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Anomalia do retorno venoso pulmonar congênita total
ORPHA:99125CID-10 · Q26.2CID-11 · LA86.20OMIM 106700DOENÇA RARA

A Drenagem Anômala Total das Veias Pulmonares (DATVP) é uma condição do coração presente desde o nascimento. Nela, todas as veias que trazem o sangue oxigenado dos pulmões, em vez de levarem esse sangue para o lado esquerdo do coração (como deveriam), o desviam para o lado direito do coração ou para outros vasos que se conectam a ele. Isso pode levar a diversos problemas, como cansaço, falta de ar ao se esforçar, pressão alta nas artérias dos pulmões, coloração azulada da pele (cianose) e insuficiência cardíaca progressiva (quando o coração vai perdendo a capacidade de bombear o sangue de forma eficiente).

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A Drenagem Anômala Total das Veias Pulmonares (DATVP) é uma condição do coração presente desde o nascimento. Nela, todas as veias que trazem o sangue oxigenado dos pulmões, em vez de levarem esse sangue para o lado esquerdo do coração (como deveriam), o desviam para o lado direito do coração ou para outros vasos que se conectam a ele. Isso pode levar a diversos problemas, como cansaço, falta de ar ao se esforçar, pressão alta nas artérias dos pulmões, coloração azulada da pele (cianose) e insuficiência cardíaca progressiva (quando o coração vai perdendo a capacidade de bombear o sangue de forma eficiente).

Publicações científicas
8 artigos
Último publicado: 2022 Jul-Aug

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
9.0
Worldwide
Início
Neonatal
🏥
SUS: Cobertura mínimaScore: 35%
Centros em: MT, PR, SC, RS, ES +10CID-10: Q26.2
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Entender a doença

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Sinais e sintomas

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

Partes do corpo afetadas

❤️
Coração
17 sintomas
🫁
Pulmão
9 sintomas
🫃
Digestivo
1 sintomas

+ 15 sintomas em outras categorias

Características mais comuns

90%prev.
Desconforto respiratório
Muito frequente (99-80%)
55%prev.
Hipertensão arterial pulmonar
Frequente (79-30%)
55%prev.
Taquicardia
Frequente (79-30%)
55%prev.
Fadiga
Frequente (79-30%)
55%prev.
Dispneia paroxística
Frequente (79-30%)
55%prev.
Conexão venosa pulmonar anômala total supracardíaca
Frequente (79-30%)
42sintomas
Muito frequente (1)
Frequente (9)
Ocasional (25)
Muito raro (5)
Sem dados (2)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 42 características clínicas mais associadas, ordenadas por frequência.

Desconforto respiratórioRespiratory distress
Muito frequente (99-80%)90%
Hipertensão arterial pulmonarPulmonary arterial hypertension
Frequente (79-30%)55%
TaquicardiaTachycardia
Frequente (79-30%)55%
FadigaFatigue
Frequente (79-30%)55%
Dispneia paroxísticaParoxysmal dyspnea
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico8PubMed
Últimos 10 anos196publicações
Pico202229 papers
Linha do tempo
2026Hoje · 2026🧪 2019Primeiro ensaio clínico📈 2022Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
·Pré-clínico5
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 5 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Anomalia do retorno venoso pulmonar congênita total

Centros de Referência SUS

24 centros habilitados pelo SUS para Anomalia do retorno venoso pulmonar congênita total

Centros para Anomalia do retorno venoso pulmonar congênita total

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.

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

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Ensaios clínicos abertos e novidades científicas recentes

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Publicações mais relevantes

Timeline de publicações
2 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

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

Interdisciplinary cardiovascular and thoracic surgery2026 Feb 05

A neonate who underwent corrective surgery for cardiac-type total anomalous pulmonary venous connection (TAPVC) was suspected of having a pseudoaneurysm of the left ventricular (LV) free wall on transthoracic echocardiography (TTE) on postoperative day 11. Emergency surgery was performed the following day, revealing LV rupture due to a congenital partial defect of the LV free wall. The defect was successfully repaired using double-patch closure reinforced with BioGlue. The postoperative course was uneventful. This case highlights that left ventricular rupture may occur due to an unrecognized congenital defect after neonatal cardiac surgery, particularly in conditions such as TAPVC, where the left ventricle is underfilled preoperatively.

