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.
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.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 23 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 63 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.
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
Medicamentos e terapias
Mecanismo: Glucocorticoid receptor agonist
Mecanismo: Cyclophilin A modulator
Mecanismo: FK506-binding protein 1A inhibitor
Variantes genéticas (ClinVar)
533 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 1,501 variantes classificadas pelo 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 do canal auriculoventricular
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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
9 ensaios clínicos encontrados, 1 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 745
'Double-ring'-like dysplastic mitral leaflet associated with atrioventricular septal defect mimicking endocarditis: computed tomography as key to diagnosis.
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.
Case Report: Anaesthetic management in a canine patient with severe atrioventricular septal defect and pulmonary hypertension undergoing non-cardiac surgery.
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.
Transcatheter Occlusion of Hepatic Vein-Atrial Communication After Modified Fontan in a Child Post Kawashima Surgery.
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.
Development of 3D-Printed Congenital Heart Disease Models Using Feasible Low-Cost Workflow - A Potential Tool to Improve Pediatric Cardiology Education.
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.
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.
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.
Left Atrial Isomerism in a Four-Year-Old Girl Diagnosed due to Gastrointestinal Bleeding: A Case Report.
Eccentricity Index Is Associated With Pulmonary Arterial Hypertension in Children After Repair of Complete Atrioventricular Septal Defects.
Pulmonary Hypertension in Down Syndrome Versus Non-syndromic Pediatric Populations With Congenital Heart Disease: A Comparative Study.
📚 EuropePMC732 artigos no totalmostrando 198
Transcatheter Occlusion of Hepatic Vein-Atrial Communication After Modified Fontan in a Child Post Kawashima Surgery.
JACC. Case reportsAtrioventricular septal defect with cor triatriatum sinister.
Anales de pediatriaDevelopment of 3D-Printed Congenital Heart Disease Models Using Feasible Low-Cost Workflow - A Potential Tool to Improve Pediatric Cardiology Education.
Arquivos brasileiros de cardiologiaPrognostic 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 anesthesiaThe Combined Double-Orifice and Single-Patch Technique for Partial Atrioventricular Septal Defect in Adults: A Novel Strategy.
Cardiovascular therapeuticsSurviving Adulthood with Rare Combined Congenital Heart Defects: Complete AV Canal Defect, Ebstein's Anomaly, and Right Ventricular Hypoplasia.
Life (Basel, Switzerland)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'Double-ring'-like dysplastic mitral leaflet associated with atrioventricular septal defect mimicking endocarditis: computed tomography as key to diagnosis.
European heart journal. Case reportsCase Report: Anaesthetic management in a canine patient with severe atrioventricular septal defect and pulmonary hypertension undergoing non-cardiac surgery.
Frontiers in veterinary scienceGenetic Syndromes and Multimorbidity in Adults with Congenital Heart Disease and Heart Failure: Insights from the PATHFINDER-CHD Registry.
Journal of clinical medicineArrhythmia Following Congenital Heart Disease Surgery in Oman: Incidence and risk factors - A prospective study.
Sultan Qaboos University medical journalRepair of Partial and Transitional Atrioventricular Septal Defects: Mid-term Outcomes in a Single-center Cohort.
Reviews in cardiovascular medicineInfant with Complex Congenital Cardiac Anomalies: A Case Report and Review of Literature.
Annals of African medicineIvemark 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 reportsLeft ventricular outflow tract obstruction repair after atrioventricular septal defect correction.
Multimedia manual of cardiothoracic surgery : MMCTSLeft atrioventricular valve durability in atrioventricular septal defects: Impact of systolic blood pressure.
JTCVS openSingle incision minimally invasive congenital heart surgery: Insights into program development and outcome.
The Journal of thoracic and cardiovascular surgeryCase Report: Concurrent atrial and ventricular septal defect in a young Sphynx cat.
Frontiers in veterinary scienceFetal presentation of a child with partial atrioventricular septal defect and calcium release deficiency syndrome.
HeartRhythm case reportsComplete atrioventricular septal defect, Australian repair: Technique and long-term results.
