Raras
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Síndrome Scimitar
ORPHA:185CID-10 · Q26.8CID-11 · LA86.22DOENÇA RARA

A síndrome da cimitarra é caracterizada por uma combinação de anomalias cardiopulmonares, incluindo conexão de retorno venoso pulmonar anômalo parcial do pulmão direito à veia cava inferior, levando à criação de um shunt da esquerda para a direita.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A síndrome da cimitarra é caracterizada por uma combinação de anomalias cardiopulmonares, incluindo conexão de retorno venoso pulmonar anômalo parcial do pulmão direito à veia cava inferior, levando à criação de um shunt da esquerda para a direita.

Publicações científicas
791 artigos
Último publicado: 2026 Apr 17

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adolescent
+ adult, antenatal, childhood, infancy, neonatal
🏥
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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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

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

Partes do corpo afetadas

❤️
Coração
13 sintomas
🫁
Pulmão
11 sintomas
🦴
Ossos e articulações
1 sintomas
🫘
Rins
1 sintomas
🫃
Digestivo
1 sintomas

+ 12 sintomas em outras categorias

Características mais comuns

55%prev.
Dextrocardia
Frequente (79-30%)
55%prev.
Morfologia anormal do coração
Frequente (79-30%)
55%prev.
Hipoplasia da artéria pulmonar
Frequente (79-30%)
55%prev.
Morfologia pulmonar anormal
Frequente (79-30%)
55%prev.
Defeito do septo atrial
Frequente (79-30%)
55%prev.
Morfologia anormal da veia cava
Frequente (79-30%)
39sintomas
Frequente (11)
Ocasional (19)
Muito raro (9)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 39 características clínicas mais associadas, ordenadas por frequência.

Dextrocardia
Frequente (79-30%)55%
Morfologia anormal do coraçãoAbnormal heart morphology
Frequente (79-30%)55%
Hipoplasia da artéria pulmonarPulmonary artery hypoplasia
Frequente (79-30%)55%
Morfologia pulmonar anormalAbnormal lung morphology
Frequente (79-30%)55%
Defeito do septo atrialAtrial septal defect
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico791PubMed
Últimos 10 anos200publicações
Pico202572 papers
Linha do tempo
2026Hoje · 2026🧪 2019Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Síndrome Scimitar

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

Nenhum ensaio clínico registrado para esta condição.

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Publicações mais relevantes

Timeline de publicações
286 papers (10 anos)
#1

Exercise Capacity and Pulmonary Function in Pediatric Patients With Anomalous Pulmonary Venous Connection Post-Surgical Repair: A Retrospective Analysis.

The Kaohsiung journal of medical sciences2026 Feb

Anomalous pulmonary venous connection (APVC), including total (TAPVC) and partial (PAPVC) forms, is a congenital heart defect with abnormal pulmonary vein drainage; and while surgical repair has improved survival, its long-term impact on cardiopulmonary function remains unclear. This retrospective study evaluated exercise capacity and pulmonary function in 26 pediatric APVC patients (17 TAPVC, 9 PAPVC) using cardiopulmonary exercise testing (CPET) and compared them with 63 age-matched healthy controls. Patients with complex defects or significant comorbidities were excluded. Results showed significantly lower anaerobic threshold VO2 (p = 0.03), peak VO2 (p < 0.001) and peak heart rate (p = 0.02) in the APVC group, indicating impaired exercise capacity; though no differences were found between TAPVC and PAPVC subgroups. Despite preserved resting lung function, these findings suggest that children with repaired APVC experience persistent exercise limitations, underscoring the importance of routine functional assessment and potential rehabilitation, with further studies needed to clarify underlying mechanisms and guide long-term care.

#2

Partial Anomalous Pulmonary Venous Return in Lung Cancer Surgery: Diagnostic Challenges and Surgical Considerations in Two Cases.

The Tokai journal of experimental and clinical medicine2026 Apr 20

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more pulmonary veins drain into the systemic venous system instead of the left atrium, in the absence of associated cardiac defects such as atrial septal defect. When only a single anomalous pulmonary vein is involved, the resulting shunt volume is typically small, allowing asymptomatic progression into advanced age. Consequently, such cases are often incidentally diagnosed or remain undetected. We report herein two cases of PAPVR that required pulmonary resection for primary or metastatic lung cancer. In Patient 1, PAPVR was successfully identified preoperatively using three-dimensional computed tomography in the lobe with cancer, enabling appropriate surgical planning for lobectomy. In Patient 2, segmentectomy was performed to preserve lung function: PAPVR was not recognized preoperatively on conventional two-dimensional computed tomography but only identified postoperatively, in a different lobe to the one with cancer. A deliberate awareness of the possible presence of PAPVR and accurate identification of PAPVR before surgery are essential in patients undergoing pulmonary resection. Segmentectomy may be a viable surgical option in select cases complicated by PAPVR because it potentially minimizes the reduction in pulmonary blood flow while preserving lung function.

