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Aneurisma dos seios de Valsalva
ORPHA:1054CID-10 · Q25.4CID-11 · LA8A.4PCDT · SUSDOENÇA RARA

O aneurisma do seio de Valsalva (SVA) é uma malformação cardíaca congênita rara de um ou mais seios aórticos, consistindo em uma dilatação que quando não rompida é geralmente assintomática, mas quando rompida apresenta dispneia progressiva aos esforços, fadiga, dor torácica e que pode levar à insuficiência cardíaca congestiva se não tratada.

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Introdução

O que você precisa saber de cara

📋

O aneurisma do seio de Valsalva (SVA) é uma malformação cardíaca congênita rara de um ou mais seios aórticos, consistindo em uma dilatação que quando não rompida é geralmente assintomática, mas quando rompida apresenta dispneia progressiva aos esforços, fadiga, dor torácica e que pode levar à insuficiência cardíaca congestiva se não tratada.

Publicações científicas
130 artigos
Último publicado: 2026 Feb

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adult
🏥
SUS: Cobertura parcialScore: 50%
PCDT disponívelTriagem neonatal (Fase 5)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

Partes do corpo afetadas

❤️
Coração
4 sintomas
🫁
Pulmão
2 sintomas

+ 5 sintomas em outras categorias

Características mais comuns

100%prev.
Dilatação do seio de Valsalva
55%prev.
Sopro cardíaco
Frequente (79-30%)
55%prev.
Dispneia
Frequente (79-30%)
55%prev.
Regurgitação aórtica
Frequente (79-30%)
17%prev.
Insuficiência cardíaca congestiva
Ocasional (29-5%)
17%prev.
Edema
Ocasional (29-5%)
11sintomas
Muito frequente (1)
Frequente (3)
Ocasional (3)
Muito raro (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 11 características clínicas mais associadas, ordenadas por frequência.

Dilatação do seio de ValsalvaDilatation of the sinus of Valsalva
Muito frequente100%
Sopro cardíacoHeart murmur
Frequente (79-30%)55%
DispneiaDyspnea
Frequente (79-30%)55%
Regurgitação aórticaAortic regurgitation
Frequente (79-30%)55%
Insuficiência cardíaca congestivaCongestive heart failure
Ocasional (29-5%)17%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico130PubMed
Últimos 10 anos26publicações
Pico20158 papers
Linha do tempo
2026Hoje · 2026🧪 2007Primeiro ensaio clínico📈 2015Ano de pico
Publicações por ano (últimos 10 anos)

Triagem neonatal (Teste do Pezinho)

👶
Teste: qPCR para deleção de SMN1 em sangue seco
Fase 5 do PNTNpending
Incidência no Brasil: 1:10.000

A triagem neonatal permite diagnóstico precoce e início imediato do tratamento.

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 31
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Aneurisma dos seios de Valsalva

🗺️

Selecione um estado ou use sua localização para ver resultados.

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

0 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
26 papers (10 anos)
#1

An unusual cause of right ventricular failure.

European heart journal. Case reports2026 Feb
#2

Dissecting aneurysm of sinus of Valsalva into interventricular septum and rupturing into left ventricle through multiple sinuses: a rare case report.

European heart journal. Case reports2024 Aug

Ruptured sinus of Valsalva (RSOV) is a rare disorder, which usually involves the right coronary sinus (RCS) or the non-coronary sinus (NCS) and ruptures usually into the right-sided chambers. Involvement of the left coronary sinus (LCS) and multiple sinuses, rupture into the left ventricle (LV), and dissecting aneurysm of the interventricular septum (IVS) have all been scarcely reported. A 24-year-old male presented with complaints of exertional fatigue, palpitations, and chest pain with signs of aortic run-off like wide pulse pressure, collapsing pulse along with cardiomegaly, and a diastolic murmur. Echocardiography revealed sinus of Valsalva aneurysms (SOVAs) involving both the RCS and LCS with RCS aneurysm dissecting the IVS and rupturing into the LV and another multilobulated aneurysm from LCS rupturing into the LV. Findings were confirmed on computed tomography (CT) aortogram, and the patient underwent successful surgical repair. Sinus of Valsalva aneurysm is a rare disorder which usually ruptures into the right-sided chambers. The involvement of multiple sinuses and rupture into the IVS is extremely rare (<2%). Aneurysm dissecting the IVS can lead to complete heart block (CHB) and sudden death. Involvement of the LCS is reported in <5% cases of RSOV, and rupture of such an aneurysm into the pericardial space may lead to cardiac tamponade and can also lead to sudden death. Clinical examination, electrocardiogram, chest X-ray, 2D echocardiography, and CT all help in the diagnosis. Treatment involves surgical repair of the defect.

#3

Aneurysms of Aortic Sinus of Valsalva Dissecting through the Interventricular Septum With Rupture into the Left Ventricle: Case Series and Literature Review.

