Raras
Buscar doenças, sintomas, genes...
Defeito do canal auriculoventricular
ORPHA:98722CID-10 · Q21.2CID-11 · LA87.4DOENÇA RARA

São vários tipos de problemas nas paredes (septos) que dividem as câmaras do coração: a parede entre os átrios (as câmaras superiores), a parede entre os ventrículos (as câmaras inferiores) e as válvulas que ficam entre eles (a tricúspide e a bicúspide/mitral). Esses defeitos acontecem porque algumas estruturas do coração do bebê em desenvolvimento, chamadas coxins endocárdicos, não crescem nem se unem completamente. Essas estruturas são cruciais para formar os dois canais que conectam as câmaras superiores às inferiores, que é o lugar onde as futuras válvulas do coração (tricúspide e bicúspide) se desenvolverão.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

São vários tipos de problemas nas paredes (septos) que dividem as câmaras do coração: a parede entre os átrios (as câmaras superiores), a parede entre os ventrículos (as câmaras inferiores) e as válvulas que ficam entre eles (a tricúspide e a bicúspide/mitral). Esses defeitos acontecem porque algumas estruturas do coração do bebê em desenvolvimento, chamadas coxins endocárdicos, não crescem nem se unem completamente. Essas estruturas são cruciais para formar os dois canais que conectam as câmaras superiores às inferiores, que é o lugar onde as futuras válvulas do coração (tricúspide e bicúspide) se desenvolverão.

Pesquisas ativas
1 ensaio
9 total registrados no ClinicalTrials.gov
Publicações científicas
1.685 artigos
Último publicado: 2026 Apr
Medicamentos
3 registrados
PREDNISOLONE, CYCLOSPORINE, EVEROLIMUS

Tem tratamento?

3 medicamentos registrados
Ver detalhes, fases e interações →
PREDNISOLONECYCLOSPORINEEVEROLIMUS
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q21.2
🇧🇷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
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

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

Partes do corpo afetadas

❤️
Coração
29 sintomas
🫁
Pulmão
5 sintomas
📏
Crescimento
2 sintomas
💪
Músculos
2 sintomas
🫃
Digestivo
1 sintomas
😀
Face
1 sintomas

+ 23 sintomas em outras categorias

Características mais comuns

Retorno venoso pulmonar anômalo
Defeito do canal atrioventricular
Eletrocardiograma anormal
Retrações intercostais
Tetralogia de Fallot
Mal-estar pós-esforço
63sintomas
Sem dados (63)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 63 características clínicas mais associadas, ordenadas por frequência.

Retorno venoso pulmonar anômaloAnomalous pulmonary venous return
Defeito do canal atrioventricularAtrioventricular canal defect
Eletrocardiograma anormalAbnormal EKG
Retrações intercostaisIntercostal retractions
Tetralogia de FallotTetralogy of Fallot

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico1.685PubMed
Últimos 10 anos200publicações
Pico202481 papers
Linha do tempo
2026Hoje · 2026🧪 2001Primeiro ensaio clínico📈 2024Ano 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

Genes associados

4 genes identificados com associação a esta condição.

NR2F2COUP transcription factor 2Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Ligand-activated transcription factor. Activated by high concentrations of 9-cis-retinoic acid and all-trans-retinoic acid, but not by dexamethasone, cortisol or progesterone (in vitro). Regulation of the apolipoprotein A-I gene transcription. Binds to DNA site A. May be required to establish ovary identity during early gonad development (PubMed:29478779)

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (1)
Transcriptional regulation of white adipocyte differentiation
MECANISMO DE DOENÇA

Congenital heart defects, multiple types, 4

A disorder characterized by congenital developmental abnormalities involving structures of the heart. Common defects include transposition of the great arteries, aortic stenosis, atrial septal defect, ventricular septal defect, pulmonic stenosis, and patent ductus arteriosus. Some patients also have cardiac arrhythmias, which may be due to the anatomic defect itself or to surgical interventions.

EXPRESSÃO TECIDUAL(Ubíquo)
Ovário
311.8 TPM
Nervo tibial
204.9 TPM
Cervix Ectocervix
190.7 TPM
Cervix Endocervix
189.9 TPM
Artéria tibial
176.0 TPM
OUTRAS DOENÇAS (4)
46,xx sex reversal 5congenital heart defects, multiple types, 4complete atrioventricular canal-ventricle hypoplasia syndromecomplete atrioventricular canal-tetralogy of fallot syndrome
HGNC:7976UniProt:P24468
GATA4Transcription factor GATA-4Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Transcriptional activator that binds to the consensus sequence 5'-AGATAG-3' and plays a key role in cardiac development and function (PubMed:24000169, PubMed:27984724, PubMed:35182466). In cooperation with TBX5, it binds to cardiac super-enhancers and promotes cardiomyocyte gene expression, while it down-regulates endocardial and endothelial gene expression (PubMed:27984724). Involved in bone morphogenetic protein (BMP)-mediated induction of cardiac-specific gene expression. Binds to BMP respons

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (10)
Developmental Lineage of Pancreatic Acinar CellsDevelopmental Lineage of Multipotent Pancreatic Progenitor CellsDevelopmental Lineage of Pancreatic Ductal CellsSynthesis, secretion, and inactivation of Glucose-dependent Insulinotropic Polypeptide (GIP)Cardiogenesis
MECANISMO DE DOENÇA

Atrial septal defect 2

A congenital heart malformation characterized by incomplete closure of the wall between the atria resulting in blood flow from the left to the right atria. Patients show other heart abnormalities including ventricular and atrioventricular septal defects, pulmonary valve thickening or insufficiency of the cardiac valves. The disease is not associated with defects in the cardiac conduction system or non-cardiac abnormalities.

EXPRESSÃO TECIDUAL(Tecido-específico)
Ovário
144.1 TPM
Coração - Átrio
53.7 TPM
Testículo
51.1 TPM
Coração - Ventrículo esquerdo
45.2 TPM
Artéria coronária
40.7 TPM
OUTRAS DOENÇAS (12)
tetralogy of fallotventricular septal defect 1testicular anomalies with or without congenital heart diseaseatrial septal defect 2
HGNC:4173UniProt:P43694
CRELD1Protein disulfide isomerase CRELD1Candidate gene tested inTolerante
FUNÇÃO

Protein disulfide isomerase (By similarity). Promotes the localization of acetylcholine receptors (AChRs) to the plasma membrane (By similarity)

LOCALIZAÇÃO

Membrane

MECANISMO DE DOENÇA

Atrioventricular septal defect 2

A congenital heart malformation characterized by a common atrioventricular junction coexisting with deficient atrioventricular septation. The complete form involves underdevelopment of the lower part of the atrial septum and the upper part of the ventricular septum; the valve itself is also shared. A less severe form, known as ostium primum atrial septal defect, is characterized by separate atrioventricular valvar orifices despite a common junction.

