O canal atrioventricular completo (CAVC), também conhecido como defeito do septo atrioventricular completo, é caracterizado por um defeito do septo atrial ostium primum, uma válvula atrioventricular comum e uma deficiência variável do influxo do septo ventricular.
Introdução
O que você precisa saber de cara
O canal atrioventricular completo (CAVC), também conhecido como defeito do septo atrioventricular completo, é caracterizado por um defeito do septo atrial ostium primum, uma válvula atrioventricular comum e uma deficiência variável do influxo do septo ventricular.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 15 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 35 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
4 genes identificados com associação a esta condição. Padrão de herança: Not applicable.
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)
Nucleus
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.
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
Nucleus
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.
Protein disulfide isomerase (By similarity). Promotes the localization of acetylcholine receptors (AChRs) to the plasma membrane (By similarity)
Membrane
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.
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
Nucleus
Variantes genéticas (ClinVar)
593 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
13 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Defeito septal atrioventricular completo
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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
Ensaios em destaque
Pesquisa e ensaios clínicos
7 ensaios clínicos encontrados, 1 ativos.
Publicações mais relevantes
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.
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.
Ivemark syndrome in an adult with complete atrioventricular septal defect and a single ventricle with L-transposition of the great arteries: a case report.
Ivemark syndrome is a rare heterotaxy disorder characterized by right atrial isomerism, complex congenital heart defects, and thoraco-abdominal anomalies. Because of such defects, survival beyond infancy is rare. We report a case of a 31-year-old North African male with lifelong cyanosis who presented with worsening exertional dyspnea and fatigue. A segmental approach revealed heterotaxy with right atrial isomerism, a complete atrioventricular septal defect, a functional single ventricle with L-transposition of the great arteries, and severe valvular and supravalvular pulmonic stenosis. Despite these complex anomalies, the patient maintained a balanced circulation that enabled survival into adulthood. A multidisciplinary team recommended a palliative care approach, including intravenous hydration, iron supplementation, allopurinol, and preventive measures for infective endocarditis. The patient remains under regular noninvasive follow-up, with a stable condition. This case highlights the rare adult presentation of Ivemark syndrome. Long-term survival is exceptionally rare and depends on balanced hemodynamics, comprehensive evaluation, and individualized multidisciplinary care.
Complete atrioventricular septal defect, Australian repair: Technique and long-term results.
BackgroundSurgical repair of complete atrioventricular septal defect (CAVSD) has evolved substantially, with early mortality markedly reduced over the past decades. However, achieving durable left atrioventricular valve (LAVV) competence and preserving left ventricular outflow tract (LVOT) geometry remain critical determinants of long-term success.MethodsThis review summarizes the anatomical principles, operative details, and clinical outcomes of the Nunn-modified single-patch ("Australian") technique for CAVSD repair.ResultsThe Australian technique-characterized by direct approximation of bridging leaflets to the septal crest without a ventricular patch-demonstrates superior preservation of LVOT architecture and promotes symmetrical LAVV coaptation. Contemporary series report >90% freedom from LAVV reoperation and >97% freedom from LVOT obstruction at 10-15 years, outperforming traditional repairs in multiple institutional and multicenter cohorts. These advantages are consistent across a wide spectrum of ventricular septal defect sizes and patient ages, provided anatomical prerequisites are met.ConclusionsBy minimizing prosthetic material and restoring native leaflet geometry, the Australian Repair achieves excellent long-term valve competence and reoperation-free survival. It should be considered a first-line option in appropriately selected CAVSD patients, particularly where durable LAVV function and LVOT integrity are paramount.
Intraoperative predictors of left atrioventricular valve reoperation after repair of complete atrioventricular septal defect.