#3

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 Association2026 Mar

Perinatal transition is uniquely complex in newborns with critical congenital heart disease (CCHD), whose cardiopulmonary physiology often diverges from the standard neonatal resuscitation framework. We developed lesion-specific clinical practice guidelines (CPGs) for delivery room (DR) management of six high-risk CCHD diagnoses - hypoplastic left heart syndrome, dextro- transposition of the great arteries, Ebstein anomaly, congenital complete heart block, total anomalous pulmonary venous return, and tetralogy of Fallot with absent pulmonary valve. Developed collaboratively between neonatologists, cardiologists, and cardiac intensivists, these guidelines standardize DR stabilization for high-risk, low frequency events, promote a shared mental model among multidisciplinary teams, and provide structured escalation thresholds for oxygen, respiratory support, intubation, and medication use. Beyond clinical standardization, these CPGs enhance provider education, team preparedness, and family counseling. They offer a scalable framework adaptable to centers with or without on-site cardiac care, bridging physiologic principles with practical implementation.

#4

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

The American journal of emergency medicine2026 Feb

Cyanotic critical congenital heart disease (CCHD) is a serious condition associated with high rate of morbidity and mortality. This review highlights key aspects of CCHD, including presentation, diagnosis, and management in the emergency department (ED), based on current evidence. CCHD encompasses several conditions, including truncus arteriosus, dextro-transposition of the great arteries (d-TGA), tricuspid atresia, tetralogy of Fallot (TOF), total anomalous pulmonary venous return (TAPVR), Ebstein's anomaly, critical pulmonary stenosis, and hypoplastic left heart syndrome (HLHS). These conditions should be considered in critically ill neonates presenting with shock and cyanosis. Evaluation involves a thorough assessment of the patient's cardiovascular and respiratory status. Diagnostic tools include pulse oximetry, laboratory testing, electrocardiogram, chest radiography, and ultrasonography. Management requires prompt recognition and hemodynamic stabilization, which may involve the administration of antibiotics, prostaglandins, vasoactive/inotropic agents, and airway management. Expert consultation and coordination of transfer to a tertiary or quaternary care center are crucial. Surgical repair serves as the definitive treatment. A thorough understanding of cyanotic congenital heart disease is essential for emergency clinicians to effectively diagnose and manage this potentially life-threatening condition.

#5

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

Children (Basel, Switzerland)2025 Dec 10

Background: Neurodevelopmental disorders (NDDs), such as autism spectrum disorder (ASD), intellectual disability (ID), and global developmental delay (GDD), frequently have underlying genetic causes. NCKAP1, a gene essential for actin cytoskeleton remodeling and neuronal development, has recently gained recognition as a promising candidate gene in NDDs. While not yet linked to a defined Mendelian disorder, damaging NCKAP1 variants have been identified in individuals with NDDs. NCKAP1 is also expressed in cardiac tissue, with emerging evidence supporting its potential involvement in cardiac development. Here, we present a case of a patient with neurodevelopmental delay and congenital heart disease (CHD) harboring a novel damaging NCKAP1 variant. Methods: Comprehensive clinical evaluations and trio exome sequencing (proband and parents) were conducted on a patient with complex cardiac and neurodevelopmental phenotypes. Results: We identified a de novo heterozygous frameshift variant in NCKAP1, NM_205842.3:c.2956_2959del p.(Ser986Hisfs*34), predicted to result in loss of function through nonsense-mediated mRNA decay. The patient's clinical features included neonatally diagnosed and surgically repaired infradiaphragmatic total anomalous pulmonary venous return (TAPVR), intellectual disability, speech delay, and autistic traits. His NDD phenotypes and variant type align well with previously described NCKAP1-associated NDD, while the cardiac anomaly adds evidence to the gene's expanding phenotypic spectrum. This represents the fourth reported case linking NCKAP1 variants to CHD and/or neurodevelopmental delay. Conclusions: This case strengthens the growing recognition of NCKAP1 in both neurodevelopment and cardiac formation. It highlights the importance of genetic testing for individuals with overlapping developmental and cardiac conditions. Further research is warranted to elucidate the role of NCKAP1 in cardiac development and its contribution to CHD.

