Raras
Buscar doenças, sintomas, genes...
Ventrículo direito com dupla saída
ORPHA:3426CID-10 · Q20.1CID-11 · LA85.2DOENÇA RARA

Ventrículo direito de dupla saída (DORV) é uma condição cardíaca rara em que a aorta e a artéria pulmonar — os dois grandes vasos que saem do coração — se originam, total ou em sua maior parte, do ventrículo direito.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Ventrículo direito de dupla saída (DORV) é uma condição cardíaca rara em que a aorta e a artéria pulmonar — os dois grandes vasos que saem do coração — se originam, total ou em sua maior parte, do ventrículo direito.

Pesquisas ativas
2 ensaios
7 total registrados no ClinicalTrials.gov
Publicações científicas
2.069 artigos
Último publicado: 2026 Apr
Medicamentos
2 registrados
PROPRANOLOL HYDROCHLORIDE, PROPRANOLOL

Tem tratamento?

2 medicamentos registrados
Ver detalhes, fases e interações →
PROPRANOLOL HYDROCHLORIDEPROPRANOLOL

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-5 / 10 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Infancy
+ neonatal
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q20.1
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

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

Partes do corpo afetadas

❤️
Coração
6 sintomas
😀
Face
4 sintomas
📏
Crescimento
3 sintomas
🦴
Ossos e articulações
1 sintomas
🫃
Digestivo
1 sintomas
🧠
Neurológico
1 sintomas

+ 11 sintomas em outras categorias

Características mais comuns

100%prev.
Ventrículo direito de saída dupla
90%prev.
Cianose
Muito frequente (99-80%)
55%prev.
Estenose pulmonar
Frequente (79-30%)
55%prev.
Defeito do septo ventricular
Frequente (79-30%)
55%prev.
Déficit de crescimento
Frequente (79-30%)
55%prev.
Ponte nasal deprimida
Frequente (79-30%)
28sintomas
Muito frequente (2)
Frequente (17)
Ocasional (9)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 28 características clínicas mais associadas, ordenadas por frequência.

Ventrículo direito de saída duplaDouble outlet right ventricle
Muito frequente100%
CianoseCyanosis
Muito frequente (99-80%)90%
Estenose pulmonarPulmonic stenosis
Frequente (79-30%)55%
Defeito do septo ventricularVentricular septal defect
Frequente (79-30%)55%
Déficit de crescimentoFailure to thrive
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico2.069PubMed
Últimos 10 anos200publicações
Pico202581 papers
Linha do tempo
2026Hoje · 2026🧪 2007Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
1Fase 13
·Pré-clínico6
Medicamentos catalogadosEnsaios clínicos· 2 medicamentos · 7 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Ventrículo direito com dupla saída

🗺️

Selecione um estado ou use sua localização para ver resultados.

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

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

2 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

7 ensaios clínicos encontrados, 2 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
709 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 709

#1

Neurodevelopmental outcomes in preschool children with congenital heart defects: A case-control study using Ages & Stages Questionnaire.

PloS one2026

Um estudo comparando o neurodesenvolvimento de crianças pré-escolares com doenças cardíacas congênitas que exigiram cirurgia (incluindo o Dupla Saída do Ventrículo Direito - DORV) e controles saudáveis, revelou que, embora a pontuação média geral fosse comparável, as crianças com defeitos cardíacos tinham cinco vezes mais chances de obter uma pontuação baixa no questionário ASQ. Este risco aumentado foi impulsionado principalmente por casos de Defeito do Septo Ventricular (DSV) não detectado prenatalmente, e uma tendência de pontuações mais baixas em Transposição das Grandes Artérias (TGA), especialmente quando não detectada antes do nascimento, sem diferenças específicas reportadas para o DORV isoladamente. Esses achados são cruciais para médicos e pais, sublinhando a importância do acompanhamento de todas as crianças com cardiopatia congênita cirúrgica, incluindo aquelas com DORV, para identificar precocemente e intervir em potenciais atrasos no desenvolvimento.

🇧🇷 traduzido
#2

Anaesthetic management in a neonate with a double-outlet right ventricle undergoing repair of leaking myelomeningocele.

BMJ case reports2026 Mar 10

Este artigo destaca a extrema complexidade do manejo anestésico em recém-nascidos com múltiplos defeitos congênitos graves, como a Dupla Via de Saída do Ventrículo Direito (DORV) e mielomeningocele. Para médicos, o relato demonstra que uma estratégia de anestesia intravenosa total, utilizando dexmedetomidina e fentanil, pode ser uma opção segura e eficaz para manter a estabilidade hemodinâmica e oferecer neuroproteção em cirurgias de alto risco nestes pacientes pediátricos.

🇧🇷 traduzido
#3

Sustained remission of post-Fontan protein-losing enteropathy achieved by combination therapy with high-dose spironolactone and the somatostatin analogue octreotide: a case report.

