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Anomalia congênita da veia cava superior
ORPHA:95498CID-11 · LA86.YDOENÇA RARA

O coração é um órgão muscular presente nos humanos e em outros animais que bombeia o sangue através dos vasos sanguíneos do sistema circulatório. O sangue fornece ao corpo oxigénio e nutrientes e ajuda a eliminar resíduos metabólicos. Nos humanos, o coração situa-se na cavidade torácica entre os pulmões, num espaço denominado mediastino.

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Introdução

O que você precisa saber de cara

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Anomalia congênita da veia cava superior é uma condição rara onde a veia cava superior não se forma corretamente durante o desenvolvimento fetal. Isso pode levar a alterações no fluxo sanguíneo para o coração, com ou sem sintomas.

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SUS: Cobertura mínimaScore: 20%
Centros em: PA, PE, BA, CE, PB +10
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Sinais e sintomas

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

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Anos de pesquisa2
Últimos 10 anos200publicações
Pico2025102 papers
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2024Hoje · 2026📈 2025Ano 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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Nenhum gene associado encontrado

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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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Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Anomalia congênita da veia cava superior

Centros de Referência SUS

24 centros habilitados pelo SUS para Anomalia congênita da veia cava superior

Centros para Anomalia congênita da veia cava superior

Detalhes dos centros

Hospital Universitário Prof. Edgard Santos (HUPES)

R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital Infantil Albert Sabin

R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876

Serviço de Referência

Rota
Anomalias CongênitasDeficiência Intelectual

Hospital de Apoio de Brasília (HAB)

AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)

Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital das Clínicas da UFG

Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital Universitário da UFJF

R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442

Atenção Especializada

Rota
Anomalias Congênitas

Hospital das Clínicas da UFMG

Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Julio Müller (HUJM)

R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092

Atenção Especializada

Rota
Anomalias Congênitas

Hospital Universitário João de Barros Barreto

R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Lauro Wanderley (HULW)

R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470

Atenção Especializada

Rota
Anomalias Congênitas

Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)

R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Pequeno Príncipe

R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805

Serviço de Referência

Rota
Anomalias CongênitasDeficiência Intelectual

Hospital Universitário Regional de Maringá (HUM)

Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108

Atenção Especializada

Rota
Anomalias Congênitas

Hospital de Clínicas da UFPR

R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Pedro Ernesto (HUPE-UERJ)

Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)

Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital São Lucas da PUCRS

Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital de Clínicas de Porto Alegre (HCPA)

Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário da UFSC (HU-UFSC)

R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital das Clínicas da FMUSP

R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital de Base de São José do Rio Preto

Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798

Atenção Especializada

Rota
Anomalias Congênitas

Hospital de Clínicas da UNICAMP

R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital de Clínicas de Ribeirão Preto (HCRP-USP)

R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

UNIFESP / Hospital São Paulo

R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo
Sobre os centros SUS: Estes centros são habilitados pelo Ministério da Saúde como Serviços de Referência em Doenças Raras ou Serviços de Atenção Especializada. O atendimento é pelo SUS, com encaminhamento da rede de atenção básica.

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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

Distal coronary sinus-left atrial fistulous communication uncovered during atrial fibrillation ablation.