Publicações recentes

Ver todas no PubMed

📚 EuropePMCmostrando 196

2026

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

Interdisciplinary cardiovascular and thoracic surgery
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
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

Thoracic Vascular Anomalies: Insights from Embryology and Imaging.

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

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

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

Scientific reports
2025

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

World journal for pediatric &amp; congenital heart surgery
2025

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

Cardiology in the young
2025

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

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

Pediatric cardiology
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

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

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

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

Cureus
2024

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

Medicine
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

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

Journal of cardiothoracic surgery
2024

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

World journal for pediatric &amp; congenital heart surgery
2024

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

International heart journal
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

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

World journal for pediatric &amp; congenital heart surgery
2024

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

Pulmonary circulation
2025

Cor Triatriatum: A Review.

Cardiology in review
2024

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

The Journal of thoracic and cardiovascular surgery
2023

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

Kyobu geka. The Japanese journal of thoracic surgery
2023

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

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

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

The American journal of cardiology
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

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

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

Annals of thoracic surgery short reports
2024

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

Pediatric cardiology
2022

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

Cureus
2023

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

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

Primary sutureless repair concomitant with the Warden procedure.

Asian cardiovascular &amp; thoracic annals
2022

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

Journal of cardiothoracic surgery
2023

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
2023

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

Birth defects research
2023

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

[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.)
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
2023

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

The Annals of thoracic 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

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

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

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

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
2022

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

Pediatric cardiology
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

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

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

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

IEEE journal of biomedical and health informatics
2022

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

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

Emergency surgical treatment of total anomalous pulmonary venous connection.

Journal of cardiac surgery
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

Surgical management of the scimitar syndrome.

Journal of cardiac surgery
2021

Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report.

Journal of cardiothoracic surgery
2021

Perioperative Anomalous Pulmonary Venous Return Evaluation With Low-Dose Cardiac Computed Tomography.

World journal for pediatric &amp; congenital heart surgery
2021

A single center, retrospective analysis of total anomalous pulmonary venous connection repair early outcome at a tertiary care center in India.

Annals of cardiac anaesthesia
2021

Mixed type TAPVR-Measure twice, cut once.

Journal of cardiac surgery
2021

Utility of a modified vascular corrosion casting technique in the diagnosis of fetal total anomalous pulmonary venous connection.

Scientific reports
2022

Effects of universal critical CHD screening of neonates at a mid-sized California congenital cardiac surgery centre.

Cardiology in the young
2022

Total anomalous pulmonary venous connection: Influence of heterotaxy and venous obstruction on outcomes.

The Journal of thoracic and cardiovascular surgery
2021

Totally anomalous pulmonary venous connection with intact atrial septum.

Journal of cardiac surgery
2021

Total anomalous pulmonary venous connection mimicking hypoplastic left heart syndrome.

Cardiology in the young
2021

Outcomes of Surgical Repair of Total Anomalous Pulmonary Venous Drainage: Role of Primary Sutureless Technique.

Pediatric cardiology
2021

Lung cancer with partial anomalous pulmonary venous connection in a different lobe: a case report.

Journal of cardiothoracic surgery
2021

Repair of intracardiac total anomalous pulmonary venous return.

Multimedia manual of cardiothoracic surgery : MMCTS
2021

Repair technique for a rare partial anomalous pulmonary venous return associated with retroaortic innominate vein.

Journal of cardiothoracic surgery
2021

Multidisciplinary management of a rare case of mixed total anomalous pulmonary venous connection.

