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Agenesia da veia cava superior, direita
ORPHA:99114CID-10 · Q26.8CID-11 · LA86.YDOENÇA RARA

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.

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Introdução

O que você precisa saber de cara

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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.

🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q26.8
🇧🇷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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Últimos 10 anos200publicações
Pico202573 papers
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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

Rebuttal: aortic coarctation and concomitant anomalies - left circumflex fistula, persistent left SVC and ventricular diverticulum.

BMJ case reports2026 Feb 24

Coarctation of the aorta (COA) is a relatively common cardiovascular congenital anomaly. It can be seen in up to 6 per 1000 live births. Rare cardiovascular anomalies have been found to be associated with COA, which may complicate management. We present a case of an adolescent male, incidentally found to be hypertensive. COA was confirmed on imaging, however, with multiple anomalies with an incidence in the general population as common as 0.4 per cent to as rare as 7 cases ever reported in the literature. The association of COA with left circumflex to coronary sinus fistula, ventricular diverticulum and persistent left superior vena cava has never been reported to our knowledge.

#2

Unroofed coronary sinus and persistent left superior vena cava: a rare neonatal venous anomaly.

BMJ case reports2026 Jan 29

Approach to abnormal cardiovascular signs in the early neonatal period is challenging due to delayed fetal adaptation, closure of the patent ductus arteriosus and physiological immaturity. We present a rare case of unroofed coronary sinus (UCS) with persistent left superior vena cava (PLSVC) in a term neonate who presented with recurrent desaturation episodes and persistent oxygen requirements. Transthoracic echocardiography (TTE) revealed this rare venous anomaly, which is mainly reported in the older population and primarily as an incidental finding. TTE with an agitated saline study was the key diagnostic modality. Early diagnosis is crucial given the potential complications of thromboembolic phenomena and brain abscesses. Clinical suspicion should be high in neonates with persistent unexplained oxygen requirements with a structurally normal heart, primarily when associated with PLSVC. This is the first-ever case report of UCS presenting in the neonatal period.

#3

Congenital absence of the portal vein identified following the onset of esophageal-gastric variceal rupture.

Clinical journal of gastroenterology2026 Feb

A 36-year-old woman with an unremarkable medical history was admitted to our hospital owing to hematemesis. Upper gastrointestinal endoscopy revealed F3 esophageal varices and F3 gastric varices with erosions, which were treated with endoscopic injection sclerotherapy with histoacryl. Contrast-enhanced computed tomography showed that the superior mesenteric vein and splenic vein converged and drained into the inferior vena cava, with no identifiable intrahepatic portal vein branches. Additionally, collateral circulation and thrombosis in the superior mesenteric vein were identified. On the basis of these findings, we diagnosed congenital absence of the portal vein and associated portal hypertension. After treatment for various complications, the patient underwent living donor liver transplantation as a curative treatment. Surgical pathology also supported the diagnosis of portal vein agenesis. Congenital absence of the portal vein is a rare congenital condition, and only one case involving variceal rupture has been reported. There are no established treatment protocols; however, given the pathophysiology, the development of varices is certainly possible. Effective bridging therapy followed by living donor liver transplantation is expected to result in a favorable prognosis. Although the patient remained free from rebleeding for seven months after initial treatment, the absence of portal flow and progression of SMV thrombosis indicated a high risk of recurrence and other complications. After multidisciplinary discussion, living donor liver transplantation was chosen as the most reliable curative treatment compared with endoscopic therapy or shunt surgery.

#4

Retrocardiac Innominate Vein: A Rare Vascular Anomaly Associated With Congenital Heart Disease.

Journal of the Saudi Heart Association2026

Atrial septal defects are common congenital heart anomalies and may coexist with rare systemic venous abnormalities. We describe a one-year-old female with a large secundum atrial septal defect and an unusual retrocardiac innominate vein. Transthoracic echocardiography suggested a persistent left superior vena cava, but the innominate vein was absent from its usual location and the coronary sinus was not dilated. Cardiac CT angiography demonstrated a retrocardiac innominate vein draining into the azygos system and subsequently to the right atrium. Surgical atrial septal defect closure was successful. Recognition of this anomaly is essential for accurate diagnosis and procedural planning.

#5

Polysplenia syndrome complicated by multiple intrahepatic bile duct stones in an adult: a case report.

