomalacia in Straight Back Syndrome.
Schmid S, Schibilsky D, Kalbhenn J, Hassan M, Loop T, Passlick B, Beyersdorf F, Czerny M
Ann Thorac Surg 2021 Jul;112(1):e41-e44. Epub 2021 Jan 6 doi: 10.1016/j.athoracsur.2020.11.045. PMID: 33421398
Atretic anomalous left subclavian artery as part of an unusual vascular ring.
Powell SK, Patel JK, Ebenroth ES
Cardiol Young 2019 Jan;29(1):85-87. Epub 2018 Oct 26 doi: 10.1017/S1047951118001737. PMID: 30360770
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
Should aortas in patients with bicuspid aortic valve really be resected at an earlier stage than tricuspid? PRO.
Wald O, Korach A, Shapira OM
Cardiol Clin 2010 May;28(2):289-98. doi: 10.1016/j.ccl.2010.01.005. PMID: 20452544

Therapy

Open Tacking of a Migrated Thoracic Endovascular Aortic Graft.
Trahanas JM, Kwolek CJ, Tolis G
Ann Vasc Surg 2020 Feb;63:461.e7-461.e9. Epub 2019 Oct 17 doi: 10.1016/j.avsg.2019.09.023. PMID: 31629854
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
Aortopulmonary fistula after a modified Bentall procedure.
Ferrari G, Anastasio G, Bianchi M, Scioti G, Guarracino F, Bortolotti U
J Heart Valve Dis 2012 Jul;21(4):505-8. PMID: 22953679
Unsuccessful treatment of a collapsed thoracic stent graft by Palmaz stent.
Pirotte M, Lacroix V, Astarci P, Nardella J, Funken JC, El Khoury G, Noirhomme P, Verhelst R
Ann Vasc Surg 2010 Nov;24(8):1137.e13-9. doi: 10.1016/j.avsg.2010.06.012. PMID: 21035713
Surgical results of anomalous origin of the right pulmonary artery from the ascending aorta including reoperation for infrequent complications.
Kajihara N, Imoto Y, Sakamoto M, Ochiai Y, Kan-o M, Joo K, Watanabe M, Yuge T, Asou T, Takeda Y, Sese A
Ann Thorac Surg 2008 Apr;85(4):1407-11. doi: 10.1016/j.athoracsur.2007.11.081. PMID: 18355536

Prognosis

Endovascular Repair of an Intrathoracic Subclavian Artery Aneurysm in a Patient With Dextrocardia.
Motaganahalli AR, Wang SK, Sawchuk AP
Vasc Endovascular Surg 2023 Jan;57(1):69-74. Epub 2022 Jun 8 doi: 10.1177/15385744221108045. PMID: 35675973
Bilateral vertebral arteries arising distal to the left subclavian artery: embryological and anatomical description.
Martín-González I, Díez-Solórzano ML, Blasco-Serra A, Valverde-Navarro AA, Colás-López A, Linuesa-Mendoza M, Cortés-Aucejo JJ, Ekuaga-Eyang MM, Jara-Jaquez A, Cortés-Martínez MC
Surg Radiol Anat 2022 Apr;44(4):531-534. Epub 2022 Mar 9 doi: 10.1007/s00276-022-02903-0. PMID: 35266028
Valve-sparing aortic root replacement in a patient with retroaortic course of the left circumflex artery.
Akiel R, Guihaire J, Isorni MA, Deleuze P
J Card Surg 2020 Oct;35(10):2817-2820. Epub 2020 Aug 2 doi: 10.1111/jocs.14740. PMID: 32740986
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
The hemi-Fontan operation.
Jacobs ML, Pourmoghadam KK
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003;6:90-7. doi: 10.1053/pcsu.2003.50007. PMID: 12740775

Clinical prediction guides

Thyroidea Ima Artery in Cadavers of a Medical College in Nepal: A Descriptive Cross-sectional Study.
Jha M, Dhungel S, Jha AK, Yadav SK
JNMA J Nepal Med Assoc 2024 Mar 31;62(272):261-263. doi: 10.31729/jnma.8540. PMID: 39356843Free PMC Article
Dual esophageal compression secondary to two vascular abnormalities.
Sánchez Fuentes PA, Ruiz Pardo J, Vidaña Márquez E, Belda Lozano R
Rev Esp Enferm Dig 2022 Oct;114(10):619-620. doi: 10.17235/reed.2022.8567/2022. PMID: 35142531
Valve-sparing aortic root replacement in a patient with retroaortic course of the left circumflex artery.
Akiel R, Guihaire J, Isorni MA, Deleuze P
J Card Surg 2020 Oct;35(10):2817-2820. Epub 2020 Aug 2 doi: 10.1111/jocs.14740. PMID: 32740986
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
A Tubular Aortopulmonary Window: An Embryological Curiosity.
Chidambarathanu S, Agarwal R, Hussain ZM, Brown NA, Anderson RH
World J Pediatr Congenit Heart Surg 2016 May;7(3):411-3. Epub 2016 Jan 21 doi: 10.1177/2150135115596586. PMID: 26795903

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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On
    • Proximal aortic arch hypoplasia
      Proximal aortic arch hypoplasia
      MedGen
    • Significant Arterial Dissection
      Significant Arterial Dissection
      MedGen
    • Sialic Acid Storage Disease
      Sialic Acid Storage Disease
      Autosomal recessive neurodegenerative disorders caused by lysosomal membrane transport defects that result in accumulation of free sialic acid (N-ACETYLNEURAMINIC ACID) within...<br/>Year introduced: 2002
      MeSH
    • Glycogen Storage Disease Type II
      Glycogen Storage Disease Type II
      An autosomal recessively inherited glycogen storage disease caused by GLUCAN 1,4-ALPHA-GLUCOSIDASE deficiency. Large amounts of GLYCOGEN accumulate in the LYSOSOMES of skeleta...<br/>Year introduced: 1989(1975)
      MeSH

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...