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Retorno venoso pulmonar anômalo congênito
ORPHA:3090CID-10 · Q26.2DOENÇA RARA

A anomalia congênita de retorno venoso pulmonar é um tipo de malformação do coração presente desde o nascimento. Nela, algumas ou todas as veias que trazem o sangue dos pulmões se conectam ao lado direito do coração ou a outras veias do corpo, em vez de se conectarem ao lado esquerdo (o correto). Essa condição pode ou não apresentar um estreitamento (obstrução) nas veias dos pulmões. Isso leva a diversos sintomas, como cansaço, falta de ar ao fazer esforço, pressão alta nas artérias do pulmão, pele azulada (cianose) e insuficiência cardíaca (coração fraco) que piora progressivamente. Os dois tipos principais são a anomalia parcial de retorno venoso pulmonar congênita (PAPVC), onde apenas uma ou algumas veias pulmonares estão afetadas, e a anomalia total de retorno venoso pulmonar congênita (TAPVC), onde todas as veias pulmonares estão afetadas.

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Introdução

O que você precisa saber de cara

📋

A anomalia congênita de retorno venoso pulmonar é um tipo de malformação do coração presente desde o nascimento. Nela, algumas ou todas as veias que trazem o sangue dos pulmões se conectam ao lado direito do coração ou a outras veias do corpo, em vez de se conectarem ao lado esquerdo (o correto). Essa condição pode ou não apresentar um estreitamento (obstrução) nas veias dos pulmões. Isso leva a diversos sintomas, como cansaço, falta de ar ao fazer esforço, pressão alta nas artérias do pulmão, pele azulada (cianose) e insuficiência cardíaca (coração fraco) que piora progressivamente. Os dois tipos principais são a anomalia parcial de retorno venoso pulmonar congênita (PAPVC), onde apenas uma ou algumas veias pulmonares estão afetadas, e a anomalia total de retorno venoso pulmonar congênita (TAPVC), onde todas as veias pulmonares estão afetadas.

🏥
SUS: Cobertura mínimaScore: 20%
Centros em: PA, PR, SC, RS, ES +10CID-10: Q26.2
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Sinais e sintomas

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

Partes do corpo afetadas

❤️
Coração
24 sintomas
🫁
Pulmão
16 sintomas
🫃
Digestivo
2 sintomas
🦴
Ossos e articulações
1 sintomas
🫘
Rins
1 sintomas

+ 23 sintomas em outras categorias

Características mais comuns

Sequestro pulmonar
Retorno venoso pulmonar anômalo parcial
Bloqueio cardíaco
Anormalidade da coluna vertebral
Hipoplasia do diafragma
Tosse
67sintomas
Sem dados (67)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 67 características clínicas mais associadas, ordenadas por frequência.

Sequestro pulmonarPulmonary sequestration
Retorno venoso pulmonar anômalo parcialPartial anomalous pulmonary venous return
Bloqueio cardíacoHeart block
Anormalidade da coluna vertebralAbnormality of the vertebral column
Hipoplasia do diafragmaHypoplasia of the diaphragm

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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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Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Retorno venoso pulmonar anômalo congênito

Centros de Referência SUS

24 centros habilitados pelo SUS para Retorno venoso pulmonar anômalo congênito

Centros para Retorno venoso pulmonar anômalo congênito

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

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

Transcatheter Correction of Bilateral Partial Anomalous Pulmonary Venous Return with Intrapulmonary Dual Drainage: A Rare Entity.

Life (Basel, Switzerland)2026 Feb 12

Partial anomalous pulmonary venous return (PAPVR) with dual drainage is a very rare congenital heart anomaly. We report the case of a 6-year-old boy with PAPVR in whom both upper pulmonary veins (PVs) drain anomalously into the systemic venous circulation, while maintaining preserved intrapulmonary collateral venous connections with the remaining pulmonary veins draining into the left atrium. Careful balloon occlusion testing of the anomalous PVs was performed, simultaneously with measurements of pulmonary pressures and control angiography, proving the absence of venous congestion in the upper lung fields during the pulmonary venous phase. Transcatheter occlusion using vascular plugs was safely and successfully performed.

#4

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.

#5

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.

Publicações recentes

Ver todas no PubMed

📚 EuropePMCmostrando 200

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

Transcatheter Correction of Bilateral Partial Anomalous Pulmonary Venous Return with Intrapulmonary Dual Drainage: A Rare Entity.

Life (Basel, Switzerland)
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
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
2026

Importance of clinical practice guidelines for specialized delivery room resuscitation of newborns with prenatally diagnosed critical congenital heart disease.

Journal of perinatology : official journal of the California Perinatal Association
2025

A De Novo Loss-of-Function NCKAP1 Variant in a Boy with Neurodevelopmental Delay and Congenital Heart Defect.