BMC pediatrics2026 Feb 27

Este relato de caso demonstrou o tratamento bem-sucedido de enteropatia perdedora de proteínas (EPP) pós-Fontan, grave e refratária, em uma criança com cardiopatia congênita complexa (incluindo ventrículo direito de dupla saída). Após falha da terapia padrão, a combinação de espironolactona em dose alta com octreotide induziu remissão completa da condição. Notavelmente, o octreotide pôde ser descontinuado com a manutenção da remissão a longo prazo, sugerindo que a espironolactona em doses elevadas pode ser uma opção eficaz para pacientes e médicos, especialmente para reduzir a exposição prolongada ao octreotide e seus potenciais efeitos adversos.

🇧🇷 traduzido
#4

Young-onset hepatocellular carcinoma following double outlet right ventricle surgery without liver congestion: a case report with genetic analysis.

Clinical journal of gastroenterology2026 Feb

Este artigo descreve o caso incomum de uma jovem de 16 anos que desenvolveu câncer de fígado (HCC) anos após uma cirurgia para cardiopatia congênita (DORV), sem as habituais complicações hepáticas como congestão ou cirrose. A análise genética aponta para fatores genéticos subjacentes, sugerindo um mecanismo diferente de desenvolvimento do câncer. Para médicos e pacientes, isso significa que o rastreamento para câncer de fígado deve ser considerado em indivíduos de alto risco com cardiopatias congênitas, mesmo na ausência de problemas hepáticos visíveis ou função hepática alterada.

🇧🇷 traduzido
#5

Long-term Outcomes of Standardized Strategy in Arterial Switch Operation: An 18-Year Review in a Single Center.

Korean circulation journal2026 Mar

Este estudo avaliou os resultados a longo prazo da Cirurgia de Switch Arterial (ASO) utilizando uma estratégia cirúrgica padronizada, mostrando desfechos geralmente favoráveis com baixa mortalidade operatória e tardia. No entanto, desafios significativos a longo prazo incluem a necessidade de reintervenção em cerca de um quarto dos pacientes e uma alta incidência de dilatação da raiz neo-aórtica (em 63% dos pacientes em 15 anos). Pacientes com subtipos mais complexos de Transposição das Grandes Artérias (TGA) têm maior risco dessas complicações e de reoperações, o que requer monitoramento contínuo.

🇧🇷 traduzido

Publicações recentes

Ver todas no PubMed

📚 EuropePMC719 artigos no totalmostrando 199

2026

Half-turned truncal switch for transposition of the great arteries with left ventricular outflow tract obstruction.

JTCVS techniques
2026

In vitro construction of handmade polytetrafluoroethylene right ventricle-to-pulmonary artery valved conduit using a modification of the Ozaki technique.

JTCVS techniques
2026

Aortic root replacement after repaired congenital conotruncal defects: A surgical challenge.

JTCVS open
2026

Switch-back Ross, Bentall/Konno and valve-sparing root replacement: three cutting-edge solutions to complex left ventricular outflow tract obstruction post arterial switch in Taussig-Bing anomaly.

Multimedia manual of cardiothoracic surgery : MMCTS
2026

Ultrasound-Guided Superficial Temporal Artery Cannulation for Proximal Arterial Pressure Monitoring During Coarctation Repair With an Aberrant Right Subclavian Artery in a Low-Birth-Weight Neonate.

Cureus
2026

Double-inlet double-outlet right ventricle: a case report and systematic review of the literature with cluster analysis.

Cardiology in the young
2026

Single Ventricle: Radiology Primer for Preprocedural Evaluation.

Radiographics : a review publication of the Radiological Society of North America, Inc
2026

Enhancer of zeste homolog 2 (Ezh2) is required in mouse Isl1-expressing progenitors for proper development of cardiac and skeletal hindlimb structures.

Biology open
2026

Neurodevelopmental outcomes in preschool children with congenital heart defects: A case-control study using Ages & Stages Questionnaire.

PloS one
2026

A Case of Restrictive Dermopathy With Atypical Cardiac Anomalies and a Novel ZMPSTE24 Variant.

American journal of medical genetics. Part A
2026

Successful palliation and Fontan completion in a patient with double outlet right ventricle and intact ventricular septum.

Cardiology in the young
2026

Norwood procedure with left coronary artery reimplantation for hypoplastic left heart syndrome combined with Anomalous left coronary artery from the right pulmonary artery: a case report.

General thoracic and cardiovascular surgery cases
2026

Durability of the Arterial Switch Operation: Long-Term Survival and Structural Sequelae in a Multicentre Cohort.

The Canadian journal of cardiology
2026

Infective endocarditis in an adult male patient with tetralogy of Fallot physiology secondary to double outlet right ventricle presenting with stroke: a complex presentation with multiorgan dysfunction-a case report.

Journal of medical case reports
2026

Anaesthetic management in a neonate with a double-outlet right ventricle undergoing repair of leaking myelomeningocele.

BMJ case reports
2026

Case Report: Parachute tricuspid valve in association with cyanotic congenital heart disease and juxtaposed atrial appendages in a 1-year-old boy-a rare anatomical association.