EXPRESSÃO TECIDUAL(Ubíquo)
Cerebelo
112.2 TPM
Cérebro - Hemisfério cerebelar
106.8 TPM
Tireoide
99.4 TPM
Pituitária
96.0 TPM
Aorta
74.3 TPM
INTERAÇÕES PROTEICAS (1)
OUTRAS DOENÇAS (5)
Jeffries-Lakhani neurodevelopmental syndromeatrioventricular septal defect, susceptibility to, 2obsolete partial atrioventricular septal defect without ventricular hypoplasiacomplete atrioventricular canal-ventricle hypoplasia syndrome
HGNC:14630UniProt:Q96HD1
GATA6Transcription factor GATA-6Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Transcriptional activator (PubMed:19666519, PubMed:22750565, PubMed:22824924, PubMed:27756709). Regulates SEMA3C and PLXNA2 (PubMed:19666519). Involved in gene regulation specifically in the gastric epithelium (PubMed:9315713). May regulate genes that protect epithelial cells from bacterial infection (PubMed:16968778). Involved in bone morphogenetic protein (BMP)-mediated cardiac-specific gene expression (By similarity). Binds to BMP response element (BMPRE) DNA sequences within cardiac activati

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (5)
Formation of definitive endodermCardiogenesisDevelopmental Lineage of Multipotent Pancreatic Progenitor CellsSurfactant metabolismFactors involved in megakaryocyte development and platelet production
EXPRESSÃO TECIDUAL(Ubíquo)
Ovário
121.3 TPM
Glândula adrenal
65.6 TPM
Fallopian Tube
61.2 TPM
Aorta
54.6 TPM
Artéria coronária
51.1 TPM
OUTRAS DOENÇAS (11)
conotruncal heart malformationstetralogy of fallotpancreatic hypoplasia-diabetes-congenital heart disease syndromeatrial septal defect 9
HGNC:4174UniProt:Q92908

Medicamentos e terapias

PREDNISOLONEPhase 3

Mecanismo: Glucocorticoid receptor agonist

CYCLOSPORINEPhase 3

Mecanismo: Cyclophilin A modulator

EVEROLIMUSPhase 3

Mecanismo: FK506-binding protein 1A inhibitor

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

533 variantes patogênicas registradas no ClinVar.

🧬 NR2F2: NM_021005.4(NR2F2):c.971-12A>G ()
🧬 NR2F2: GRCh38/hg38 15q26.1-26.3(chr15:90068508-101888837)x3 ()
🧬 NR2F2: NM_021005.4(NR2F2):c.1133T>A (p.Ile378Lys) ()
🧬 NR2F2: NM_021005.4(NR2F2):c.139_155dup (p.Thr55fs) ()
🧬 NR2F2: GRCh37/hg19 15q26.1-26.3(chr15:90569376-102369410)x1 ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 1,501 variantes classificadas pelo ClinVar.

826
675
VUS (55.0%)
Benigna (45.0%)
VARIANTES MAIS SIGNIFICATIVAS
GATA4: NM_001308093.3(GATA4):c.1220C>T (p.Ser407Phe) [Uncertain significance]
GATA6: NM_005257.6(GATA6):c.137G>T (p.Cys46Phe) [Uncertain significance]
GATA6: NM_005257.6(GATA6):c.998A>G (p.His333Arg) [Uncertain significance]
GATA6: NM_005257.6(GATA6):c.565G>C (p.Val189Leu) [Uncertain significance]
GATA6: NM_005257.6(GATA6):c.743G>C (p.Gly248Ala) [Uncertain significance]

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.
3Fase 34
2Fase 23
1Fase 11
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 3 medicamentos · 6 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Defeito do canal auriculoventricular

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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

Pesquisa e ensaios clínicos

9 ensaios clínicos encontrados, 1 ativos.

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

Timeline de publicações
745 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 745

#1

'Double-ring'-like dysplastic mitral leaflet associated with atrioventricular septal defect mimicking endocarditis: computed tomography as key to diagnosis.

European heart journal. Case reports2026 Feb

Atrioventricular septal defect (AVSD) is categorized into 3 anatomical types: complete, partial, and transitional (or intermediate). Complete AVSDs feature both an atrial and a ventricular septal defect, as well as a common AV valve. Partial AVSDs contain an interatrial communication (primum atrial septal defect) but lack an interventricular communication. Transitional AVSDs are a variant of complete AVSDs, characterized by an interatrial communication and a restrictive interventricular communication. Complete AVSDs can be further classified using the Rastelli classification into types A, B, and C, based on the anatomical variations of the superior bridging leaflet and its attachments to the ventricular septum. Additionally, AVSDs can be categorized as balanced or unbalanced. A balanced AVSD features a single, large AV valve that opens nearly equally into both ventricles, resulting in similarly sized ventricles. Conversely, an unbalanced AVSD has the common valve displaced toward 1 ventricle, resulting in that ventricle being significantly larger. This distinction influences whether a biventricular or single-ventricle repair is recommended. Pathophysiology and clinical presentation vary considerably by AVSD subtype. Mortality approaches 50% within the first year of life among children with unrepaired complete AVSD, most commonly due to congestive heart failure and pneumonia. Survors develop irreversible pulmonary arterial hypertension; unfortunately, long-term survival is low. Surgical management has evolved significantly, ranging from palliative pulmonary artery banding to complete AVSD repair. Early detection through echocardiography, appropriate preoperative care, and meticulous management of the AV valve morphology are essential to ensure optimal long-term survival and functional outcomes in this group.

#2

Case Report: Anaesthetic management in a canine patient with severe atrioventricular septal defect and pulmonary hypertension undergoing non-cardiac surgery.