Frontiers in medicine2026

Polysplenia syndrome is a rare congenital disorder characterized by multiple spleens and complex visceral and vascular anomalies. We report an adult case admitted for multiple intrahepatic bile duct stones. Imaging revealed multiple congenital abnormalities, including polysplenia, a truncated pancreas, and absence of the superior inferior vena cava segment. Intraoperative findings confirmed extensive intrahepatic bile duct stones and biliary malformations. Surgical treatment included partial hepatectomy, bile duct exploration, and T-tube drainage. Postoperatively, the patient developed bacteremia, which resolved with targeted antibiotics. This case highlights the link between polysplenia syndrome and hepatobiliary anomalies, emphasizing the need for careful anatomical evaluation, early recognition of biliary complications, and close clinical monitoring in affected patients.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC2 artigos no totalmostrando 200

2026

Retrocardiac Innominate Vein: A Rare Vascular Anomaly Associated With Congenital Heart Disease.

Journal of the Saudi Heart Association
2025

The Effect of Aspirin Administration on Fetal Cardiovascular Function Between 18 to 24 Weeks of Gestation: A New Perspective on ASA Indication in Obstetrics.

Journal of reproduction & infertility
2026

Polysplenia syndrome complicated by multiple intrahepatic bile duct stones in an adult: a case report.

Frontiers in medicine
2026

Acute Superior Vena Cava Obstruction Mimicking Aortic Dissection: A Case of Primary Mediastinal Large B-cell Lymphoma.

Cureus
2026

Rebuttal: aortic coarctation and concomitant anomalies - left circumflex fistula, persistent left SVC and ventricular diverticulum.

BMJ case reports
2026

Prenatal ultrasound diagnosis and prognosis of persistent left superior vena cava: a 10-year retrospective cohort study at a single center in China.

Frontiers in medicine
2026

Atypical Presentation of Downhill Esophageal Varices: A Diagnostic Enigma.

ACG case reports journal
2026

Computed tomography angiography imaging of double superior vena cava in a case of tetralogy of fallot.

Radiology case reports
2026

The Bachmann's bundle connection: from obesity and aging to atrial arrhythmia development.

Folia morphologica
2026

Management of Acute Cholecystitis in a Patient With Eisenmenger Syndrome and Abdominal Heterotaxy: A Case Report.

The American journal of case reports
2026

Unroofed coronary sinus and persistent left superior vena cava: a rare neonatal venous anomaly.

BMJ case reports
2026

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.

Annals of cardiac anaesthesia
2026

Interatrial Septum Assessment Part 2: Sinus Venosus Defects.

Methodist DeBakey cardiovascular journal
2025

Transcatheter resolution of a post-surgical "Iatrogenic" circular shunt in a child.

Cardiology in the young
2025

Virtual Reality to Enhance Understanding of Congenital Heart Disease.

Journal of cardiovascular development and disease
2025

Beyond the Pulmonary Veins: The Influence of Pulsed Field Ablation on the Superior Vena Cava.

Journal of arrhythmia
2025

A Case of a Rare Vascular Phenomenon: Superior Vena Cava Aneurysm.

Cureus
2026

Complex Fetal Anomalies: Kluth Type Xc2 Variant Tracheoesophageal Fistula, Srikanth Group III Bronchopulmonary Foregut Malformation, Mirror-Image Dextrocardia with L-Looped Ventricles, Anomalous Coronary Origin from the Pulmonary Artery, and Cardiac-Type Total Anomalous Pulmonary Venous Connection.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2025

Radiofrequency ablation of persistent atrial fibrillation in a patient with dextrocardia and interruption of inferior vena cava in the fluoroless catheterization laboratory: a case report.

Journal of medical case reports
2025

Intraoperative recognition of persistent left superior vena cava during right internal jugular central line placement in mitral valve replacement: a case report.

Journal of medical case reports
2025

VATS thymectomy in left atrial isomerism.

BMJ case reports
2026

Congenital absence of the portal vein identified following the onset of esophageal-gastric variceal rupture.

Clinical journal of gastroenterology
2025

[Mitral Valvuloplasty in a Patient with Left Inferior Vena Cava with Hemiazygos Continuation:Report of a Case].

Kyobu geka. The Japanese journal of thoracic surgery
2025

Frequency of Persistent Left Superior Vena Cava and Its Impact on Outcomes in Children Undergoing Congenital Heart Surgery.

Brazilian journal of cardiovascular surgery
2025

Anatomical considerations in fixation of sternal fractures and nonunions.

Injury
2025

MYH6 in Congenital Heart Defects: A Genotype-Phenotype Characterization in a French Cohort.

Pediatric cardiology
2025

Prenatal Diagnosis of a Rare Congenital Right Subclavian Artery-Superior Vena Cava Fistula.