#3

Scimitar Syndrome Incidentally Identified During the Workup for Acute Appendicitis in a Young Adult Female: A Case Report.

Cureus2026 Jan

While congenital pulmonary venolobar syndrome (CPVS), including Scimitar syndrome and horseshoe lung, is highly uncommon in adults, acute appendicitis is a common surgical emergency. This case report emphasizes the difficulties in diagnosing patients and the possibility of misdiagnosis, highlighting the significance of clinical awareness for the best possible care. A laparoscopic appendectomy was performed on a 21-year-old female patient who presented with symptoms of acute appendicitis. During the procedure, a suspicious cecal tumor and a subserosal, dilated retrocecal appendix were observed. The patient was identified as having right lung hypoplasia, cardiac dextroposition, and partial anomalous pulmonary venous return (PAPVR), indicating the presence of Scimitar syndrome along with horseshoe lung. These findings were confirmed by post-operative CT imaging. The appendix histopathology and colonoscopy were unremarkable, and the cardiac assessment, including the electrocardiogram and echocardiogram, was also normal. Incidental CPVS in adults requires diligent recognition for optimal management. This case contributes to the limited research on adult manifestations of Scimitar syndrome and associated abnormalities, which is expanded by this instance.

#4

Unmasking an Anomalous Vein: Surgical Repair of Left Upper Pulmonary Vein Drainage into the Innominate Vein.

Portuguese journal of cardiac thoracic and vascular surgery2026 Feb 17

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly characterized by one or more pulmonary veins draining into the systemic venous system. We present the case of a 51-year-old woman with PAPVR involving the left upper pulmonary vein (LUPV) draining into the left innominate vein via a vertical vein, diagnosed after investigation of progressive dyspnea and lower limb edema. The diagnosis was confirmed through contrast-enhanced CT and echocardiography. The patient underwent surgical correction of the anomalous return along with tricuspid valve repair. Postoperative recovery was uneventful. This case highlights the importance of recognizing PAPVR in adult patients presenting with unexplained right heart dilation or symptoms of right heart overload.

#5

Computational Predictive Modeling of Surgical Outcomes in Total Anomalous Pulmonary Venous Connection: Assessing the Impact of Pulmonary Venous Confluence Size on Preoperative Planning.

Journal of cardiovascular translational research2026 Feb 04

To develop a predictive model for optimal anastomosis sizing in TAPVC surgery, focusing on the role of pulmonary venous confluence (PVC) size. A patient-specific fluid-structure interaction (FSI) model simulated blood flow through various anastomosis sizes. Key variables included body weight, anastomosis length, and PVC size. The model's predictions were validated against postoperative echocardiographic measurements from nine TAPVC cases. A strong positive correlation was found between flow velocity and the ratio of body weight to anastomosis length and PVC circumference. Including PVC size significantly improved predictive accuracy. No significant difference was observed between predicted and measured velocities. PVC size is a critical factor for planning TAPVC surgery. Incorporating it into computational models enhances the prediction of flow dynamics and supports personalized surgical decision-making.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC616 artigos no totalmostrando 197

2026

Partial Anomalous Pulmonary Venous Return in Lung Cancer Surgery: Diagnostic Challenges and Surgical Considerations in Two Cases.

The Tokai journal of experimental and clinical medicine
2026

Scimitar Syndrome Incidentally Identified During the Workup for Acute Appendicitis in a Young Adult Female: A Case Report.

Cureus
2026

Unmasking an Anomalous Vein: Surgical Repair of Left Upper Pulmonary Vein Drainage into the Innominate Vein.

Portuguese journal of cardiac thoracic and vascular surgery
2026

Computational Predictive Modeling of Surgical Outcomes in Total Anomalous Pulmonary Venous Connection: Assessing the Impact of Pulmonary Venous Confluence Size on Preoperative Planning.

Journal of cardiovascular translational research
2025

Horseshoe Lung Associated with Left-sided Scimitar Syndrome in an Infant with Complex Congenital Heart Disease.

Journal of cardiovascular echography
2025

Don't Miss the Sword in the Lung: Unveiling a Previously Undiagnosed Congenital Syndrome in a Suspected Myocardial Infarction with Nonobstructive Coronary Arteries Patient.

Journal of cardiovascular echography
2026

Left Ventricular Rupture Due to Congenital Partial Defect of the Left Ventricular Free Wall.

Interdisciplinary cardiovascular and thoracic surgery
2026

Transcatheter Management of Atypical Scimitar Syndromes With Dual Venous Drainage.

JACC. Case reports
2025

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

Cardiology in the young
2025

Embolización de secuestro pulmonar en paciente con síndrome de cimitarra: reporte de caso.