Although outcomes of repair of complete atrioventricular septal defect have improved in recent years, the burden of reoperation on the left atrioventricular valve (LAVV) remains high. We investigated predictors of LAVV reoperation on the intraoperative echocardiogram that may help guide surgical decision making. All patients who underwent repair of complete atrioventricular septal defect at the Royal Children's Hospital from 2010 to 2020 were included in the study. Intraoperative postbypass echocardiograms were individually reviewed. Long-term data were obtained from review of medical records. Of 134 patients underwent repair of atrioventricular septal defect, early mortality was 1.5% (2 out of 134). After first bypass run, 20.1% (27 out of 134) had moderate or greater residual LAVV regurgitation (LAVVR). A second bypass run to re-repair the LAVV was performed in 8.2% (11 out of 134), after which the mean grade of LAVVR significantly improved from 2.2 to 1.3 (P = .004). Freedom from LAVV reoperation at 10 years was 89.6% in those with no LAVVR, 86.1% in those with mild LAVVR, compared with 61.2% in those with moderate LAVVR (P = .002). Freedom from reoperation at 10 years was 92.7% in patients with no significant LAVVR, 84.2% in those with central LAVV compared with 65.4% in those with eccentric LAVVR (P = .001). Moderate or greater LAVVR and an eccentric jet of LAVVR on the intraoperative postbypass echocardiogram predict need for reoperation. A second bypass run to re-repair was associated with an improvement in LAVVR. These results should help guide surgeons when deciding on the need for a second bypass run to re-repair the LAVV.
The Fate of the Left Atrioventricular Valve After Atrioventricular Septal Defect Repair: Long-Term Outcomes.
While repair of complete atrioventricular septal defects (CAVSD) has shown excellent results, the need for left atrioventricular valve (LAVV) intervention after the initial CAVSD repair presents therapeutic challenges and potential morbidity. Data on incidence and outcomes of LAVV intervention after primary CAVSD repair remain limited. Retrospective review of the Pediatric Health Information System (PHIS) from 1/2004 to 12/2023. All patients who underwent CAVSD repair were included. LAVV reintervention was defined as mitral valve repair or replacement after initial CAVSD. International Classification of Diseases 9/10 codes were used to identify diagnoses and procedures. 7745 patients underwent CAVSD repair with 4,430 (57.4%) females, 4430 (57.2%) non-Hispanic White, 4250 (54.9%) with Down syndrome, and a median age of 5.4 [IQR: 3.7-10.2] months at repair. Following CAVSD repair, 503 (6.5%) required LAVV reintervention, with 405 (80.5%) repairs and 98 (19.5%) replacements. Median total follow up was 1.99[IQR: 0.09-9.13] years. Freedom from reintervention was 93.8% at 1 year, 88.3% at 5 years, 83.8% at 10 years, and 79.1% at 15 years. Factors associated with LAVV repair included government insurance (HR 0.72; 95% CI 0.57-0.92, p = 0.008), age (per month at CAVSD repair) (HR 0.98; 95% CI 0.97-0.99, p < 0.001), Down syndrome (HR 0.56; 95% CI 0.45-0.88, p = 0.006), and middle-tertile volume center compared to highest tertile (HR 1.24; 95% CI 1.00-1.55; p = 0.044). For LAVV replacement, Down syndrome (HR 0.47; 95% CI 0.30-0.72, p < 0.001) was the only significant factor. In a large multicenter dataset study, LAVV reintervention after CAVSD repair is not uncommon, with an incidence of 7%, emphasizing its long-term management challenges. Our study demonstrated that older age at CAVSD repair and a diagnosis of Down Syndrome were associated with a decreased risk for LAVV reintervention. These findings can help counsel patients/families and help guide surveillance protocols and long-term management in this population.
Publicações recentes
Echocardiographic Assessment of Pulmonary Arterial Hypertension After Complete Atrioventricular Septal Defect Repair.
Cleft-to-commissure conversion in atrioventricular septal defect using artificial chordae: Technical report and case illustration.
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.
Sustained remission of post-Fontan protein-losing enteropathy achieved by combination therapy with high-dose spironolactone and the somatostatin analogue octreotide: a case report.
Heart failure in a patient with Glenn circulation during secondary pregnancy with a single ventricle: A rare case report.
📚 EuropePMC200 artigos no totalmostrando 195
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 anesthesiaHeart 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 ObstetricsIvemark 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 reportsComplete atrioventricular septal defect, Australian repair: Technique and long-term results.
Asian cardiovascular & thoracic annalsPredictive Factors of Prolonged Mechanical Ventilation After Complete Atrioventricular Septal Defect Surgery.
World journal for pediatric & congenital heart surgeryModified 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 : MMCTSUnilateral absence of the pulmonary artery in a paediatric cohort.
Cardiology in the youngCommentary: Second cardiopulmonary bypass run after complete atrioventricular septal defect repair: To perform or not to perform?
The Journal of thoracic and cardiovascular surgeryIntraoperative predictors of left atrioventricular valve reoperation after repair of complete atrioventricular septal defect.
The Journal of thoracic and cardiovascular surgeryHeterotaxy syndrome with biliary atresia: a case report.