The journal of obstetrics and gynaecology research
2025

Prenatal Diagnosis of Joubert Syndrome 23 With Left Isomerism: A Novel Phenotype Associated With Pathogenic KIAA0586 Variant.

Prenatal diagnosis
2025

Conduction system pacing in a patient with persistent left superior vena cava and absent right superior vena cava: a case report.

Journal of medical case reports
2025

Pulse Field Ablation for Persistent Atrial Fibrillation: Targeting the Persistent Superior Vena Cava.

Pacing and clinical electrophysiology : PACE
2025

Persistent left superior vena cava discovered during central line insertion in a patient with Joubert syndrome: a case report.

Journal of medical case reports
2025

A Rare Congenital Triad in an Adult With Coronary Artery Disease: A Case Report From Georgia.

Cureus
2025

New anatomical classification to plan transcatheter correction of sinus venosus defect based on 3D models.

Journal of cardiovascular computed tomography
2025

A novel NOTCH1 nonsense variant in a bicuspid aortic valve family with intrafamilial clinical heterogeneity.

BMC cardiovascular disorders
2025

[Minimally Invasive Cardiac Surgery for Partial Anomalous Pulmonary Venous Connection and Sinus Venosus Atrial Septal Defect].

Kyobu geka. The Japanese journal of thoracic surgery
2026

Prenatal Diagnosis of Fetal Heart Malformation With Abnormal Number of Pulmonary Artery Branches as the Initial Clue.

Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine
2025

Hot Quadrant Sign in a Lung Cancer Patient: A Rare Hepatic Pseudo-Lesion Unveiling Superior Vena Cava Obstruction.

Cureus
2025

A Rare Variant of Caval Malformation: Left-Dominant Caval System With Anomalous Cardiac Drainage.

Cureus
2025

Dynamic Cause for Elevated Pulmonary Venous and Left Ventricular Inflow Gradients.

Journal of cardiothoracic and vascular anesthesia
2025

Case report of anomalous venous drainage of the right upper pulmonary lobe.

International journal of surgery case reports
2025

Atypical course of the azygos vein and its tributaries: a case report.

Folia morphologica
2025

Intracardiac repair of Raghib syndrome along with ventricular septal defect closure: a successful single-surgery outcome.

Cardiology in the young
2025

Preference for double-patch technique in adult patients with partial anomalous pulmonary venous return.

Cardiovascular journal of Africa
2025

Venovenous extracorporeal membrane oxygenation catheter related superior vena cava syndrome without pre-existing stenosis in an adult patient: a case report.

BMC pulmonary medicine
2026

Transcatheter Versus Surgical Correction of Superior Sinus Venosus Defects: Comparison of the Outcomes and Late Complications.

Pediatric cardiology
2025

Cardiovascular CT angiography in systemic and pulmonary venous anomalies of the thorax.

Clinical imaging
2025

Thoracic Vascular Anomalies: Insights from Embryology and Imaging.

Radiographics : a review publication of the Radiological Society of North America, Inc
2025

A case of fatal infective endocarditis in uncorrected tetralogy of Fallot with persistent left superior vena cava.

Journal of forensic sciences
2025

Chylothorax after patent ductus arteriosus ligation in a dog with persistent left cranial vena cava.

Australian veterinary journal
2026

Prenatal diagnosis of absent right with persistent left superior vena cava: a case series.

Pediatric cardiology
2025

Splenic infarction secondary to multi-site thrombosis in lung adenocarcinoma with EGFR-L858R mutation: A case report.

Oncology letters
2025

Late Presentation of Isolated Persistent Left Superior Vena Cava with Absent Right Superior Vena Cava Manifesting as Heart Failure: A Case Report.

International medical case reports journal
2025

Partial anomalous pulmonary venous return: diagnostic challenges and successful correction in a patient with progressive pulmonary hypertension.

BMJ case reports
2025

Postpartum ischemic stroke due to persistent left superior vena cava to left atrium after DORV repair: a case report and literature review.

BMC cardiovascular disorders
2025

Borderline left ventricular hypoplasia without significant aortic or mitral stenosis: cardiac magnetic resonance criteria for biventricular repair.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2025

Prenatal Diagnosis of Arboleda-Tham Syndrome Associated With KAT6A Variants Presented With Interrupted Inferior Vena Cava and Fetal Growth Restriction.

Prenatal diagnosis
2025

The Surgical Considerations of Intracardiac or Extracardiac Aorto-Right Atrial Tunnels.

JACC. Case reports
2025

Dynamics of a Distorted Waveform and Elevated Central Venous Pressure (CVP) Resulting From Coiling of a Central Venous Catheter: A Case Report.