Archivos de cardiologia de Mexico
2025

Ascending Vertical Vein Aneurysm.

Methodist DeBakey cardiovascular journal
2025

[Utility of Low-Dose CT Angiography in the Diagnosis of Neonatal Scimitar Syndrome].

RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin
2025

Total Anomalous Pulmonary Venous Connections.

Clinics in perinatology
2025

Cardiac Magnetic Resonance Imaging in the Evaluation and Management of Scimitar Syndrome.

Annals of thoracic surgery short reports
2025

Minimally Invasive Repair of Sinus Venosus Atrial Septal Defects and Anomalous Pulmonary Venous Connections via Vertical Right Axillary Thoracotomy.

Journal of cardiovascular development and disease
2025

[Agenesis of the left pulmonary artery with hypoplasia of the homolateral lung].

La Revue du praticien
2025

Practical Considerations for Endovascular Treatment of Pulmonary Sequestration-Induced Hemoptysis: Two Case Reports and Review of Literature.

Journal of the Korean Society of Radiology
2025

Scimitar Syndrome: A Rare Congenital Conundrum in a Child With Stridor.

Pediatric pulmonology
2025

'Congenitally corrected' Scimitar syndrome? An anomalous unilateral single pulmonary vein variant.

BMJ case reports
2025

Transcatheter correction of the scimitar variant with dual pulmonary venous drainage-an international multicentre series.

Cardiology in the young
2025

Congenital extrahepatic portosystemic shunt with partial anomalous pulmonary venous return and aneurysmal dilation of the pulmonary artery: successful management of a complex multisystem congenital anomaly.

Cardiology in the young
2025

The unconventional surgical management of an infant with rare Scimitar syndrome variant.

Cardiology in the young
2025

An Adult With Giant Vertical Vein in Total Anomalous Pulmonary Venous Connection Combined With Pulmonary Valve Stenosis.

Echocardiography (Mount Kisco, N.Y.)
2025

Left Atrial Mechanics in Isolated Total Anomalous Pulmonary Venous Connection after Repair.

Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography
2026

Exercise Capacity and Pulmonary Function in Pediatric Patients With Anomalous Pulmonary Venous Connection Post-Surgical Repair: A Retrospective Analysis.

The Kaohsiung journal of medical sciences
2025

Total Anomalous Pulmonary Venous Connection: A Case Series.

The Journal of the Association of Physicians of India
2025

Nationwide registry study of long term survival and comorbidities in total anomalous pulmonary venous connection in Denmark.

Scientific reports
2025

Successful treat-and-repair strategy in a 5 year old with partial anomalous pulmonary venous connection, atrial septal defect, and severe pulmonary hypertension.

Cardiology in the young
2025

MYRF gene mutation leading to coronary artery anomaly combined with 46,XY sex development disorder, a case report and literature review.

BMC pediatrics
2025

Hemi-Senning baffle for scimitar syndrome correction.

JTCVS techniques
2025

Scimitar syndrome: Updated follow-up of the multipatch technique.

JTCVS techniques
2025

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

Cardiovascular journal of Africa
2025

CRB2-Related Syndrome in 2 New Patients: Three Novel Variants.

Molecular syndromology
2025

The Long Shadow of Repair: Late-Onset Atrioventricular Block and Atrial Arrhythmias After Scimitar Syndrome and Mitral Annuloplasty.

Reports (MDPI)
2025

Modified double-decker repair for pediatric scimitar syndrome.

JTCVS techniques
2025

Ductus arteriosus obstruction induced by vertical vein stenting in single-ventricle physiology with supracardiac total anomalous pulmonary venous connection.

Cardiology in the young
2025

Infracardiac total anomalous pulmonary venous drainage with concomitant hypoplastic aortic arch in a preterm neonate: a rare and complex surgical challenge.

Cardiology in the young
2025

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

BMJ case reports
2025

The double scimitar sign.

Cardiology in the young
2025

Multimodality Imaging in Presurgical Evaluation of Total Anomalous Pulmonary Venous Connection: Single-Center Practice Variability and a Systematic Review of Diagnostic Error.

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

Atrial Fibrillation-Induced Cardiomyopathy in Scimitar Syndrome With Electrically Silent Anomalous Pulmonary Vein: An Adult Case Report.

CJC pediatric and congenital heart disease
2025

Postrepair Pulmonary Vein Stenosis: Addressing Anatomic and Patient Risk Factors to Improve Outcomes.

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual
2024

Neonatal Atrial Flutter: A Novel Triad for Immediate Electrocardiographic Diagnosis.

The journal of Tehran Heart Center
2025

Unveiling the Mystery of Horseshoe Lung: A Case Report.

Cureus
2025

Robotic approach for surgical correction of scimitar syndrome via direct anastomosis.