Frontiers in cardiovascular medicine
2026

Sustained remission of post-Fontan protein-losing enteropathy achieved by combination therapy with high-dose spironolactone and the somatostatin analogue octreotide: a case report.

BMC pediatrics
2026

One-stage repair of Taussig-Bing anomaly in a neonate with aortic arch hypoplasia and single coronary artery.

Multimedia manual of cardiothoracic surgery : MMCTS
2026

Surgical management of double outlet right ventricle with outflow obstruction: a systematic review of biventricular strategies.

Cardiology in the young
2026

Physics-Informed Emulation of Systemic Circulation for Fast Parameter Estimation and Uncertainty Quantification.

International journal for numerical methods in biomedical engineering
2026

Reference standards and diagnosis-specific trends in cardiorespiratory fitness in paediatric patients with repaired CHD.

Cardiology in the young
2025

Double-outlet Right Ventricle with Subaortic Ventricular Septal Defect and Severe Pulmonary Valve Stenosis, but Only Minimal Cyanosis and No Murmur: A Paradox?

Journal of cardiovascular echography
2025

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

Journal of cardiovascular echography
2025

Reversal of Glenn-associated diffuse pulmonary arteriovenous malformations after total cavopulmonary connection: a case report.

Frontiers in cardiovascular medicine
2026

Accuracy of fetal echocardiography in detecting lesions associated with, and predicting surgical plan for, dextro-transposition of the great arteries and double outlet right ventricle with subpulmonary ventricular septal defect and predicted transposition physiology.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2026

Infant with Complex Congenital Cardiac Anomalies: A Case Report and Review of Literature.

Annals of African medicine
2026

Double-Drainage Levoatrial Cardinal Vein: Cardiac CT Evaluation of Compensatory Pulmonary Venous Pathway in Univentricular Heart.

JACC. Case reports
2026

Pulmonary Vein-Triggered Atrial Fibrillation in Unrepaired Double-Outlet Right Ventricle With Pulmonary Atresia.

JACC. Case reports
2026

A One-Dimensional (1D) Computational Fluid Dynamics Study of Fontan-Associated Liver Disease (FALD).

International journal for numerical methods in biomedical engineering
2026

Haploinsufficiency of Endothelial Nitric Oxide Synthase Mitigates Beneficial Effects of Maternal Exercise on Fetal Heart Development During Pregestational Diabetes.

Journal of the American Heart Association
2025

Transcatheter pulmonary valve replacement with the venusP-valve system in a patient with double outlet right ventricle and transposition of the great arteries a first-in-human case report.

Frontiers in cardiovascular medicine
2025

The 24-Segment Global Sphericity Index Is Useful for Assessing Fetal Cardiac Function and as an Indicator of Fetal Well-Being.

Cureus
2025

Double Outlet Right Ventricle With a Not-Directly-Committed Ventricular Septal Defect.

Korean circulation journal
2026

Multimodality Imaging of Double-Outlet Right Ventricle, Superior-Inferior Ventricles, and Malposed Great Arteries.

JACC. Case reports
2025

A Novel STAG2 Frameshift Variant in Mullegama-Klein-Martinez Syndrome with Complex Conotruncal Heart Defect.

Genes
2025

Criss cross heart: an outflow tract defect?

Orphanet journal of rare diseases
2025

Right Ventricular Myocardial Strain and Proteomic Analysis of Fetuses With Left Ventricular Hypoplasia: A Cross-Sectional Study.

Health science reports
2026

Young-onset hepatocellular carcinoma following double outlet right ventricle surgery without liver congestion: a case report with genetic analysis.

Clinical journal of gastroenterology
2026

Long-term Outcomes of Standardized Strategy in Arterial Switch Operation: An 18-Year Review in a Single Center.

Korean circulation journal
2025

Identifying Pathogenic Variants in Vietnamese Children with Functional Single Ventricle Based on Whole-Exome Sequencing.

Diagnostics (Basel, Switzerland)
2025

Balloon dilation of the para-device space to alleviate severe flow restriction in an oversized pulmonary flow restrictor: a case report.

BMC cardiovascular disorders
2025

Successful Transcatheter Pulmonary Valve Replacement Via the Azygous Vein in a Patient With Interrupted Inferior Vena Cava.

JACC. Case reports
2025

Long-Term Surgical Outcomes in Double Outlet Right Ventricle Based on Detailed Anatomical Sub-Typology.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2025

Clarifying the Cardiovascular Morphology and Associated Abnormalities in Patients with Double Inlet Left Ventricle Using Multidetector CT Angiography.

Pediatric cardiology
2025

Optimising pre-operative assessment in double-outlet right ventricle: the complementary role of echocardiography and CT angiography.

Cardiology in the young
2025

Multisystem Phenotypic Spectrum in Pediatric Heterotaxy Syndrome: A Case Series.