Cureus
2025

Is There an Ideal Age or Weight for the Warden Procedure in Patients With Partial Anomalous Pulmonary Venous Return?

The Annals of thoracic surgery
2025

Prenatal diagnosis of Type I persistent left superior vena cava and its correlation with congenital cardiac comorbidities.

Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology
2025

Acute thrombosis of an atrial flow regulator in a failing Fontan circulation: recanalization and management.

BMC cardiovascular disorders
2025

[Azygos continuation of the inferior vena cava associated with "H syndrome"].

La Revue de medecine interne
2025

Kawashima procedure in single ventricle palliation: the factors affecting clinical outcomes.

Journal of cardiothoracic surgery
2025

Case report: right radical nephrectomy with thrombectomy in a patient with renal tumor, retrohepatic inferior vena cava agenesis, and tumor extension to the azygos vein.

Journal of surgical case reports
2025

Right Internal Jugular Vein Catheterization for Hemodialysis Reveals Persistent Left Superior Vena Cava: A Rare Case Study.

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions
2025

Validation of the peripherally inserted central venous catheter insertion length prediction formula using height in children.

Pediatrics international : official journal of the Japan Pediatric Society
2025

Mini-Sternotomy for Aortic Valve Replacement with Concomitant Ablation of the Pulmonary Vein and Persistent Left Superior Vena Cava: A Case Report.

Brazilian journal of cardiovascular surgery
2025

Prenatal Genetic Diagnosis of Williams-Beuren Syndrome with Atypical and Complex Phenotypes.

Clinical laboratory
2025

Invasive management of unroofed coronary sinus: A systematic review.

Trends in cardiovascular medicine
2025

Chromosomal Microarray Analysis and Karyotype Analysis for Prenatal Diagnosis of Fetuses With Abnormal Ultrasound Soft Markers.

Journal of clinical laboratory analysis
2025

Case Report: Atrial baffles of pulmonary and systemic veins for the anatomic and physiologic repair of left atrial isomerism heterotaxy-pre- and post-operative three-dimensional reconstructions of two mirror-image pediatric hearts.

Frontiers in cardiovascular medicine
2025

Anatomical Left Superior Vena Cava Correction: An Option for Left Ventricular Recruitment?

The Thoracic and cardiovascular surgeon
2025

Catheter for Hemodialysis in Persistent Left Superior Vena Cava in a Patient with Aortic Valve Endocarditis.

Brazilian journal of cardiovascular surgery
2025

The Appearance of a Secondary Bone Lesion in Place of Nonspecific Vertebral Anomalies Related to Mediastinal Venous Thrombosis: Coincidence?

Clinical nuclear medicine
2025

Clinical outcomes of Kawashima procedure and subsequent hepatic vein incorporation†.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2026

A rare case of mal-positioning of an internal jugular central venous catheter due to an anomalous right upper pulmonary venous return.

The journal of vascular access
2024

[A Case of Distal Bile Duct Cancer Associated with Preduodenal Portal Vein].

Gan to kagaku ryoho. Cancer & chemotherapy
2025

Prenatal Diagnosis and Classification of Type I Persistent Left Superior Vena Cava.

Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine
2025

Ultrasonographic diagnosis of secondary cardiac position alterations led by fetal thoraco-abdominal anomalies: a report of three cases.

Translational pediatrics
2025

A rare, asymptomatic case of an extrahepatic portocaval shunt between the posterior aspect of the portal vein and the inferior vena cava.

Surgical and radiologic anatomy : SRA
2025

Covered stent repair of a veno-venous cavopulmonary window: a case report.

Cardiology in the young
2025

3D Computed Tomography Reconstruction for Pre-Operative Diagnosis of Anomalous Venous Drainage of the Right Upper Lobe.

Thoracic cancer
2024

An Accessory Right Hepatic and Cystic Arteries Derived from the Superior Mesenteric Artery: A Cadaveric Case Report.

The American journal of case reports
2025

An uncommon cardiovascular abnormality: Case report of core triatriatum associated with persistent left superior vena cava and coronary sinus dilation.

Radiology case reports
2025

A rare case of idiopathic portal hypertension with portopulmonary hypertension occurred following splenectomy with a change in portal hemodynamics.

Clinical journal of gastroenterology
2024

Glenn circulation causes early and progressive shunting in a surgical model of pulmonary arteriovenous malformations.

Physiological reports
2025

Vascular abnormalities not to miss on routine chest CT: A pictorial review.

European journal of radiology
2024

Retroesophageal Left Brachiocephalic Vein with Right-Sided Aortic Arch in Tetralogy of Fallot: A Rare Venous Anomaly Demonstrated on Computed Tomography Angiography.