JTCVS techniques
2025

Prognostic factors in pediatrics TAPVC: a 10-year retrospective study.

Scientific reports
2025

Over 3,000 Minimally Invasive Thoracotomies From the European Congenital Heart Surgeons Association for Quality Repairs of the Most Common Congenital Heart Defects: Safe and Routine for Selected Repairs.

World journal for pediatric &amp; congenital heart surgery
2025

A "hybrid" approach to total anomalous pulmonary venous return repair.

Cardiology in the young
2025

A bleeding sword: Scimitar syndrome in a child with hematemesis due to esophageal varices - a case report.

Annals of medicine and surgery (2012)
2025

Delayed Diagnosis of Partial Anomalous Pulmonary Venous Return in an Adult With Pulmonary Hypertension.

Journal of investigative medicine high impact case reports
2025

Computed tomography for major aortopulmonary collateral arteries: a roadmap to unifocalized pulmonary arterial flow.

Pediatric radiology
2025

Scimitar Syndrome: Unveiling the Complexities of a rare Congenital Cardiopulmonary Anomaly.

European journal of case reports in internal medicine
2025

Prenatal diagnosis of a de novo 17q25.3 microdeletion encompassing RAC3 and CSNK1D in a fetus associated with partial agenesis of the corpus callosum, small brain volume, micrognathia and total anomalous pulmonary venous return.

Taiwanese journal of obstetrics &amp; gynecology
2025

Predicting the Need for Pulmonary Venous Reintervention in Total Anomalous Pulmonary Venous Connection: The Role of Preoperative Echocardiographic Metrics.

Echocardiography (Mount Kisco, N.Y.)
2025

Scimitar syndrome: A case report.

Radiology case reports
2025

Adult-type scimitar syndrome.

European heart journal. Cardiovascular Imaging
2025

A Case of Incomplete Scimitar Syndrome in a Quinquagenarian Male.

Cureus
2025

Total Anomalous Pulmonary Venous Drainage Caused by Malposition of Septum Primum in Adults.

Echocardiography (Mount Kisco, N.Y.)
2025

Comparison of different approaches applied for surgical correction of partial anomalous pulmonary venous connection.

Cardiology in the young
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

A Rare Case of Anomalous Pulmonary Venous Return.

Echocardiography (Mount Kisco, N.Y.)
2025

Total Anomalous Pulmonary Venous Return, Marfan Syndrome, and Severe Combined Immunodeficiency: A Lethal Combination?

World journal for pediatric &amp; congenital heart surgery
2025

Outcomes in Late Presenting Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection-Single Centre Experience.

World journal for pediatric &amp; congenital heart surgery
2025

The Role of Fetal Echocardiography in Predicting Postnatal Pulmonary Venous Obstruction in Neonates with TAPVR: A Single Center Experience.

Pediatric cardiology
2024

A Curious Case of Scimitar Syndrome That Defies Embryology.

Annals of thoracic surgery short reports
2025

Anatomy vs. shunt fraction: haemodynamic consequences in atrial septal defects and partial anomalous pulmonary venous connection-a comprehensive CMR study.

European heart journal. Cardiovascular Imaging
2024

Transcatheter Embolization in Congenital Cardiovascular Malformations-Variable Use of Vascular Plugs.

Cardiovascular therapeutics
2025

Percutaneous Transcatheter Approach to Partial Anomalous Pulmonary Venous Return: A Case Series.

The American journal of cardiology
2024

Factor Analysis of the Missed Diagnosis of Total Anomalous Pulmonary Venous Connection in Prenatal Echocardiography.

Birth defects research
2024

New Fetal Sonographic Marker to Rule Out Total Anomalous Pulmonary Venous Return: Color Doppler Drainage From Pulmonary Vein to Atrium to Ventricle (CDDVAV).

Echocardiography (Mount Kisco, N.Y.)
2025

Prenatal MR Diagnosis of Total Anomalous Pulmonary Venous Connection and Related Brain Growth Changes.

Journal of magnetic resonance imaging : JMRI
2025

Unveiling the anomaly: Scimitar syndrome in a middle-aged female patient.

Radiology case reports
2024

An explainable deep learning model to predict partial anomalous pulmonary venous connection for patients with atrial septal defect.

BMC pediatrics
2025

Single Meandering Right Pulmonary Vein With Potential Systemic Arterial Fistula: Serial Cardiac MRI Assessment With 4D Flow Characterization.

Circulation. Cardiovascular imaging
2025

Analysis and Visualization of Inter-/Intra-surgeon Variability Using Surgical Simulation for Partial Anomalous Pulmonary Venous Connection Repair.

Pediatric cardiology
2024

Pseudo hepatic vein thrombosis in a newborn with infracardiac total anomalous pulmonary venous connection.