Frontiers in veterinary science2026

A 9-year-old, 6.8-kg intact male Petit Brabançon with a congenital partial atrioventricular septal defect (AVSD) presented for dental extractions. Pre-anaesthetic echocardiographic assessment revealed a large ostium primum atrial septal defect with bidirectional interatrial shunting, severe right atrial and ventricular enlargement, abnormal atrioventricular valves with severe tricuspid regurgitation, mild relative pulmonic stenosis, and moderate pulmonary hypertension (PH). Anaesthetic goals were to minimize increases in pulmonary vascular resistance (PVR), preserve right ventricular perfusion and cardiac output (CO), and avoid alterations in intracardiac shunt dynamics that could promote right-to-left shunting. Premedication consisted of methadone [0.2 mg/kg intramuscularly (IM)], lidocaine (1 mg/kg intravenously (IV)) and midazolam (0.3 mg/kg IV). Continuous infusions of lidocaine (30-50 μg/kg/min) and remifentanil (10-30 μg/kg/h) were used as inhalant minimum alveolar concentration (MAC)-sparing analgesic adjuncts. Anaesthesia was induced with ketamine (1 mg/kg IV) and propofol (2 mg/kg IV) and maintained with sevoflurane in oxygen and air. Controlled ventilation targeted normocapnia and the fraction of inspired oxygen (FiO₂) was titrated to maintain pulse oximeter (SpO₂) values above 95%, while avoiding unnecessary hyperoxia. A norepinephrine infusion (0.2-0.4 μg/kg/min) supported mean arterial pressure (MAP) > 65 mmHg. Regional dental nerve blocks supplemented analgesia. Fourteen teeth were extracted during a 64-min anaesthetic. Recovery was complicated by transient hypoxemia attributed to upper airway obstruction, resolving with oxygen supplementation and nebulized adrenaline. No arrhythmias or sustained right-to-left shunting occurred. The dog was discharged the same day and returned to normal activity. Survival after the procedure was 14 months before euthanasia for acute respiratory decompensation. This report highlights practical, physiology-guided strategies to conduct anaesthesia in non-cardiac procedures in dogs with severe AVSD and PH, in hospital settings without advanced equipment such as intra-operative transesophageal echocardiography or direct CO measurements. It also serves as a reminder not to let the anaesthetic risk in patients with severe cardiac disease be the reason for refraining from quality of life improving dental procedures.

#3

Transcatheter Occlusion of Hepatic Vein-Atrial Communication After Modified Fontan in a Child Post Kawashima Surgery.

JACC. Case reports2026 Mar 18

The hepatic vein-azygos vein connection is a rare Fontan modification used in patients with single ventricle physiology and interrupted inferior vena cava, following an initial Kawashima procedure. We report an unusual case of severe, late-onset hypoxemia due to recanalization of a hepatic vein-atrial communication after this operation. A girl with heterotaxy, mesocardia, unbalanced atrioventricular septal defect, and interrupted inferior vena cava underwent Kawashima surgery at 1 year of age, followed by modified Fontan completion at 4 years, in which hepatic veins were connected to the azygos vein. Nine years later, she presented with profound cyanosis (SpO2 35% to 40%) and polycythemia. Imaging demonstrated recanalization of hepatic vein-atrial communication resulting in significant right to left shunt and diffuse pulmonary arterio-venous malformations. Transcatheter closure of the communication using a 12-mm muscular ventricular septal defect occluder improved oxygen saturation to 65% with symptomatic relief. The hepatic vein-azygos vein connection represents an uncommon Fontan modification designed to ensure uniform distribution of hepatic venous flow to both the lungs. Late recanalization of a hepatic vein-atrial communication following this procedure has not been previously reported. In our case, transcatheter occlusion of the communication successfully redirected the hepatic venous flow to the pulmonary circuit. We describe a previously unreported complication of a rare Fontan modification. Transcatheter closure of a recanalized hepatic vein-atrial communication can effectively restore Fontan physiology and improve systemic oxygenation.

#4

Development of 3D-Printed Congenital Heart Disease Models Using Feasible Low-Cost Workflow - A Potential Tool to Improve Pediatric Cardiology Education.

Arquivos brasileiros de cardiologia2026 Jan

Congenital heart diseases (CHD) require an in-depth anatomopathological understanding. 3D printing is a promising educational tool for creating physical prototypes from imaging data, but costs are a major issue. This study aimed to describe a feasible low-cost workflow for the development of 3D-printed (3DP) CHD models and create a shareable educational set. Data from cardiac tomography images were used, following the steps of image acquisition, segmentation, digital design, slicing, 3D printing, and post-printing. Valvar structures were created from echocardiographic data using the cartographic heightmap technique and inserted into prototypes. 3DP models were evaluated by an expert team, enhanced, and applied to medical residents during an educational session. Free software, a desktop 3D printer, and low-cost materials were used. Twelve 3DP models were developed, including ventricular septal defect with patent ductus arteriosus, atrial septal defect, Tetralogy of Fallot, transposition of the great arteries, atrioventricular septal defect, coarctation of the Aorta, hypoplastic left heart syndrome, tricuspid atresia, pulmonary atresia, total anomalous pulmonary venous connection, Truncus arteriosus, and interrupted aortic arch. All residents (100%) agreed that prototypes were "faithful to the anatomy", "visually appealing", "motivated the study", and "allowed better spatial conceptualization". They related 3DP CHD models "are an important pedagogical resource" and can potentially benefit the education of "undergraduate students" (100%), "interdisciplinary team" (100%), "cardiologists, surgeons and residents" (100%), and "families" (93%). Development of 3DP CHD models using a low-cost workflow is feasible. Models developed are freely available for download and printing, intending to promote education to all interested in CHD. As cardiopatias congênitas (CC) demandam profundo conhecimento anatomopatológico. A impressão 3D (Imp3D) é uma ferramenta de ensino promissora para a criação de protótipos físicos a partir de dados de imagem, mas os custos representam um grande obstáculo. Descrever um fluxo de trabalho factível e de baixo custo para o desenvolvimento de modelos de CC em Imp3D e criar um conjunto de modelos de ensino compartilháveis. Foram utilizados conjuntos de imagens de tomografia cardíaca, seguindo as etapas de aquisição, segmentação, design digital, fatiamento, Imp3D e pós-impressão. Estruturas valvares foram criadas a partir de dados ecocardiográficos utilizando a técnica cartographic heightmap e inseridas nos protótipos. Os modelos de CC em Imp3D foram avaliados por uma equipe de especialistas, aprimorados e aplicados a residentes de cardiologia e cirurgia cardíaca durante uma sessão de ensino. Foram utilizados softwares gratuitos, impressora 3D desktop e materiais de baixo custo. Doze modelos de CC em Imp3D foram desenvolvidos, incluindo Comunicação Interventricular, Persistência do Canal Arterial, Comunicação Interatrial, Tetralogia de Fallot, Transposição das Grandes Artérias, Defeito do Septo Atrioventricular, Coarctação de Aorta, Síndrome do Coração Esquerdo Hipoplásico, Atresia Tricúspide, Atresia Pulmonar, Conexão Anômala Total de Veias Pulmonares, Truncus Arteriosus e Interrupção de Arco Aórtico. Todos os residentes (100%) concordaram que os protótipos eram “fiéis à anatomia”, “visualmente atraentes”, “motivaram o estudo” e “permitiram uma melhor conceitualização espacial”. Eles relataram que os modelos em Imp3D “são um importante recurso pedagógico” e podem potencialmente beneficiar a educação de “estudantes de graduação” (100%), “equipe interdisciplinar” (100%), “cardiologistas, cirurgiões e residentes” (100%) e “famílias” (93%). O desenvolvimento de modelos de CC em Imp3D utilizando metodologia de baixo custo é factível. Os arquivos digitais dos modelos desenvolvidos estão disponíveis gratuitamente para download e impressão, com o objetivo de promover a educação de todos os interessados no estudo das CC.