World journal for pediatric &amp; congenital heart surgery2024 May

Aneurysm of Aortic sinus of Valsalva (ASOV) dissecting into the interventricular septum (IVS) and rupturing into the left ventricle (LV) is a rare clinical diagnosis. Systemic inflammatory diseases like tuberculosis can aggravate this condition. We describe three cases of ASOV dissecting into the IVS and rupturing into the LV. All three patients underwent surgical intervention; two had a successful outcome. A literature review was conducted and19 previously reported cases were studied. The extent and direction of septal dissection determined the associated cardiac valvular and rhythm problems. Patch closure of the mouth of the aneurysm is the surgical method of choice. In the presence of multiple sinus tracts or if there is recurrence after surgical closure, aortic sinus or root replacement techniques have better outcomes.

#4

[A case of cerebral embolism treated with early surgical intervention for aneurysm of sinus of Valsalva as embolic source].

Rinsho shinkeigaku = Clinical neurology2022 Jun 24

The patient was a 78-year-old man. He was transferred to the emergency room presenting with aphasia and right hemiplegia. Head CT and CT angiography demonstrated a narrow territory of early ischemic signs and occlusion of the horizontal segment of the left middle cerebral artery (MCA), respectively. Endovascular thrombectomy was performed and complete recanalization of MCA was achieved. After admission, transthoracic echocardiography revealed a cystic mass near the left coronary apex of the aortic valve, which was diagnosed as an aneurysm of the sinus of Valsalva (ASV) by contrast-enhanced chest computed tomography. Transesophageal echocardiography showed a severe smoke-like echo within ASV, despite being in sinus rhythm. Intravenous anticoagulant therapy was started, and patch closure was performed by cardiovascular surgeons on the 13th day despite of early period after stroke onset. During the operation, no thrombus was seen around the inlet of ASV. On the two days after the operation, paroxysmal atrial fibrillation was detected and anticoagulant therapy was continued. In this patient, ASV was regarded as the embolic source of cerebral embolism, and anticoagulant therapy and surgical intervention were selected in the early period after stroke onset to prevent embolism recurrence, resulting in a favorable clinical course.

#5

Multilobulated Sinus of Valsalva Aneurysm Dissecting into the Interventricular Septum (DAIS) and Rupturing into Left Ventricle: A Case Report.

European heart journal. Case reports2022 Feb

Dissecting aneurysm of sinus of Valsalva (SOV) into the interventricular septum is a rare entity. Multilobulated form of dissection rupturing into the left ventricle (LV) has never been reported in the literature. A 52-year-old male presented with dyspnoea and palpitation with wide pulse pressure and peripheral signs of distal run-off and a continuous murmur along the left parasternal area. Echocardiography revealed dilated right coronary cusp (RCC), which burrowed into the interventricular septum (IVS), forming multi-loculated cystic lesion which ruptured into LV with associated restrictive ventricular septal defect (VSD) and severe aortic regurgitation. Computed tomography (CT) angiography confirmed a 4.8 cm × 5.3 cm × 5.4 cm multiseptated aneurysm. The surgery involved excision of the aortic valve (AV) with the sinus, ligation of its penetrating portion at the crest of IVS, closure of VSD, and AV replacement. Postoperative echocardiography showed the complete collapse of the IVS component of the SOV aneurysm and the normally functioning mechanical AV. Dissecting aneurysm into the IVS is a rare variant of SOV aneurysm, usually arising from RCC. It is mostly congenital in origin and has wide variety of presentations like congestive heart failure, palpitations, recurrent syncope, chest pain, sudden cardiac arrest, infective endocarditis, cerebral infarction, or asymptomatic. Aortic regurgitation is present in 30-50% of cases. Conduction disturbances from first-degree block to complete heart block are common. Echocardiogram, CT angio, and magnetic resonance imaging are useful for diagnosis. Surgical repair is the only option for treatment.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC90 artigos no totalmostrando 26

2026

An unusual cause of right ventricular failure.

European heart journal. Case reports
2024

Dissecting aneurysm of sinus of Valsalva into interventricular septum and rupturing into left ventricle through multiple sinuses: a rare case report.

European heart journal. Case reports
2024

Aneurysms of Aortic Sinus of Valsalva Dissecting through the Interventricular Septum With Rupture into the Left Ventricle: Case Series and Literature Review.

World journal for pediatric &amp; congenital heart surgery
2022

A giant aneurysm of sinus of Valsalva presenting with frequent syncopal attacks.

Asian cardiovascular &amp; thoracic annals
2022

[A case of cerebral embolism treated with early surgical intervention for aneurysm of sinus of Valsalva as embolic source].

Rinsho shinkeigaku = Clinical neurology
2022

Multilobulated Sinus of Valsalva Aneurysm Dissecting into the Interventricular Septum (DAIS) and Rupturing into Left Ventricle: A Case Report.

European heart journal. Case reports
2021

Sinus of Valsalva Rupture or VSD Shunt: Mystery Solved by Cardiac CT.

The Indian journal of radiology &amp; imaging
2020

Management of ruptured sinus of valsalva for device closure in a patient with haemophilia.