Cureus
2025

Anterior Pulmonary Artery Translocation with the Damus-Kaye-Stansel and Rastelli Procedures for Multilevel Tracheobronchomalacia with Double-Outlet Right Ventricle (S, D, L) and Right Pulmonary Agenesis.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2025

Endoscopic Third Ventriculostomy in Hydrocephalus Due to Complex Congenital Heart Disease: A Case Report.

Cureus
2025

Iatrogenic aorto-right ventricular fistula after transcatheter pulmonary valvuloplasty.

Asian cardiovascular &amp; thoracic annals
2025

Double-Outlet Right Ventricular Malformation in a Two-Year-Old Aberdeen Angus Cow.

Animals : an open access journal from MDPI
2026

Comparison for Long-Term Results of the Modified Réparation à l´étage Ventriculaire and Rastelli Repair.

World journal for pediatric &amp; congenital heart surgery
2025

Acute stent placement for dynamic airway collapse after cardiac surgery in congenital heart disease with bronchial stenosis: A case report.

Medicine
2025

Conotruncal Anomalies of the Fetus.

Ultraschall in der Medizin (Stuttgart, Germany : 1980)
2025

Successful ablation of ventricular tachycardia in which resected infundibular septum served as a critical isthmus in a patient with double-outlet right ventricle after Rastelli surgery.

HeartRhythm case reports
2025

Classification of Fetal Congenital Heart Disease by Prenatal Ultrasound and Its Diagnostic Value.

International journal of general medicine
2025

Technical Advances and Outcomes of Fetal Atrial Septal Intervention for Restrictive or Intact Atrial Septum.

Circulation. Cardiovascular interventions
2025

Genetic Variants Associated With Congenital Heart Disease: A Meta-Analysis of Ethnicity and Subtype-Specific Susceptibility.

Circulation. Genomic and precision medicine
2026

First-Trimester Detection of Conotruncal Anomalies Using Transabdominal SlowflowHD: A Case Series and Proof-of-Concept Study.

Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine
2026

Utility of inhaled nitric oxide for pulmonary hypertension in cyanotic congenital heart disease: a cohort study with propensity score matching.

Medical gas research
2026

Outcomes of Fontan patients undergoing combined heart-liver transplantation in pediatric hospitals across the United States.

The Journal of thoracic and cardiovascular surgery
2025

KRAS4B is required for placental development.

Cellular and molecular life sciences : CMLS
2025

Infective endocarditis leading to pulmonary pseudoaneurysm and severe pulmonary valve regurgitation: A case report.

Medicine
2025

Reaching consensus as to how knowledge of development underscores our understanding of deficient ventricular septation.

Cardiology in the young
2025

Straddling of the mitral valve in the set of situs solitus in dextrocardia with atrio-ventricular connection discordance and double-outlet right-ventricle: a rare case report.

Archivos de cardiologia de Mexico
2025

Heterotaxy syndrome with biliary atresia: a case report.

The Pan African medical journal
2025

A rare complication of cyanotic CHD: brain abscess.

Cardiology in the young
2025

The Ultrasound and Genetic Characteristics of Fetuses With Laterality Defects-A Prenatal Cohort in Asian Population.

Prenatal diagnosis
2025

Incidence, Associated Abnormalities, and Outcomes of Dextrocardia: A Registry-based Study in Saudi Arabia.

Journal of the Saudi Heart Association
2025

Double-Outlet Right Ventricle in an Adult With a Univentricular Heart and Total Situs Inversus.

JACC. Case reports
2025

Postpartum ischemic stroke due to persistent left superior vena cava to left atrium after DORV repair: a case report and literature review.

BMC cardiovascular disorders
2025

Congenital Conundrum: Unraveling the Puzzle of Double Outlet Right Ventricle for the Neonatologist.

NeoReviews
2025

Using Multimodality Imaging and 3-Dimensional Printed Models to Guide Decision-Making for Complex Congenital Biventricular Repair.

JACC. Case reports
2025

Mortality Associated With Viral Bronchiolitis in a Pediatric Department: A Retrospective Analysis.

Cureus
2025

Significance of Papillary and Trabecular Muscular Volume in Right Ventricular Volumetry with Cardiac MR Imaging.

Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in Medicine
2025

Navigating Complexity: Advanced Visualization for Müllerian Anomalies and its Implications for Diagnoses and Surgical Planning.

Journal of pediatric and adolescent gynecology
2025

A Retrospective Study on the Imaging Spectrum of Functional Single Ventricle and Its Associations.

The Indian journal of radiology &amp; imaging
2025

Aortic Disturbed Flow Is Associated with Aortic Angle in Patients with Tetralogy of Fallot or Double-Outlet Right Ventricle.

Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in Medicine
2025

Coarctation Stenting When Origin of Aberrant Right Subclavian Artery Is Before Stenotic Segment.

JACC. Case reports
2025

Valve-preserving technique for tetralogy of fallot by transannular delamination.

Asian cardiovascular &amp; thoracic annals
2025

Unraveling the complexities of double outlet right ventricle (DORV): A rare case of congenital heart disease with bidirectional shunting in a young girl.