Methodist DeBakey cardiovascular journal
2025

Techniques and outcomes of atrial fibrillation ablation in patients with persistent left superior vena cava.

Journal of cardiovascular electrophysiology
2024

Coronary arteriovenous fistula originating from the left coronary artery and draining into the superior vena cava.

Asian cardiovascular & thoracic annals
2024

Successful placement of a chest wall venous infusion port via persistent left superior vena cava: A case report.

Medicine
2024

Dual-chamber leadless pacemaker in complex adult congenital heart disease: a case report.

European heart journal. Case reports
2025

Outcomes of the Modified Warden Procedure for Partial Anomalous Pulmonary Venous Connection Drainage to a High Location in the Superior Vena Cava.

Heart, lung & circulation
2024

Living Donor Liver Transplantation for Congenital Portosystemic Shunt Presenting With Hyperinsulinemic Hypoglycemia.

Pediatric transplantation
2024

Prenatal diagnosis in fetal right aortic arch using chromosomal microarray analysis and whole exome sequencing: a Chinese single-center retrospective study.

Molecular cytogenetics
2025

Surgical Treatment of Superior Vena Cava Syndrome in a Preterm Neonate.

Vascular and endovascular surgery
2024

Unraveling fetal venous disorders: An integrated approach in fetal echocardiography and their clinical significance.

Taiwanese journal of obstetrics & gynecology
2024

Left-bundle branch optimized CRT implantation in a patient with persistent left superior vena cava.

Pacing and clinical electrophysiology : PACE
2024

Co-existing Mediastinal Venous Malformation and Fusiform Superior Vena Cava Aneurysm in a Patient with Ischemic Stroke: A Case Report and Review of Literature.

Current medical imaging
2024

Surgical Repair of Total Anomalous Pulmonary Venous Connection into the Superior Vena Cava: A Case Report.

World journal for pediatric & congenital heart surgery
2024

A Rare Variant of Aorto-Atrial Fistula; Left Sinus of Valsalva Fistula to Superior Vena Cava and Right Atrium in a Neonate.

World journal for pediatric & congenital heart surgery
2024

Prenatal Diagnosis of Persistent Left Superior Vena Cava Using High-Definition Flow Render Mode and Spatiotemporal Image Correlation.

Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine
2024

Persistent left cranial vena cava in a dog.

The Journal of small animal practice
2024

Polysplenia with situs inversus totalis, azygos continuation of the inferior vena cava, and duplication of the superior vena cava in a healthy adult: A case report.

Radiology case reports
2024

Percutaneous transcatheter treatment for partial anomalous pulmonary right venous connection to the superior caval vein without atrial septal defect.

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions
2024

Persistent left superior vena cava: clinical and radiological significance.

Radiologia
2024

RF catheter ablation of AVNRT in a patient with interrupted inferior vena cava anomaly with hemiazygos continuity with persistent left superior vena cava.

Journal of cardiothoracic surgery
2024

A 24-Year-Old Male Patient With an Ostium Secundum Complex Atrial Defect Secondary to a Perforated Aneurysm With Inferior Vena Cava Agenesis.

Cureus
2024

Ischemic Heart Disease with In-Stent Re-Stenosis with Complete Heart Block and Isolated Persistent Left Superior Vena Cava - Rare Case Report with Challenges and Clinical Implications.

Annals of cardiac anaesthesia
2024

Thoracic duct drainage patterns in heterotaxy.

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance
2025

Brachiocephalic Vein Duplication: Case Report of a Double Left Brachiocephalic Vein in an Infant.

Pediatric cardiology
2024

TAPVR: Molecular Pathways and Animal Models.

Advances in experimental medicine and biology
2024

Adult Partial Anomalous Pulmonary Venous Connection: Case Reports (Including a Case of Scimitar Syndrome).

The Journal of the Association of Physicians of India
2024

A Double Rarity: Lost intravascular Catheter Guidewire in Persistent Left Superior Vena Cava and Coronary Sinus - A Case Report.

Vascular health and risk management
2024

Transcatheter Correction of Superior Venacaval Form of Sinus Venosus Defects Using Balloon-Mounted Covered STENTS.

Interventional cardiology clinics
2025

Congenital Heart Defects in Patients with Anorectal Malformations: A Retrospective Cohort Study of 281 Patients.

Pediatric cardiology
2024

Agenesis of inferior vena cava in pregnancy: A case report and narrative review.