Asian cardiovascular & thoracic annalsLeft 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 reportsPathogenic variant in GATA4 associated with atrioventricular septal defect and congenital diaphragmatic hernia: A case report.
European journal of medical geneticsFrequency of Persistent Left Superior Vena Cava and Its Impact on Outcomes in Children Undergoing Congenital Heart Surgery.
Brazilian journal of cardiovascular surgeryUtility of Three-Dimensional Holographic Workstation for Preoperative Simulation of Complex Congenital Heart Surgery.
Interdisciplinary cardiovascular and thoracic surgeryManaging Pregnancy in a Patient With Unrepaired Congenital Heart Disease.
JACC. Case reportsMyocardial reprogramming by HMGN1 underlies heart defects in trisomy 21.
NaturePredictive 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 : MMCTSSurgical correction of anomalous pulmonary veinous return and atrioventricular septal defect in a patient with left lung agenesis.
JTCVS techniquesPartial Atrioventricular Septal Defect Repair through Right Thoracotomy in A 73-Year-Old Woman.
Surgical case reportsMultisystem Phenotypic Spectrum in Pediatric Heterotaxy Syndrome: A Case Series.
CureusUnilateral absence of the pulmonary artery in a paediatric cohort.
Cardiology in the youngAorto-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 AssociationCommentary: Second cardiopulmonary bypass run after complete atrioventricular septal defect repair: To perform or not to perform?
The Journal of thoracic and cardiovascular surgeryClues on the Three-Vessel View for Fetal Diagnosis of Supracardiac Total Anomalous Pulmonary Venous Connection.
CASE (Philadelphia, Pa.)Long-term outcomes following atrioventricular septal defect in adult patients.
Annals of medicineIntraoperative predictors of left atrioventricular valve reoperation after repair of complete atrioventricular septal defect.
The Journal of thoracic and cardiovascular surgeryConcurrent 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 scienceClinical outcomes of tetralogy canal repair: A multidisciplinary perspective.
Saudi medical journalRare Presentation of Partial Atrioventricular Septal Defect in the Elderly: A Case Report.
CureusUnbalanced Atrioventricular Septal Defect: Could This Be the Best Substrate for Recruitment of a Borderline Left Ventricle?
The Annals of thoracic surgeryIndividualized approach to early surgery for complete repair of atrioventricular septal defect.
The Journal of thoracic and cardiovascular surgeryStenotic left atrioventricular valve after atrioventricular septal defect repair: A distinct surgical phenotype.
The Journal of thoracic and cardiovascular surgeryHigher Incidence of Moderate and Severe Congenital Heart Disease in Patients With Neonatal Abstinence Syndrome.
Neonatal network : NNStaged right ventricular recruitment of unbalanced atrioventricular septal defect.
JTCVS techniquesPartial AVSD with cortriatriatum sinister with unroofed coronary sinus in an adult.
Annals of pediatric cardiologyTruncus bicaroticus with aberrant origin of right subclavian artery in a patient with atrioventricular septal defect: A rare aortic branch anomaly.
Annals of pediatric cardiologyRe-operative minimally invasive endoscopic mitral valve repair after partial atrioventricular canal repair.
Multimedia manual of cardiothoracic surgery : MMCTSA Rare Case of Dextrocardia With Atrioventricular Septal Defect and Situs Inversus in an 8-Month-Old Infant Complicated by Severe Acute Malnutrition.
Clinical case reportsRenal Hemosiderosis Associated with Mitral Regurgitation Detected by Severe Proteinuria Due to Congestion.
Internal medicine (Tokyo, Japan)Heterotaxy 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?
CureusIatrogenic coronary sinus diversion to left atrium mimicking paravalvular leak: the importance of documentation.
Indian journal of thoracic and cardiovascular surgeryAtrioventricular Inflow and Subsequent Ventricular Growth After Staged Ventricular Recruitment for Unbalanced Atrioventricular Canal Defect.
The Annals of thoracic surgerySurgical 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 reportsLeft-sided valve dysfunction with extreme giant left atrium and anomalous coronary sinus connection after atrioventricular septal defect repair.
JTCVS techniquesUtility of Intraoperative 3D TEE to Unmask a Hidden Intracardiac Fistula.