Radiology case reports
2025

Hemodynamics of a right ventricular outflow tract aneurysm assessed with 4D flow MRI before and after unilateral pulmonary artery balloon angioplasty: A case report.

Radiology case reports
2025

Prominent Chiari Network as a Cause of Cyanosis in Infants: A Case Report and Implications for Pediatric Cardiology Practice.

Journal of clinical ultrasound : JCU
2025

A new association between Kleefstra syndrome and Panayiotopoulos epilepsy.

Italian journal of pediatrics
2025

Longitudinal Trends in Pediatric Survival by Congenital Heart Defect in Texas, 1999 to 2017.

JACC. Advances
2025

An isolated double-inlet right ventricular malformation: a case report and review of the literature.

Journal of medical case reports
2025

Single and Double Root Translocation for Transposition and Malposition of the Great Arteries.

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual
2025

Can We Better Understand the Anatomy of Channels Between the Ventricles on the Basis of Knowledge of Their Development?

World journal for pediatric &amp; congenital heart surgery
2025

Aspirin Use Following the Arterial Switch Operation in the United States: Variability, Trends, and Perioperative Outcomes.

World journal for pediatric &amp; congenital heart surgery
2024

Hemodynamic rounds: Transcatheter creation of ventricular septal defect in pulmonary arterial hypertension with suprasystemic pressures.

Annals of pediatric cardiology
2024

Management dilemma of a child with long-segment tracheal stenosis, double outlet right ventricle, and left pulmonary artery sling: For single or staged repair?

Annals of pediatric cardiology
2025

Total anomalous pulmonary venous connection - prenatal echocardiography and neonatal follow-up.

Journal of ultrasonography
2024

[MÖB-03] Extra-Pericardial Modified Blalock-Taussig Shunt Via Sternotomy in Patients with A Right Aortic Arch.

Turk gogus kalp damar cerrahisi dergisi
2025

Born to be Bad: Combination of Aorticopulmonary Window With Double-Outlet Right Ventricle-Pulmonary Atresia.

Echocardiography (Mount Kisco, N.Y.)
2025

Complex Management of Thromboembolism in Double-Outlet Right Ventricle.

JACC. Case reports
2025

An extreme case of Raghib syndrome: combination of mirror-image dextrocardia, single atrium, double-outlet right ventricle, and patent ductus arteriosus.

European heart journal
2025

Practical approach to using cardiac magnetic resonance imaging for pre-surgical planning in complex paediatric congenital heart disease.

Pediatric radiology
2025

Enhancing management of double outlet right ventricle when the interventricular communication is remote from the arterial roots through three-dimensional printing.

3D printing in medicine
2025

Criss-cross heart with complex cardiac malformation, situs inversus totalis and bronchial dysplasia.

BMC cardiovascular disorders
2025

Cumulative radiation dose in children with congenital heart disease: national data 2000-2021, stratified by diagnosis, age, and imaging modality.

Acta radiologica (Stockholm, Sweden : 1987)
2025

Criss-cross heart with double-outlet right ventricle and interrupted aortic arch in a rare adult survivor: computed tomography insights.

European heart journal
2025

Utility of 3-Dimensional Echocardiography in Patients With Complex Intracardiac Connections and Ventricular Septal Defects.

JACC. Case reports
2025

Repeated Systolic Anterior Motion of the Mitral Valve After Double Outlet Right Ventricle Repair.

Annals of thoracic surgery short reports
2025

Outcomes of Fontan operation in patients with conotruncal anomalies from Australia and New Zealand Fontan Registry: a review of 1835 patients†.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2025

Pregnancy in ACHD women: crucial role of multidisciplinary clinical roadmap.

The journal of maternal-fetal &amp; neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians
2025

Parameter selection and optimization of a computational network model of blood flow in single-ventricle patients.

Journal of the Royal Society, Interface
2024

[Double Outlet Right Ventricle (DORV)].

The Thoracic and cardiovascular surgeon
2024

Pediatric Three-dimensional Transesophageal Echocardiography: A Game Changer in Congenital Heart Disease.

Journal of cardiovascular echography
2025

Surgical Repair of Double Outlet Right Ventricle Infants Guided by Three Dimensional-Computed Tomography Cardiac Modeling and Printing.

World journal for pediatric &amp; congenital heart surgery
2025

A De Novo Frameshift Variant in SMC1A Causes Non-Classic Cornelia de Lange Syndrome With Epilepsy: A Case Report and Literature Review.

Molecular genetics &amp; genomic medicine
2024

Prevalence of immunological aberrations and 22q11.2 deletion in children with conotruncal anomalies: A cross-sectional study.

Annals of pediatric cardiology
2025

Double Outlet Right Ventricle: A Rare Finding in a 15-Month-Old Female With Failure to Thrive.

Clinical case reports
2026

Printed Models for Better Prediction of Surgery in Patients with Double Outlet Right Ventricle.

Pediatric cardiology
2026

Clarifying the Cardiovascular Morphology and Associated Abnormalities in Patients with Double Inlet Right Ventricle Using Multidetector CT Angiography.