European journal of obstetrics, gynecology, and reproductive biology
2024

Malposition of Central Venous Catheter into Coronary Sinus throughout the Persistent Left Superior Vena Cava and Other Complications Related to Catheterization.

Diagnostics (Basel, Switzerland)
2024

Prenatal echocardiography diagnosis of a novel combination of bilateral ductus arteriosus and cardiovascular anomalies: a case report and literature review.

Frontiers in cardiovascular medicine
2024

Rare Combined Congenital Pulmonary and Cardiovascular Malformations Revealed by Acute Hemoptysis.

Case reports in pulmonology
2024

Challenge Accepted: Lead Extraction in a Patient with Persistent Left Superior Vena Cava and Right Superior Vena Cava Occlusion.

Cardiac electrophysiology clinics
2024

NASCI case of the month: "Unroofed coronary sinus without persistent left-sided superior vena cava".

The international journal of cardiovascular imaging
2025

Minimally invasive redo tricuspid valve replacement in patient with persistent left superior vena cava.

Perfusion
2024

Intracardiac Echocardiogram from Persistent Left Superior Vena Cava.

The American journal of cardiology
2024

Illuminating the Genetic Basis of Congenital Heart Disease in Patients with Kabuki Syndrome.

Diagnostics (Basel, Switzerland)
2024

A rare case of isolated persistent left superior vena cava diagnosed by echocardiography.

Journal of cardiothoracic surgery
2024

Elucidating left atrial electrical potential with microelectrode catheter: A case of coronary sinus ostial atresia with small persistent left superior vena cava.

Pacing and clinical electrophysiology : PACE
2024

Giant coronary sinus aneurysm: an incidental discovery in a case of acute coronary syndrome.

BMJ case reports
2024

Transthoracic Echocardiography-Guided Placement of a Pulmonary Artery Catheter in a Patient With a Known Persistent Left but Unknown Absent Right Superior Vena Cava.

Circulation. Cardiovascular imaging
2024

Massive right pulmonary artery to superior vena cava shunt following extracorporeal membrane oxygenation.

European heart journal. Cardiovascular Imaging
2024

Rare case of total anomalous pulmonary venous return into the right atrium in situs solitus.

Cardiology in the young
2024

Right S3 segmentectomy for lung cancer with partial anomalous pulmonary venous return in the right upper pulmonary vein: A case report.

Thoracic cancer
2024

Novel variant in LRP6 associated with unusual and severe clinical presentation: Case report.

Clinical genetics
2024

Association of Prenatally Diagnosed Isolated Single Left Superior Vena Cava and Postnatal Development of Coarctation of the Aorta.

Pediatric cardiology
2024

It's a long way to the top! Congenital venous anomalies and left isomerism limiting atrial fibrillation transcatheter ablation.

Current problems in cardiology
2024

Challenges and pitfalls during CRT implantation in patients with persistent left superior vena cava.

Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing
2024

Foramen ovale flap aneurysm in fetuses associated with and without heart defects: Prenatal diagnosis, imaging, in-utero hemodynamics, pregnancy, and postnatal outcomes.

Echocardiography (Mount Kisco, N.Y.)
2024

Increased Pulmonary-Aortic Interspace in Fetal Right Aortic Arch: A Matched Case-Control Study.

Fetal diagnosis and therapy
2024

A 15-Year Experience With Total Anomalous Pulmonary Venous Connection in Vietnam.

World journal for pediatric & congenital heart surgery
2024

Major issues for supraventricular tachycardia ablation in patients with persistent left superior vena cava.

Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing
2024

Developmental or Procedural Vena Cava Interruption and Venous Thromboembolism: A Review.

Seminars in thrombosis and hemostasis
2024

Optimal Pacemaker Implantation in Patients with Isolated Persistent Left Superior Vena Cava.

Internal medicine (Tokyo, Japan)
2024

Author's reply to Optimal Pacemaker Implantation in Patients with Isolated Persistent Left Superior Vena Cava.

Internal medicine (Tokyo, Japan)
2025

Intracavitary electrocardiogram guidance for peripherally inserted central catheter placement in a patient with persistent left superior vena cava: A case report.

The journal of vascular access
2023

The Sinus Venosus Veno-Venous Bridge: Not a septal defect.

Sultan Qaboos University medical journal
2023

Device Closure of Superior Sinus Venosus Atrial Septal Defects: A single centre experience.

Sultan Qaboos University medical journal
2023

Adrenal Vein Sampling in the Management of Primary Aldosteronism: The Added Value of Intraprocedural Cortisol Assessment.

International journal of endocrinology
2024

A novel genotype-phenotype between persistent-cloaca-related VACTERL and mutations of 8p23 and 12q23.1.

