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Quinto arco aórtico persistente
ORPHA:99076CID-10 · Q25.4CID-11 · LA8B.2YDOENÇA RARA

A segunda temporada do drama médico americano Grey's Anatomy, começou a ser exibida nos Estados Unidos na American Broadcasting Company (ABC) em 25 de setembro de 2005, e terminou em 15 de maio de 2006. A temporada foi produzida pela Touchstone Television, em associação com a produtora Shondaland e The Mark Gordon Company, sendo Shonda Rhimes a showrunner do programa. Ellen Pompeo, Sandra Oh, Katherine Heigl, Justin Chambers e T.R. Knight reprisaram seus papéis como os internos de cirurgia Meredith Grey, Cristina Yang, Izzie Stevens, Alex Karev e George O'Malley, respectivamente. Os membros principais do elenco anterior, Chandra Wilson, James Pickens, Jr.

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Introdução

O que você precisa saber de cara

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Anomalia congênita rara onde o quinto arco aórtico embrionário não involui, podendo formar um vaso anômalo entre a aorta ascendente e a artéria pulmonar. Frequentemente assintomático, pode causar sintomas de compressão ou fluxo sanguíneo anormal.

Publicações científicas
93 artigos
Último publicado: 2025 Aug
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q25.4
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2025
Total histórico93PubMed
Últimos 10 anos39publicações
Pico20216 papers
Linha do tempo
2025Hoje · 2026📈 2021Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

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Publicações mais relevantes

Timeline de publicações
39 papers (10 anos)
#1

Case Report: A case of persistent fifth aortic arch associated with stenosis and interrupted aortic arch.

Frontiers in pediatrics2025

Persistent fifth aortic arch (PFAA) is a rare congenital cardiovascular anomaly, often associated with other defects such as interrupted aortic arch (IAA) or patent ductus arteriosus (PDA). We report a 12-day-old male neonate presenting with respiratory distress, edema, and oliguria. Physical examination revealed a grade 2/6 systolic murmur at the left sternal border. Echocardiography identified PFAA with coarctation, IAA, PDA, and an atrial septal defect (ASD), confirmed by cardiac CT. Surgical intervention included aortic arch repair, fifth arch excision, and ASD closure. The postoperative recovery was smooth, and the patient remained clinically well with normal cardiac function during the 1-year follow-up period. This case underscores the critical role of echocardiography in early diagnosis and surgical planning for complex congenital heart disease.

#2

Histological investigation of persistent fifth aortic arch with arch obstruction in a neonate.

Journal of cardiology cases2025 Aug

Persistent fifth aortic arch (PFAA) is a rare congenital heart disease caused by maldevelopment of the primitive aortic arch and can cause obstruction of the aortic arch. The histological structure of a fifth aortic arch differs from that of normal aortic arches. Therefore, it is unclear whether the tissue of a fifth aortic arch can be safely utilized to reconstruct the aortic arch during surgical repair of arch obstruction. We herein report the case of a neonate who underwent surgical repair of a PFAA with interrupted aortic arch, right aortic arch, and aberrant left subclavian artery. Similar to those for a ductus arteriosus, the histological findings of the resected PFAA in this case showed intimal thickening, myxoid deposition in the intima and media, and proliferation and migration of smooth muscle cells into the intima. When reconstructing the aortic arch, we suggest that, if possible, the fifth aortic arch tissue be resected. The histological findings of a persistent fifth aortic arch (PFAA) are important in determining the treatment strategy because the tissue may be unsuitable for reconstruction of the aortic arch. In the present case, the tissue of the PFAA was histologically similar to that of a ductus arteriosus and thus may have caused re-stenosis or formation of an aneurysm in the future. We therefore recommend resecting the PFAA tissue during aortic arch reconstruction.

#3

Persistent Fifth Aortic Arch in a Right-Sided Aortic Arch Associated With Tetralogy of Fallot.