Pediatric cardiology
2025

Double-outlet right ventricle in a patient with Takenouchi-Kosaki syndrome.

Pediatrics and neonatology
2025

Interventional Treatment of Acute Right Ventricular Outflow Tract Infectious Endocarditis: A Bridge to Surgical or Percutaneous Pulmonary Valve Replacement.

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography &amp; Interventions
2024

Technique for staged biventricular repair of heterotaxy, double-outlet right ventricle, unbalanced atrioventricular canal defect, and transposition.

JTCVS techniques
2025

Congenital heart defects differ following left versus right avian cardiac neural crest ablation.

Developmental biology
2025

Cyclin-dependent kinase 13 is indispensable for normal mouse heart development.

Journal of anatomy
2025

Relating normal human cardiac development to the anatomical findings in the congenitally malformed heart.

Clinical anatomy (New York, N.Y.)
2024

Successful surgical repair of Taussig Bing anomaly with pulmonary artery and pulmonary valve stenosis in a neonate patient: A case report.

International journal of surgery case reports
2025

Contemporary prenatal diagnosis of congenital heart disease in a regional perinatal center lacking onsite pediatric cardiac surgery: obstetrical and neonatal outcomes.

Journal of perinatal medicine
2025

A Prevalent TMEM260 Deletion Causes Conotruncal Heart Defects, Including Truncus Arteriosus.

American journal of medical genetics. Part A
2024

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

Asian cardiovascular &amp; thoracic annals
2024

Very high take-off the right coronary artery with double outlet right ventricle.

Cardiology in the young
2024

Prenatally Diagnosed Case of Successful Survival of an Unguarded Mitral Valve Orifice.

JACC. Case reports
2024

Investigation of the Clinical Value of Four Visualization Modalities for Congenital Heart Disease.

Journal of cardiovascular development and disease
2024

Double-Outlet Right Ventricle With Intact Ventricular Septum and Left Atrioventricular Valve Regurgitation in a Patient With Right Atrial Isomerism.

World journal for pediatric &amp; congenital heart surgery
2025

High-resolution three-dimensional atlas of congenital heart defects based on micro-CT images of human postmortem wax-infiltrated heart specimens.

Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology
2024

Nationwide Birth Prevalence of Crucial Congenital Heart Defects From 2014 to 2018 in Korea.

Korean circulation journal
2024

Spectrum of Heart Diseases in Children in a National Cardiac Referral Center Tanzania, Eastern Africa: A Six-Year Overview.

Global heart
2024

Surgical correction of total anomalous pulmonary venous connection to persistent left-sided superior vena cava: a case report.

European heart journal. Case reports
2024

Left ventricular apical pacing-induced heart failure in a child after congenital heart surgery: a case report.

European heart journal. Case reports
2025

Long-term Outcome After Repair of Transposition of the Great Arteries With Aortic Arch Obstruction.

The Annals of thoracic surgery
2024

Application of array comparative genomic hybridization on the DNA extracted from cultured amniocytes for rapid diagnosis of 22q11.2 microdeletion in a pregnancy with a normal karyotype at amniocentesis and double outlet right ventricle and transposition of great arteries on level II ultrasound.

Taiwanese journal of obstetrics &amp; gynecology
2024

[Analysis of Prenatal Ultrasound Manifestations in 15 Cases of Cantrell Syndrome].

Sichuan da xue xue bao. Yi xue ban = Journal of Sichuan University. Medical science edition
2024

Follow-up of a Term Infant with a Prenatal Diagnosis of Double-Outlet Right Ventricle.

NeoReviews
2024

Arterial Switch Operation for Transposition of the Great Arteries: Can We Do it Better?

JACC. Advances
2024

Clinical Course of TGA After Arterial Switch Operation in the Current Era.

JACC. Advances
2024

Long-term Outcomes of Surgical Repair for Corrected Transposition of the Great Arteries.

The Annals of thoracic surgery
2025

Clinical Course of Residual Ventricular Septal Defects After Congenital Heart Disease Repair.

Pediatric cardiology
2024

A new approach to surgical correction of double-outlet right ventricle with remote interventricular communication.

JTCVS techniques
2024

Human Genetics of Defects of Situs.

Advances in experimental medicine and biology
2024

Molecular Pathways and Animal Models of Tetralogy of Fallot and Double Outlet Right Ventricle.

Advances in experimental medicine and biology
2024

Human Genetics of Tetralogy of Fallot and Double-Outlet Right Ventricle.

Advances in experimental medicine and biology
2024

Clinical Presentation and Therapy of Tetralogy of Fallot and Double-Outlet Right Ventricle.

Advances in experimental medicine and biology
2024

Cardiac Transcription Factors and Regulatory Networks.

Advances in experimental medicine and biology
2024

Cardiac Development and Animal Models of Congenital Heart Defects.

Advances in experimental medicine and biology
2024

Reoperations in Adolescents and Adults After Prior Arterial Switch Operation: The Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis.