#5

Prognostic Value of Leuko-glycemic Index as a Marker of Immediate Postoperative Events After Repair of Complete Atrioventricular Septal Defect in Infants With Down Syndrome.

Journal of cardiothoracic and vascular anesthesia2026 Feb 05

The prognostic value of the leuko-glycemic index (LGI) as a marker of postoperative outcomes in pediatric cardiac patients is not known. This study aimed to determine the predictive value of LGI in predicting composite outcomes, including prolonged mechanical ventilation, after the repair of a complete atrioventricular septal defect in infants with Down syndrome. An ambidirectional cohort study. A single tertiary cardiac care center. Patients with Down syndrome and a complete atrioventricular septal defect. All patients underwent primary intracardiac repair of a complete atrioventricular septal defect and received standard perioperative anesthesia and hemodynamic management. A total of 110 patients were enrolled, of whom 104 completed the study and were included in the final analysis. Prolonged mechanical ventilation was defined as a duration ≥24 hours. We found a statistically significant correlation between postoperative LGI and prolonged mechanical ventilation (p = 0.042). A postoperative LGI cutoff value >1,640.16 was associated with the need for prolonged mechanical ventilation. The area under the receiver operating characteristic curve (AUC) was 0.659 (95% CI, 0.548-0.771) with a sensitivity of 47.50% and a specificity of 81.25% (p = 0.005). A cutoff LGI value >2,657.88 was found to differentiate between patients who developed renal failure and those who did not, with an AUC of 0.723 (p = 0.019). The LGI, a cost-effective and easily measured index, could be a valuable prognostic tool to stratify children with Down syndrome in the immediate postoperative period after complete atrioventricular septal defect correction. High LGI values may identify patients who could benefit from greater monitoring and early therapeutic strategies to reduce the duration of mechanical ventilation and the incidence of acute kidney injury.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC732 artigos no totalmostrando 198

2026

Transcatheter Occlusion of Hepatic Vein-Atrial Communication After Modified Fontan in a Child Post Kawashima Surgery.

JACC. Case reports
2026

Atrioventricular septal defect with cor triatriatum sinister.

Anales de pediatria
2026

Development of 3D-Printed Congenital Heart Disease Models Using Feasible Low-Cost Workflow - A Potential Tool to Improve Pediatric Cardiology Education.

Arquivos brasileiros de cardiologia
2026

Prognostic Value of Leuko-glycemic Index as a Marker of Immediate Postoperative Events After Repair of Complete Atrioventricular Septal Defect in Infants With Down Syndrome.

Journal of cardiothoracic and vascular anesthesia
2026

The Combined Double-Orifice and Single-Patch Technique for Partial Atrioventricular Septal Defect in Adults: A Novel Strategy.

Cardiovascular therapeutics
2026

Surviving Adulthood with Rare Combined Congenital Heart Defects: Complete AV Canal Defect, Ebstein's Anomaly, and Right Ventricular Hypoplasia.

Life (Basel, Switzerland)
2026

Heart failure in a patient with Glenn circulation during secondary pregnancy with a single ventricle: A rare case report.

International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics
2026

'Double-ring'-like dysplastic mitral leaflet associated with atrioventricular septal defect mimicking endocarditis: computed tomography as key to diagnosis.

European heart journal. Case reports
2026

Case Report: Anaesthetic management in a canine patient with severe atrioventricular septal defect and pulmonary hypertension undergoing non-cardiac surgery.

Frontiers in veterinary science
2026

Genetic Syndromes and Multimorbidity in Adults with Congenital Heart Disease and Heart Failure: Insights from the PATHFINDER-CHD Registry.

Journal of clinical medicine
2026

Arrhythmia Following Congenital Heart Disease Surgery in Oman: Incidence and risk factors - A prospective study.

Sultan Qaboos University medical journal
2026

Repair of Partial and Transitional Atrioventricular Septal Defects: Mid-term Outcomes in a Single-center Cohort.

Reviews in cardiovascular medicine
2026

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

Annals of African medicine
2026

Ivemark syndrome in an adult with complete atrioventricular septal defect and a single ventricle with L-transposition of the great arteries: a case report.

Journal of medical case reports
2025

Left ventricular outflow tract obstruction repair after atrioventricular septal defect correction.

Multimedia manual of cardiothoracic surgery : MMCTS
2025

Left atrioventricular valve durability in atrioventricular septal defects: Impact of systolic blood pressure.

JTCVS open
2025

Single incision minimally invasive congenital heart surgery: Insights into program development and outcome.

The Journal of thoracic and cardiovascular surgery
2025

Case Report: Concurrent atrial and ventricular septal defect in a young Sphynx cat.

Frontiers in veterinary science
2025

Fetal presentation of a child with partial atrioventricular septal defect and calcium release deficiency syndrome.

HeartRhythm case reports
2026

Complete atrioventricular septal defect, Australian repair: Technique and long-term results.

Asian cardiovascular & thoracic annals
2025

Left ventricular outflow tract obstruction caused by abnormal mitral valve tendinous Chordae manifesting in the extremely remote period after surgery for partial atrioventricular septal defect: a case report.

European heart journal. Case reports
2025

Pathogenic variant in GATA4 associated with atrioventricular septal defect and congenital diaphragmatic hernia: A case report.

European journal of medical genetics
2025

Frequency of Persistent Left Superior Vena Cava and Its Impact on Outcomes in Children Undergoing Congenital Heart Surgery.

Brazilian journal of cardiovascular surgery
2025

Utility of Three-Dimensional Holographic Workstation for Preoperative Simulation of Complex Congenital Heart Surgery.

Interdisciplinary cardiovascular and thoracic surgery
2025

Managing Pregnancy in a Patient With Unrepaired Congenital Heart Disease.

JACC. Case reports
2025

Myocardial reprogramming by HMGN1 underlies heart defects in trisomy 21.

Nature
2025

Predictive Factors of Prolonged Mechanical Ventilation After Complete Atrioventricular Septal Defect Surgery.

World journal for pediatric & congenital heart surgery
2025

Modified two-patch repair with left atrioventricular valve augmentation for complete atrioventricular septal defect: a novel modification to an older concept.

Multimedia manual of cardiothoracic surgery : MMCTS
2025

Surgical correction of anomalous pulmonary veinous return and atrioventricular septal defect in a patient with left lung agenesis.

JTCVS techniques
2025

Partial Atrioventricular Septal Defect Repair through Right Thoracotomy in A 73-Year-Old Woman.

Surgical case reports
2025

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

Cureus
2025

Unilateral absence of the pulmonary artery in a paediatric cohort.