Pediatric research
2024

Prenatal Detection and Postnatal Outcome of Persistent Left Superior Vena Cava and Agenesis of Ductus Venosus Associated with Postnatal Bovine Aortic Arch.

Zeitschrift fur Geburtshilfe und Neonatologie
2024

Persistant Left Superior Vena Cava with and Without Right Superior Vena Cava: Significance of Prenatal Diagnosis.

Pediatric cardiology
2023

A Rare Case of Lung Hypoplasia, Cardiac Anomalies and Ovarian Tumour in a Patient with Mayer-Rokitansky-Küster-Hauser Syndrome.

Sultan Qaboos University medical journal
2023

A practical method for prenatal diagnosis of anal atresia by second trimester ultrasound screening - A retrospective study.

Taiwanese journal of obstetrics & gynecology
2024

A case of novel combined variations of the renal and testicular vessels.

Folia morphologica
2023

Radiofrequency ablation of persistent atrial fibrillation in a patient with situs inversus totalis and interrupted inferior vena cava.

Journal of cardiovascular electrophysiology
2024

Interrupted inferior vena cava draining into the coronary sinus associated with circumflex artery to coronary sinus fistula.

Current problems in cardiology
2023

Integrated analysis of comorbidity, pregnant outcomes, and amniotic fluid cytogenetics of fetuses with persistent left superior vena cava.

World journal of cardiology
2023

Transcatheter Closure of Superior Sinus Venosus Defects.

JACC. Cardiovascular interventions
2024

Persistent 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.

Internal medicine (Tokyo, Japan)
2023

Fetal de novo heterozygous variant in the isocitrate dehydrogenase 1 gene associated with growth restriction, skeletal, cerebral and vascular anomalies.

Prenatal diagnosis
2023

Left main coronary artery aneurysm with fistula to superior vena cava: A challenging case.

Annals of cardiac anaesthesia
2024

Left Superior Vena Cava Draining to Left Atrium: A Case Report, Review of the Literature, and Classification.

Pediatric cardiology
2023

Giant right atrial aneurysm associated with persistent left cranial vena cava in a cat.

Journal of veterinary cardiology : the official journal of the European Society of Veterinary Cardiology
2023

Surgical treatment of anomalous right upper lobe pulmonary vein obstruction caused by compression between pulmonary artery and trachea: a case report.

Cardiology in the young
2024

Absent Superior Vena Cava: An Uncommon Cause of Anomalous Systemic Venous Return.

World journal for pediatric & congenital heart surgery
2022

Unusual Left Superior Vena Cava, Connected to the Left Atrium via the Left Superior Pulmonary Vein.

Archives of Iranian medicine
2023

Prenatal diagnosis of Myhre syndrome with a heterozygous pathogenic variant in SMAD4 gene presented with thick nuchal translucency and cardiac abnormalities.

Prenatal diagnosis
2024

Catheter ablation of the left-sided variant of right top pulmonary vein in a case with persistent left superior vena cava.

Pacing and clinical electrophysiology : PACE
2023

A missense variant at the RAC1-PAK1 binding site of RAC1 inactivates downstream signaling in VACTERL association.

Scientific reports
2023

Stroke 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.

Circulation. Cardiovascular imaging
2023

Biomechanical performance of the Bicaval Transcatheter System for the treatment of severe tricuspid regurgitation.

Frontiers in bioengineering and biotechnology
2023

Concomitant Coronary Artery Disease and Persistent Left Superior Vena Cava in a Patient Presenting With Symptomatic Sinus Bradycardia: A Case Report.

Cureus
2023

Isolated persistent left superior vena cava associated with anomalous left hepatic vein drainage into the right atrium accidentally discovered after sternotomy.

Kardiologia polska
2023

Anterior and Posterior Nutcracker Syndrome Combined with May-Thurner Syndrome: First Report of This Unique Case.

Diagnostics (Basel, Switzerland)
2023

Modification of the Warden Procedure for Surgical Repair of Partial Anomalous Pulmonary Venous Connection.

World journal for pediatric & congenital heart surgery
2023

Incidental finding of a bilateral absence of the superior vena cava.

Clinical case reports
2024

Cardiovascular Manifestations of Turner Syndrome: Phenotypic Differences Between Karyotype Subtypes.

Pediatric cardiology
2023

Bilateral inferior venae cava combined with the persistent left superior vena cava and hemiazygos continuation of left inferior vena cava with drainage into right atrium: A case report.

Echocardiography (Mount Kisco, N.Y.)
2023

[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 genetics
2023

Quantitative measurements and morphological evaluation of fetal cardiovascular structures with fetal cardiac MRI.

