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.
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.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 23 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 67 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — 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
Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
Serviço de Referência
Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
Serviço de Referência
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
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
Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
Atenção Especializada
Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
Serviço de Referência
Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
Atenção Especializada
Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
Serviço de Referência
Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
Atenção Especializada
Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
Serviço de Referência
Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
Serviço de Referência
Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
Atenção Especializada
Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
Serviço de Referência
Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
Serviço de Referência
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
Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
Serviço de Referência
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
Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
Serviço de Referência
Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
Serviço de Referência
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
Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
Serviço de Referência
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
UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
Serviço de Referência
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
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Publicações mais relevantes
Exercise Capacity and Pulmonary Function in Pediatric Patients With Anomalous Pulmonary Venous Connection Post-Surgical Repair: A Retrospective Analysis.
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.
Partial Anomalous Pulmonary Venous Return in Lung Cancer Surgery: Diagnostic Challenges and Surgical Considerations in Two Cases.
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.
Transcatheter Correction of Bilateral Partial Anomalous Pulmonary Venous Return with Intrapulmonary Dual Drainage: A Rare Entity.
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.
Scimitar Syndrome Incidentally Identified During the Workup for Acute Appendicitis in a Young Adult Female: A Case Report.
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.
Unmasking an Anomalous Vein: Surgical Repair of Left Upper Pulmonary Vein Drainage into the Innominate Vein.
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.
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World journal for pediatric & congenital heart surgeryA rare case on anomalous pulmonary venous drainage in congenital cardiovascular disease.
Journal of cardiac surgeryThe Lugones Procedure for Scimitar Syndrome Repair Through Right Axillary Thoracotomy: Not Only a Cosmetically Superior Approach.
World journal for pediatric & congenital heart surgeryTrans-catheter closure of ASD and abnormal connection of left pulmonary vein to vertical vein: A case report.
MedicineFetal Diagnosis of Scimitar Syndrome in the Presence of Complex Congenital Heart Disease.
Pediatric cardiologyCase report of a left superior vena cava to left atrial connection treated with percutaneous covered stent placement.
European heart journal. Case reports[Emergent Operation for Total Anomalous Pulmonary Venous Return].
Kyobu geka. The Japanese journal of thoracic surgerySurgical correction of total anomalous pulmonary venous return in an adult patient.
Journal of cardiothoracic surgeryPrenatal diagnosis of total anomalous pulmonary venous connection using 2D and HDlive flow combined with spatiotemporal image correlation.
Echocardiography (Mount Kisco, N.Y.)Treatment and prognosis of Scimitar syndrome: A retrospective analysis in a single center of East China.
Frontiers in cardiovascular medicineThe Double Whammy of Total Anomalous Pulmonary Venous Connection and Single Ventricle Palliation.
The Annals of thoracic surgeryWindow anastomosis technique for repair of supracardiac total anomalous pulmonary venous connection in infants.
Journal of cardiac surgeryApplication of sutureless technique in total anomalous pulmonary venous connection repair.
Journal of cardiac surgeryA rare pulmonary-to-systemic venous connection associated with the partially anomalous pulmonary venous connection.
Journal of cardiac surgeryImpact of Total Anomalous Pulmonary Venous Connection in Staged Single Ventricle Palliation.
The Annals of thoracic surgeryTetralogy of Fallot associated with a rare type partially anomalous pulmonary venous connection and levoatrial cardinal vein.
Journal of cardiac surgeryFetal 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 EchocardiographyInvited commentary: Total anomalous pulmonary venous connection remains a challenging pediatric disease.
Journal of cardiac surgerySurgical and Cardiac Catheterization Outcomes of Scimitar Syndrome Patients: A Three Decade Single-Center Experience.
Pediatric cardiologyPartial anomalous pulmonary venous return with secundum atrial septal defect: A case report.
Radiology case reportsSupracardiac Total Anomalous Pulmonary Venous Return Repair in a 7-Month-Old Infant.
Texas Heart Institute journalTotal Anomalous Pulmonary Venous Connection Repair: Single-Center Outcomes in a Lower-Middle Income Region.
World journal for pediatric & congenital heart surgeryIsolated Anomalous Right Superior Pulmonary Venous Return to the Inferior Vena Cava.
World journal for pediatric & congenital heart surgeryVascular anomaly diagnosis by central venous catheter misplacement: a case report.
Journal of medical case reportsTotal anomalous pulmonary venous connection: 16 years of surgical results in a single center.
Journal of cardiac surgeryRisk 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 surgeryPrognosis and related risk factors analysis of total anomalous pulmonary venous connection correction.
Journal of cardiac surgerySurgical Outcomes of Total Anomalous Pulmonary Venous Connection Repair.
Medicina (Kaunas, Lithuania)Multimodality Approach to a Complex Scimitar Syndrome: How Advanced Diagnostics Can Guide Therapeutic Strategies.
JACC. Case reportsInfradiaphragmatic partial anomalous pulmonary venous connection in adulthood. Considerations for management: a single centre experience.
Cardiology in the youngClinical implications of airway anomalies and stenosis in patients with heterotaxy syndrome.
Pediatric pulmonologyCardiac-type total anomalous pulmonary venous return is not benign.
The Journal of thoracic and cardiovascular surgeryLong-term results of the Warden procedure for right partial anomalous pulmonary venous connection.
Cardiology in the youngTBX5 variant with the novel phenotype of mixed‑type total anomalous pulmonary venous return in Holt‑Oram Syndrome and variable intrafamilial heart defects.
Molecular medicine reportsScimitar syndrome - An incidental diagnosis in a case of fibroadenoma.
Radiology case reportsAnomalous Connection of the Left Pulmonary Vein to the Coronary Sinus with Intact Atrial Septum in a Young Woman.