Cardiology in the young
2025

Aorto-septal Angle as a Predictor of Left Ventricular Outflow Tract Obstruction in Pediatric Patients With Subaortic Stenosis and Subaortic Membrane Development: A Study at Prince Sultan Cardiac Center, Buraydah, Saudi Arabia 2023.

Journal of the Saudi Heart Association
2026

Commentary: Second cardiopulmonary bypass run after complete atrioventricular septal defect repair: To perform or not to perform?

The Journal of thoracic and cardiovascular surgery
2025

Clues on the Three-Vessel View for Fetal Diagnosis of Supracardiac Total Anomalous Pulmonary Venous Connection.

CASE (Philadelphia, Pa.)
2025

Long-term outcomes following atrioventricular septal defect in adult patients.

Annals of medicine
2026

Intraoperative predictors of left atrioventricular valve reoperation after repair of complete atrioventricular septal defect.

The Journal of thoracic and cardiovascular surgery
2025

Concurrent incomplete atrioventricular septal defect and supravalvular mitral stenosis causing heart failure in a 13-year-old cat: a case report.

The Journal of veterinary medical science
2025

Clinical outcomes of tetralogy canal repair: A multidisciplinary perspective.

Saudi medical journal
2025

Rare Presentation of Partial Atrioventricular Septal Defect in the Elderly: A Case Report.

Cureus
2026

Unbalanced Atrioventricular Septal Defect: Could This Be the Best Substrate for Recruitment of a Borderline Left Ventricle?

The Annals of thoracic surgery
2025

Individualized approach to early surgery for complete repair of atrioventricular septal defect.

The Journal of thoracic and cardiovascular surgery
2026

Stenotic left atrioventricular valve after atrioventricular septal defect repair: A distinct surgical phenotype.

The Journal of thoracic and cardiovascular surgery
2025

Higher Incidence of Moderate and Severe Congenital Heart Disease in Patients With Neonatal Abstinence Syndrome.

Neonatal network : NN
2025

Staged right ventricular recruitment of unbalanced atrioventricular septal defect.

JTCVS techniques
2025

Partial AVSD with cortriatriatum sinister with unroofed coronary sinus in an adult.

Annals of pediatric cardiology
2025

Truncus bicaroticus with aberrant origin of right subclavian artery in a patient with atrioventricular septal defect: A rare aortic branch anomaly.

Annals of pediatric cardiology
2025

Re-operative minimally invasive endoscopic mitral valve repair after partial atrioventricular canal repair.

Multimedia manual of cardiothoracic surgery : MMCTS
2025

A Rare Case of Dextrocardia With Atrioventricular Septal Defect and Situs Inversus in an 8-Month-Old Infant Complicated by Severe Acute Malnutrition.

Clinical case reports
2026

Renal Hemosiderosis Associated with Mitral Regurgitation Detected by Severe Proteinuria Due to Congestion.

Internal medicine (Tokyo, Japan)
2025

Heterotaxy syndrome with biliary atresia: a case report.

The Pan African medical journal
2025

Experience of the Pediatric Department at the Mohammed VI University Hospital Center in Oujda on Trisomy 21 and Congenital Heart Defects: What Is the Reality in the Oriental Region of Morocco?

Cureus
2025

Iatrogenic coronary sinus diversion to left atrium mimicking paravalvular leak: the importance of documentation.

Indian journal of thoracic and cardiovascular surgery
2026

Atrioventricular Inflow and Subsequent Ventricular Growth After Staged Ventricular Recruitment for Unbalanced Atrioventricular Canal Defect.

The Annals of thoracic surgery
2025

Surgical management of hiatal hernia in a 3-year-old child with asplenia syndrome, congenital heart disease, thoracic scoliosis, and intestinal malrotation: A case report.

International journal of surgery case reports
2025

Left-sided valve dysfunction with extreme giant left atrium and anomalous coronary sinus connection after atrioventricular septal defect repair.

JTCVS techniques
2025

Utility of Intraoperative 3D TEE to Unmask a Hidden Intracardiac Fistula.

JACC. Case reports
2025

Thirty years of left atrioventricular valve reoperations following atrioventricular septal defect repair: Impact of valvar stenosis.

The Journal of thoracic and cardiovascular surgery
2025

Outcomes of 3-Dimensional Total Endoscopic Surgery for Partial Atrioventricular Septal Defect in Children: A Single-Center Experience in Vietnam.

Innovations (Philadelphia, Pa.)
2025

Single center experience with surgically implanted Melody and Sapien 3 valves in the mitral position in young children.

Frontiers in cardiovascular medicine
2025

Absence of the Left Circumflex Artery in Congenital Heart Disease With Vasospastic Myocardial Infarction.

JACC. Case reports
2025

Duplication of the umbilical vein along with an extrahepatic, intrathoracic right umbilical vein in the setting of Taussig-Bing anomaly with atrioventricular septal defect.

Indian journal of thoracic and cardiovascular surgery
2026

Status and Operative Outcomes of the Fontan Procedure Performed Beyond the First Decade of Life in the United States.

World journal for pediatric & congenital heart surgery
2025

Mitral and tricuspid valve repair in an operated case of complete atrioventricular septal defect: A surgical challenge case report!

SAGE open medical case reports
2025

Unanticipated Abdominal Compartment Syndrome in an Infant Undergoing Atrioventricular Septal Defect Repair.

Journal of cardiothoracic and vascular anesthesia
2025

Systematic analysis of the atrioventricular canal in 129 heart specimens identified anatomic and microarchitectural advantages in Down syndrome.

The Journal of thoracic and cardiovascular surgery
2025

Surgical repair of partial atrioventricular septal defect (pAVSD) in adulthood: A rare case report.

International journal of surgery case reports
2024

[MSB-26] Minimally Invasive Right Vertical Axillary Thoracotomy for Repair of Congenital Heart Defects: Azerbaijan Experience.

Turk gogus kalp damar cerrahisi dergisi
2025

Rhythm Disturbances in Children With Down Syndrome who Underwent Surgery for Congenital Heart Defects.

World journal for pediatric & congenital heart surgery
2025

Cor triatriatum sinister with partial atrioventricular septal defect in a cat.

Veterinarni medicina
2025

Coronary Sinus Blues: A Word of Caution When Placing the Coronary Sinus Ostium in the Left Atrium.

World journal for pediatric & congenital heart surgery
2025

A case of complete atrioventricular septal defect in which extracorporeal membrane oxygenation could be removed after performing the bilateral Glenn procedure for severe cardiac dysfunction after tricuspid valve replacement: a case report.

BMC pediatrics
2025

Pulmonary Artery Banding in a Cat with Atrioventricular Canal Defect Type A with Concurrent Muscular Septal Defect.

Animals : an open access journal from MDPI
2026

The Fate of the Left Atrioventricular Valve After Atrioventricular Septal Defect Repair: Long-Term Outcomes.

