Agenesia da veia cava é uma malformação rara. Sua causa mais comum é a disgenesia durante a embriogênese, mas também pode estar relacionada a trombose intrauterina ou perinatal. Normalmente é assintomática, em associação, ou não, com outras malformações congênitas, e pode cursar com maior risco de insuficiência venosa crônica e trombose venosa profunda, especialmente em jovens. Seu diagnóstico frequentemente é acidental, durante cirurgias ou procedimentos radiológicos.
Introdução
O que você precisa saber de cara
Agenesia da veia cava superior direita é uma anomalia congênita rara onde a veia cava superior direita não se forma. Isso pode levar ao retorno venoso alternativo, muitas vezes assintomático, mas pode causar sintomas como edema facial e de membros superiores.
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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 — Agenesia da veia cava superior, direita
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Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
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Taiwanese journal of obstetrics & gynecologyA case of novel combined variations of the renal and testicular vessels.
Folia morphologicaRadiofrequency ablation of persistent atrial fibrillation in a patient with situs inversus totalis and interrupted inferior vena cava.
Journal of cardiovascular electrophysiologyInterrupted inferior vena cava draining into the coronary sinus associated with circumflex artery to coronary sinus fistula.
Current problems in cardiologyIntegrated analysis of comorbidity, pregnant outcomes, and amniotic fluid cytogenetics of fetuses with persistent left superior vena cava.
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JACC. Cardiovascular interventionsPersistent Left Superior Vena Cava with the Absence of the Right Superior Vena Cava and Atrial Appendage: Complex Pacemaker Implantation in a Patient Presenting with a Rare Vascular Anomaly.
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Annals of cardiac anaesthesiaLeft Superior Vena Cava Draining to Left Atrium: A Case Report, Review of the Literature, and Classification.
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Journal of veterinary cardiology : the official journal of the European Society of Veterinary CardiologySurgical treatment of anomalous right upper lobe pulmonary vein obstruction caused by compression between pulmonary artery and trachea: a case report.
Cardiology in the youngAbsent Superior Vena Cava: An Uncommon Cause of Anomalous Systemic Venous Return.
World journal for pediatric & congenital heart surgeryUnusual Left Superior Vena Cava, Connected to the Left Atrium via the Left Superior Pulmonary Vein.
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Pacing and clinical electrophysiology : PACEA missense variant at the RAC1-PAK1 binding site of RAC1 inactivates downstream signaling in VACTERL association.
Scientific reportsStroke Caused by a Paradoxical Embolus From a Rare Congenital Anomaly in the Adult: Persistent Left Superior Vena Cava Draining into the Left Upper Pulmonary Vein.
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Frontiers in bioengineering and biotechnologyConcomitant Coronary Artery Disease and Persistent Left Superior Vena Cava in a Patient Presenting With Symptomatic Sinus Bradycardia: A Case Report.
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Echocardiography (Mount Kisco, N.Y.)[Genetic analysis of a fetus with de novo 46,X,der(X)t(X;Y)(q26;q11)].
Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical geneticsQuantitative measurements and morphological evaluation of fetal cardiovascular structures with fetal cardiac MRI.
European journal of radiologyPersistent Left Superior Vena Cava: Rare Location of Hemodialysis Catheter.
CureusAscites in Animals With Right Heart Failure: Correlation With Lymphatic Dysfunction.
Journal of the American Heart AssociationMultiple accessory pathways coexisting with a persistent left superior vena cava: a case report.
Journal of medical case reportsRepair of Partial Anomalous Pulmonary Venous Connection to the Azygous Vein via Right Thoracotomy.
World journal for pediatric & congenital heart surgeryHigh prevalence of left superior vena cava and congenital heart disease in patients with pre- and postnatally diagnosed esophageal atresia/tracheoesophageal fistula.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyHeart Transplant Using a Donor Heart With Only a Persistent Left Superior Vena Cava: A Case Report.
Transplantation proceedingsIdentifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.
World journal for pediatric & congenital heart surgerySubjective and Objective Sonographic Assessment for the Prenatal Detection of Neonatal Coarctation of the Aorta.
Fetal diagnosis and therapyMultiple ventricular septal defects associated to anomalous venous returns, mitral valve disease, myocardium hypertrophy, and right outflow obstruction: a multimodality imaging assessment.
Echocardiography (Mount Kisco, N.Y.)Long-Term Surgical Outcomes of Patients With Isomeric Right and Left Atrial Appendages.
World journal for pediatric & congenital heart surgeryInferior Vena Cava Agenesis: An Underrated Cause of Deep Venous Thrombosis.
CureusMalposition of the central venous catheter secondary to accessory hemiazygos vein variant.
The journal of vascular accessUltrasound Imaging of the Superior Vena Cava: A State-of-the-Art Review.
Journal of the American Society of Echocardiography : official publication of the American Society of EchocardiographyPre-procedural imaging and atrial tachycardia ablation in a patient with complex congenital heart disease.
Pacing and clinical electrophysiology : PACECoronary Sinus Draining Into the Right Superior Vena Cava: A Rare Anomaly.
JACC. Cardiovascular interventionsComputed tomography and magnetic resonance imaging of congenital thoracic systemic venous anomalies.
Pediatric radiologyAnomalous Connection of Superior Vena Cava to Left Atrium-A Rare Congenital Cardiac Anomaly and Its Implications.
A&A practicePersistent left superior vena cava in a patient with an implanted dual-chamber pacing system in atrial fibrillation with tachycardia-bradycardia syndrome.
Bratislavske lekarske listyEndobronchial Ultrasound Diagnosis of a Malignant Superior Vena Cava Tumor Thrombus Extending Into the Right Atrium: An Unusual Cause of Recurrent Syncope.
CureusPrimary sutureless repair concomitant with the Warden procedure.
Asian cardiovascular & thoracic annalsMal-positioning of a dialysis catheter: One lumen with arterial blood and the other with venous blood?
The journal of vascular accessAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Agenesia da veia cava superior, direita.
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Agenesia da veia cava superior, direita
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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.
- Rebuttal: aortic coarctation and concomitant anomalies - left circumflex fistula, persistent left SVC and ventricular diverticulum.
- Unroofed coronary sinus and persistent left superior vena cava: a rare neonatal venous anomaly.
- Congenital absence of the portal vein identified following the onset of esophageal-gastric variceal rupture.
- Retrocardiac Innominate Vein: A Rare Vascular Anomaly Associated With Congenital Heart Disease.
- Polysplenia syndrome complicated by multiple intrahepatic bile duct stones in an adult: a case report.
- Anaesthetic Management of a Case of Minimally Invasive Surgical Repair of Sinus Venosus Atrial Septal Defect (SVASD) and Persistent Left Superior Vena Cava (LSVC), Assisted by Unique Bilateral Percutaneous Superior Vena Cava Cannulation.
- Interatrial Septum Assessment Part 2: Sinus Venosus Defects.
- Transcatheter resolution of a post-surgical "Iatrogenic" circular shunt in a child.
- Intraoperative recognition of persistent left superior vena cava during right internal jugular central line placement in mitral valve replacement: a case report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:99114(Orphanet)
- MONDO:0020445(MONDO)
- GARD:19657(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55789384(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