Annals of cardiac anaesthesia
2019

[Giant Aneurysm of Sinus of Valsalva;Report of a Case].

Kyobu geka. The Japanese journal of thoracic surgery
2019

[Aortic Valve-preserving Root Remodeling in a Patient with Bicuspid Aortic Valve and Severe Annulo-aortic Ectasia].

Kyobu geka. The Japanese journal of thoracic surgery
2018

[Application of Combined Special Staining in the Observation of Vascular Pathology].

Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae
2017

Congenital unruptured aneurysm of sinus of Valsalva, quadricuspid aortic valve, and ascending aortic aneurysm associated with genetic leukoencephalopathy in an infant: A new syndrome?

Echocardiography (Mount Kisco, N.Y.)
2017

[Ruptured Aneurysm of the Sinus of Valsalva Accompanied with a Bicuspid Aortic Valve in an Elderly Man;Report of a Case].

Kyobu geka. The Japanese journal of thoracic surgery
2017

Diagnostic paradox: Ruptured aneurysm of sinus of Valsalva simulating tricuspid valve endocarditis.

Echocardiography (Mount Kisco, N.Y.)
2016

[Comment on "Recurrent aneurysm of sinus of Valsalva, the world´s first case report"].

Revista medica del Instituto Mexicano del Seguro Social
2016

Ruptured Aneurysm of Sinus of Valsalva: A Case Report.

Mymensingh medical journal : MMJ
2016

A Constellation of Cardiac Anomalies: Beyond Shone's Complex.

Heart views : the official journal of the Gulf Heart Association
2016

Unusual clinical course after surgical repair of unruptured aneurysm of sinus of Valsalva.

Journal of medical ultrasonics (2001)
2015

Giant Unruptured Sinus of Valsalva Aneurysm: An Unusual Cause of Right Heart Failure.

Journal of clinical imaging science
2015

[Recurrent aneurysm of sinus of Valsalva, the world's first case report].

Revista medica del Instituto Mexicano del Seguro Social
2015

Unruptured Aneurysm of Sinus of Valsalva Coexisting with the Large Ventricular Septal Defect and Severe Aortic Regurgitation in a Young Man.

Case reports in medicine
2015

Supracristal ventricular septal defect in adults: Is it time for a paradigm shift?

International journal of cardiology
2015

[Aneurysm of sinus of Valsalva as a complication of infective endocarditis in a patient after implantation of biological aortic valve].

Kardiologia polska
2015

Complete heart block with diastolic heart failure and pulmonary edema secondary to enlarging previously diagnosed thrombosed aneurysm of sinus of valsalva in a patient with history of autosomal dominant polycystic kidney disease.

Case reports in cardiology
2015

Cesarean section under epidural anesthesia in a documented case of ruptured aneurysm of the sinus of valsalva.

Journal of anaesthesiology, clinical pharmacology
2015

The dilemma of evaluating a continuous murmur in a patient of aneurysm of sinus of Valsalva and coronary cameral fistula presenting with supraventricular tachycardia.

BMJ case reports
Ver todos os 90 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

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. An unusual cause of right ventricular failure.
    European heart journal. Case reports· 2026· PMID 41635601mais citado
  2. Dissecting aneurysm of sinus of Valsalva into interventricular septum and rupturing into left ventricle through multiple sinuses: a rare case report.
    European heart journal. Case reports· 2024· PMID 39205792mais citado
  3. Aneurysms of Aortic Sinus of Valsalva Dissecting through the Interventricular Septum With Rupture into the Left Ventricle: Case Series and Literature Review.
    World journal for pediatric &amp; congenital heart surgery· 2024· PMID 38646718mais citado
  4. [A case of cerebral embolism treated with early surgical intervention for aneurysm of sinus of Valsalva as embolic source].
    Rinsho shinkeigaku = Clinical neurology· 2022· PMID 35644577mais citado
  5. Multilobulated Sinus of Valsalva Aneurysm Dissecting into the Interventricular Septum (DAIS) and Rupturing into Left Ventricle: A Case Report.
    European heart journal. Case reports· 2022· PMID 35233482mais citado
  6. A giant aneurysm of sinus of Valsalva presenting with frequent syncopal attacks.
    Asian Cardiovasc Thorac Ann· 2022· PMID 36071598recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:1054(Orphanet)
  2. MONDO:0015197(MONDO)
  3. Esclerose Lateral Amiotrofica(PCDT · Ministério da Saúde)
  4. GARD:670(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. Q4761767(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.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Aneurisma dos seios de Valsalva
Compêndio · Raras BR

Aneurisma dos seios de Valsalva

ORPHA:1054 · MONDO:0015197
🇧🇷 Brasil SUS
Triagem
qPCR para deleção de SMN1 em sangue seco
PNTN
Fase 5
Incidência BR
1:10.000
Geral
Prevalência
Unknown
Herança
Unknown
CID-10
Q25.4 · Outras malformações congênitas da aorta
CID-11
Início
Adult
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C2239253
EuropePMC
Wikidata
Papers 10a
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