JACC. Case reports2025 Oct 08

Persistent fifth aortic arch is a rare congenital cardiac anomaly. We report the imaging findings of a subtype of persistent fifth aortic arch in an infant with tetralogy of Fallot and a right-sided aortic arch.

#4

Persistent fifth aortic arch in a neonate with interrupted aortic arch: an unexpected intraoperative finding.

Interdisciplinary cardiovascular and thoracic surgery2025 Jun 04

Persistent fifth aortic arch (PFAA) is a rare variant of the aortic arch that may be associated with coarctation or interrupted aortic arch. We report the case of a neonate initially referred for coarctation repair. After a left thoracotomy was performed, a rare diagnosis of PFAA associated with interrupted aortic arch was made. Despite this unusual anatomy, the repair was successfully performed via a lateral approach. This unusual anatomy of the aortic arch deserves special consideration in case of association with coarctation. Indeed, repair from the side may not be possible due to the common origin of the neck-vessels, and resection should be extended as far as possible to eliminate remaining ductal tissue and prevent recoarctation.

#5

Persistent fifth aortic arch complicated by Williams-Beuren syndrome: a case description.

Quantitative imaging in medicine and surgery2025 Jul 01

Publicações recentes

Ver todas no PubMed

📚 EuropePMC81 artigos no totalmostrando 39

2025

Histological investigation of persistent fifth aortic arch with arch obstruction in a neonate.

Journal of cardiology cases
2025

Case Report: A case of persistent fifth aortic arch associated with stenosis and interrupted aortic arch.

Frontiers in pediatrics
2025

Persistent Fifth Aortic Arch in a Right-Sided Aortic Arch Associated With Tetralogy of Fallot.

JACC. Case reports
2025

Persistent fifth aortic arch complicated by Williams-Beuren syndrome: a case description.

Quantitative imaging in medicine and surgery
2025

Persistent fifth aortic arch in a neonate with interrupted aortic arch: an unexpected intraoperative finding.

Interdisciplinary cardiovascular and thoracic surgery
2024

Echocardiography of persistent fifth aortic arch confirmed by computed tomography angiography or surgery in children: a case series.

BMC cardiovascular disorders
2024

Confusion from prenatal diagnosis: Type C persistent fifth aortic arch or right-side ductus arteriosus.

Echocardiography (Mount Kisco, N.Y.)
2024

Cinematic Rendering of Persistent Fifth Aortic Arch with Aortic Coarctation and Bicuspid Aortic Valve.

The Indian journal of radiology & imaging
2023

Persistent fifth aortic arch: a comprehensive literature review.

Frontiers in pediatrics
2023

Common Arterial Trunk Coexisting With Double-Barreled Aorta.

JACC. Case reports
2022

Surgical repair of persistent fifth aortic arch coarctation and interrupted fourth aortic arch without cardiopulmonary bypass: a case report.

Translational pediatrics
2022

A new association of Oculoauriculovertebral spectrum and persistent fifth aortic arch -double lumen aorta: a case report.

BMC pediatrics
2022

A Case of Persistent Fifth Aortic Arch With an Interrupted Fourth Aortic Arch.

The Annals of thoracic surgery
2021

A double-barrelled aorta with high aortic Arch.

Journal of cardiology cases
2021

Stridor as a Harbinger of Congenital Cardiovascular Anomaly.

Acta Cardiologica Sinica
2021

Persistent fifth aortic arch associated with aortic coarctation: a case of surgical correction without artificial material.

Journal of cardiothoracic surgery
2020

Neonatal repair of persistent fifth aortic arch and aortic coarctation.

JTCVS techniques
2021

Persistent fifth aortic arch: a single-center experience, case series.

Translational pediatrics
2021

Is It Indeed the Persistent Fifth Aortic Arch?

The Annals of thoracic surgery
2021

About a Definition of the Persistent Fifth Aortic Arch.

The Annals of thoracic surgery
2020

Anomalous Left-to-Right Shunting Vessel between the Ascending Aorta and Right Pulmonary Artery and Concurrent Left Peripheral Pulmonary Artery Stenosis in a Dog.