Children (Basel, Switzerland)
2026

High risk and low incidence diseases: Cyanotic critical congenital heart disease.

The American journal of emergency medicine
2025

Total Anomalous Pulmonary Venous Connections.

Clinics in perinatology
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

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
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

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

Thoracic Vascular Anomalies: Insights from Embryology and Imaging.

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

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

Molecular syndromology
2025

A case report on myocarditis and right chamber dilation: cardiac magnetic resonance acquisition for accurate diagnosis.

Future cardiology
2025

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

Reports (MDPI)
2025

A Hidden Congenital Secret: Unmasking a Rare Right Upper Lobe PAPVR Draining into the IVC.

Heart, lung &amp; circulation
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

Cardiorespiratory transition in CDH.

Seminars in fetal &amp; neonatal medicine
2025

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

BMJ case reports
2025

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
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

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

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

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

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

A Case of Incomplete Scimitar Syndrome in a Quinquagenarian Male.

Cureus
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

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

Pediatric cardiology
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

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

Pediatric cardiology
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

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

Taiwanese journal of obstetrics &amp; gynecology
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

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

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

Revue medicale de Liege
2024

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

Cureus
2023

Scimitar syndrome in a four-month-old infant.

Ghana medical journal
2024

Double Connection of Left-Sided Partial Anomalous Pulmonary Vein Return in a Young Man.

JACC. Case reports
2024

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

Children (Basel, Switzerland)
2024

A Case Report of an Uncommon Association of Total Intracardiac Anomalous Pulmonary Venous Return and Tetralogy of Fallot.

Cureus
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

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

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

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

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

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

Cureus
2024

[Contribution of cardiac CT in the management of congenital heart disease : experience of the Abidjan cardiology institute in 27 cases].

Annales de cardiologie et d'angeiologie
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

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
2024

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

Pediatric pulmonology
2023

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

CASE (Philadelphia, Pa.)
2025

Cor Triatriatum: A Review.

Cardiology in review
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

[An unexpected case of right heart dilation in the elderly].

Giornale italiano di cardiologia (2006)
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

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

European journal of human genetics : EJHG
2023

Extensive Deep Vein Thrombosis With Intraabdominal Extension Triggered by Oral Contraceptive Use in a Young Patient With Interrupted Inferior Vena Cava With Azygos Continuation: A Case Report.

Cureus
2023

A 20-year follow-up of successful surgical management for a complex case of pentalogy of fallot and dextrocardia with systemic and pulmonary venous anomalies: A rare case report.

International journal of surgery case reports
2023

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

Multimedia manual of cardiothoracic surgery : MMCTS
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

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
2023

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

Pediatric radiology
2023

Prevalence and detection rate of major congenital heart disease in twin pregnancies in Denmark.

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

Retroaortic Innominate Vein in Association With Pulmonary Atresia/Major Aortopulmonary Collaterals.

Annals of thoracic surgery short reports
2023

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

Saudi journal of anaesthesia
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
2024

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

Pediatric cardiology
2023

Partial anomalous pulmonary venous connection diagnosed prenatally.

Pediatrics international : official journal of the Japan Pediatric Society
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

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

Complex congenital cardiovascular anomaly in a patient with AGO1-associated disorder.

American journal of medical genetics. Part A
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

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

Anaesthesia Challenges in a Neonate Having Supracardiac Total Anomalous Pulmonary Venous Return With Incidental Stridor for Non-operating Room Anaesthesia (NORA): Feed and Wrap Technique as a Rescue.

Cureus
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
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

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
2022

Trans-catheter closure of ASD and abnormal connection of left pulmonary vein to vertical vein: A case report.

Medicine
2023

Fetal Diagnosis of Scimitar Syndrome in the Presence of Complex Congenital Heart Disease.

Pediatric cardiology
2022

Case report of a left superior vena cava to left atrial connection treated with percutaneous covered stent placement.

European heart journal. Case reports
2022

[Emergent Operation for Total Anomalous Pulmonary Venous Return].

Kyobu geka. The Japanese journal of thoracic surgery
2022

Surgical correction of total anomalous pulmonary venous return in an adult patient.

Journal of cardiothoracic surgery
2022

Prenatal diagnosis of total anomalous pulmonary venous connection using 2D and HDlive flow combined with spatiotemporal image correlation.

Echocardiography (Mount Kisco, N.Y.)
2022

Treatment and prognosis of Scimitar syndrome: A retrospective analysis in a single center of East China.

Frontiers in cardiovascular medicine
2023

The Double Whammy of Total Anomalous Pulmonary Venous Connection and Single Ventricle Palliation.

The Annals of thoracic surgery
2022

Window anastomosis technique for repair of supracardiac total anomalous pulmonary venous connection in infants.

Journal of cardiac surgery
2022

Application of sutureless technique in total anomalous pulmonary venous connection repair.