European heart journal. Case reports2026 Feb

Congenital anomalies of the coronary sinus (CS) are rare and often overlooked on standard imaging. Among these, the unroofed coronary sinus (URCS) is the most recognized, typically involving the proximal CS and frequently associated with a persistent left superior vena cava. In contrast, fistulous communications between the distal CS and the left atrium (LA) are exceptionally uncommon. Their recognition is important during atrial fibrillation (AF) ablation, where unanticipated channels may complicate catheter manipulation and lesion delivery. A 76-year-old woman with recurrent paroxysmal AF underwent catheter ablation. A lumen-equipped multipolar catheter was positioned in the CS, and transseptal access to the LA was achieved. During electroanatomical mapping with a high-density catheter, entanglement with the CS catheter suggested an anomalous connection. Contrast injection through the CS catheter opacified the left atrial appendage (LAA), and selective angiography revealed simultaneous filling of the LAA and the left inferior pulmonary vein (LIPV), confirming a fistulous tract between the distal CS, LAA, and LIPV. Pulmonary vein isolation was completed without complication. Retrospective review of pre-procedural computed tomography demonstrated a connecting vein from the distal CS to the LA at the LAA-LIPV ridge, corroborating the procedural findings. This case highlights a rare distal CS-LA fistulous communication involving the LAA and LIPV. It broadens the anatomical spectrum between URCS and partial anomalous pulmonary venous connection. Recognition of these anomalies is critical for procedural safety during AF ablation, underscoring the value of multimodality imaging and intra-procedural venography.

#3

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.

#4

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.

#5

The Selection of an Interposition Graft for Portal Vein Reconstruction: Maximizing the Availability of Autologous Vessels.

Pediatric transplantation2026 Mar

Portal vein (PV) reconstruction is a crucial step in pediatric liver transplantation (LT). Pediatric recipients with a hypoplastic or sclerotic PV often require an interposition vein graft. Allogeneic grafts from donors are generally preferred. However, if they are not available, the use of autologous vessels is necessary. An 8-month-old girl with biliary atresia (BA) underwent living donor LT with a left lateral segment graft. During the operation, portal vein thrombosis (PVT) developed after direct anastomosis between the recipient PV and graft left PV. We used several techniques to resolve this problem, including ligation of collateral circulation and use of interposition grafts from the left internal jugular vein (IJV) and left renal vein (LRV). On postoperative day 1, PVT reoccurred. Emergency exploratory laparotomy was performed for thrombectomy, using the retrohepatic inferior vena cava (IVC) as an additional vascular graft. The final reconstruction successfully utilized a combination of LRV and IVC grafts between the superior mesenteric vein and graft left PV. Postoperatively, the patient received thrombolytic therapy followed by anticoagulants for thrombus prevention, along with immunosuppressive drugs. The patient's postoperative clinical course was uneventful. Although the IJV and LRV are established options for PV reconstruction in pediatric LT, we propose retrohepatic IVC as a feasible and effective alternative option. This approach maximizes the availability of autologous vessels; however, selection must be individualized based on graft availability and the patient condition.

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The Selection of an Interposition Graft for Portal Vein Reconstruction: Maximizing the Availability of Autologous Vessels.

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

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Persistent Double Dorsal Aorta and Complex Congenital Heart Disease - A Case Report.

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Feasibility of Left Bundle Branch Area Pacing via a Persistent Left Superior Vena Cava in a Patient with Repaired Ventricular Septal Defect.

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2026

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

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2026

Late Diagnosis of Unroofed Coronary Sinus Associated with Persistent Left Superior Vena Cava in Patient with Repaired Tetralogy of Fallot: Case Report.

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2026

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

BMJ case reports
2026

Distal coronary sinus-left atrial fistulous communication uncovered during atrial fibrillation ablation.

European heart journal. Case reports
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

Plasma non-targeted metabolomics unveils the metabolic signatures of trans-right ventricle in pulmonary arterial hypertension associated with atrial septal defect.

Clinica chimica acta; international journal of clinical chemistry
2025

Double Superior Vena Cava and Coexisting Vascular Variants: A Case Report and Review of Clinical Implications.

Cureus
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
2026

Incidental Detection of Persistent Left Superior Vena Cava During Lung Perfusion Scan.

Journal of nuclear medicine technology
2025

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

Cardiology in the young
2025

Cardiovascular phenotypes of children and adolescents with Turner syndrome from a single-center cohort study.

Orphanet journal of rare diseases
2025

Virtual Reality to Enhance Understanding of Congenital Heart Disease.

