Uma ligação errada de uma ou mais veias pulmonares, que faz com que o sangue rico em oxigênio retorne para o átrio direito do coração.
Introdução
O que você precisa saber de cara
Uma ligação errada de uma ou mais veias pulmonares, que faz com que o sangue rico em oxigênio retorne para o átrio direito do coração.
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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
+ 41 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 129 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
Genes associados
1 gene identificado com associação a esta condição.
Probable transcription activator for a number of lung-specific genes
Nucleus
Alveolar capillary dysplasia with misalignment of pulmonary veins
A rare developmental disorder characterized by abnormal development of the capillary vascular system in the lungs. Histological features include failure of formation and ingrowth of alveolar capillaries, medial muscular thickening of small pulmonary arterioles with muscularization of the intraacinar arterioles, thickened alveolar walls, and anomalously situated pulmonary veins running alongside pulmonary arterioles and sharing the same adventitial sheath. Less common features include a reduced number of alveoli and a patchy distribution of the histopathologic changes. Affected infants present with respiratory distress and the disease is fatal within the newborn period. Additional features include multiple congenital anomalies affecting the cardiovascular, gastrointestinal, genitourinary, and musculoskeletal systems, as well as disruption of the normal right-left asymmetry of intrathoracic or intraabdominal organs. ACDMPV is a rare cause of persistent pulmonary hypertension of the newborn, an abnormal physiologic state caused by failure of transition of the pulmonary circulation from the high pulmonary vascular resistance of the fetus to the low pulmonary vascular resistance of the newborn.
Variantes genéticas (ClinVar)
119 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
2 vias biológicas associadas aos genes desta condição.
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 — Anomalia do retorno venoso
Centros de Referência SUS
24 centros habilitados pelo SUS para Anomalia do retorno venoso
Centros para Anomalia do retorno venoso
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
Adjunctive vein of marshall ethanol infusion for pulmonary vein isolation in unilateral left pulmonary artery agenesis: a case report.
Isolated unilateral agenesis of the pulmonary artery (UAPA) is an exceptionally rare congenital anomaly. In adults, it may promote structural remodelling and predispose to atrial fibrillation (AF). Pulmonary vein (PV) isolation in UAPA poses unique challenges due to anatomical variations, hypoplastic pulmonary veins (PVs), and altered venous drainage, potentially reducing ablation efficacy. An 80-year-old woman with persistent AF and heart failure, and left UAPA, underwent PV isolation using a size-adjustable cryoballoon (POLARx FIT, Boston Scientific, Marlborough, MA, USA). Computed tomography demonstrated the absence of the left pulmonary artery and systemic collateral supply to the left lung. Left superior PV (LSPV) occlusion with a 31-mm cryoballoon achieved complete contrast seal (nadir -56°C) but failed electrical isolation. A subsequent 28-mm application with similar occlusion and nadir temperature achieved isolation with a prolonged time-to-isolation of 97 s. The hypoplastic left inferior PV (LIPV) was not ablated to avoid stenosis risk. During a second procedure, LSPV reconnection and residual LIPV potentials were detected. Ethanol infusion into the vein of Marshall (VOM) successfully isolated both veins. In UAPA, extensive systemic collateral circulation to the affected lung may cause heat dissipation ('heat sink effect'), limiting the efficacy of thermal energy delivery. Moreover, hypoplastic PVs carry an increased risk of stenosis after thermal ablation. Additional non-thermal lesion-creation techniques, such as VOM ethanol infusion, may be necessary to achieve durable and safe isolation when conventional thermal methods are insufficient.
Noncirrhotic Portopulmonary Hypertension Due to Hepatoportal Sclerosis in Adams-Oliver Syndrome.