The Annals of thoracic surgery
2024

Surgical repair of unusual variant of double-outlet right ventricle with discordant atrioventricular connections and superior-inferior ventricles.

JTCVS techniques
2024

Relationship between diagnosis of conus arteriosus malformation and genetic diagnosis results in fetal cardiac axis abnormalities by echocardiography during middle pregnancy.

Frontiers in cardiovascular medicine
2024

The developmental processes of ventricular septal defects with outflow tract malalignment.

Annals of anatomy = Anatomischer Anzeiger : official organ of the Anatomische Gesellschaft
2024

Clinical implications of dextrocardia based on four visceroatrial situs studies.

Pediatrics and neonatology
2024

Recent advances in multimodal imaging in tetralogy of fallot and double outlet right ventricle.

Current opinion in cardiology
2024

Impact of right ventriculotomy on cardiac function after pulmonary valve sparing repair of tetralogy of Fallot and double outlet right ventricle with pulmonary stenosis.

Cardiology in the young
2024

Repair of the complete atrioventricular septal defect-impact of postoperative moderate or more regurgitation.

Interdisciplinary cardiovascular and thoracic surgery
2024

Atrioventricular valve surgery in patients with univentricular heart and two separate atrioventricular valves.

Cardiology in the young
2024

En Bloc Rotation of the Outflow Tracts - Double Root Translocation.

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

Two-Dimensional and Doppler trans-thoracic echocardiographic patterns of suspected pediatric heart diseases at Tibebe--Ghion specialized Teaching Hospital and Adinas General Hospital, Bahir Dar, North-west Ethiopia:-An experience from an LMIC.

PloS one
2024

Navigating the spectrum of double-outlet right ventricle presentations: Outcomes from a contemporary cohort based on subtypes.

Echocardiography (Mount Kisco, N.Y.)
2022

Three-dimensional printed moulds to obtain silicone hearts with congenital defects for paediatric heart-surgeon training.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2024

Preoperative surgical planning for biventricular repair of double outlet right ventricle by using a 3D heart model.

Quantitative imaging in medicine and surgery
2024

Double outlet right ventricle - the 50% rule has always been about the conus.

Current opinion in cardiology
2024

Antifungal medication use during early pregnancy and the risk of congenital heart defects in the National Birth Defects Prevention Study, 1997-2011.

Birth defects research
2024

What are the conotruncal malformations?

The Journal of thoracic and cardiovascular surgery
2023

Gaming on an Immersive Virtual Reality Platform to Ameliorate the Level of Anxiety in Patients Undergoing Congenital Heart Disease.

Cureus
2024

Different leadless pacemakers working in harmony (Aveir in the atrium/Micra AV2 in the ventricle) in a patient with dextrocardia and double outlet right ventricle after high-risk infected device extraction.

Journal of cardiovascular electrophysiology
2023

Long-term results after the réparation à l'étage ventriculaire procedure for transposition of the great arteries and double-outlet right ventricle with pulmonary stenosis.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2024

Multicentre study on late outcomes of biventricular repair of double outlet right ventricle.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2024

Persistent neo-aortic root dilatation and aortic valve insufficiency after arterial switch operation following prior pulmonary artery banding.

Indian journal of thoracic and cardiovascular surgery
2023

Comprehensive Multiomics Analysis of Monozygotic Twin Discordant for Double Outlet Right Ventricle.

Twin research and human genetics : the official journal of the International Society for Twin Studies
2024

How best can we name the channels seen in the setting of deficient ventricular septation?

Cardiology in the young
2024

Intracardiac Three-Dimensional Image as Surgical Decision-Making Tool of Congenital Heart Disease.

Pediatric cardiology
2023

3D Printed Cardiac Models as an Adjunct to Traditional Teaching of Anatomy in Congenital Heart Disease-A Randomised Controlled Study.

Heart, lung &amp; circulation
2023

Recanalized vein of marshall following bidirectional glenn surgery.

The Journal of invasive cardiology
2024

A Rare Entity: Double Outlet of Both Ventricles.

World journal for pediatric &amp; congenital heart surgery
2024

Growth of the Neo-Aortic Root and Prognosis of Transposition of the Great Arteries.

Journal of the American College of Cardiology
2024

Cardiovascular anomalies in patients with Tessier syndrome: a systematic review.

European journal of pediatrics
2023

Trans-aortic DORV repair.

Indian journal of thoracic and cardiovascular surgery
2024

Lower Socioeconomic Status is Associated with an Increased Incidence and Spectrum of Major Congenital Heart Disease and Associated Extracardiac Pathology.

Pediatric cardiology
2023

Complex cyanotic congenital heart disease and cervical spine surgery: Ticking all the right boxes.

Annals of cardiac anaesthesia
2023

Improved Surgical Outcomes in Adults with Congenital Heart Disease and Decompensated Heart Failure: The Role of Perioperative Medical Optimization.

The American journal of case reports
2023

Double outlet right ventricle.