European journal of radiology
2023

Persistent Left Superior Vena Cava: Rare Location of Hemodialysis Catheter.

Cureus
2023

Ascites in Animals With Right Heart Failure: Correlation With Lymphatic Dysfunction.

Journal of the American Heart Association
2023

Multiple accessory pathways coexisting with a persistent left superior vena cava: a case report.

Journal of medical case reports
2023

Repair of Partial Anomalous Pulmonary Venous Connection to the Azygous Vein via Right Thoracotomy.

World journal for pediatric & congenital heart surgery
2023

High 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 Gynecology
2023

Heart Transplant Using a Donor Heart With Only a Persistent Left Superior Vena Cava: A Case Report.

Transplantation proceedings
2023

Identifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.

World journal for pediatric & congenital heart surgery
2023

Subjective and Objective Sonographic Assessment for the Prenatal Detection of Neonatal Coarctation of the Aorta.

Fetal diagnosis and therapy
2023

Multiple 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.)
2023

Long-Term Surgical Outcomes of Patients With Isomeric Right and Left Atrial Appendages.

World journal for pediatric & congenital heart surgery
2023

Inferior Vena Cava Agenesis: An Underrated Cause of Deep Venous Thrombosis.

Cureus
2024

Malposition of the central venous catheter secondary to accessory hemiazygos vein variant.

The journal of vascular access
2023

Ultrasound 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 Echocardiography
2023

Pre-procedural imaging and atrial tachycardia ablation in a patient with complex congenital heart disease.

Pacing and clinical electrophysiology : PACE
2023

Coronary Sinus Draining Into the Right Superior Vena Cava: A Rare Anomaly.

JACC. Cardiovascular interventions
2023

Computed tomography and magnetic resonance imaging of congenital thoracic systemic venous anomalies.

Pediatric radiology
2022

Anomalous Connection of Superior Vena Cava to Left Atrium-A Rare Congenital Cardiac Anomaly and Its Implications.

A&A practice
2023

Persistent left superior vena cava in a patient with an implanted dual-chamber pacing system in atrial fibrillation with tachycardia-bradycardia syndrome.

Bratislavske lekarske listy
2022

Endobronchial Ultrasound Diagnosis of a Malignant Superior Vena Cava Tumor Thrombus Extending Into the Right Atrium: An Unusual Cause of Recurrent Syncope.

Cureus
2023

Primary sutureless repair concomitant with the Warden procedure.

Asian cardiovascular & thoracic annals
2024

Mal-positioning of a dialysis catheter: One lumen with arterial blood and the other with venous blood?

The journal of vascular access

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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. Rebuttal: aortic coarctation and concomitant anomalies - left circumflex fistula, persistent left SVC and ventricular diverticulum.
    BMJ case reports· 2026· PMID 41734959mais citado
  2. Unroofed coronary sinus and persistent left superior vena cava: a rare neonatal venous anomaly.
    BMJ case reports· 2026· PMID 41611327mais citado
  3. Congenital absence of the portal vein identified following the onset of esophageal-gastric variceal rupture.
    Clinical journal of gastroenterology· 2026· PMID 41313388mais citado
  4. Retrocardiac Innominate Vein: A Rare Vascular Anomaly Associated With Congenital Heart Disease.
    Journal of the Saudi Heart Association· 2026· PMID 41847120mais citado
  5. Polysplenia syndrome complicated by multiple intrahepatic bile duct stones in an adult: a case report.
    Frontiers in medicine· 2026· PMID 41783070mais citado
  6. 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.
    Ann Card Anaesth· 2026· PMID 41543421recente
  7. Interatrial Septum Assessment Part 2: Sinus Venosus Defects.
    Methodist Debakey Cardiovasc J· 2026· PMID 41542142recente
  8. Transcatheter resolution of a post-surgical "Iatrogenic" circular shunt in a child.
    Cardiol Young· 2025· PMID 41527731recente
  9. Intraoperative recognition of persistent left superior vena cava during right internal jugular central line placement in mitral valve replacement: a case report.
    J Med Case Rep· 2025· PMID 41353337recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:99114(Orphanet)
  2. MONDO:0020445(MONDO)
  3. GARD:19657(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. 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

Agenesia da veia cava superior, direita
Compêndio · Raras BR

Agenesia da veia cava superior, direita

ORPHA:99114 · MONDO:0020445
CID-10
Q26.8 · Outras malformações congênitas das grandes veias
CID-11
MedGen
UMLS
C0265929
EuropePMC
Wikidata
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