Journal of cardiac surgery
2021

Surgical correction for scimitar syndrome by right thoracotomy and direct anastomosis in children.

Pediatric investigation
2021

Anomalous branch of right pulmonary artery supplying the left lung: A "pseudo-pulmonary sling".

Journal of cardiac surgery
2021

Early and Intermediate-Term Results for the Combined Superior Approach Correction of Supracardiac Total Anomalous Pulmonary Venous Connection in Neonatal Patients.

Heart, lung &amp; circulation
2021

Smaller right pulmonary artery is associated with longer survival time without scimitar vein repair.

Journal of cardiac surgery
2021

Re: Primary sutureless repair for infracardiac total anomalous pulmonary venous connection.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2021

Prenatal diagnosis of total anomalous pulmonary venous connection: 2D and 3D echocardiographic findings.

Journal of clinical ultrasound : JCU
2021

Repair of a mixed form of supracardiac total anomalous pulmonary venous connection.

Cardiology in the young
2020

Supracardiac total anomalous pulmonary venous connection with cor triatriatum sinister: A rare diagnosis confirmed by saline contrast echocardiography.

Echocardiography (Mount Kisco, N.Y.)
2020

A Scimitar Syndrome Variant Associated with Critical Aortic Coarctation in a Newborn.

The American journal of case reports
2020

Automatic Pulmonary Vein and Left Atrium Segmentation for TAPVC Preoperative Evaluation Using V-Net with Grouped Attention.

Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International Conference
2021

Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection.

General thoracic and cardiovascular surgery
2021

Sustained Total All-Region (STAR) perfusion for Norwood reconstruction with complex intracardiac repair.

Perfusion
2021

Single-institution outcomes of surgical repair of infracardiac total anomalous pulmonary venous connection.

The Journal of thoracic and cardiovascular surgery
2020

Congenital superior vena cava (SVC) stenosis and obstructed supracardiac total anomalous pulmonary venous connection (TAPVC)-A surgical perspective.

Journal of cardiac surgery
2021

Younger Age at Operation Is Associated With Reinterventions After the Warden Procedure.

The Annals of thoracic surgery
2020

The use of an autologous right atrial free wall as a patch for closure of atrial septal defects.

Journal of cardiac surgery
2020

Congenital extracardiac venous system anomaly in two siblings with normal karyotype and increased nuchal translucency thickness: a case report.

Oxford medical case reports
2020

Continuous real-time monitoring of pulmonary arterial pressure using a tip deflecting microcatheter in an infant with pulmonary arterial hypertension.

Cardiology in the young
2020

Adults forms of scimitar syndrome.

Journal of cardiac surgery
2020

Approach to Cyanotic Congenital Heart Disease in Children.

Indian journal of pediatrics
2020

Double drainage of total anomalous pulmonary venous connection: An extremely rare variant.

Journal of cardiac surgery
2020

Isomerism of the atrial appendages: morphology and terminology.

Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology
2020

Surgical Management of Heterotaxy Syndrome: Current Challenges and Opportunities.

World journal for pediatric &amp; congenital heart surgery
2020

Outcomes of the Warden Procedure for Partial Anomalous Pulmonary Venous Drainage.

Pediatric cardiology
2020

Trends in Infant Mortality After TAPVR Repair over 18 Years in Texas and Impact of Hospital Surgical Volume.

Pediatric cardiology
2020

d-Transposition of Great Arteries and Total Anomalous Pulmonary Venous Connection With Left Ventricular Regression-A Rarity.

World journal for pediatric &amp; congenital heart surgery
2019

Assessing the recently noted surgical outcome of isolated total anomalous pulmonary venous connection repair: A single-secondary center experience.

Journal of cardiac surgery
2019

A Rare Case of Cardiogenic Shock: Cor Triatriatum With Anomalous Pulmonary Venous Return.

World journal for pediatric &amp; congenital heart surgery
2020

A Wide Comparison of Techniques for Repair of PAPVCs: One Institution's 20-Year Experience.