CASE (Philadelphia, Pa.)
2020

Persistent Fifth Aortic Arch With Left Ventricular Dysfunction and Left Bronchial Obstruction.

The Annals of thoracic surgery
2020

Assessment of persistent fifth aortic arch by echocardiography and computed tomography angiography.

Medicine
2019

Persistent fifth aortic arch stenosis associated with type A interruption of the aortic arch: a report of six cases.

Chinese medical journal
2019

"Double-lumen" aortic arch with "double-lumen" brachiocephalic artery.

Annals of pediatric cardiology
2019

True bovine aortic arch combined with coarctation of persistent fifth aortic arch in a child.

European heart journal. Cardiovascular Imaging
2020

Type 2 persistent fifth aortic arch: An elusive entity diagnosed on computed tomography angiography.

Journal of cardiovascular computed tomography
2018

Persistent fifth aortic arch: the "great pretender" in clinical practice.

Cardiology in the young
2017

Neonatal Repair of Persistent Fifth Aortic Arch Coarctation and Interrupted Fourth Aortic Arch.

The Annals of thoracic surgery
2017

Aortic arch advancement for type A interrupted aortic arch with persistent fifth aortic arch type B.

Cardiology in the young
2016

CT imaging of a rare case of persistent fifth aortic arch in newborn.

BJR case reports
2016

Double-Lumen Aortic Arch: Persistence of the Fifth Aortic Arch.

The Annals of thoracic surgery
2016

Persistent Fifth Aortic Arch with Coarctation.

The Korean journal of thoracic and cardiovascular surgery
2016

Coarctation of Persistent Fifth Aortic Arch With Interrupted Fourth Arch: First Pediatric Report of Stent Intervention.

Revista espanola de cardiologia (English ed.)
2016

Iatrogenic aortic dissection in an infant with persistent fifth aortic arch and congenital heart defects.

The Journal of thoracic and cardiovascular surgery
2015

Persistent Fifth Aortic Arch Confirmed by Computed Tomography Angiography.

World journal for pediatric & congenital heart surgery
2015

Aortic Arch Interruption and Persistent Fifth Aortic Arch in Phace Syndrome: Prenatal Diagnosis and Postnatal Course.

Echocardiography (Mount Kisco, N.Y.)
2015

Interruption of the fourth aortic arch and a persistent fifth aortic arch with coarctation: a rare morphological finding.

Kardiologia polska
2015

[Persistent fifth aortic arch with patent ductus arteriosus].

Archivos de cardiologia de Mexico
Ver todos os 81 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Case Report: A case of persistent fifth aortic arch associated with stenosis and interrupted aortic arch.
    Frontiers in pediatrics· 2025· PMID 41395307mais citado
  2. Histological investigation of persistent fifth aortic arch with arch obstruction in a neonate.
    Journal of cardiology cases· 2025· PMID 41635833mais citado
  3. Persistent Fifth Aortic Arch in a Right-Sided Aortic Arch Associated With Tetralogy of Fallot.
    JACC. Case reports· 2025· PMID 40920148mais citado
  4. Persistent fifth aortic arch in a neonate with interrupted aortic arch: an unexpected intraoperative finding.
    Interdisciplinary cardiovascular and thoracic surgery· 2025· PMID 40574475mais citado
  5. Persistent fifth aortic arch complicated by Williams-Beuren syndrome: a case description.
    Quantitative imaging in medicine and surgery· 2025· PMID 40727346mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:99076(Orphanet)
  2. MONDO:0020414(MONDO)
  3. GARD:19636(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55789350(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

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Quinto arco aórtico persistente
Compêndio · Raras BR

Quinto arco aórtico persistente

ORPHA:99076 · MONDO:0020414
CID-10
Q25.4 · Outras malformações congênitas da aorta
CID-11
MedGen
UMLS
C0345066
EuropePMC
Wikidata
Papers 10a
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