The Pan African medical journalExperience 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?
CureusMitral and tricuspid valve repair in an operated case of complete atrioventricular septal defect: A surgical challenge case report!
SAGE open medical case reportsRhythm Disturbances in Children With Down Syndrome who Underwent Surgery for Congenital Heart Defects.
World journal for pediatric & congenital heart surgeryA 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 pediatricsThe Fate of the Left Atrioventricular Valve After Atrioventricular Septal Defect Repair: Long-Term Outcomes.
Pediatric cardiologyComplete Atrioventricular Septal Defect Repair in Patients With Down Syndrome Presenting Beyond Six Months- A Single Center Experience.
World journal for pediatric & congenital heart surgeryCase report: morphological challenges to surgical repair in complete atrioventricular septal defect with isolated ventricular component.
Cardiology in the youngA 27-Year Experience with Atrioventricular Septal Defect Correction.
The Thoracic and cardiovascular surgeonCan Fetal Heterotaxy Syndrome Be Diagnosed Through Prenatal Ultrasound in the First Trimester (GA 11+0-13+6 Weeks)?
Prenatal diagnosisPrimary repair versus pulmonary artery banding in complete atrioventricular canal defects in the modern surgical era.
The Journal of thoracic and cardiovascular surgeryNeonatal 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 surgeryThe Usefulness of Intraoperative Indocyanine Green Fluorescence Imaging in Surgical Treatment of Refractory Chylothorax in Pediatric Patients: A Case Report.
Surgical case reportsThe Association Between Residual Lesion Score and Long-term Outcomes of Congenital Cardiac Operations.
The Annals of thoracic surgeryOutcomes of Atrioventricular Septal Defect Repair: Two-Institutions, 10-Year Experience in Cordoba, Argentina.
World journal for pediatric & congenital heart surgerySuccessful 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 youngFetal Characteristics and Perinatal Outcomes in Tetralogy of Fallot Without a Ductus Arteriosus.
Pediatric cardiology[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[Patch Closure of the Ventricular Septal Defect].
Kyobu geka. The Japanese journal of thoracic surgeryLeft-Sided Atrioventricular Valve Regurgitation After the Repair of Complete Atrioventricular Septal Defect.
Pediatric cardiologyEarly 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 surgeryDiagnosis and surgical treatment for complete atrioventricular septal defect with intact atrial septum.
Cardiology in the youngLeft atrioventricular valve regurgitation repair with concomitant outflow obstruction release on repaired atrioventricular septal defect case.
Journal of cardiothoracic surgeryImpact of Age and of the Patent Ductus Arteriosus on Pulmonary Hemodynamics in Children with Complete Atrioventricular Septal Defect.
Pediatric cardiologyMinimally Invasive Surgical Repair of Simple Congenital Heart Defects Using the Right Vertical Infra-Axillary Thoracotomy Approach.
Innovations (Philadelphia, Pa.)Left Atrioventricular Valve Regurgitation After Atrioventricular Septal Defect Repair.
The Annals of thoracic surgeryDihydropyrimidinase deficiency with atrioventricular septal defect: a case report.
Journal of pediatric endocrinology & metabolism : JPEMPredicting surgical outcome by echocardiography in complete atrioventricular canal defect repair.
Echocardiography (Mount Kisco, N.Y.)Predictors of post-operative left atrioventricular valve regurgitation in pediatric patients with complete atrioventricular canal defects.
Echocardiography (Mount Kisco, N.Y.)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)Repair of the complete atrioventricular septal defect-impact of postoperative moderate or more regurgitation.
Interdisciplinary cardiovascular and thoracic surgeryRisk of infective endocarditis after hybrid melody mitral valve replacement in infants: the French experience.
Interdisciplinary cardiovascular and thoracic surgeryCanonical Wnt signaling directs the generation of functional human PSC-derived atrioventricular canal cardiomyocytes in bioprinted cardiac tissues.
Cell stem cellTricuspid 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 surgeryUse of Interactive Visualization and 3D Printing in the Repair of Complex Congenital Heart Disease Presenting in Adult Life.
World journal for pediatric & congenital heart surgeryAssociation 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 SurgeryPartial heart transplantation of atrioventricular valves in complete atrioventricular septal defect-simulation of techniques using silicone-molded heart models.
JTCVS techniquesUse of glutaraldehyde-treated autologous pericardial patch in complete atrioventricular septal defect repair.