Journal of cardiovascular development and disease
2025

Inadvertent Left Superior Vena Cava Catheterization via the Axillary Vein in a Patient With Persistent Left Superior Vena Cava.

Case reports in medicine
2025

Isolated Persistent Left Superior Vena Cava Causing Hemodialysis Catheter Dysfunction: An Uncommon Challenge in Vascular Access.

Cureus
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A Case of Right-Sided Heart Failure and Severe Pulmonary Hypertension Secondary to Partial Anomalous Pulmonary Venous Connection.

Respirology case reports
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
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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
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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
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Percutaneous Fontan by inferior vena cava banding and subsequent creation of an intraatrial stent-conduit: a clinical renaissance of a less invasive approach?

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

Pediatric cardiology
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Preserving Pathways: Strategies for Left Superior Vena Cava Management in Pediatric Cardiac Surgery.

Annals of thoracic surgery short reports
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Prenatal Diagnosis of a Rare Congenital Right Subclavian Artery-Superior Vena Cava Fistula.

The journal of obstetrics and gynaecology research
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Prenatal Diagnosis of Joubert Syndrome 23 With Left Isomerism: A Novel Phenotype Associated With Pathogenic KIAA0586 Variant.

Prenatal diagnosis
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Beyond the murmur: unmasking a rare sinus venosus ASD in an adolescent with chronic respiratory infections: A case report.

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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
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Echocardiographic characteristics of 14 cases with uncommon manifestation of Ebstein anomaly in the fetus: a case series report.

Archives of gynecology and obstetrics
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Pulse Field Ablation for Persistent Atrial Fibrillation: Targeting the Persistent Superior Vena Cava.

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

Journal of medical case reports
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A Rare Congenital Triad in an Adult With Coronary Artery Disease: A Case Report From Georgia.

Cureus
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Optimising pre-operative assessment in double-outlet right ventricle: the complementary role of echocardiography and CT angiography.

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

Journal of cardiovascular computed tomography
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A novel NOTCH1 nonsense variant in a bicuspid aortic valve family with intrafamilial clinical heterogeneity.

BMC cardiovascular disorders
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Whole-heart magnetic resonance imaging in 1 minute: Three-dimensional fast cardiac magnetic resonance imaging technique employing the non-Cartesian ultrashort echo time sequence.

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance
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Persistent Left Superior Vena Cava in the Setting of Granulicatella adiacens Bactermia and Crescentic Glomerulonephritis.

Journal of investigative medicine high impact case reports
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[Minimally Invasive Cardiac Surgery for Partial Anomalous Pulmonary Venous Connection and Sinus Venosus Atrial Septal Defect].

Kyobu geka. The Japanese journal of thoracic surgery
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Interventional Partial Cavopulmonary Connection: Initial Clinical Experience With Acute Procedural up to Midterm Results.

Journal of the American Heart Association
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Impact of Persistent Left Superior Vena Cava on Outcomes With Atrial Fibrillation Ablation.

Pacing and clinical electrophysiology : PACE
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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
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Persistent Left Superior Vena Cava: Implications for Long-Term Arteriovenous Fistula Patency and Potential Cardiac Complications.

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

Cureus
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Interventional treatment for azygos vein steal syndrome after bidirectional Glenn procedure in cyanotic congenital heart disease: a retrospective study.

PeerJ
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Impact of Fetal Diagnosis on the Management of Right Superior Vena Cava to Left Atrium.

JACC. Case reports
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Mitral and tricuspid valve surgery in an adult with aberrant left brachiocephalic vein: a case report.

Journal of surgical case reports
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Plasma non-targeted metabolomics unravels the metabolic features of normal trans-right heart.

Metabolomics : Official journal of the Metabolomic Society
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Multidisciplinary Approach to Closure of a Late-Presenting Large Coronary Artery Fistula.

JACC. Case reports
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Double Aortic Arch, Double SVC, and Coronary Artery Anomaly in a 38-Year-Old Woman.