Pediatric radiology
2024

Successful repair of infracardiac total anomalous pulmonary venous connection with nonconfluent bilateral pulmonary veins.

Asian cardiovascular &amp; thoracic annals
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 &amp; circulation
2024

Hybrid stage 1 Norwood combined with reverse Blalock-Taussig shunt and sutureless repair of total anomalous pulmonary venous connections.

Multimedia manual of cardiothoracic surgery : MMCTS
2025

Impact of the pulmonary venous entry site morphology on postoperative pulmonary vein stenosis in total anomalous pulmonary venous connection patients.

Journal of the Formosan Medical Association = Taiwan yi zhi
2024

Scimitar Syndrome: A Thorough Diagnosis in a Pediatric Patient.

Cureus
2024

Outcomes of Adults With Unrepaired Partial Anomalous Pulmonary Venous Return and Intact Atrial Septum.

Journal of the American Heart Association
2024

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

World journal for pediatric &amp; congenital heart surgery
2024

Atrial thrombus after total anomalous pulmonary venous connection repair.

Asian cardiovascular &amp; thoracic annals
2024

Prenatal diagnosis of scimitar syndrome.

Ginekologia polska
2024

[Mispositioned central line with catheterization of partial anomalous pulmonary venous return].

Revue medicale de Liege
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 &amp; Interventions
2024

Images in Medicine: Inability to Advance Pulmonary Artery Catheter Unmasking Anomalous Infra-Cardiac Right Pulmonary Venous Return in a Middle-Aged Adult.

South Dakota medicine : the journal of the South Dakota State Medical Association
2025

Clinical Outcomes, Predictors, and Surgical Management of Infracardiac Total Anomalous Pulmonary Venous Connection.

Pediatric cardiology
2024

Advancing prenatal diagnosis: Echocardiographic detection of Scimitar syndrome in China - A case series.

Open medicine (Warsaw, Poland)
2024

Scimitar Syndrome in Adulthood: Challenges in Management and Individualized Approaches.

Cureus
2025

Diagnostic value of reversed differential cyanosis in (supra)cardiac total anomalous pulmonary venous return.

Pediatric research
2023

Scimitar syndrome in a four-month-old infant.

Ghana medical journal
2024

Congenital Lung Malformations: A Pictorial Review of Imaging Findings and a Practical Guide for Diagnosis.

Children (Basel, Switzerland)
2025

Long-Term Outcomes of Surgical Repair of Supracardiac Total Anomalous Pulmonary Venous Connection.

Pediatric cardiology
2024

TAPVR: Molecular Pathways and Animal Models.

Advances in experimental medicine and biology
2024

Total Anomalous Pulmonary Venous Connections, Human Genetics.

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

Infracardiac total anomalous pulmonary venous connection with dual drainage into the hepatic and portal venous system.

Journal of cardiovascular computed tomography
2024

Factors associated with early postoperative mortality after total anomalous pulmonary venous connection repair: A retrospective cohort study.

Medicine
2024

Scimitar Syndrome in a Pediatric Cohort.

World journal for pediatric &amp; congenital heart surgery
2024

Dual Drainage Total Anomalous Pulmonary Venous Connection With Infracardiac and Supracardiac Drainage.

World journal for pediatric &amp; congenital heart surgery
2024

Valley Index as a Predictor of Prenatal Diagnosis of Total Anomalous Pulmonary Venous Connection.

Fetal diagnosis and therapy
2024

Infracardiac Total Anomalous Pulmonary Venous Connection.

Radiology. Cardiothoracic imaging
2024

Advanced Imaging Guidance for Ductus Venosus Stenting in Obstructed Total Anomalous Pulmonary Venous Return.

World journal for pediatric &amp; congenital heart surgery
2024

Ultrasound and computed tomography angiography diagnosis of fetal right atrial isomerism with cardiac total anomalous pulmonary venous connection and intestinal malrotation: a case report and literature review.

Pediatric radiology
2024

Scimitar syndrome with vein stenosis in an infant.

BMJ case reports
2024

Insight into the research history and trends of total anomalous pulmonary venous connection: a bibliometric analysis.

Journal of cardiothoracic surgery
2024

Imaging spectrum of horseshoe lung: A series of four cases.

SA journal of radiology
2024

Percutaneous closure of a sinus venosus defect with partial anomalous pulmonary venous return.

The Journal of invasive cardiology
2024

Eleven Years After Scimitar Syndrome Repair With the Lugones Technique: Usefulness of Four-Dimensional Flow Magnetic Resonance Imaging.

World journal for pediatric &amp; congenital heart surgery
2024

Total Anomalous Pulmonary Venous Connection in Adults: Should We Offer Surgery?

World journal for pediatric &amp; congenital heart surgery
2024

Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary referral healthcare center in Colombia.