Pediatric cardiology
2025

Complete Atrioventricular Septal Defect Repair in Patients With Down Syndrome Presenting Beyond Six Months- A Single Center Experience.

World journal for pediatric & congenital heart surgery
2025

Case report: morphological challenges to surgical repair in complete atrioventricular septal defect with isolated ventricular component.

Cardiology in the young
2025

Diastolic Mitral Regurgitation With Interatrial Left-to-Right Shunt.

JACC. Case reports
2025

A 27-Year Experience with Atrioventricular Septal Defect Correction.

The Thoracic and cardiovascular surgeon
2025

Intermediate-Type Atrioventricular Septal Defect Repair With Atrioventricular Valve Annuloplasty via Minimally Invasive Approach.

Innovations (Philadelphia, Pa.)
2025

Can Fetal Heterotaxy Syndrome Be Diagnosed Through Prenatal Ultrasound in the First Trimester (GA 11+0-13+6 Weeks)?

Prenatal diagnosis
2025

A cohort evaluation of surgical pathways reported to a national audit of children undergoing treatment for congenital heart disease in England and Wales.

The Journal of thoracic and cardiovascular surgery
2025

Primary repair versus pulmonary artery banding in complete atrioventricular canal defects in the modern surgical era.

The Journal of thoracic and cardiovascular surgery
2025

Neonatal enlargement of the aortic arch roof without cardiopulmonary bypass using ductal patency for lower body perfusion: impact on long-term growth, function and shape of the aortic arch†.

Interdisciplinary cardiovascular and thoracic surgery
2025

The Usefulness of Intraoperative Indocyanine Green Fluorescence Imaging in Surgical Treatment of Refractory Chylothorax in Pediatric Patients: A Case Report.

Surgical case reports
2025

The Association Between Residual Lesion Score and Long-term Outcomes of Congenital Cardiac Operations.

The Annals of thoracic surgery
2025

Peripandemic outcomes of infants treated for sentinel congenital heart diseases in England and Wales.

Open heart
2025

The particular anatomy of atrioventricular septal defect with a common valvar orifice in patients with Down syndrome: an echocardiographic study.

International journal of cardiology
2025

Outcomes of Atrioventricular Septal Defect Repair: Two-Institutions, 10-Year Experience in Cordoba, Argentina.

World journal for pediatric & congenital heart surgery
2025

Berlin Heart EXCOR sVAD implantation technique for neonates and infants with functionally univentricular ductal-dependent systemic circulation.

Multimedia manual of cardiothoracic surgery : MMCTS
2025

A case report of a rare cardiac anomaly associated with Ellis-van Creveld syndrome: common atrium, partial atrioventricular septal defect, and hypoplastic left ventricle.

European heart journal. Case reports
2025

Successful biventricular repair following strategic treatment focused on left ventricular growth in a patient with hypoplastic left heart complex and unbalanced right dominant atrioventricular septal defect: a case report.

Cardiology in the young
2025

[Atrioventricular Septal Defect (AVSD) in a 44-Year-Old Male Incidentally Detected by CTA prior to Mitral Valve Surgery].

RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin
2024

Rescuing the Right Ventricle: Mechanical Support After Pediatric Heart Transplantation.

Annals of thoracic surgery short reports
2026

Fetal Characteristics and Perinatal Outcomes in Tetralogy of Fallot Without a Ductus Arteriosus.

Pediatric cardiology
2024

Can we diagnose aortic arch obstruction in a fetus with an atrioventricular septal defect?

Cardiology in the young
2024

[Replacement of Left Atrioventricular Valve and Patch Augmentation of Right Atrioventricular Valve After Repair of Complete Atrioventricular Septal Defect:Report of a Case].

Kyobu geka. The Japanese journal of thoracic surgery
2024

Transcatheter Edge-to-Edge Repair of Common Atrioventricular Valve Regurgitation in the Setting of a Lateral Tunnel Fontan Circulation.

Structural heart : the journal of the Heart Team
2024

[Patch Closure of the Ventricular Septal Defect].

Kyobu geka. The Japanese journal of thoracic surgery
2024

Epidemiology of Congenital Heart Defects in Perinatal Infants in Guangxi, China.

International journal of general medicine
2025

Left-Sided Atrioventricular Valve Regurgitation After the Repair of Complete Atrioventricular Septal Defect.

Pediatric cardiology
2025

Early Outcomes for Management of Atrioventricular Septal Defect-Tetralogy of Fallot in the Last Decade: A Congenital Heart Surgeons' Society Study.

World journal for pediatric & congenital heart surgery
2025

Sinus node dysfunction in children: different aetiologies, similar clinical course in two-centre experience.

Cardiology in the young
2024

Berlin Heart EXCOR sVAD upsizing and exchange technique.

Multimedia manual of cardiothoracic surgery : MMCTS
2024

Identification of two novel genetic variants for Ellis-van Creveld syndrome from a Chinese family through whole exome sequencing.

European journal of obstetrics, gynecology, and reproductive biology
2024

Hemi-Commando with aortic homograft and atrioventricular septal defect repair for recurrent triple valve endocarditis.

JTCVS techniques
2024

Diagnosis and surgical treatment for complete atrioventricular septal defect with intact atrial septum.

Cardiology in the young
2024

Evaluating Long-Term Outcomes of Children Undergoing Surgical Treatment for Congenital Heart Disease for National Audit in England and Wales.

Journal of the American Heart Association
2024

A case report: pitfalls in antibacterial therapy with rifampicin for mechanical valve endocarditis-the king of drug interactions.

European heart journal. Case reports
2024

Staged ventricular recruitment following single-ventricular palliation in unbalanced atrioventricular septal defect with heterotaxy syndrome.

Cardiology in the young
2024

Evaluation of mid-term outcomes of partial and intermediate atrioventricular canal defect surgery.

Cardiology in the young
2025

Outcomes of left atrioventricular valve operation following atrioventricular septal defect repair.

The Journal of thoracic and cardiovascular surgery
2024

Left atrioventricular valve regurgitation repair with concomitant outflow obstruction release on repaired atrioventricular septal defect case.

Journal of cardiothoracic surgery
2024

Missense mutations in the CITED2 gene may contribute to congenital heart disease.

BMC cardiovascular disorders
2024

Fetal cardiovascular magnetic resonance feature tracking myocardial strain analysis in congenital heart disease.

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance
2025

Partial atrioventricular septal defect: An unexpected anomaly in a 68-year-old patient.

Kardiologia polska
2025

Compound heterozygosity for two variants in BMP5 in human skeletal dysostosis with atrioventricular septal defect.

Clinical genetics
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 & congenital heart surgery
2024

Arrhythmias in congenital heart disease: A nationwide cohort study.