JACC. Case reports
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Isolated subpulmonary ventricular assist device support for failing Fontan circulation in hypoplastic left heart syndrome.

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

Cardiology in the young
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Resynchronization Therapy in a Coronary Venous Sinus Anomaly.

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

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

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

Clinical imaging
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Thoracic Vascular Anomalies: Insights from Embryology and Imaging.

Radiographics : a review publication of the Radiological Society of North America, Inc
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A case of fatal infective endocarditis in uncorrected tetralogy of Fallot with persistent left superior vena cava.

Journal of forensic sciences
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Supracardiac total anomalous pulmonary venous drainage with right-sided vertical vein draining into the proximal superior vena cava.

Indian journal of thoracic and cardiovascular surgery
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Duplicated superior vena cava in a patient with tetralogy of Fallot: A rare vascular anomaly.

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

Australian veterinary journal
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Characterizing fetoplacental response to acute maternal hyperoxygenation in suspected coarctation of aorta using fetal cardiac MRI.

Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
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Prenatal diagnosis of absent right with persistent left superior vena cava: a case series.

Pediatric cardiology
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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
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Atrial Fistulas as Unusual Cause of Paradoxical Embolism: Prevalence and Percutaneous Treatment.

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions
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Partial anomalous pulmonary venous return: diagnostic challenges and successful correction in a patient with progressive pulmonary hypertension.

BMJ case reports
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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
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Unexpected migraine improvement following surgical ligation of the right superior vena cava drainage into the left atrium: a case report.

European heart journal. Case reports
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Telangiectasia in the Distribution of the Superior Vena Cava: A Novel Dermatological Manifestation of 22q11.2 Deletion Syndrome.

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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
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Duplication of the umbilical vein along with an extrahepatic, intrathoracic right umbilical vein in the setting of Taussig-Bing anomaly with atrioventricular septal defect.

Indian journal of thoracic and cardiovascular surgery
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Relationship Between the Pulmonary-to-systemic-blood-flow Ratio and the Cross-sectional Area of the Right Internal Jugular Vein in Pediatric Patients With Congenital Heart Disease.

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

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

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Percutaneous repair of biatrial drainage of the right superior vena cava using a covered stent.

International journal of cardiology. Congenital heart disease
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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
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Acute thrombosis of an atrial flow regulator in a failing Fontan circulation: recanalization and management.

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

La Revue de medecine interne
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Kawashima procedure in single ventricle palliation: the factors affecting clinical outcomes.

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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

Transjugular Intrahepatic Portosystemic Shunt Creation in Isolated Persistent Left Superior Vena Cava and Portal Vein Thrombosis.

Interventional radiology (Higashimatsuyama-shi (Japan)
2025

Role of sequential functional echocardiography in predicting clinically apparent patent ductus arteriosus in preterm very low birth weight newborns: An observational study.

Journal of neonatal-perinatal medicine
2025

Prenatal ultrasound prediction of coarctation of the aorta: a nomogram for risk stratification.

Pediatric radiology
2025

Rare Anomalous Left Brachiocephalic Venous Drainage Affecting Pacemaker Placement.

JACC. Case reports
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

Duplication of the inferior vena cava: Case report of an incidental radiological finding.

Radiology case reports
2025

Persistent Levoatriocardinal Vein in Association with Complete Duplication of SVC and IVC.

The Indian journal of radiology & imaging
2025

An Alternative Technique of Performing the Bidirectional Superior Cavopulmonary Connection without Cardiopulmonary Bypass.

World journal for pediatric & congenital heart 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

Highly accelerated 4D flow MRI with respiratory compensation and cardiac view sharing: a cross-sectional study of flow in the great vessels of pediatric congenital heart disease.

Pediatric radiology
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

Minimally invasive anterior thoracotomy approach for complex sinus venosus atrial septal defect with anomalous pulmonary venous return: A case report.

