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.
Introdução
O que você precisa saber de cara
Visão geral
O quinto arco aórtico persistente é uma condição vascular rara identificada pelos códigos MONDO:0020414 e CID-10 Q25.4. No momento, não há informações consolidadas sobre sua prevalência populacional, padrão de herança genética ou faixa etária típica de início.[1]
Causas genéticas
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
O **quinto arco aórtico persistente** é uma alteração anatômica congênita muito rara na estrutura da aorta, decorrente do desenvolvimento embrionário. Com frequência, essa condição é identificada juntamente com outros defeitos no coração e nos vasos sanguíneos, como a coarctação ou a interrupção do arco aórtico. O diagnóstico e a confirmação são realizados por exames detalhados de imagem, como o ecocardiograma e a angiotomografia computadorizada. O manejo envolve reparo cirúrgico especializado, e no SUS o acompanhamento pode contar com procedimentos de atendimento em reabilitação para doenças raras. A doença não possui um Protocolo Clínico e Diretrizes Terapêuticas (PCDT) próprio formulado pelo Ministério da Saúde.
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.
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Quinto arco aórtico persistente
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Case Report: A case of persistent fifth aortic arch associated with stenosis and interrupted aortic arch.
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.
Histological investigation of persistent fifth aortic arch with arch obstruction in a neonate.
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.
Persistent Fifth Aortic Arch in a Right-Sided Aortic Arch Associated With Tetralogy of Fallot.
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.
Persistent fifth aortic arch in a neonate with interrupted aortic arch: an unexpected intraoperative finding.
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.
Persistent fifth aortic arch complicated by Williams-Beuren syndrome: a case description.
Publicações recentes
Histological investigation of persistent fifth aortic arch with arch obstruction in a neonate.
Case Report: A case of persistent fifth aortic arch associated with stenosis and interrupted aortic arch.
Persistent Fifth Aortic Arch in a Right-Sided Aortic Arch Associated With Tetralogy of Fallot.
Persistent fifth aortic arch complicated by Williams-Beuren syndrome: a case description.
Persistent fifth aortic arch in a neonate with interrupted aortic arch: an unexpected intraoperative finding.
📚 EuropePMC81 artigos no totalmostrando 39
Histological investigation of persistent fifth aortic arch with arch obstruction in a neonate.
Journal of cardiology casesCase Report: A case of persistent fifth aortic arch associated with stenosis and interrupted aortic arch.
Frontiers in pediatricsPersistent Fifth Aortic Arch in a Right-Sided Aortic Arch Associated With Tetralogy of Fallot.
JACC. Case reportsPersistent fifth aortic arch complicated by Williams-Beuren syndrome: a case description.
Quantitative imaging in medicine and surgeryPersistent fifth aortic arch in a neonate with interrupted aortic arch: an unexpected intraoperative finding.
Interdisciplinary cardiovascular and thoracic surgeryEchocardiography of persistent fifth aortic arch confirmed by computed tomography angiography or surgery in children: a case series.
BMC cardiovascular disordersConfusion from prenatal diagnosis: Type C persistent fifth aortic arch or right-side ductus arteriosus.
Echocardiography (Mount Kisco, N.Y.)Cinematic Rendering of Persistent Fifth Aortic Arch with Aortic Coarctation and Bicuspid Aortic Valve.
The Indian journal of radiology & imagingPersistent fifth aortic arch: a comprehensive literature review.
Frontiers in pediatricsCommon Arterial Trunk Coexisting With Double-Barreled Aorta.
JACC. Case reportsSurgical repair of persistent fifth aortic arch coarctation and interrupted fourth aortic arch without cardiopulmonary bypass: a case report.
Translational pediatricsA new association of Oculoauriculovertebral spectrum and persistent fifth aortic arch -double lumen aorta: a case report.
BMC pediatricsA Case of Persistent Fifth Aortic Arch With an Interrupted Fourth Aortic Arch.
The Annals of thoracic surgeryA double-barrelled aorta with high aortic Arch.
Journal of cardiology casesStridor as a Harbinger of Congenital Cardiovascular Anomaly.
Acta Cardiologica SinicaPersistent fifth aortic arch associated with aortic coarctation: a case of surgical correction without artificial material.
Journal of cardiothoracic surgeryNeonatal repair of persistent fifth aortic arch and aortic coarctation.
JTCVS techniquesPersistent fifth aortic arch: a single-center experience, case series.
Translational pediatricsIs It Indeed the Persistent Fifth Aortic Arch?
The Annals of thoracic surgeryAbout a Definition of the Persistent Fifth Aortic Arch.
The Annals of thoracic surgeryAnomalous 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.)Persistent Fifth Aortic Arch With Left Ventricular Dysfunction and Left Bronchial Obstruction.
The Annals of thoracic surgeryAssessment of persistent fifth aortic arch by echocardiography and computed tomography angiography.
MedicinePersistent fifth aortic arch stenosis associated with type A interruption of the aortic arch: a report of six cases.
Chinese medical journal"Double-lumen" aortic arch with "double-lumen" brachiocephalic artery.
Annals of pediatric cardiologyTrue bovine aortic arch combined with coarctation of persistent fifth aortic arch in a child.
European heart journal. Cardiovascular ImagingType 2 persistent fifth aortic arch: An elusive entity diagnosed on computed tomography angiography.
Journal of cardiovascular computed tomographyPersistent fifth aortic arch: the "great pretender" in clinical practice.
Cardiology in the youngNeonatal Repair of Persistent Fifth Aortic Arch Coarctation and Interrupted Fourth Aortic Arch.
The Annals of thoracic surgeryAortic arch advancement for type A interrupted aortic arch with persistent fifth aortic arch type B.
Cardiology in the youngCT imaging of a rare case of persistent fifth aortic arch in newborn.
BJR case reportsDouble-Lumen Aortic Arch: Persistence of the Fifth Aortic Arch.
The Annals of thoracic surgeryPersistent Fifth Aortic Arch with Coarctation.
The Korean journal of thoracic and cardiovascular surgeryCoarctation of Persistent Fifth Aortic Arch With Interrupted Fourth Arch: First Pediatric Report of Stent Intervention.
Revista espanola de cardiologia (English ed.)Iatrogenic aortic dissection in an infant with persistent fifth aortic arch and congenital heart defects.
The Journal of thoracic and cardiovascular surgeryPersistent Fifth Aortic Arch Confirmed by Computed Tomography Angiography.
World journal for pediatric & congenital heart surgeryAortic Arch Interruption and Persistent Fifth Aortic Arch in Phace Syndrome: Prenatal Diagnosis and Postnatal Course.
Echocardiography (Mount Kisco, N.Y.)Interruption of the fourth aortic arch and a persistent fifth aortic arch with coarctation: a rare morphological finding.
Kardiologia polska[Persistent fifth aortic arch with patent ductus arteriosus].
Archivos de cardiologia de MexicoAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Quinto arco aórtico persistente.
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Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Ainda não achamos doenças com sintomas parecidos o suficiente.
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
É uma alteração morfológica congênita vascular muito rara do arco aórtico, catalogada sob o código ORPHA:99076. Ela ocorre pela permanência de um vaso embrionário transitório durante a formação do sistema cardiovascular.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
3 fontes citadas no texto · 5 publicações do grafo RarasNet (PubMed) · 4 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (2026). Quinto arco aórtico persistente. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/quinto-arco-aortico-persistente
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Quinto arco aórtico persistente
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Dado público estruturado
- fonte: Wikidata