American heart journal
2025

Impact of Age and of the Patent Ductus Arteriosus on Pulmonary Hemodynamics in Children with Complete Atrioventricular Septal Defect.

Pediatric cardiology
2024

Minimally Invasive Surgical Repair of Simple Congenital Heart Defects Using the Right Vertical Infra-Axillary Thoracotomy Approach.

Innovations (Philadelphia, Pa.)
2024

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

Korean circulation journal
2024

Extracorporeal Membrane Oxygenation after Pediatric Cardiac Surgery: A Single-Center Experience.

Turkish archives of pediatrics
2025

Racial disparities in maternal exposure to ambient air pollution during pregnancy and prevalence of congenital heart defects.

American journal of epidemiology
2024

Down syndrome and associated atrioventricular septal defects in a nationwide Norwegian cohort: Prevalence, time trends, and outcomes.

Acta obstetricia et gynecologica Scandinavica
2024

Pulmonary Artery Banding and Arch Repair vs Norwood for Unbalanced Atrioventricular Canal Defect.

The Annals of thoracic surgery
2024

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

Global heart
2025

Left Atrioventricular Valve Regurgitation After Atrioventricular Septal Defect Repair.

The Annals of thoracic surgery
2024

Partial atrioventricular canal defect and aortic coarctation associated with variants in GDF1 and NOTCH1 genes: A case report.

Birth defects research
2024

Congenital heart disease: types, pathophysiology, diagnosis, and treatment options.

MedComm
2024

Dihydropyrimidinase deficiency with atrioventricular septal defect: a case report.

Journal of pediatric endocrinology & metabolism : JPEM
2024

Intra-aortic balloon pump treatment in an adult patient with a Fontan circulation and acute heart failure: a case report.

European heart journal. Case reports
2024

Molecular Pathways and Animal Models of Atrioventricular Septal Defect.

Advances in experimental medicine and biology
2024

Human Genetics of Atrioventricular Septal Defect.

Advances in experimental medicine and biology
2024

Clinical Presentation and Therapy of Atrioventricular Septal Defect.

Advances in experimental medicine and biology
2024

Molecular Pathways and Animal Models of Atrial Septal Defect.

Advances in experimental medicine and biology
2024

Cardiac Development and Animal Models of Congenital Heart Defects.

Advances in experimental medicine and biology
2024

Risk factors for the recurrence of left atrioventricular valvular regurgitation after surgical repair of partial and transitional atrioventricular septal defect.

Journal of thoracic disease
2024

Predicting surgical outcome by echocardiography in complete atrioventricular canal defect repair.

Echocardiography (Mount Kisco, N.Y.)
2024

Staged repair of borderline hypoplastic heart disease with early biventricular conversion.

JTCVS techniques
2024

Congenital heart "Challenges" in Down syndrome.

World journal of cardiology
2024

Outcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus.

The Journal of pediatrics
2024

Comparison of post-operative transesophageal and transthoracic echocardiogram findings following atrioventricular septal defect repair.

Cardiology in the young
2024

Predictors of post-operative left atrioventricular valve regurgitation in pediatric patients with complete atrioventricular canal defects.

Echocardiography (Mount Kisco, N.Y.)
2024

A novel MED12 pathogenic variant in a female fetus with facial cleft and cardiac defects identified in the first trimester.

Prenatal diagnosis
2024

Impact of trisomy 21 on late surgical management results in patients with common atrioventricular septal defect: single-center experience.

Polish archives of internal medicine
2024

Cardiac Complications in Trisomy 21 Patients in a Secondary Hospital: A Descriptive Study.

Cureus
2024

Tumour lysis syndrome in a neonate with transient abnormal myelopoiesis.

Journal of neonatal-perinatal medicine
2024

PRKD1-related telangiectasia-ectodermal dysplasia-brachydactyly-cardiac anomaly syndrome: Case report and review of the literature.

European journal of medical genetics
2024

Fetal left and right ventricular strain parameters using speckle tracking in congenital heart diseases.

The international journal of cardiovascular imaging
2024

Management of Left Ventricular Intramural Hematoma- an Unusual Complication of Complete Atrioventricular Septal Defect Repair in a Child.

ASAIO journal (American Society for Artificial Internal Organs : 1992)
2024

Revisiting Atrioventricular Septal Defects: Exploring Chromosomal Abnormalities, Cardiac and Extracardiac Anomalies in a Contemporary Prenatal Cohort.

Pediatric cardiology
2024

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

Interdisciplinary cardiovascular and thoracic surgery
2024

Successful surgical correction of an incomplete atrioventricular septal defect in a 76-year-old female patient.

Journal of surgical case reports
2024

Cleft closure and other predictors of contemporary outcomes after atrioventricular canal repair in patients with parachute left atrioventricular valve.

Interdisciplinary cardiovascular and thoracic surgery
2024

Risk of infective endocarditis after hybrid melody mitral valve replacement in infants: the French experience.

Interdisciplinary cardiovascular and thoracic surgery
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
2025

Preoperative Feeding Fortification Among Infants with Congenital Heart Disease is Associated with Higher Growth Velocity in the First 30 Days Post-repair and Lower BMI Percentile for Age at 10 Years: A Retrospective Cohort Study.

Pediatric cardiology
2024

Left ventricular outflow tract obstruction after repair of atrioventricular septal defect.

JTCVS open
2024

Role of ECG in the Accidental Finding of an Atrioventricular Septal Defect in an Asymptomatic Patient Undergoing Cosmetic Surgery.

Cureus
2024

Canonical Wnt signaling directs the generation of functional human PSC-derived atrioventricular canal cardiomyocytes in bioprinted cardiac tissues.

Cell stem cell
2024

Efficacy of atrioventricular valve regurgitation in the first trimester for the diagnosis of atrioventricular septal defect.

Journal of clinical ultrasound : JCU
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

Tricuspid mechanical valve replacement for severe tricuspid stenosis in a child who underwent surgical correction of complete atrioventricular septal defect, a rare long-term complications.

Journal of cardiothoracic surgery
2024

Application of neural networks in prenatal diagnosis of atrioventricular septal defect.

Translational pediatrics
2024

Use of Interactive Visualization and 3D Printing in the Repair of Complex Congenital Heart Disease Presenting in Adult Life.

World journal for pediatric & congenital heart surgery
2024

Association of tetralogy of Fallot and complete atrioventricular canal: a single-centre 40-year experience.

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

National population-based estimates for major birth defects, 2016-2020.

Birth defects research
2024

Simultaneous Bentall Procedure and Partial Atrioventricular Septal Defect Repair in a 64-Year-Old Patient with Idiopathic Thrombocytopenic Purpura: A Case Report and Literature Review.

