Persistent fifth aortic arch

A rare, congenital anomaly of the great arteries characterized by an extrapericardial vessel arising from the ascending aorta proximal to the brachiocephalic artery and terminating either in the dorsal aorta or in pulmonary arteries via a persistently patent arterial duct. The resulting connection is a systemic-to-systemic or systemic-to-pulmonary. Clinical manifestation include exercise intolerance, reduced femoral pulses, cyanosis with or without pulmonary hypertension and heart failure. Other congenital cardiovascular anomalies are often present and influence the clinical presentation.



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Total: 19 (papers)

  


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)
PMID
(PMCID)
1
(4.0%)
30363644
(6180890)
CT imaging of a rare case of persistent fifth aortic arch in newborn.
Schicchi N, Agliata G, Giovagnoni A.
BJR Case Rep. 2016;2(2):20150048.
Left-to-right shunt
1
(4.0%)
28260541
Aortic arch advancement for type A interrupted aortic arch with persistent fifth aortic arch type B.
Binsalamah ZM, Chen P, McKenzie ED.
Cardiol Young. 2017;27(5):1018-1021.
Interrupted aortic arch
Echocardiography Females Homo sapiens Infant, Newborn Thoracotomy X-Ray Computed Tomography
1
(4.0%)
27106464
Double-Lumen Aortic Arch: Persistence of the Fifth Aortic Arch.
Naimo PS, Vazquez-Alvarez Mdel C, d'Udekem Y, Jones B, Konstantinov IE.
Ann Thorac Surg. 2016;101(5):e155-6.
Perimembranous ventricular septal defect
Cornelia De Lange Syndrome Females Homo sapiens Infant Ventricular Septal Defects
1
(4.0%)
25809619
Aortic Arch Interruption and Persistent Fifth Aortic Arch in Phace Syndrome: Prenatal Diagnosis and Postnatal Course.
Chiappa E, Greco A, Fainardi V, Passantino S, Serranti D, Favilli S.
Echocardiography. 2015;32(9):1441-3.
Hemangioma
Congenital Heart Defects Differential Diagnosis Females Hemangioma Homo sapiens Infant Infant, Newborn Male Pregnancy Syndrome Ultrasonography, Prenatal
1
(4.0%)
25576260
[Persistent fifth aortic arch with patent ductus arteriosus].
Tamayo-Espinosa T, Erdmenger-Orellana J, Becerra-Becerra R, Balderrabano-Saucedo N, Arevalo-Salas LA.
Arch Cardiol Mex. 2015;85(2):161-3.
Patent ductus arteriosus
Child Females Homo sapiens
1
(4.0%)
25195807
Persistent fifth aortic arch with interrupted aortic arch.
Tang X, Wang L, Wu Q, Tong X.
J Card Surg. 2015;30(3):284-7.
Interrupted aortic arch
Aorta Cardiovascular Abnormalities Echocardiography, Doppler, Color Homo sapiens Infant Male Multidetector Computed Tomography
1
(4.0%)
24463916
A case report of persistent fifth aortic arch presenting with severe left ventricular dysfunction.
Nakashima K, Oka N, Hayashi H, Shibata M, Kitamura T, Itatani K, Miyaji K.
Int Heart J. 2014;55(1):87-8.
Hypertension
Females Homo sapiens Infant Severity of Illness Index Ventricular Dysfunction, Left
1
(4.0%)
23960580
Persistent fifth aortic arch diagnosed by echocardiography and confirmed by angiography: Case report and literature review.
Al Akhfash AA, Al Mutairi MB, Al Habshan FM.
J Saudi Heart Assoc. 2009;21(4):245-8.
Left-to-right shunt
1
(4.0%)
23774614
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.
Aortic arch aneurysm
Aortic Aneurysm, Thoracic Aortography Blood Vessel Prosthesis Implantation Females Homo sapiens Middle Aged Vascular Surgical Procedures X-Ray Computed Tomography
1
(4.0%)
23129912
(3487211)
A rare association with patent ductus arteriosus.
Warrier D, Shah S, John C, Dayananda L.
Ann Pediatr Cardiol. 2012;5(2):191-3.
Patent ductus arteriosus
        

Phenotype(s) retrieved from Orphanet

    Total: 0

HPO ID Term Frequency


Phenotype(s) retrieved from case reports

    Total: 8

HPO ID Term # of case reports
HP:0011611 Interrupted aortic arch 3
HP:0000822 Hypertension 1
HP:0001643 Patent ductus arteriosus 1
HP:0001680 Coarctation of aorta 1
HP:0005162 Left ventricular dysfunction 1
HP:0010775 Vascular ring 1
HP:0030148 Heart murmur 1
HP:0031273 Shock 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID