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Pseudocoarctation of the aorta

MedGen UID:
138015
Concept ID:
C0345088
Congenital Abnormality
Synonyms: Kinking of descending aorta; Pseudo-coarctation; Pseudocoarctation of aorta; Pseudocoarctation of aorta, congenital
SNOMED CT: Pseudocoarctation of aorta (70602002); Pseudo-coarctation (70602002); Kinking of descending aorta (70602002); Pseudocoarctation of aorta, congenital (70602002)
 
HPO: HP:0005295

Definition

Pseudocoarctation is a congenital anomaly of kinking, or buckling, of the aorta without a pressure gradient across the lesion. It is characterized by elongation and kinking of the aorta at the level of the ligamentum arteriosum. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPseudocoarctation of the aorta

Conditions with this feature

Patent ductus arteriosus-bicuspid aortic valve-hand anomalies syndrome
MedGen UID:
346902
Concept ID:
C1858420
Disease or Syndrome
Patent ductus arteriosus - bicuspid aortic valve - hand anomalies syndrome is a very rare heart-hand syndrome (see this term) that is characterized by a variety of cardiovascular anomalies including patent arterial duct, bicuspid aortic valve and pseudocoarctation of the aorta in conjunction with hand anomalies such as brachydactyly and ulnar ray derivative i.e. fifth metacarpal hypoplasia. Transmission is most likely autosomal dominant.

Professional guidelines

PubMed

Cheng TO
Am J Med 1970 Oct;49(4):551-5. doi: 10.1016/s0002-9343(70)80050-x. PMID: 5477640

Recent clinical studies

Etiology

Kouchoukos NT, Scharff JR, Castner CF
J Thorac Cardiovasc Surg 2015 Feb;149(2 Suppl):S83-5. Epub 2014 Aug 4 doi: 10.1016/j.jtcvs.2014.07.071. PMID: 25212057
Rabin J, DuBose J, Sliker CW, O'Connor JV, Scalea TM, Griffith BP
J Thorac Cardiovasc Surg 2014 Jan;147(1):143-9. doi: 10.1016/j.jtcvs.2013.08.053. PMID: 24331909
Atalay MK, Kochilas LK
Pediatr Cardiol 2011 Jun;32(5):692-5. Epub 2011 Feb 25 doi: 10.1007/s00246-011-9933-8. PMID: 21347833
Takach TJ, Anstadt MP, Moore HV
Tex Heart Inst J 2005;32(1):16-20. PMID: 15902816Free PMC Article
Argotte AF, Giron F, Bilfinger TV
J Cardiovasc Surg (Torino) 1998 Dec;39(6):747-50. PMID: 9972892

Diagnosis

Mahadevappa M, Kulkarni P, Attri L, Basavaraj N
Curr Cardiol Rev 2023;19(5):73-82. doi: 10.2174/1573403X19666230329135028. PMID: 36999416Free PMC Article
Arıkan AA, Omay O, Çakır Ö, Cesur S, Çardaközü T, Durmaz A, Küçük B, Kanko M
Vasc Endovascular Surg 2021 Nov;55(8):889-896. Epub 2021 Jun 18 doi: 10.1177/15385744211022536. PMID: 34142624
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
Atalay MK, Kochilas LK
Pediatr Cardiol 2011 Jun;32(5):692-5. Epub 2011 Feb 25 doi: 10.1007/s00246-011-9933-8. PMID: 21347833
Adaletli I, Kurugoglu S, Davutoglu V, Ozer H, Besirli K, Sayin AG
Can J Cardiol 2007 Jun;23(8):675-6. doi: 10.1016/s0828-282x(07)70232-5. PMID: 17593995Free PMC Article

Therapy

Kouchoukos NT, Scharff JR, Castner CF
J Thorac Cardiovasc Surg 2015 Feb;149(2 Suppl):S83-5. Epub 2014 Aug 4 doi: 10.1016/j.jtcvs.2014.07.071. PMID: 25212057
Rabin J, DuBose J, Sliker CW, O'Connor JV, Scalea TM, Griffith BP
J Thorac Cardiovasc Surg 2014 Jan;147(1):143-9. doi: 10.1016/j.jtcvs.2013.08.053. PMID: 24331909
Lioupis C, MacKenzie KS, Corriveau MM, Obrand DI, Abraham CZ, Steinmetz OK
Vasc Endovascular Surg 2012 Feb;46(2):109-16. Epub 2012 Feb 5 doi: 10.1177/1538574411431648. PMID: 22308206
Tanju S, Ustuner E, Erden I, Aytac SK
Cardiovasc Intervent Radiol 2007 Jan-Feb;30(1):146-9. doi: 10.1007/s00270-005-0316-5. PMID: 16802075
Gysi J, Schaffner T, Mohacsi P, Aeschbacher B, Althaus U, Carrel T
Eur J Cardiothorac Surg 1997 Jun;11(6):1163-9; discussion 1169-70. doi: 10.1016/s1010-7940(97)00091-2. PMID: 9237604

Prognosis

Sekelyk R, Abdurakhmanov Z, Kurkevych A, Tammo R, Mykychak Y, Yemets I
World J Pediatr Congenit Heart Surg 2021 May;12(3):436-438. Epub 2019 May 20 doi: 10.1177/2150135118825266. PMID: 31109242
Atalay MK, Kochilas LK
Pediatr Cardiol 2011 Jun;32(5):692-5. Epub 2011 Feb 25 doi: 10.1007/s00246-011-9933-8. PMID: 21347833
Takach TJ, Anstadt MP, Moore HV
Tex Heart Inst J 2005;32(1):16-20. PMID: 15902816Free PMC Article
Morocutti G, Minen G, Vendrametto F, Miani D, Spedicato L, Bernardi G
Minerva Cardioangiol 1999 Sep;47(9):301-7. PMID: 10630068
Argotte AF, Giron F, Bilfinger TV
J Cardiovasc Surg (Torino) 1998 Dec;39(6):747-50. PMID: 9972892

Clinical prediction guides

Minici R, Serra R, Ierardi AM, Petullà M, Bracale UM, Carrafiello G, Laganà D
Vascular 2024 Feb;32(1):5-18. Epub 2022 Sep 19 doi: 10.1177/17085381221127740. PMID: 36121832
Mecklai A, Rosenzweig B, Applebaum R, Axel L, Grossi E, Chan A, Saric M
Tex Heart Inst J 2014 Aug;41(4):433-6. Epub 2014 Aug 1 doi: 10.14503/THIJ-13-3448. PMID: 25120401Free PMC Article
Rabin J, DuBose J, Sliker CW, O'Connor JV, Scalea TM, Griffith BP
J Thorac Cardiovasc Surg 2014 Jan;147(1):143-9. doi: 10.1016/j.jtcvs.2013.08.053. PMID: 24331909
Kuhlman JE, Pozniak MA, Collins J, Knisely BL
Radiographics 1998 Sep-Oct;18(5):1085-106; discussion 1107-8; quiz 1. doi: 10.1148/radiographics.18.5.9747609. PMID: 9747609
Mirowitz SA, Lee JK, Gutierrez FR, Brown JJ, Eilenberg SS
J Comput Assist Tomogr 1990 Sep-Oct;14(5):753-5. PMID: 2398154

Recent systematic reviews

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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