Journal of cardiac surgery
2022

A rare pulmonary-to-systemic venous connection associated with the partially anomalous pulmonary venous connection.

Journal of cardiac surgery
2023

Impact of Total Anomalous Pulmonary Venous Connection in Staged Single Ventricle Palliation.

The Annals of thoracic surgery
2022

Tetralogy of Fallot associated with a rare type partially anomalous pulmonary venous connection and levoatrial cardinal vein.

Journal of cardiac surgery
2022

Fetal Doppler Echocardiographic Assessment Predicts Severe Postnatal Obstruction in Total Anomalous Pulmonary Venous Connection.

Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography
2022

Invited commentary: Total anomalous pulmonary venous connection remains a challenging pediatric disease.

Journal of cardiac surgery
2023

Surgical and Cardiac Catheterization Outcomes of Scimitar Syndrome Patients: A Three Decade Single-Center Experience.

Pediatric cardiology
2022

Partial anomalous pulmonary venous return with secundum atrial septal defect: A case report.

Radiology case reports
2022

Supracardiac Total Anomalous Pulmonary Venous Return Repair in a 7-Month-Old Infant.

Texas Heart Institute journal
2022

Total Anomalous Pulmonary Venous Connection Repair: Single-Center Outcomes in a Lower-Middle Income Region.

World journal for pediatric &amp; congenital heart surgery
2022

Isolated Anomalous Right Superior Pulmonary Venous Return to the Inferior Vena Cava.

World journal for pediatric &amp; congenital heart surgery
2022

Vascular anomaly diagnosis by central venous catheter misplacement: a case report.

Journal of medical case reports
2022

Total anomalous pulmonary venous connection: 16 years of surgical results in a single center.

Journal of cardiac surgery
2022

Risk factors for postoperative pulmonary venous obstruction after surgical repair of total anomalous pulmonary venous connection: a systemic review and meta-analysis.

Interactive cardiovascular and thoracic surgery
2022

Prognosis and related risk factors analysis of total anomalous pulmonary venous connection correction.

Journal of cardiac surgery
2022

Surgical Outcomes of Total Anomalous Pulmonary Venous Connection Repair.

Medicina (Kaunas, Lithuania)
2022

Multimodality Approach to a Complex Scimitar Syndrome: How Advanced Diagnostics Can Guide Therapeutic Strategies.

JACC. Case reports
2023

Infradiaphragmatic partial anomalous pulmonary venous connection in adulthood. Considerations for management: a single centre experience.

Cardiology in the young
2022

Clinical implications of airway anomalies and stenosis in patients with heterotaxy syndrome.

Pediatric pulmonology
2023

Cardiac-type total anomalous pulmonary venous return is not benign.

The Journal of thoracic and cardiovascular surgery
2023

Long-term results of the Warden procedure for right partial anomalous pulmonary venous connection.

Cardiology in the young
2022

TBX5 variant with the novel phenotype of mixed‑type total anomalous pulmonary venous return in Holt‑Oram Syndrome and variable intrafamilial heart defects.

Molecular medicine reports
2022

Scimitar syndrome - An incidental diagnosis in a case of fibroadenoma.

Radiology case reports
2022

Anomalous Connection of the Left Pulmonary Vein to the Coronary Sinus with Intact Atrial Septum in a Young Woman.

The heart surgery forum
2022

Invited commentary for: Essential role of cardiac computed tomography for surgical decision making in children with total anomalous pulmonary venous connection and single ventricle.

Journal of cardiac surgery
2023

Successful occlusion of a feeding artery with Amplatzer Piccolo Occluder in a patient diagnosed with Scimitar syndrome.

Cardiology in the young
2022

Sutureless Closure Versus Conventional Technique in the Primary Surgery of Total Anomalous Pulmonary Venous Connection: A Systematic Review and Meta-analysis.

Pediatric cardiology
2021

Horseshoe lung in a young child at Tygerberg Hospital, South Africa.

African journal of thoracic and critical care medicine
2022

Prenatal Diagnosis and Outcome of Scimitar Syndrome: A Case Series of Six Patients.

Journal of clinical medicine
2022

Essential role of cardiac computed tomography for surgical decision making in children with total anomalous pulmonary venous connection and single ventricle.

Journal of cardiac surgery
2022

New trend: Surgical correction of Ebstein's anomaly using modified cone repair and a combination of different techniques.

Journal of cardiac surgery
2022

Laryngeal atresia, esophageal atresia, and total anomalous pulmonary venous return in a monochorionic twin.

Pediatrics international : official journal of the Japan Pediatric Society
2022

"Letter to the Editor: Emergency surgical treatment of total anomalous pulmonary venous connection".

Journal of cardiac surgery
2022

Multifactorial pulmonary hypertension in infantile scimitar syndrome.