European review for medical and pharmacological sciencesPrimary 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 surgeryAnatomic considerations in the management of complete atrioventricular canal.
Cardiology in the youngLecompte maneuver for compressed left coronary artery after pulmonary artery banding.
Asian cardiovascular & thoracic annalsA Case of Successful Biventricular Repair of the Transposition of the Great Arteries with a Coronary Anomaly Associated with an Atrioventricular Septal Defect.
Pediatric cardiologyDiscussion to: Left atrioventricular valve repair after primary atrioventricular canal surgery: Predictors of durability.
The Journal of thoracic and cardiovascular surgeryCardiac magnetic resonance predictors for successful primary biventricular repair of unbalanced complete common atrioventricular canal.
Cardiology in the youngStaged repair for complete atrioventricular septal defect in patients weighing less than 4.0 kg.
The Journal of thoracic and cardiovascular surgeryLeft atrioventricular valve repair after primary atrioventricular canal surgery: Predictors of durability.
The Journal of thoracic and cardiovascular surgeryZebrafish smarcc1a mutants reveal requirements for BAF chromatin remodeling complexes in distinguishing the atrioventricular canal from the cardiac chambers.
Developmental dynamics : an official publication of the American Association of AnatomistsLong-Term Surgical Outcomes of Patients With Isomeric Right and Left Atrial Appendages.
World journal for pediatric & congenital heart surgerySimplified primum ASD closure technique in complete VSD repair: shallow suture crossing on the AV node.
General thoracic and cardiovascular surgeryComplete atrioventricular septal defect: Modified 2-patch technique.
Multimedia manual of cardiothoracic surgery : MMCTSEchocardiographic Evaluation of Postoperative Coaptation Geometry of Left AV Valve in Complete Atrioventricular Septal Defect.
Clinical medicine insights. PediatricsINDIAMAN-20 (INstant DIAgnosis of 20 Major ANomalies) protocol: application of IOTA diagnostic strategy to fetal anomalies.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyHigh temperature and hyperkalemia cause exit block of action potentials at the atrioventricular junction of rainbow trout (Oncorhynchus mykiss) heart.
Journal of thermal biologyKawashima Procedure: The Impact of Age.
The Annals of thoracic surgeryDirect ventriculoatrial shunt in a pediatric patient: case report and technical note.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgerySurgical Outcomes of Congenital Heart Disease in Down Syndrome: Tertiary Center Experience-Focus on the Electrical Conduction System.
Pediatric cardiologyExtracardiac Fontan completion after the Kawashima procedure with a custom-made bifurcated graft.
Multimedia manual of cardiothoracic surgery : MMCTSPredictors of increased postoperative length of stay after complete atrioventricular canal repair.
Cardiology in the youngRare association of absent pulmonary valve syndrome, complete atrioventricular canal defect, double outlet right ventricle, right aortic arch, and aberrant right subclavian artery in a fetal case.
Echocardiography (Mount Kisco, N.Y.)Modified single-patch repair for atrioventricular septal defects results in good functional outcomes in the absence of deep ventricular septal defects.
The Journal of thoracic and cardiovascular surgeryTiming of surgical repair and resource utilisation in infants with complete atrioventricular septal defect.
Cardiology in the youngA word of caution regarding early repair of complete atrioventricular septal defect: Don't let the pendulum swing too far.
JTCVS openSurgical Repair of Complete Common Atrioventricular Canal in a Conjoined Twin Following Separation.
World journal for pediatric & congenital heart surgeryPrenatal Diagnosis, Associations and Outcome for Fetuses with Congenital Absence of the Pulmonary Valve Syndrome.
Anatolian journal of cardiologyThe V-shaped double-layer patch technique for complete atrioventricular septal defect: A novel surgical technique.
The Journal of thoracic and cardiovascular surgeryIsolated Balanced Complete Atrioventricular Septal Defects: Prenatal Detection and Outcome in Nevada.
Clinical pediatricsThe influence of age and weight on the outcomes of complete atrioventricular septal defect repair.
The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of CardiologyCommon arterial trunk in functionally univentricular hearts: a case series.
Cardiology in the youngTechnique for Neo-Pulmonary Valve Creation With Living Tissue for Repair of Atrioventricular Septal Defect and Tetralogy of Fallot.
World journal for pediatric & congenital heart surgeryReoperations After Repair for Atrioventricular Septal Defects: >25 Years Experience at a Single Center.