The international journal of cardiovascular imaging
2024

Complex pulmonary venous drainage pattern with obstructive partial anomalous pulmonary venous connection and associated meandering pulmonary vein.

Pediatric pulmonology
2024

Partial Anomalous Pulmonary Venous Return Incidentally Detected at Lung Cancer Screening With Low-dose Thoracic CT.

Archivos de bronconeumologia
2024

Isolated Partial Anomalous Pulmonary Veins: A 10-Year Experience at a Single Center.

The Journal of surgical research
2025

ALCAPA in Children with Complex Congenital Heart Disease: A Multicenter Study.

Pediatric cardiology
2024

Congenital Pulmonary Lymphangiectasia Masked by Postoperative Pulmonary Venous Obstruction in an Infant with Total Anomalous Pulmonary Venous Connection.

International heart journal
2024

Multimodality assessment of a child with Turner syndrome and a late diagnosis of mixed partial anomalous pulmonary venous return without atrial septal defect. Case report.

Archivos de cardiologia de Mexico
2024

Prenatal diagnosis of total anomalous pulmonary venous connection with vertical vein branching into two.

Journal of medical ultrasonics (2001)
2024

Unveiling a Unique Case of Scimitar Syndrome: Clinical Significance and Multidisciplinary Management Challenges in Pakistan.

Cureus
2024

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

Cardiology in the young
2024

Dual drainage in "partial" anomalous pulmonary venous connection.

Journal of cardiovascular computed tomography
2024

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

Thoracic cancer
2023

Left sided Scimitar syndrome.

Annals of pediatric cardiology
2024

Unilateral Anomalous Pulmonary Venous Return into Coronary Sinus.

Radiology. Cardiothoracic imaging
2024

A rare variant of scimitar syndrome characterised by right pulmonary vein drainage to portal vein with eventeration of diaphragm.

Cardiology in the young
2024

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

World journal for pediatric &amp; congenital heart surgery
2024

Bronchial Asthma With Scimitar Syndrome: A Case Report.

Cureus
2024

A rare case of scimitar syndrome with pulmonary arterial hypertension in an adult female.

Pulmonary circulation
2025

Persistent Patent Vertical Vein After Repair of Total Anomalous Pulmonary Venous Connection (TAPVR): A Rare Cause of Hypoxemia Post-Fontan Procedure.

Pediatric cardiology
2024

STIC-HD live flow technology in the antenatal diagnosis of scimitar syndrome: A case report.

Echocardiography (Mount Kisco, N.Y.)
2024

An 8-year-old boy with Scimitar syndrome: Presentation with gastroesophageal reflux symptoms and concurrent associations, including ectopic herniated liver.

Pediatric pulmonology
2024

Scimitar syndrome, bronchiectasis, haemoptysis and a pneumonectomy.

Respirology case reports
2023

The Sword and the Crown: Echocardiography for the Detection of a Rare Combination of Congenital Heart Disease.

CASE (Philadelphia, Pa.)
2023

Partial anomalous pulmonary venous return: a casual finding in many cases.

Archivos de cardiologia de Mexico
2024

Prenatal diagnosis of isolated total anomalous pulmonary venous connection (TAPVC) to coronary sinus.

Ginekologia polska
2023

Scimitar syndrome with large atrial septal defect and a rare partial anomalous venous drainage in an adult: a case report.

European heart journal. Case reports
2024

Contemporary outcomes for functional single ventricle with total anomalous pulmonary venous connection.

The Journal of thoracic and cardiovascular surgery
2023

[Left Metastatic Lung Tumor for a Patient with Scimitar Syndrome:Report of a Case].

Kyobu geka. The Japanese journal of thoracic surgery
2023

Unilateral bilobar pulmonary aplasia with anomalous pulmonary venous return: A rare combination of congenital airway and vascular anomalies.

Pediatric pulmonology
2023

Risk factors associated with prolonged intensive care unit stay following surgery for total anomalous pulmonary venous connection: a retrospective study.

Journal of cardiothoracic surgery
2023

Long-term outcomes of functional single ventricles associated with heterotaxy syndrome†.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2023

Modified L-shaped incision technique for supracardiac total anomalous pulmonary venous connection as an alternative to sutureless technique.

International journal of surgery (London, England)
2023

Clinical exome sequencing efficacy and phenotypic expansions involving anomalous pulmonary venous return.

European journal of human genetics : EJHG
2023

Intracardiac total anomalous pulmonary venous return or septum primum malposition? Complete repair in left atrial isomerism.

Multimedia manual of cardiothoracic surgery : MMCTS
2023

A rare cause of angina: scimitar syndrome case report.

European heart journal. Case reports
2023

Case report: A unique quadruple coexisting anomaly-scimitar syndrome, atrial septal defect, vascular ring, and pulmonary sequestration.

Frontiers in pediatrics
2023

Scimitar syndrome with hemi-cor triatriatum.