International journal of surgery case reports
2025

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

Brazilian journal of cardiovascular surgery
2025

Management of Cardiovascular Health Issues in Turner Syndrome: Expert Insights and Expanded Recommendations From the 2024 Guideline Development Team.

American journal of medical genetics. Part C, Seminars in medical genetics
2025

Evaluation of Three-Vessel View Intervessel Distance Measurements in Fetuses Without Congenital Cardiac Defects.

Journal of clinical ultrasound : JCU
2025

Persistent Left Superior Vena Cava in Pacemaker Implantation for Digoxin-Induced Complete Heart Block: Clinical Considerations and Challenges.

Clinical case reports
2025

Transvenous Dual-Chamber Pacemaker Implantation in a Patient with Persistent Left Superior Vena Cava Undergoing Maintenance Hemodialysis.

International medical case reports journal
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
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

Isolated right superior vena cava drainage into the left atrium.

Indian journal of thoracic and cardiovascular surgery
2025

Transthoracic Echocardiography for Central Venous Catheter Tip Positioning in Children: An Observational Study Using Transesophageal Echocardiography as Reference Method.

Paediatric anaesthesia
2025

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

Radiology case reports
2026

Iatrogenic Post-Surgical Cyanosis Due to Vena Caval Redirection to the Left Atrium and Its Transcatheter Management.

Pediatric cardiology
2025

Left-Sided Atrioventricular Valve Regurgitation After the Repair of Complete Atrioventricular Septal Defect.

Pediatric cardiology
2024

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

Physiological reports
2025

Thoracoscopic closure of atrial septal defect in perfused beating hearts.

Surgical endoscopy
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

Experience and Results of Liver Transplantation in Patients With Alagille Syndrome at Our Center.

Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation
2024

Incidental Finding of a Persistent Left Superior Vena Cava During Permanent Dual-Chamber Pacemaker Implantation: A Case Report.

Cureus
2024

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

Asian cardiovascular & thoracic annals
2024

Special considerations for the stabilization and resuscitation of patients with cardiac disease in the Neonatal Intensive Care Unit.

Seminars in perinatology
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
2024

Right-to-left-shunts in patients scheduled for neurosurgical intervention in semi-sitting position - a literature review based on two case scenarios.

BMC anesthesiology
2024

Complexities in Managing Wolf-Parkinson-White Syndrome: A Case of Congenital Inferior Vena Cava Anomaly With Azygos Continuation.

Cureus
2024

Homograft implant for prosthetic aortic endocarditis with paravalvular abscess in a patient with persistent left superior vena cava.

Multimedia manual of cardiothoracic surgery : MMCTS
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

Persistent Left Superior Vena Cava: Unanticipated Stumbling Block in Cardiac Resynchronization Therapy.

Cureus
2024

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

Molecular cytogenetics
2024

Persistent Left-Sided Superior Vena Cava: A Case Report.

Cureus
2024

Persistent left cranial vena cava and right cranial vena cava aplasia in a French bulldog and a Cavalier King Charles spaniel with severe pulmonic stenosis.

Journal of veterinary cardiology : the official journal of the European Society of Veterinary Cardiology
2024

Management of Persistent Left Superior Vena Cava: Overcoming Lead and Vascular Complications With Transcatheter Pacing System Implantation.

Cureus
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

Unintended Sequalae of Long-Term Transvenous Pacemaker Leads in a Patient With Congenital Heart Disease.

World journal for pediatric & congenital heart surgery
2024

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

Pacing and clinical electrophysiology : PACE
2025

Maternal exposure to ambient ozone and fetal congenital heart defects: a national multicenter study in China.

Journal of exposure science & environmental epidemiology
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

Left superior vena cava's unconventional path to left atrium drainage: A case report.

Radiology case reports
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

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

A Comparison of Perinatal Circulatory Transition in Critical Right and Left Heart Obstructive Lesions.

Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography
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

Transcatheter Fontan completion: Creation of an extracardiac Fontan.