Seminars in thoracic and cardiovascular surgeryVisualization and Quantification of the Unrepaired Complete Atrioventricular Canal Valve Using Open-Source Software.
Journal of the American Society of Echocardiography : official publication of the American Society of EchocardiographyBeyond the Syndrome: Extensive Congenital Abnormalities in an Infant With Trisomy 21.
Clinical pathology (Thousand Oaks, Ventura County, Calif.)Teaching the Modified Single-Patch Technique for Complete Atrioventricular Septal Defect.
World journal for pediatric & congenital heart surgeryContemporary Outcomes After Pulmonary Artery Banding in Complete Atrioventricular Septal Defect.
The Annals of thoracic surgeryRepair of complete atrioventricular septal defect between 2 and 3.5 kilograms: Defining the limits of safe repair.
The Journal of thoracic and cardiovascular surgeryContemporary surgical management of complete atrioventricular septal defect with tetralogy of Fallot in Japan.
General thoracic and cardiovascular surgeryComplete repair for Scimitar syndrome with transitional atrioventricular canal.
Asian cardiovascular & thoracic annalsEarly outcomes of fenestrated intra-extracardiac Fontan procedure: Insights, experiences, and expectations.
Journal of cardiac surgeryHome High-Flow Nasal Cannula Therapy in Children with Congenital Heart Disease.
Pediatric cardiologyCongenital heart defects in molecularly confirmed KBG syndrome patients.
American journal of medical genetics. Part ALong-Term Outcome Up To 40 Years after Single Patch Repair of Complete Atrioventricular Septal Defect in Infancy or Childhood.
The Thoracic and cardiovascular surgeonLV outflow obstruction after repair of atrioventricular septal defect: an uncommon but challenging problem.
Interactive cardiovascular and thoracic surgeryCorrelation of ventricular septal defect height and outcomes after complete atrioventricular septal defect repair.
Interactive cardiovascular and thoracic surgeryStent implantation of an unusual morphology patent ductus arteriosus via Glidesheath slender.
Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidirImpact of concomitant complex cardiac anatomy in nonsyndromic patients with complete atrioventricular septal defect.
The Journal of thoracic and cardiovascular surgeryPropensity Score Matched Analysis of Cleft Closure in Complete Atrioventricular Septal Defect Repair.
The Annals of thoracic surgeryThe effect of surgical technique, age, and Trisomy 21 on early outcome of surgical management of complete atrioventricular canal defect.
Cardiology in the youngMitral valve replacement in infants and younger children.
Scientific reportsMultifactorial origin of pulmonary hypertension in a child with congenital heart disease, Down syndrome, and BMPR-2 mutation.
Pulmonary circulationComplete atrioventricular septal defect with absent or diminutive primum component: Incidence, anatomic characteristics, and outcomes.
The Journal of thoracic and cardiovascular surgeryA rare case of pulmonary lymphangiectasia associated with CHD.
Cardiology in the youngPost-operative course of pulmonary artery pressure after complete atrioventricular canal defect repair.
Cardiology in the youngSurgery for severe congenital heart diseases in children from developing nations.
The Journal of thoracic and cardiovascular surgeryPredictors of reoperation and mortality after complete atrioventricular septal defect repair.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryImpact of Major Residual Lesions on Outcomes After Surgery for Congenital Heart Disease.
Journal of the American College of CardiologyRepair of complete atrioventricular canal defects: Early and midterm results and comparison of the left anterior leaflet augmentation technique with traditional technics.
Journal of cardiac surgeryUpper mini sternotomy approach for pulmonary artery banding: A single centre experience.
Journal of cardiac surgeryEarly postoperative arrhythmias in patients undergoing congenital heart surgery.
Turk gogus kalp damar cerrahisi dergisiLevosimendan Versus Milrinone and Release of Myocardial Biomarkers After Pediatric Cardiac Surgery: Post Hoc Analysis of Clinical Trial Data.
Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care SocietiesMelody valve to replace the mitral valve in small children: Lessons learned.
Annals of pediatric cardiologyCommentary: Early repair of complete atrioventricular septal defect is the forward move.
The Journal of thoracic and cardiovascular surgeryTwo-patch transannular repair of tetralogy of Fallot with complete atrioventricular canal defect.
Multimedia manual of cardiothoracic surgery : MMCTSModified Single-Patch Technique Versus Two-Patch Technique for the Repair of Complete Atrioventricular Septal Defect: An Updated Meta-Analysis.