Archives of cardiovascular diseases
2022

Is horseshoe lung a component of VACTERL spectrum? Case report and review of literature.

Radiology case reports
2022

Surgical Treatment of Total Anomalous Pulmonary Venous Drainage with Atrial Fibrillation in an Adult: A Case Report and Literature Review.

The heart surgery forum
2022

Misclassification of Pulmonary Hypertension in Partial Anomalous Pulmonary Venous Return.

The Annals of thoracic surgery
2022

A new association of Oculoauriculovertebral spectrum and persistent fifth aortic arch -double lumen aorta: a case report.

BMC pediatrics
2023

Mini right axillary thoracotomy for congenital heart defect repair can become a safe surgical routine.

Cardiology in the young
2022

"Dual" drainage in cardiac partial anomalous pulmonary venous return.

Journal of cardiac surgery
2022

Building a Risk Prediction Model for Postoperative Pulmonary Vein Obstruction via Quantitative Analysis of CTA Images.

IEEE journal of biomedical and health informatics
2022

Pulmonary Arteriovenous Malformation and Its Vascular Mimickers.

Korean journal of radiology
2022

Transplantation of Donor Lung With Partial Anomalous Pulmonary Venous Return Using a Carrel Patch.

The Annals of thoracic surgery
2022

Extremely tortuous vertical vein in supracardiac totally anomalous pulmonary venous connection.

Journal of cardiac surgery
2022

Sutureless Repair With Extended Atriotomy for Post-Repair Pulmonary Venous Obstruction.

The Annals of thoracic surgery
2022

Reconstruction of Donor Anomalous Pulmonary Vein During Lung Transplantation.

The Annals of thoracic surgery
2021

Perioperative Management of Scimitar Syndrome Mimicking Total Anomalous Pulmonary Venous Return Posing a Diagnostic Dilemma: A Compilation of Two Cases.

Cureus
2022

Congenital anomalies of the large intrathoracic airways.

Paediatric anaesthesia
2022

Association of PLXND1 with a novel subtype of anomalous pulmonary venous return.

Human molecular genetics
2022

Staged approach for correction of anomalous venous return to the azygos vein.

Journal of cardiac surgery
2021

Leftward deviation of the primary septum or dividing left atrial shelf?

Cardiology in the young
2022

Budd-Chiari syndrome: A rare association of Scimitar syndrome.

Journal of cardiovascular computed tomography
2022

Emergency surgical treatment of total anomalous pulmonary venous connection.

Journal of cardiac surgery
2022

Cor Triatriatum in Association With a Unique Form of Partial Anomalous Pulmonary Venous Connection.

World journal for pediatric &amp; congenital heart surgery
2021

3D reconstruction for preoperative planning of partial anomalous pulmonary venous return.

Kardiologia polska
2021

Late-onset total anomalous pulmonary venous connection in a 70-year-old woman.

BMJ case reports
2021

Rare presentation of transposition of great arteries and total anomalous pulmonary venous connection in an 18-year-old.

Echocardiography (Mount Kisco, N.Y.)
2021

Atypical presentation of scimitar syndrome with severe hepatomegaly: a case report.

European heart journal. Case reports
2021

Posterior trans-mediastinal lung herniation in a postoperative Marfan's patient.

Clinical imaging
2021

Surgical management of the scimitar syndrome.

Journal of cardiac surgery

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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. Transcatheter Correction of Bilateral Partial Anomalous Pulmonary Venous Return with Intrapulmonary Dual Drainage: A Rare Entity.
    Life (Basel, Switzerland)· 2026· PMID 41752952mais citado
  4. Scimitar Syndrome Incidentally Identified During the Workup for Acute Appendicitis in a Young Adult Female: A Case Report.
    Cureus· 2026· PMID 41728402mais citado
  5. 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
  6. Left Ventricular Rupture Due to Congenital Partial Defect of the Left Ventricular Free Wall.
    Interdiscip Cardiovasc Thorac Surg· 2026· PMID 41601237recente
  7. Total Anomalous Pulmonary Venous Connections.
    Clin Perinatol· 2025· PMID 41233009recente
  8. 'Congenitally corrected' Scimitar syndrome? An anomalous unilateral single pulmonary vein variant.
    BMJ Case Rep· 2025· PMID 41087087recente
  9. Transcatheter correction of the scimitar variant with dual pulmonary venous drainage-an international multicentre series.
    Cardiol Young· 2025· PMID 41070433recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:3090(Orphanet)
  2. MONDO:0017705(MONDO)
  3. GARD:4599(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q1877483(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

Retorno venoso pulmonar anômalo congênito
Compêndio · Raras BR

Retorno venoso pulmonar anômalo congênito

ORPHA:3090 · MONDO:0017705
CID-10
Q26.2 · Comunicação venosa pulmonar anormal total
MedGen
UMLS
C0265916
Wikidata
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