A 13-year-old girl presented for evaluation of pulmonary hypertension after symptoms of dyspnea and exercise intolerance. Full evaluation was negative except for abdominal ultrasonography with splenomegaly and esophageal varices suggestive of portal hypertension. Cardiac and hepatic vein catheterization confirmed portopulmonary hypertension. Liver biopsy demonstrated sinusoidal dilatation, nodularity, and minimal fibrosis, which was interpreted as possible nodular regenerative hyperplasia but not cirrhosis. Radiographic imaging, including computed tomography venography, demonstrated an elongated and severely stenotic extrahepatic portal vein, and portal hypertension was presumed to be secondary to congenital portal vein hypoplasia. She was treated with ambrisentan with initial improvement in symptoms and estimated pulmonary pressure. Whole-exome sequencing revealed a likely pathogenic missense mutation in Delta-like canonical Notch ligand 4 associated with Adams-Oliver syndrome. After 2 years, pulmonary hypertension and right heart failure symptoms worsened, along with liver failure. She then had fulminant liver failure and cardiorespiratory arrest. Resuscitative efforts included extracorporeal membranous oxygenation (ECMO), but because of hypoxic brain injury, care was compassionately withdrawn. Autopsy limited to the thorax and abdomen revealed high-grade pulmonary plexiform arteriopathy, splenomegaly, esophageal varices, and large splenorenal shunt. The liver was small with a nodular surface but not fibrotic. The entire length of the extrahepatic portal vein was severely stenotic, and intrahepatic portal veins were missing or diminutive-findings diagnostic of hepatoportal sclerosis. Noncirrhotic portopulmonary hypertension is rare and should include evaluation of immunologic, infectious, toxic, thrombotic, and genetic etiologies. Unfortunately, there is no known treatment of hepatoportal sclerosis.
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.
Publicações recentes
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CureusQuantitative plasma proteomic analysis in children after superior cavopulmonary anastomosis with pulmonary arteriovenous malformations.
Pediatric research[Mispositioned central line with catheterization of partial anomalous pulmonary venous return].
Revue medicale de LiegeOutcomes of Patients Undergoing the Kawashima Procedure at an Early Age: A Single Center Experience.
World journal for pediatric & congenital heart surgeryModified Senning Procedure for Treatment of Transposition of the Great Arteries with Crisscross Heart.
Brazilian journal of cardiovascular surgeryEndothelial-to-Mesenchymal Transition in an Hereditary Hemorrhagic Telangiectasia-like Pediatric Case of Multiple Pulmonary Arteriovenous Malformations.
International journal of molecular sciencesClinical Presentation and Therapy of Hypoplastic Left Heart Syndrome.
Advances in experimental medicine and biologyClinical Presentation and Therapy of Total Anomalous Pulmonary Venous Return.
Advances in experimental medicine and biologyA Case Report of an Uncommon Association of Total Intracardiac Anomalous Pulmonary Venous Return and Tetralogy of Fallot.
CureusAdult Partial Anomalous Pulmonary Venous Connection: Case Reports (Including a Case of Scimitar Syndrome).
The Journal of the Association of Physicians of IndiaFactors associated with early postoperative mortality after total anomalous pulmonary venous connection repair: A retrospective cohort study.
MedicineScimitar Syndrome in a Pediatric Cohort.
World journal for pediatric & congenital heart surgeryValley Index as a Predictor of Prenatal Diagnosis of Total Anomalous Pulmonary Venous Connection.
Fetal diagnosis and therapyInfracardiac Total Anomalous Pulmonary Venous Connection.
Radiology. Cardiothoracic imagingAdvanced Imaging Guidance for Ductus Venosus Stenting in Obstructed Total Anomalous Pulmonary Venous Return.
World journal for pediatric & congenital heart surgeryScimitar syndrome with vein stenosis in an infant.
BMJ case reportsN-Butyl cyanoacrylate embolization of an extremely rare variant of sequestration complex - a high-flow left-to-left shunt between systemic artery and pulmonary vein.
Indian journal of thoracic and cardiovascular surgeryRare and potentially lethal case of a round lesion in the lung.
BMJ case reportsTotal Anomalous Pulmonary Venous Connection in Adults: Should We Offer Surgery?
World journal for pediatric & congenital heart surgeryIsolated Partial Anomalous Pulmonary Veins: A 10-Year Experience at a Single Center.
The Journal of surgical researchPredicting High-Risk Fetal Cardiac Disease Anticipated to Need Immediate Postnatal Stabilization and Intervention with Planned Pediatric Cardiac Operating Room Delivery.