Frontiers in pediatrics
2024

Optimizing Surgical Selection for Transposition With Left Ventricular Outflow Tract Obstruction.

The Annals of thoracic surgery
2024

Outcomes of neo-aortic valve and root surgery late after arterial switch operation.

The Journal of thoracic and cardiovascular surgery
2024

Early Outcomes of Pulmonary Valve Replacement With the Edwards Inspiris Resilia Pericardial Bioprosthesis.

World journal for pediatric &amp; congenital heart surgery
2023

Stenting for Left Ventricular Outflow Tract Stenosis after Double Outlet Right Ventricle Repair.

Acta Cardiologica Sinica
2024

Outcomes of atrioventricular septal defects with and without down syndrome: analysis of the national inpatient database.

Cardiology in the young
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
2024

The Arterial Switch Operation in the Developing World: Risk Factors and Current Outcomes.

The Annals of thoracic surgery
2023

Exploring the Role of Modified Vascular Anatomical Molding (MVAM) in Prenatal Diagnosis Teaching and Prognosis Prediction of Fetal Complex Congenital Heart Disease (CCHD): A Preliminary Study.

International journal of general medicine
2023

Atrioventricular conduction recovery immediately after the re-operation in a repaired CHD patient.

Cardiology in the young
2023

Perioperative Management of Emergency Craniotomes in Children With Cyanotic Congenital Heart Disease: A Case Series.

Cureus
2023

Characterization of ultrasound and postnatal pathology in fetuses with heterotaxy syndrome.

Frontiers in cardiovascular medicine
2023

A rare case of diagnosed absent aortic valve and severely hypoplastic pulmonary valve with double outlet right ventricle: A case report.

Heliyon
2024

The retirement years of Doctor Helen B. Taussig: an intersection of art and medicine.

Cardiology in the young
2023

Outcomes after implantation of right-sided mechanical valve prostheses in congenital heart disease.

Heart (British Cardiac Society)
2023

Ten-year follow-up of dilatation of aortic structures in Fallot-type anomalies.

Pediatric cardiology
2023

Sinus of Valsalva Aneurysm in a Preschooler With Schuurs-Hoeijmakers Syndrome.

Annals of thoracic surgery short reports
2023

Right Aortic Arch with Isolated Left Subclavian Artery in Association with Double Outlet Right Ventricle and Cor Triatriatum Sinister: Hitherto Unreported Association.

The Indian journal of radiology &amp; imaging
Ver todos os 719 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Neurodevelopmental outcomes in preschool children with congenital heart defects: A case-control study using Ages &amp; Stages Questionnaire.
    PloS one· 2026· PMID 41880298mais citado
  2. Anaesthetic management in a neonate with a double-outlet right ventricle undergoing repair of leaking myelomeningocele.
    BMJ case reports· 2026· PMID 41806986mais citado
  3. Sustained remission of post-Fontan protein-losing enteropathy achieved by combination therapy with high-dose spironolactone and the somatostatin analogue octreotide: a case report.
    BMC pediatrics· 2026· PMID 41761153mais citado
  4. Young-onset hepatocellular carcinoma following double outlet right ventricle surgery without liver congestion: a case report with genetic analysis.
    Clinical journal of gastroenterology· 2026· PMID 41251886mais citado
  5. Long-term Outcomes of Standardized Strategy in Arterial Switch Operation: An 18-Year Review in a Single Center.
    Korean circulation journal· 2026· PMID 41168126mais citado
  6. Half-turned truncal switch for transposition of the great arteries with left ventricular outflow tract obstruction.
    JTCVS Tech· 2026· PMID 41971863recente
  7. In vitro construction of handmade polytetrafluoroethylene right ventricle-to-pulmonary artery valved conduit using a modification of the Ozaki technique.
    JTCVS Tech· 2026· PMID 41971850recente
  8. Aortic root replacement after repaired congenital conotruncal defects: A surgical challenge.
    JTCVS Open· 2026· PMID 41960099recente
  9. Ultrasound-Guided Superficial Temporal Artery Cannulation for Proximal Arterial Pressure Monitoring During Coarctation Repair With an Aberrant Right Subclavian Artery in a Low-Birth-Weight Neonate.
    Cureus· 2026· PMID 41930035recente
  10. Double-inlet double-outlet right ventricle: a case report and systematic review of the literature with cluster analysis.
    Cardiol Young· 2026· PMID 41891181recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:3426(Orphanet)
  2. MONDO:0018089(MONDO)
  3. GARD:1908(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q361648(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

Ventrículo direito com dupla saída
Compêndio · Raras BR

Ventrículo direito com dupla saída

ORPHA:3426 · MONDO:0018089
Prevalência
1-5 / 10 000
Herança
Multigenic/multifactorial
CID-10
Q20.1 · Ventrículo direito com dupla via de saída
CID-11
Ensaios
2 ativos
Medicamentos
2 registrados
Início
Infancy, Neonatal
Prevalência
0.0 (Europe)
MedGen
UMLS
C0013069
EuropePMC
Wikidata
Papers 10a
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