Pediatric cardiologyA case of a complete atrioventricular canal defect in a ferret.
BMC veterinary researchDynamic Annular Modeling of the Unrepaired Complete Atrioventricular Canal Annulus.
The Annals of thoracic surgeryCharacteristics of the pulmonary circulation in infants with complete atrioventricular septal defect.
Cardiology in the youngCommentary: Repair of complete atrioventricular septal defect: The bar has been set.
The Journal of thoracic and cardiovascular surgeryLong-Term Follow-Up of Pericardium for the Ventricular Component in Atrioventricular Septal Defect Repair.
World journal for pediatric & congenital heart surgeryCommentary: Complete atrioventricular septal defect in young infants: The advantages of early repair.
The Journal of thoracic and cardiovascular surgeryShort to medium term outcomes of right ventricular outflow tract stenting as initial palliation for symptomatic infants with complete atrioventricular septal defect with associated tetralogy of Fallot.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & InterventionsCommentary: To band or not to band-is that really the question?
The Journal of thoracic and cardiovascular surgeryEarly repair of complete atrioventricular septal defect has better survival than staged repair after pulmonary artery banding: A propensity score-matched study.
The Journal of thoracic and cardiovascular surgeryLong-term outcome after early repair of complete atrioventricular septal defect in young infants.
The Journal of thoracic and cardiovascular surgeryAdult case of tetralogy of Fallot associated with atrioventricular septal defect.
General thoracic and cardiovascular surgeryMajor Septal Defects: Comparative Study of Down Syndrome and Non-Down Syndrome Infants, Before and After Surgery.
Pakistan journal of medical sciencesStaged repair of transposition of the great arteries with anomalous coronary anatomy and complete atrioventricular canal.
Cardiology in the youngSurgical Management for Complete Atrioventricular Septal Defects: A Systematic Review and Meta-Analysis.
Pediatric cardiologyThree-dimensional printing of the fetal heart with complete atrioventricular septal defect based on ultrasound data.
Kardiologia polskaMonitored Anesthesia Care of Two Patients with Highly Elevated Subpulmonic Ventricular Pressure due to Adult Congenital Heart Disease.
Case reports in cardiologyVentriculoatrial malalignment in atrioventricular septal defect resulting in uniatrial biventricular connection: surgical options.
Journal of cardiothoracic surgeryLevosimendan Versus Milrinone for Inotropic Support in Pediatric Cardiac Surgery: Results From a Randomized Trial.
Journal of cardiothoracic and vascular anesthesiaAtrioventricular Valve Function Predicts Reintervention in Complete Atrioventricular Septal Defect.
World journal for pediatric & congenital heart surgeryModified Single Repair Technique for Complete Atrioventricular Septal Defect: A Propensity Score Matching Analysis.
Pediatric cardiologyOutcomes of Supra-annular Mechanical Atrioventricular Valve Replacement with Polytetrafluoroethylene Graft in Infants and Children.
Pediatric cardiologyThe Pediatric Heart Network Residual Lesion Score Study: Design and objectives.
The Journal of thoracic and cardiovascular surgeryCommentary: Pulmonary artery banding in infants with atrioventricular septal defect, valid strategy or backward move?
The Journal of thoracic and cardiovascular surgeryTwelve Years of Complete Atrioventricular Septal Defect Repair.
Revista portuguesa de cirurgia cardio-toracica e vascular : orgao oficial da Sociedade Portuguesa de Cirurgia Cardio-Toracica e VascularIvabradine as an Adjunct for Refractory Junctional Ectopic Tachycardia Following Pediatric Cardiac Surgery: A Preliminary Study.
World journal for pediatric & congenital heart surgeryEarly and midterm outcomes of a modified single-patch technique for repairing complete atrioventricular septal defect in children and adults.
Journal of cardiac surgeryLeft bundle branch pacing, the only feasible physiological pacing modality for a patient with complete atrioventricular septal defect after surgical correction.
Journal of cardiovascular electrophysiologyCommentary: No harm, no foul-Staged pulmonary artery banding in complete canal defects.
The Journal of thoracic and cardiovascular surgeryPulmonary artery banding in complete atrioventricular septal defect.
The Journal of thoracic and cardiovascular surgeryCommentary: Atrioventricular canal repair: Surgeon preference reigns supreme.
The Journal of thoracic and cardiovascular surgeryAdipose‑derived stem cells overexpressing SK4 calcium‑activated potassium channel generate biological pacemakers.