Acta cardiologica
2023

Novel operative technique for Scimitar syndrome: Posterior flap technique.

Asian cardiovascular &amp; thoracic annals
2023

Outcomes and Risk Factors of Interventions for Pediatric Post-operative Pulmonary Vein Stenosis.

Pediatric cardiology
2023

Long-Term Outcomes of Unrepaired Isolated Partial Anomalous Pulmonary Venous Connection With an Intact Atrial Septum.

The American journal of cardiology
2023

Whale's tail sign in fetus with coronary sinus total anomalous pulmonary venous connection.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2023

Surgical repair of mitral valve regurgitation with anomalous unilateral single pulmonary vein.

Journal of cardiology cases
2023

[Integrated management during the perinatal period for total anomalous pulmonary venous connection].

Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics
2023

Successful Surgical Management of Incidentally Diagnosed Scimitar Syndrome With Anomalous Connection of the Left Hepatic Vein to Coronary Sinus and Persistent Left Superior Vena Cava.

Cureus
2024

Sutureless Repair of Extracardiac Univentricular Total Anomalous Pulmonary Venous Connection.

The Annals of thoracic surgery
2023

Multimodality diagnostic imaging for anomalous pulmonary venous connections: a pictorial essay.

Pediatric radiology
2023

A unique bizarre subtype of mixed total anomalous pulmonary venous connection.

Cardiology in the young
2023

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

World journal for pediatric &amp; congenital heart surgery
2022

Extracardiac conduit as alternative repair for scimitar syndrome.

Annals of pediatric cardiology
2023

Rare case of horseshoe lung associated with scimitar syndrome in Palestine: a case report.

Annals of medicine and surgery (2012)
2023

Total or Partial? Rare Cases of Partial Anomalous Pulmonary Venous Return: Three Pulmonary Veins Connected to the Coronary Sinus.

Balkan medical journal
2023

Unrecognized Patent Foramen Ovale in Patient With Sinus Venosus-Type Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return.

A&amp;A practice
2023

Anesthesia management of living donor liver transplantation in a patient with scimitar syndrome.

Saudi journal of anaesthesia
2023

Worsening Dyspnea and New-Onset Atrial Fibrillation in a 79-Year-Old Woman.

JAMA cardiology
2023

Better Late Than Never - A Case of a Congenital Left Partial Anomalous Pulmonary Venous Drainage Diagnosed and Treated in The Sixth Decade of Life.

The heart surgery forum
2023

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

World journal for pediatric &amp; congenital heart surgery
2023

Rarer Components of a Rare Disease: Accessory Hemidiaphragm and Horseshoe Lung with Scimitar Syndrome; Two Case Reports.

Current medical imaging
2023

Comparison of the effects of conventional method and primary sutureless techniques on early postoperative rhythm problems in patients with total abnormal pulmonary venous return anomaly.

Cardiology in the young
2024

Palliation of a Heterotaxy Single Ventricle Neonate with Pulmonary Atresia and Obstructed TAPVR.

Pediatric cardiology
2023

Total Anomalous Pulmonary Venous Return.

Radiology
2023

Partial anomalous pulmonary venous connection diagnosed prenatally.

Pediatrics international : official journal of the Japan Pediatric Society
2023

Midline crossing right pulmonary veins with dual drainage to the left atrium.

Cardiology in the young
2023

RE: Wang et al. Diagnosis of fetal total anomalous pulmonary venous connection based on the post-left atrium space ratio using artificial intelligence.

Prenatal diagnosis
2023

Supracardiac total anomalous pulmonary venous connection type Ib: Morphology and outcomes.

The Journal of thoracic and cardiovascular surgery
2023

Infracardiac obstructive total anomalous pulmonary venous return with ventricular septal defect: a rare anomaly.

Multimedia manual of cardiothoracic surgery : MMCTS
2022

Proximal Interruption of Pulmonary Artery: Spectrum of Radiological Findings With Emphasis on Chest Radiograph and Contrast-Enhanced Computed Tomography (CECT).

Cureus
2023

Adult congenital horseshoe lung with bilateral pulmonary sequestration: A case report.

Acta radiologica open
2023

Two sisters with cardiac-urogenital syndrome secondary to pathogenic splicing variant in the MYRF gene with unaffected parents: A case of gonadal mosaicism?

Molecular genetics &amp; genomic medicine
2023

Reintervention and mortality risk after total anomalous pulmonary venous connection repair.

Cardiology in the young
2023

[Scimitar syndrome with pulmonary sequestration containing carcinoma: a case report].

Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases
2023

Anatomical attention-based prediction of postoperative pulmonary venous obstruction via CTA images.

Computerized medical imaging and graphics : the official journal of the Computerized Medical Imaging Society
2023

Primary sutureless repair concomitant with the Warden procedure.