Journal of the American Heart Association[Resected Right Upper Lobe Lung Cancer with Displaced Bronchus and Anomalous Pulmonary Vein].
Kyobu geka. The Japanese journal of thoracic surgeryUnveiling a Unique Case of Scimitar Syndrome: Clinical Significance and Multidisciplinary Management Challenges in Pakistan.
CureusSeparating Out Pulmonary Sequestration.
CureusRight S3 segmentectomy for lung cancer with partial anomalous pulmonary venous return in the right upper pulmonary vein: A case report.
Thoracic cancerA 15-Year Experience With Total Anomalous Pulmonary Venous Connection in Vietnam.
World journal for pediatric & congenital heart surgeryA Rare Case of Partial Anomalous Pulmonary Venous Connection.
CureusPeripheral Connection of the Right Pulmonary Vein.
Internal medicine (Tokyo, Japan)Hitherto Unreported Pattern of Complex Obstructive Partial Anomalous Pulmonary Venous Drainage with Dual Drainage of Accessory Pulmonary Veins.
The Indian journal of radiology & imagingTrans-catheter closure of an unusual type of Abernethy malformation in a child presenting with severe pulmonary hypertension.
Cardiology in the youngTranscatheter correction of sinus venosus defect in a patient with a challenging anatomical configuration: From bench testing to clinical success.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & InterventionsLutembacher's Syndrome Accompanied by Partial Anomalies of Pulmonary Veins in a 70-Year-Old Male: A Case of Congenital Heart Disease in the Elderly.
European journal of case reports in internal medicineTranscatheter Closure of Superior Sinus Venosus Defects.
JACC. Cardiovascular interventionsLooking at a baby's heart through the lens of the mother's blood.
EMBO molecular medicinePulmonary arteriovenous malformation and a concurrent patent ductus arteriosus in a Doberman.
Journal of veterinary cardiology : the official journal of the European Society of Veterinary CardiologyContemporary outcomes for functional single ventricle with total anomalous pulmonary venous connection.
The Journal of thoracic and cardiovascular surgeryUnilateral bilobar pulmonary aplasia with anomalous pulmonary venous return: A rare combination of congenital airway and vascular anomalies.
Pediatric pulmonologyLetter by Geva Regarding Article, "Stroke Caused by a Paradoxical Embolus From a Rare Congenital Anomaly in the Adult: Persistent Left Superior Vena Cava Draining Into the Left Upper Pulmonary Vein".
Circulation. Cardiovascular imagingDifferentiating primary sarcomeric hypertrophic cardiomyopathy from Noonan syndrome: can the electrocardiogram be of use?
Cardiology in the youngAdded value of the pulmonary vein pulsatility index and its correlation to neonatal umbilical artery pH in fetal growth restrictions: a Vietnamese matched cohort study.
BMC pregnancy and childbirthSurgical treatment of anomalous right upper lobe pulmonary vein obstruction caused by compression between pulmonary artery and trachea: a case report.
Cardiology in the youngIntracardiac total anomalous pulmonary venous return or septum primum malposition? Complete repair in left atrial isomerism.
Multimedia manual of cardiothoracic surgery : MMCTSClinical Relevance of Rapid FOXF1-Targeted Sequencing in Patients Suspected of Alveolar Capillary Dysplasia With Misalignment of Pulmonary Veins.
Laboratory investigation; a journal of technical methods and pathologyMorphogenesis of the pulmonary vein and left atrial appendage in human embryos and early fetuses.
Journal of anatomyOpitz GBBB syndrome with total anomalous pulmonary venous connection: A new MID1 gene variant.
Molecular genetics & genomic medicineDuplex ultrasound abnormalities of the lower limb veins might precede clinical venous reflux signs in post-Fontan adolescents and young adults.
Journal of vascular surgery. Venous and lymphatic disordersOutcomes and Risk Factors of Interventions for Pediatric Post-operative Pulmonary Vein Stenosis.
Pediatric cardiologyLong-Term Outcomes of Unrepaired Isolated Partial Anomalous Pulmonary Venous Connection With an Intact Atrial Septum.
The American journal of cardiologyRESULTS OF TREATMENT OF DEEP VEINS THROMBOSIS IN PATIENTS WITH CONGENITAL ANOMALIES OF THE INFERIOR VENA CAVA.