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

Outcomes Following Surgical Repair of Sinus Venosus Atrial Septal Defects: A Systematic Review and Meta-Analysis.

Journal of the American Heart Association
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

Transcatheter Covered Stent Exclusion of Superior Sinus Venosus Defects.

Journal of the American College of Cardiology
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

A case report of superior vena cava/right coronary artery fistula secondary to chronic endocarditis.

European heart journal. Case reports
2024

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

Case reports in pulmonology
2024

Lead Extraction and Baffle Stenting in a Patient with Transposition of the Great Arteries.

Cardiac electrophysiology clinics
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

Intracranial Pressure and Cerebral Hemodynamics in Infants Before and After Glenn Procedure.

Critical care explorations
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 Coarse-Fine Collaborative Learning Model for Three Vessel Segmentation in Fetal Cardiac Ultrasound Images.

IEEE journal of biomedical and health informatics
2024

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

Journal of cardiothoracic surgery
2024

Impact of Age-Related Change in Caval Flow Ratio on Hepatic Flow Distribution in the Fontan Circulation.

Circulation. Cardiovascular imaging
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

Fetal Hemodynamics, Early Survival, and Neurodevelopment in Patients With Cyanotic Congenital Heart Disease.

Journal of the American College of Cardiology
2024

Impact of 4D-Flow CMR Parameters on Functional Evaluation of Fontan Circulation.

Pediatric cardiology
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

Association between patent ductus arteriosus flow and home oxygen therapy in extremely preterm infants.

Pediatric research
2024

Clinical implication of persistent left superior vena cava isolation for atrial fibrillation: Author's reply.

Pacing and clinical electrophysiology : PACE
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

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

Complete free wall isolation of arrhythmogenic persistent left superior vena cava.

Journal of cardiovascular electrophysiology
2024

Prenatal Diagnosis and Postnatal Outcomes of Persistent Left Superior Vena Cava Associated With Mild Narrow Aorta: A Cohort Study.

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

A successful cardiac transplant in a patient with situs inversus totalis and congenitally corrected transposition of the great arteries.

Multimedia manual of cardiothoracic surgery : MMCTS
2024

Usefulness of perioperative transoesophageal echocardiography during paediatric cardiac surgery.

Archives of cardiovascular diseases
2024

[An unusual comeback: a case report of persistent left superior vena cava].

Giornale italiano di cardiologia (2006)
2024

A short RP tachycardia in congenitally corrected transposition. What is the mechanism?

Pacing and clinical electrophysiology : PACE
2024

Extremely Long-Term Follow-Up of Dr William Glenn's Original Patient Cohort With Superior Cavopulmonary Anastomosis (1958-1990).

World journal for pediatric & congenital heart surgery
2024

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

World journal for pediatric & congenital heart surgery
2024

Persistent Left Superior Vena Cava with Hemiazygos Continuation of Left Inferior Vena Cava.

Radiology

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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. Distal coronary sinus-left atrial fistulous communication uncovered during atrial fibrillation ablation.
    European heart journal. Case reports· 2026· PMID 41669320mais citado
  3. Unroofed coronary sinus and persistent left superior vena cava: a rare neonatal venous anomaly.
    BMJ case reports· 2026· PMID 41611327mais citado
  4. Congenital absence of the portal vein identified following the onset of esophageal-gastric variceal rupture.
    Clinical journal of gastroenterology· 2026· PMID 41313388mais citado
  5. The Selection of an Interposition Graft for Portal Vein Reconstruction: Maximizing the Availability of Autologous Vessels.
    Pediatric transplantation· 2026· PMID 41872734mais 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:95498(Orphanet)
  2. MONDO:0019829(MONDO)
  3. GARD:19275(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55788903(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

Anomalia congênita da veia cava superior
Compêndio · Raras BR

Anomalia congênita da veia cava superior

ORPHA:95498 · MONDO:0019829
CID-11
MedGen
UMLS
C0265928
Wikidata
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