The Thoracic and cardiovascular surgeon
2019

Clinical outcomes after the endovascular treatments of pulmonary vein stenosis in patients with congenital heart disease.

Cardiology in the young
2019

Prenatal diagnosis and prevalence of critical congenital heart defects: an international retrospective cohort study.

BMJ open
2019

A new palliative surgical technique for high risk Total anomalous pulmonary venous connection (Sarmast-Takriti shunt).

Journal of cardiothoracic surgery
2019

Infracardiac total anomalous pulmonary venous return in a patient with Williams syndrome: A case report.

Medicine
2019

Long-term Outcomes of the Fontan Operation in Patients With Total Anomalous Pulmonary Venous Drainage.

The Annals of thoracic surgery
2019

Computed Tomography Angiography and Magnetic Resonance Angiography of Congenital Anomalies of Pulmonary Veins.

Journal of computer assisted tomography
2020

Diagnosis and Management of the Unligated Vertical Vein in Repaired Total Anomalous Pulmonary Venous Connection.

World journal for pediatric &amp; congenital heart surgery
2019

A rare association of left pulmonary artery sling with right pulmonary hypoplasia and total anomalous pulmonary venous connection.

Cardiology in the young
2019

Total Anomalous Pulmonary Venous Connection Beyond the First Decade of Life.

World journal for pediatric &amp; congenital heart surgery
2020

Successful Surgical Management of a Rare Case of Mixed Type of Total Anomalous Pulmonary Venous Connection in a Very Low Birth Weight Preterm Child Using a Novel Surgical Technique.

World journal for pediatric &amp; congenital heart surgery
2018

Total anomalous pulmonary vein drainage in a 60-year-old woman diagnosed in an ECG-gated multidetector computed tomography - a case report and review of literature.

Polish journal of radiology
2019

Evaluation of the post-LA space index in the normal fetus.

Prenatal diagnosis
2019

A Novel Mouse Model for Cilia-Associated Cardiovascular Anomalies with a High Penetrance of Total Anomalous Pulmonary Venous Return.

Anatomical record (Hoboken, N.J. : 2007)
2019

Surgical results of total anomalous pulmonary venous connection repair in 256 patients.

Interactive cardiovascular and thoracic surgery
2019

Is Sutureless Technique Beneficial in the Primary Repair of Total Anomalous Pulmonary Venous Connection? A Systematic Review and Meta-Analysis.

Pediatric cardiology
2018

Prevalence of congenital cardiac anomalies in patients with cleft lip and palate - Its implications in surgical management.

Journal of oral biology and craniofacial research
2018

Familial total anomalous pulmonary venous return with 15q11.2 (BP1-BP2) microdeletion.

Journal of human genetics
2018

Single Ventricle and Total Anomalous Pulmonary Venous Connection: Implications of Prenatal Diagnosis.

World journal for pediatric &amp; congenital heart surgery
2018

Computed tomography findings and preoperative risk factors for mortality of total anomalous pulmonary venous connection.

The international journal of cardiovascular imaging
2018

Maternal Obesity as a Risk Factor for the Development of Total Anomalous Pulmonary Venous Connection in Their Offspring.

Archives of medical research
2018

Sutureless technique versus conventional surgery in the primary treatment of total anomalous pulmonary venous connection: a systematic review and meta-analysis.

Journal of cardiothoracic surgery
2018

Postoperative Obstruction of the Pulmonary Veins in Mixed Total Anomalous Pulmonary Venous Connection.

Pediatric cardiology
2018

Neonatal Outcomes in Total Anomalous Pulmonary Venous Return: The Role of Prenatal Diagnosis and Pulmonary Venous Obstruction.

Pediatric cardiology
2018

Dual drainage total anomalous pulmonary venous connection: a rare mixed variant.

Asian cardiovascular &amp; thoracic annals
2018

Pulmonary vein obstruction after primary sutureless pericardial repair of a total anomalous pulmonary venous connection.

Interactive cardiovascular and thoracic surgery
2018

Beating the odds: a rare case of supracardiac total anomalous pulmonary venous return (TAPVR) in an adult patient.