International journal of molecular medicineCommentary: This case is closed.
The Journal of thoracic and cardiovascular surgeryCommentary: Complete Atrioventricular Canal Defects: Is There a Superior Repair Technique?
Seminars in thoracic and cardiovascular surgeryComplete atrioventricular septal defect repair in Australia: Results over 25 years.
The Journal of thoracic and cardiovascular surgeryMosaic complete tetrasomy 21 in a fetus with complete atrioventricular septal defect and minor morphological variations.
Molecular genetics & genomic medicineModified-Single Patch vs Double Patch Repair of Complete Atrioventricular Septal Defects.
Seminars in thoracic and cardiovascular surgeryThe Effect of Levosimendan Versus Milrinone on the Occurrence Rate of Acute Kidney Injury Following Congenital Heart Surgery in Infants: A Randomized Clinical Trial.
Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care SocietiesConcurrent right atrial isomerism, complete atrioventricular septal defect, and single ventricle in an L-transposition of great arteries patient complicated by brain abscess.
Cardiology in the youngAtrioventricular septal defect and tetralogy of Fallot - A single tertiary center experience: A retrospective review.
Annals of pediatric cardiologyPreoperative Clinical and Echocardiographic Factors Associated with Surgical Timing and Outcomes in Primary Repair of Common Atrioventricular Canal Defect.
Pediatric cardiologyPropensity-matched comparison of the long-term outcome of the Nunn and two-patch techniques for the repair of complete atrioventricular septal defects.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryCase 2 / 2019 - Complete Atrioventricular Septal Defect, with Down Syndrome, without Pulmonary Hypertension and Natural History at 33 Years of Age.
Arquivos brasileiros de cardiologiaSurgical Treatment for Congenital Heart Defects in Down Syndrome Patients.
Brazilian journal of cardiovascular surgeryComplete Atrioventricular Septal Defect: Evolution of Results in a Single Center During 50 Years.
The Annals of thoracic surgeryNovel Left Atrioventricular Valvuloplasty for Atrioventricular Septal Defect.
The Annals of thoracic surgeryEvaluation of septal insertion of atrioventricular valves in fetuses by postmortem 4.7 Tesla cardiac MRI: A feasibility study.
Diagnostic and interventional imagingMitral valve replacement with a pulmonary autograft: long-term follow-up in an infant.
Interactive cardiovascular and thoracic surgeryDysrhythmias in patients with a complete atrioventricular septal defect: From surgery to early adulthood.
Congenital heart diseaseEchocardiographic Assessment of Complete Atrioventricular Canal Defects: A Balancing Act.
Seminars in thoracic and cardiovascular surgeryStaged Repair of Truncus Arteriosus Associated with Complete Atrioventricular Septal Defect.
The Korean journal of thoracic and cardiovascular surgeryProximal Isovelocity Surface Area Method for Diagnosing Isolated Pulmonary Vein Stenosis in a Case of Complete Atrioventricular Septal Defect With Increased Pulmonary Venous Blood Flow.
A&A practiceBloodless Heart Surgery for an 11-kg Infant of the Jehovah's Witness Faith Undergoing Second Repair for Complete Atrioventricular Canal.
The journal of extra-corporeal technologyComplete atrioventricular canal repair with a decellularized porcine small intestinal submucosa patch.
Congenital heart diseaseIs the modified single-patch repair superior to the double-patch repair of complete atrioventricular septal defects?
Interactive cardiovascular and thoracic surgeryContemporary Outcomes After Repair of Isolated and Complex Complete Atrioventricular Septal Defect.
The Annals of thoracic surgeryHybrid approach: an alternative prior to corrective surgery in a patient with arch hypoplasia and complete atrioventricular septal defect.
Turk gogus kalp damar cerrahisi dergisiBradyarrhythmias in Repaired Atrioventricular Septal Defects: Single-Center Experience Based on 34 Years of Follow-Up of 522 Patients.
Pediatric cardiologyVertical Right Axillary Mini-Thoracotomy for Correction of Ventricular Septal Defects and Complete Atrioventricular Septal Defects.
The Annals of thoracic surgeryA review of the Nunn modified single patch technique for atrioventricular septal defect repair.
Translational pediatricsThe role of intraoperative epicardial echocardiography in pediatric cardiac surgery.