Georgian medical newsPulmonary Vein Isolation in a Patient with Superior Vena Cava Atresia: Challenges and Solutions.
The Journal of innovations in cardiac rhythm managementStroke Caused by a Paradoxical Embolus From a Rare Congenital Anomaly in the Adult: Persistent Left Superior Vena Cava Draining into the Left Upper Pulmonary Vein.
Circulation. Cardiovascular imaging[Integrated management during the perinatal period for total anomalous pulmonary venous connection].
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatricsUnilateral pulmonary vein atresia presenting with recurrent hemoptysis and bronchial varices in an Ethiopian adolescent: a case report.
Journal of medical case reportsIntracardiac thrombosis after congenital heart disease surgeries in neonates: a report of two cases.
BMC pediatricsRobotic magnetic navigation-guided pulmonary vein isolation in an atrial fibrillation patient with dextrocardia situs inversus: techniques and potential advantages.
BMC cardiovascular disordersMultimodality diagnostic imaging for anomalous pulmonary venous connections: a pictorial essay.
Pediatric radiologyModification of the Warden Procedure for Surgical Repair of Partial Anomalous Pulmonary Venous Connection.
World journal for pediatric & congenital heart surgeryInfra-cardiac TAPVC with an intrahepatic portosystemic shunt: A rare association.
Annals of pediatric cardiologyComputed tomographic parenchymal lung findings in premature infants with pulmonary vein stenosis.
Pediatric radiologyQuantitative measurements and morphological evaluation of fetal cardiovascular structures with fetal cardiac MRI.
European journal of radiologyAnesthesia management of living donor liver transplantation in a patient with scimitar syndrome.
Saudi journal of anaesthesiaPulsed-field-ablation for the treatment of atrial fibrillation in patients with congenital anomalies of cardiac veins.
Journal of cardiovascular electrophysiologyUncovering an Easily Overlooked Cause of Dyspnea: Partial Anomalous Pulmonary Venous Connection of the Right Pulmonary Vein to the Superior Vena Cava Leading to Right Heart Enlargement.
CureusBetter 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 forumNovel Diabolo Configuration of a Large Covered Stent to Treat Cyanosis Related to Pulmonary Arteriovenous Malformations.
World journal for pediatric & congenital heart surgeryDemonstration of Safety in Wild Type Mice of npFOXF1, a Novel Nanoparticle-Based Gene Therapy for Alveolar Capillary Dysplasia with Misaligned Pulmonary Veins.
Biologics : targets & therapyNeonatal Diagnosis of Alveolar Capillary Dysplasia via Rapid Genomic Sequencing: A New Gold Standard?
NeonatologyIdentifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.
World journal for pediatric & congenital heart surgery[Thoracoscopic Right Lower Lobectomy Safely Performed after the Identification of Anomalous Middle Pulmonary Vein by Preoperative Three-dimensional Computed Tomography Angiography: Report of a Case].
Kyobu geka. The Japanese journal of thoracic surgeryLong-Term Surgical Outcomes of Patients With Isomeric Right and Left Atrial Appendages.
World journal for pediatric & congenital heart surgeryPartial anomalous pulmonary venous connection diagnosed prenatally.
Pediatrics international : official journal of the Japan Pediatric SocietyAlveolar capillary dysplasia with misalignment of pulmonary veins in a premature newborn: the role of lung ultrasound.
The ultrasound journalTotal anomalous pulmonary venous connection in 80 patients: Primary sutureless repair and outcomes.
Frontiers in surgeryAdult congenital horseshoe lung with bilateral pulmonary sequestration: A case report.
Acta radiologica openA Case of Congenital Pulmonary Vein Stenosis with Secondary Post-Capillary Pulmonary Hypertension and Left Sided Congestive Heart Failure in a Cat.
Veterinary sciencesAnatomical attention-based prediction of postoperative pulmonary venous obstruction via CTA images.
Computerized medical imaging and graphics : the official journal of the Computerized Medical Imaging SocietyPrimary sutureless repair concomitant with the Warden procedure.