Asian cardiovascular &amp; thoracic annals
2022

Ductus arteriosus banding to regulate excessive pulmonary blood flow in a neonate with necrotizing enterocolitis and complex congenital heart disease, including pulmonary atresia and total anomalous pulmonary venous return: a case report.

Journal of cardiothoracic surgery
2023

Echocardiographic evaluation of supracardiac anomalous pulmonary venous connection in children: comparison with multilayer spiral CT.

The international journal of cardiovascular imaging
2023

Hypogenetic right lung with partial anomalous pulmonary venous return and accessory diaphragm: a case of "scimitar lung".

Folia morphologica
2023

Total anomalous pulmonary venous connection with another major congenital cardiac defect in biventricular hearts.

Asian cardiovascular &amp; thoracic annals
2022

Complete transcatheter correction of variant scimitar syndrome-a case report.

European heart journal. Case reports
2023

Influenza vaccination during pregnancy and risk of selected major structural congenital heart defects, National Birth Defects Prevention Study 2006-2011.

Birth defects research
2023

Novel marker for diagnosis of supracardiac total anomalous pulmonary venous connection in first trimester.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2022

Surgical correction of partial anomalous left superior pulmonary venous connection to the innominate vein.

Multimedia manual of cardiothoracic surgery : MMCTS
2023

Computed Tomographic 3-Dimensional Virtual Dissection Aiding in Diagnosis and Surgical Planning of a Rare Form of Obstructed Supracardiac Total Anomalous Pulmonary Venous Connection With an Unusual Scimitar-Like Arrangement.

World journal for pediatric &amp; congenital heart surgery
2022

Minimally invasive versus sternotomy approach for double-patch repair of partial anomalous pulmonary venous connection and sinus venosus defect in pediatric and adult patients: Mid to long-term outcomes.

Journal of cardiac surgery
2022

A rare case on anomalous pulmonary venous drainage in congenital cardiovascular disease.

Journal of cardiac surgery
2022

The Lugones Procedure for Scimitar Syndrome Repair Through Right Axillary Thoracotomy: Not Only a Cosmetically Superior Approach.

World journal for pediatric &amp; congenital heart surgery
Ver todos os 616 no EuropePMC

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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. Exercise Capacity and Pulmonary Function in Pediatric Patients With Anomalous Pulmonary Venous Connection Post-Surgical Repair: A Retrospective Analysis.
    The Kaohsiung journal of medical sciences· 2026· PMID 40899442mais citado
  2. Partial Anomalous Pulmonary Venous Return in Lung Cancer Surgery: Diagnostic Challenges and Surgical Considerations in Two Cases.
    The Tokai journal of experimental and clinical medicine· 2026· PMID 41859801mais citado
  3. Scimitar Syndrome Incidentally Identified During the Workup for Acute Appendicitis in a Young Adult Female: A Case Report.
    Cureus· 2026· PMID 41728402mais citado
  4. Unmasking an Anomalous Vein: Surgical Repair of Left Upper Pulmonary Vein Drainage into the Innominate Vein.
    Portuguese journal of cardiac thoracic and vascular surgery· 2026· PMID 41719571mais citado
  5. Computational Predictive Modeling of Surgical Outcomes in Total Anomalous Pulmonary Venous Connection: Assessing the Impact of Pulmonary Venous Confluence Size on Preoperative Planning.
    Journal of cardiovascular translational research· 2026· PMID 41639534mais citado
  6. Surgical and Catheter-Based Intervention in Pediatric Pulmonary Vein Stenosis.
    Pediatr Cardiol· 2026· PMID 41995823recente
  7. [Scimitar Syndrome in a young adult with an arteriovenous fistula from the celiac trunk - A rare adult presentation].
    Rofo· 2026· PMID 41985470recente
  8. Intra-atrial Baffling of an Infradiaphragmatic Scimitar Vein without Circulatory Arrest.
    Methodist Debakey Cardiovasc J· 2026· PMID 41971523recente
  9. Discovery and management of accessory hemidiaphragm during video-assisted thoracoscopic segmentectomy for pulmonary nodule: a case report.
    Front Surg· 2026· PMID 41971064recente
  10. Early outcomes of a formal robotic congenital cardiac surgery program.
    JTCVS Open· 2026· PMID 41960109recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:185(Orphanet)
  2. MONDO:0015987(MONDO)
  3. GARD:18680(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q2557206(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

Síndrome Scimitar
Compêndio · Raras BR

Síndrome Scimitar

ORPHA:185 · MONDO:0015987
Prevalência
Unknown
Herança
Not applicable
CID-10
Q26.8 · Outras malformações congênitas das grandes veias
CID-11
Início
Adolescent, Adult, Antenatal, Childhood, Infancy, Neonatal
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0036400
EuropePMC
Wikidata
Papers 10a
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