Echocardiography (Mount Kisco, N.Y.)The Congenital Heart Surgeon's Society Complete Atrioventricular Septal Defect Cohort: Baseline, Preintervention Echocardiographic Characteristics.
Seminars in thoracic and cardiovascular surgeryAcute Effect of Inhaled Iloprost in Children with Pulmonary Arterial Hypertension Associated with Simple Congenital Heart Defects.
Pediatric cardiologyDoes biventricular conversion bring survival benefits to patients with an unbalanced atrioventricular septal defect?
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryRetrospective study of complete atrioventricular canal defects: Anesthetic and perioperative challenges.
Annals of cardiac anaesthesia[Surgical treatment and early-mid follow-up results of complete atrioventricular septal].
Zhonghua wai ke za zhi [Chinese journal of surgery]Analysis of Copy Number Variants on Chromosome 21 in Down Syndrome-Associated Congenital Heart Defects.
G3 (Bethesda, Md.)Current status of cardiovascular surgery in Japan 2013 and 2014: A report based on the Japan Cardiovascular Surgery Database. 2: Congenital heart surgery.
General thoracic and cardiovascular surgeryInfluence of staged repair and primary repair on outcomes in patients with complete atrioventricular septal defect and tetralogy of Fallot: a systematic review and meta-analysis.
Interactive cardiovascular and thoracic surgeryOne-stage definitive repair of complete atrioventricular septal defect and pulmonary atresia with major aortopulmonary collateral arteries.
Interactive cardiovascular and thoracic surgeryDown-Klinefelter syndrome (48,XXY,+21) in a neonate associated with congenital heart disease.
Genetics and molecular research : GMRModified Single-Patch Technique Versus Two-Patch Technique for the Repair of Complete Atrioventricular Septal Defect: A Meta-Analysis.
Pediatric cardiologyComplete Atrioventricular Canal Defect: Towards a More Physiological Repair.
Heart, lung & circulationTechnical Performance Score: Predictor of Outcomes in Complete Atrioventricular Septal Defect Repair.
The Annals of thoracic surgeryMid-term outcomes in unbalanced complete atrioventricular septal defect: role of biventricular conversion from single-ventricle palliation.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryRepair of Complete Atrioventricular Septal Defects With Decellularized Extracellular Matrix: Initial and Midterm Outcomes.
World journal for pediatric & congenital heart surgeryMacroreentrant atrial tachycardia around the native common atrioventricular valve in a surgically corrected complete 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 CardiologyCardiac Function After Tetralogy of Fallot/Complete Atrioventricular Canal Repair.
World journal for pediatric & congenital heart surgeryRepair of complete atrioventricular septal defect with tetralogy of Fallot.
Translational pediatricsProphylactic Pulmonary Artery Reduction in a Young Female with Severe Pulmonary Hypertension from Complete Atrioventricular Septal Defect.
Korean circulation journalSurgical repair of a complete atrioventricular septal defect with tetralogy of Fallot using a classical one-patch technique and pulmonary valve preservation.
Multimedia manual of cardiothoracic surgery : MMCTSComplete atrioventricular septal defect in the era of prenatal diagnosis.
Medical ultrasonographyEarly pulmonary arterial hypertension immediately after closure of a ventricular or complete atrioventricular septal defect beyond 6months of age.
International journal of cardiologyAnesthetic management of a child with complete atrioventricular septal defect and single ventricle posted for noncardiac surgery.
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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.
- 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.
- Ivemark syndrome in an adult with complete atrioventricular septal defect and a single ventricle with L-transposition of the great arteries: a case report.
- Complete atrioventricular septal defect, Australian repair: Technique and long-term results.
- Intraoperative predictors of left atrioventricular valve reoperation after repair of complete atrioventricular septal defect.
- The Fate of the Left Atrioventricular Valve After Atrioventricular Septal Defect Repair: Long-Term Outcomes.
- Echocardiographic Assessment of Pulmonary Arterial Hypertension After Complete Atrioventricular Septal Defect Repair.
- Cleft-to-commissure conversion in atrioventricular septal defect using artificial chordae: Technical report and case illustration.
- Sustained remission of post-Fontan protein-losing enteropathy achieved by combination therapy with high-dose spironolactone and the somatostatin analogue octreotide: a case report.
- Heart failure in a patient with Glenn circulation during secondary pregnancy with a single ventricle: A rare case report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:1329(Orphanet)
- MONDO:0015273(MONDO)
- GARD:1454(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q55785365(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