Asian cardiovascular & thoracic annalsFOXF1 Regulates Alveolar Epithelial Morphogenesis through Transcriptional Activation of Mesenchymal WNT5A.
American journal of respiratory cell and molecular biologyMal-positioning of a dialysis catheter: One lumen with arterial blood and the other with venous blood?
The journal of vascular accessHypogenetic right lung with partial anomalous pulmonary venous return and accessory diaphragm: a case of "scimitar lung".
Folia morphologicaTotal anomalous pulmonary venous connection with another major congenital cardiac defect in biventricular hearts.
Asian cardiovascular & thoracic annalsPartial anomalous pulmonary venous connection: state-of-the-art review with assessment using a multidetector computed tomography angiography.
Polish journal of radiologyUncommon variant of total anomalous pulmonary venous drainage in an infant with sudden death: a case report and review of the literature.
Forensic science, medicine, and pathologyLingular division of the left lung: Only superior and inferior bronchi?
Clinical anatomy (New York, N.Y.)Surgical correction of partial anomalous left superior pulmonary venous connection to the innominate vein.
Multimedia manual of cardiothoracic surgery : MMCTSHaploinsufficiencies of FOXF1, FOXC2 and FOXL1 genes originated from deleted 16q24.1q24.2 fragment related with alveolar capillary dysplasia with misalignment of pulmonary veins and lymphedema-distichiasis syndrome: relationship to phenotype.
Molecular cytogeneticsComputed 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 & congenital heart surgeryA rare case on anomalous pulmonary venous drainage in congenital cardiovascular disease.
Journal of cardiac surgeryThe anomalous drainage of the inferior vena cava into the left atrium.
The Turkish journal of pediatricsTrans-catheter closure of ASD and abnormal connection of left pulmonary vein to vertical vein: A case report.
MedicinePrenatal ultrasonographic features and follow-up outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts diagnosed during the foetal period.
Insights into imagingAbernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis.
Brazilian journal of cardiovascular surgeryCase report of a left superior vena cava to left atrial connection treated with percutaneous covered stent placement.
European heart journal. Case reportsUltrasound findings in neonates with alveolar capillary dysplasia with misalignment of the pulmonary veins: report of two cases.
The Journal of international medical researchObstetrical and neonatal complications in hereditary haemorrhagic telangiectasia: A retrospective study.
BJOG : an international journal of obstetrics and gynaecology[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 surgeryThe 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 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 surgeryPartial anomalous pulmonary venous drainage to persistent left superior caval vein: Or is it a "levoatriocardinal vein"?
Journal of cardiac surgeryTetralogy of Fallot associated with a rare type partially anomalous pulmonary venous connection and levoatrial cardinal vein.
Journal of cardiac surgeryCongenital unilateral pulmonary vein atresia: A rare disease with successful outcome in a child.
Asian cardiovascular & thoracic annalsInvited commentary: Total anomalous pulmonary venous connection remains a challenging pediatric disease.
Journal of cardiac surgeryConservative treatment vs thrombus removal for Iliofemoral vein thrombosis in patients with congenital abnormalities of the inferior vena cava: a case report and systematic review of the literature.
Journal of thrombosis and thrombolysisSuture connection of overlapping covered CP stents for transcatheter treatment of sinus venosus atrial septal defect with anomalous pulmonary venous connection.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & InterventionsAssociações
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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.
- Adjunctive vein of marshall ethanol infusion for pulmonary vein isolation in unilateral left pulmonary artery agenesis: a case report.
- Noncirrhotic Portopulmonary Hypertension Due to Hepatoportal Sclerosis in Adams-Oliver Syndrome.
- 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.
- Unmasking an Anomalous Vein: Surgical Repair of Left Upper Pulmonary Vein Drainage into the Innominate Vein.
- False Thrombus in the Pulmonary Vein: Multimodality in Post-Lung Transplantation.
- Transparavertebral Occlusion of a Large Venovenous Collateral in a Fontan Patient.
- Interorgan Communication-Pulmonary Vein Stenosis in Children-A Review of Epidemiology, Pathophysiology, and Current Management Principles.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:98729(Orphanet)
- MONDO:0020295(MONDO)
- GARD:19559(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q55789287(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