The heart surgery forumInvited 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 surgerySuccessful occlusion of a feeding artery with Amplatzer Piccolo Occluder in a patient diagnosed with Scimitar syndrome.
Cardiology in the youngSutureless Closure Versus Conventional Technique in the Primary Surgery of Total Anomalous Pulmonary Venous Connection: A Systematic Review and Meta-analysis.
Pediatric cardiologyHorseshoe lung in a young child at Tygerberg Hospital, South Africa.
African journal of thoracic and critical care medicinePrenatal Diagnosis and Outcome of Scimitar Syndrome: A Case Series of Six Patients.
Journal of clinical medicineEssential role of cardiac computed tomography for surgical decision making in children with total anomalous pulmonary venous connection and single ventricle.
Journal of cardiac surgeryNew trend: Surgical correction of Ebstein's anomaly using modified cone repair and a combination of different techniques.
Journal of cardiac surgeryLaryngeal atresia, esophageal atresia, and total anomalous pulmonary venous return in a monochorionic twin.
Pediatrics international : official journal of the Japan Pediatric Society"Letter to the Editor: Emergency surgical treatment of total anomalous pulmonary venous connection".
Journal of cardiac surgeryMultifactorial pulmonary hypertension in infantile scimitar syndrome.
Archives of cardiovascular diseasesIs horseshoe lung a component of VACTERL spectrum? Case report and review of literature.
Radiology case reportsSurgical Treatment of Total Anomalous Pulmonary Venous Drainage with Atrial Fibrillation in an Adult: A Case Report and Literature Review.
The heart surgery forumMisclassification of Pulmonary Hypertension in Partial Anomalous Pulmonary Venous Return.
The Annals of thoracic surgeryA new association of Oculoauriculovertebral spectrum and persistent fifth aortic arch -double lumen aorta: a case report.
BMC pediatricsMini right axillary thoracotomy for congenital heart defect repair can become a safe surgical routine.
Cardiology in the young"Dual" drainage in cardiac partial anomalous pulmonary venous return.
Journal of cardiac surgeryBuilding a Risk Prediction Model for Postoperative Pulmonary Vein Obstruction via Quantitative Analysis of CTA Images.
IEEE journal of biomedical and health informaticsPulmonary Arteriovenous Malformation and Its Vascular Mimickers.
Korean journal of radiologyTransplantation of Donor Lung With Partial Anomalous Pulmonary Venous Return Using a Carrel Patch.
The Annals of thoracic surgeryExtremely tortuous vertical vein in supracardiac totally anomalous pulmonary venous connection.
Journal of cardiac surgerySutureless Repair With Extended Atriotomy for Post-Repair Pulmonary Venous Obstruction.
The Annals of thoracic surgeryReconstruction of Donor Anomalous Pulmonary Vein During Lung Transplantation.
The Annals of thoracic surgeryPerioperative Management of Scimitar Syndrome Mimicking Total Anomalous Pulmonary Venous Return Posing a Diagnostic Dilemma: A Compilation of Two Cases.
CureusCongenital anomalies of the large intrathoracic airways.
Paediatric anaesthesiaAssociation of PLXND1 with a novel subtype of anomalous pulmonary venous return.
Human molecular geneticsStaged approach for correction of anomalous venous return to the azygos vein.
Journal of cardiac surgeryLeftward deviation of the primary septum or dividing left atrial shelf?
Cardiology in the youngBudd-Chiari syndrome: A rare association of Scimitar syndrome.
Journal of cardiovascular computed tomographyEmergency surgical treatment of total anomalous pulmonary venous connection.
Journal of cardiac surgeryCor Triatriatum in Association With a Unique Form of Partial Anomalous Pulmonary Venous Connection.
World journal for pediatric & congenital heart surgery3D reconstruction for preoperative planning of partial anomalous pulmonary venous return.
Kardiologia polskaLate-onset total anomalous pulmonary venous connection in a 70-year-old woman.
BMJ case reportsRare presentation of transposition of great arteries and total anomalous pulmonary venous connection in an 18-year-old.
Echocardiography (Mount Kisco, N.Y.)Atypical presentation of scimitar syndrome with severe hepatomegaly: a case report.
European heart journal. Case reportsPosterior trans-mediastinal lung herniation in a postoperative Marfan's patient.
Clinical imagingSurgical management of the scimitar syndrome.
Journal of cardiac surgeryAssociações
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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.
- Exercise Capacity and Pulmonary Function in Pediatric Patients With Anomalous Pulmonary Venous Connection Post-Surgical Repair: A Retrospective Analysis.
- Partial Anomalous Pulmonary Venous Return in Lung Cancer Surgery: Diagnostic Challenges and Surgical Considerations in Two Cases.
- Transcatheter Correction of Bilateral Partial Anomalous Pulmonary Venous Return with Intrapulmonary Dual Drainage: A Rare Entity.
- Scimitar Syndrome Incidentally Identified During the Workup for Acute Appendicitis in a Young Adult Female: A Case Report.
- Unmasking an Anomalous Vein: Surgical Repair of Left Upper Pulmonary Vein Drainage into the Innominate Vein.
- Left Ventricular Rupture Due to Congenital Partial Defect of the Left Ventricular Free Wall.
- Total Anomalous Pulmonary Venous Connections.
- 'Congenitally corrected' Scimitar syndrome? An anomalous unilateral single pulmonary vein variant.
- Transcatheter correction of the scimitar variant with dual pulmonary venous drainage-an international multicentre series.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:3090(Orphanet)
- MONDO:0017705(MONDO)
- GARD:4599(GARD (NIH))
- Busca completa no PubMed(PubMed)
- 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