BMJ case reports
2018

Long-term outcomes of total correction for isolated total anomalous pulmonary venous connection: lessons from 50-years' experience.

Interactive cardiovascular and thoracic surgery
2018

Superior Approach for Supracardiac Total Anomalous Pulmonary Venous Connection.

The Annals of thoracic surgery
2017

Congenital heart defects in Williams syndrome.

The Turkish journal of pediatrics
2018

Morphological dilemma: Anomalous pulmonary venous confluence or cor triatriatum-does it matter?

Journal of the Saudi Heart Association
2017

Prenatal diagnosis of total anomalous pulmonary venous connection by 2D and 3D fetal echocardiography.

Echocardiography (Mount Kisco, N.Y.)
2017

Outcomes of Surgery for Mixed Total Anomalous Pulmonary Venous Drainage in Children.

Seminars in thoracic and cardiovascular surgery
2017

A rare variant of intracardiac total anomalous pulmonary venous connection.

Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology
2019

Retroesophageal Infracardiac Total Anomalous Pulmonary Venous Drainage.

World journal for pediatric &amp; congenital heart surgery
2018

Prenatal diagnosis of total and partial anomalous pulmonary venous connection: multicenter cohort study and meta-analysis.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2017

Total anomalous pulmonary venous connection (TAPVC): A familial cluster of 3 siblings.

Echocardiography (Mount Kisco, N.Y.)
2017

Prenatal diagnosis of isolated total anomalous systemic venous connection to the left atrium.

Cardiology in the young
2017

Total Anomalous Pulmonary Venous Connection in Children: Preoperative Evaluation with Low-Dose Multidetector Computed Tomographic Angiography.

Texas Heart Institute journal
2017

Whole-exome sequencing identifies SGCD and ACVRL1 mutations associated with total anomalous pulmonary venous return (TAPVR) in Chinese population.

Oncotarget
2017

Type IV Total Anomalous Pulmonary Venous Connection.

World journal for pediatric &amp; congenital heart surgery
2017

Obstructed Infradiaphragmatic Total Anomalous Pulmonary Venous Return in a 13-Day-Old Infant Presenting Acutely to the Emergency Department: A Case Report.

The Journal of emergency medicine
2017

Prenatal diagnoses of an uncommon isolated obstructed supracardiac total anomalous pulmonary venous connection: Case report and review of the literature (CARE compliant).

Medicine
2017

Surgical Considerations in Total Anomalous Pulmonary Venous Connection.

Seminars in cardiothoracic and vascular anesthesia
2016

Anomalous pulmonary venous connection: An underestimated entity.

Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology
2016

Continuous Cardiopulmonary Bypass During the Repair of Total Anomalous Pulmonary Venous Return.

World journal for pediatric &amp; congenital heart surgery
2017

Total Anomalous Pulmonary Venous Connection: Preoperative Anatomy, Physiology, Imaging, and Interventional Management of Postoperative Pulmonary Venous Obstruction.

Seminars in cardiothoracic and vascular anesthesia
2017

Multidetector Computed Tomographic Angiography Imaging of Congenital Pulmonary Venous Anomalies: A Pictorial Review.

Canadian Association of Radiologists journal = Journal l'Association canadienne des radiologistes
2017

Perioperative and Anesthetic Considerations in Total Anomalous Pulmonary Venous Connection.

Seminars in cardiothoracic and vascular anesthesia
2016

[Tetralogy of Fallow with Total Anomalous Pulmonary Vein Return and Atrial Septal Defect;Successful Two-staged Surgical Management;Report of a Case].

Kyobu geka. The Japanese journal of thoracic surgery
2017

Unusual presentation of total anomalous systemic venous connection.

Asian cardiovascular &amp; thoracic annals
2016

Descending aortic flow reversal in obstructed total anomalous pulmonary venous connection.

Cardiology in the young
2016

Bodily isomerism is an independent risk factor for pulmonary hypertension in adults with congenital heart disease.