Anatolian journal of cardiology
2024

Interaction between clinicians and artificial intelligence to detect fetal atrioventricular septal defects on ultrasound: how can we optimize collaborative performance?

Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2024

Percutaneous Closure of Hemodynamically Significant Postoperative Residual Ventricular Septal Defects.

Pediatric cardiology
2024

[Genetic analysis of the false positive trisomy 7 and false negative trisomy 18 by NIPT-PLUS].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2024

Outcomes and characteristics in term infants with necrotising enterocolitis and CHD.

Cardiology in the young
2023

Partial heart transplantation of atrioventricular valves in complete atrioventricular septal defect-simulation of techniques using silicone-molded heart models.

JTCVS techniques
2024

Introduction of transcatheter edge-to-edge repair in patients with congenital heart disease at a children's hospital.

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

Fetal single ventricle journey to first postnatal procedure: a multicentre UK cohort study.

Archives of disease in childhood. Fetal and neonatal edition
2023

Use of glutaraldehyde-treated autologous pericardial patch in complete atrioventricular septal defect repair.

European review for medical and pharmacological sciences
2024

Double-orifice mitral valve and partial atrioventricular septal defect: A rare combination.

Asian journal of surgery
2025

Long-Term Results Following Combined Repair of Atrioventricular Septal Defect with Tetralogy of Fallot.

Pediatric cardiology
2023

Minimally Invasive Surgery for Simple Congenital Heart Defects: Preserving Aesthetics without Jeopardizing Patient Safety.

Journal of cardiovascular development and disease
2023

Successful Treatment of Pulmonary Artery Aneurysm With Atrioventricular Septal Defect and Pulmonary Hypertension: Clinical and Pathologic Features.

CJC pediatric and congenital heart disease
2024

Primary or Delayed Repair for Complete Atrioventricular Septal Defect, Tetralogy of Fallot, and Ventricular Septal Defect: Relationship to Country Economic Status.

World journal for pediatric & congenital heart surgery
2023

Early and Mid-Term Outcomes of Primary Repair After Atrioventricular Canal Defect: A Single-Center Eight-Year Experience.

Cureus
2023

A retrospective audit of young adults who received permanent pacemakers at a teaching hospital in the Western Cape, South Africa.

Frontiers in cardiovascular medicine
2023

Double outlet right ventricle.

Frontiers in pediatrics
2024

Anatomic considerations in the management of complete atrioventricular canal.

Cardiology in the young
2023

Plasma proteoglycan 4: a novel biomarker for acute lung injury after pediatric cardiac surgery.

Translational pediatrics
2023

Redo mitral valve replacement in an adult with severe pulmonary hypertension resulting from structural valve deterioration and left ventricular outflow tract obstruction and a history of atrioventricular septal defect repair: a case report.

Journal of cardiothoracic surgery
2023

Role of autopsy in diagnosing asplenia and right bilobed lung in a fetus with dextrocardia.

BMJ case reports
2023

Lecompte maneuver for compressed left coronary artery after pulmonary artery banding.

Asian cardiovascular & thoracic annals
2023

Transcatheter aortic valve-in-valve implantation in right ventricle-aorta conduit in an adult patient with Fontan circulation.

International journal of cardiology. Congenital heart disease
2024

Atrioventricular Septal Defect With Tetralogy of Fallot and Exclusive Shunting at the Ventricular Level.

World journal for pediatric & congenital heart surgery
2023

Catheter ablation of atrial tachyarrhythmias in patients with atrioventricular septal defect.

Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology
2023

Clinical profile, prenatal detection and predictors of outcome of heterotaxy syndromes in Western Australia.

International journal of cardiology. Congenital heart disease
2024

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

Cardiology in the young
2024

Detection of severe congenital heart defects in live-born infants in Norway 2017-2020.

Acta paediatrica (Oslo, Norway : 1992)
2023

Combined tricuspid atresia- AV septal defect-a rare congenital cardiac abnormality.

Echocardiography (Mount Kisco, N.Y.)
2024

A Case of Successful Biventricular Repair of the Transposition of the Great Arteries with a Coronary Anomaly Associated with an Atrioventricular Septal Defect.

Pediatric cardiology
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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. 'Double-ring'-like dysplastic mitral leaflet associated with atrioventricular septal defect mimicking endocarditis: computed tomography as key to diagnosis.
    European heart journal. Case reports· 2026· PMID 41704836mais citado
  2. Case Report: Anaesthetic management in a canine patient with severe atrioventricular septal defect and pulmonary hypertension undergoing non-cardiac surgery.
    Frontiers in veterinary science· 2026· PMID 41696011mais citado
  3. Transcatheter Occlusion of Hepatic Vein-Atrial Communication After Modified Fontan in a Child Post Kawashima Surgery.
    JACC. Case reports· 2026· PMID 41854327mais citado
  4. Development of 3D-Printed Congenital Heart Disease Models Using Feasible Low-Cost Workflow - A Potential Tool to Improve Pediatric Cardiology Education.
    Arquivos brasileiros de cardiologia· 2026· PMID 41779486mais citado
  5. Prognostic Value of Leuko-glycemic Index as a Marker of Immediate Postoperative Events After Repair of Complete Atrioventricular Septal Defect in Infants With Down Syndrome.
    Journal of cardiothoracic and vascular anesthesia· 2026· PMID 41764019mais citado
  6. Echocardiographic Assessment of Pulmonary Arterial Hypertension After Complete Atrioventricular Septal Defect Repair.
    Echocardiography· 2026· PMID 41979870recente
  7. Cleft-to-commissure conversion in atrioventricular septal defect using artificial chordae: Technical report and case illustration.
    JTCVS Tech· 2026· PMID 41971883recente
  8. Left Atrial Isomerism in a Four-Year-Old Girl Diagnosed due to Gastrointestinal Bleeding: A Case Report.
    Cureus· 2026· PMID 41952947recente
  9. Eccentricity Index Is Associated With Pulmonary Arterial Hypertension in Children After Repair of Complete Atrioventricular Septal Defects.
    Echocardiography· 2026· PMID 41949040recente
  10. Pulmonary Hypertension in Down Syndrome Versus Non-syndromic Pediatric Populations With Congenital Heart Disease: A Comparative Study.
    Cureus· 2026· PMID 41939622recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:98722(Orphanet)
  2. MONDO:0020290(MONDO)
  3. GARD:802(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Artigo Wikipedia(Wikipedia)
  7. Q390211(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

Defeito do canal auriculoventricular
Compêndio · Raras BR

Defeito do canal auriculoventricular

ORPHA:98722 · MONDO:0020290
CID-10
Q21.2 · Comunicação atrioventricular
CID-11
Ensaios
1 ativos
Medicamentos
3 registrados
MedGen
UMLS
C0014116
EuropePMC
Wikidata
Wikipedia
Papers 10a
DiscussaoAtiva

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