Therapeutic advances in respiratory disease
2017

Total anomalous pulmonary venous connection with ventricular septal defects.

Asian cardiovascular &amp; thoracic annals
2015

Early- and intermediate-term results of surgical correction in 122 patients with total anomalous pulmonary venous connection and biventricular physiology.

Journal of cardiothoracic surgery
2015

Modified Senning Procedure for Correction of Atrioventricular Discordance With Total Anomalous Pulmonary Venous Return, Atrial Situs Inversus, Dextrocardia, and Bilateral Superior Venae Cavae.

The Annals of thoracic surgery
2016

Magnetic resonance imaging in the assessment of anomalous pulmonary venous connections.

Radiologia
2015

Portal Vein Thrombosis of a Newborn with Corrected Total Anomalous Pulmonary Venous Return.

Turkish journal of haematology : official journal of Turkish Society of Haematology
2015

[Total Anomalous Pulmonary Venous Connection].

Kyobu geka. The Japanese journal of thoracic surgery
2015

[Total isolated anomalous pulmonary venous return: Are there any clues for prenatal screening?].

Gynecologie, obstetrique &amp; fertilite
2015

Repair Type Influences Mode of Pulmonary Vein Stenosis in Total Anomalous Pulmonary Venous Drainage.

The Annals of thoracic surgery
2015

Shared Segment Analysis and Next-Generation Sequencing Implicates the Retinoic Acid Signaling Pathway in Total Anomalous Pulmonary Venous Return (TAPVR).

PloS one
2015

Profile and outcome of congenital heart diseases in children: a preliminary experience from a tertiary center in sokoto, north Western Nigeria.

The Nigerian postgraduate medical journal
2015

Adult Scimitar Syndrome: A Surgical Approach.

Heart, lung &amp; circulation
2014

Total anomalous pulmonary venous return in siblings.

Journal of cardiovascular ultrasound

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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. Left Ventricular Rupture Due to Congenital Partial Defect of the Left Ventricular Free Wall.
    Interdisciplinary cardiovascular and thoracic surgery· 2026· PMID 41601237mais citado
  3. 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· 2026· PMID 41490934mais citado
  4. High risk and low incidence diseases: Cyanotic critical congenital heart disease.
    The American journal of emergency medicine· 2026· PMID 41237673mais citado
  5. A De Novo Loss-of-Function NCKAP1 Variant in a Boy with Neurodevelopmental Delay and Congenital Heart Defect.
    Children (Basel, Switzerland)· 2025· PMID 41462819mais citado
  6. Left superior vena cava or left vertical vein in supracardiac total anomalous pulmonary venous drainage - Does it make a difference?
    Ann Pediatr Cardiol· 2022· PMID 36935838recente
  7. Determination of anomalous pulmonary venous return with high-pitch low-dose computed tomography in paediatric patients.
    Folia Morphol (Warsz)· 2021· PMID 32459365recente
  8. Neonatal repair of truncus arteriosus with "scimitar-like" mixed total pulmonary venous return.
    Ann Thorac Surg· 2014· PMID 24882335recente
  9. Atresia of the common pulmonary vein.
    Cardiol Young· 2006· PMID 16839434recente
  10. Stenting of the vertical vein in obstructed total anomalous pulmonary venous return as rescue procedure in a neonate.
    Catheter Cardiovasc Interv· 2006· PMID 16575922recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:99125(Orphanet)
  2. OMIM OMIM:106700(OMIM)
  3. MONDO:0007130(MONDO)
  4. GARD:16896(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. Q2557206(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Anomalia do retorno venoso pulmonar congênita total
Compêndio · Raras BR

Anomalia do retorno venoso pulmonar congênita total

ORPHA:99125 · MONDO:0007130
Prevalência
1-9 / 100 000
Herança
Not applicable
CID-10
Q26.2 · Comunicação venosa pulmonar anormal total
CID-11
Início
Neonatal
Prevalência
9.0 (Worldwide)
MedGen
UMLS
C0036400
Wikidata
Papers 10a
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