JACC. Case reportsThirty years of left atrioventricular valve reoperations following atrioventricular septal defect repair: Impact of valvar stenosis.
The Journal of thoracic and cardiovascular surgeryOutcomes of 3-Dimensional Total Endoscopic Surgery for Partial Atrioventricular Septal Defect in Children: A Single-Center Experience in Vietnam.
Innovations (Philadelphia, Pa.)Single center experience with surgically implanted Melody and Sapien 3 valves in the mitral position in young children.
Frontiers in cardiovascular medicineAbsence of the Left Circumflex Artery in Congenital Heart Disease With Vasospastic Myocardial Infarction.
JACC. Case reportsDuplication 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 surgeryStatus and Operative Outcomes of the Fontan Procedure Performed Beyond the First Decade of Life in the United States.
World journal for pediatric & congenital heart surgeryMitral and tricuspid valve repair in an operated case of complete atrioventricular septal defect: A surgical challenge case report!
SAGE open medical case reportsUnanticipated Abdominal Compartment Syndrome in an Infant Undergoing Atrioventricular Septal Defect Repair.
Journal of cardiothoracic and vascular anesthesiaSystematic analysis of the atrioventricular canal in 129 heart specimens identified anatomic and microarchitectural advantages in Down syndrome.
The Journal of thoracic and cardiovascular surgerySurgical repair of partial atrioventricular septal defect (pAVSD) in adulthood: A rare case report.
International journal of surgery case reports[MSB-26] Minimally Invasive Right Vertical Axillary Thoracotomy for Repair of Congenital Heart Defects: Azerbaijan Experience.
Turk gogus kalp damar cerrahisi dergisiRhythm Disturbances in Children With Down Syndrome who Underwent Surgery for Congenital Heart Defects.
World journal for pediatric & congenital heart surgeryCor triatriatum sinister with partial atrioventricular septal defect in a cat.
Veterinarni medicinaCoronary Sinus Blues: A Word of Caution When Placing the Coronary Sinus Ostium in the Left Atrium.
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 pediatricsPulmonary Artery Banding in a Cat with Atrioventricular Canal Defect Type A with Concurrent Muscular Septal Defect.
Animals : an open access journal from MDPIThe 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 youngDiastolic Mitral Regurgitation With Interatrial Left-to-Right Shunt.
JACC. Case reportsA 27-Year Experience with Atrioventricular Septal Defect Correction.
The Thoracic and cardiovascular surgeonIntermediate-Type Atrioventricular Septal Defect Repair With Atrioventricular Valve Annuloplasty via Minimally Invasive Approach.
Innovations (Philadelphia, Pa.)Can Fetal Heterotaxy Syndrome Be Diagnosed Through Prenatal Ultrasound in the First Trimester (GA 11+0-13+6 Weeks)?
Prenatal diagnosisA 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 surgeryPrimary 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 surgeryPeripandemic outcomes of infants treated for sentinel congenital heart diseases in England and Wales.
Open heartThe particular anatomy of atrioventricular septal defect with a common valvar orifice in patients with Down syndrome: an echocardiographic study.
International journal of cardiologyOutcomes of Atrioventricular Septal Defect Repair: Two-Institutions, 10-Year Experience in Cordoba, Argentina.
World journal for pediatric & congenital heart surgeryBerlin Heart EXCOR sVAD implantation technique for neonates and infants with functionally univentricular ductal-dependent systemic circulation.
Multimedia manual of cardiothoracic surgery : MMCTSA 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 reportsSuccessful 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[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 NuklearmedizinRescuing the Right Ventricle: Mechanical Support After Pediatric Heart Transplantation.
Annals of thoracic surgery short reportsFetal Characteristics and Perinatal Outcomes in Tetralogy of Fallot Without a Ductus Arteriosus.
Pediatric cardiologyCan we diagnose aortic arch obstruction in a fetus with an atrioventricular septal defect?
Cardiology in the young[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 surgeryTranscatheter 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[Patch Closure of the Ventricular Septal Defect].
Kyobu geka. The Japanese journal of thoracic surgeryEpidemiology of Congenital Heart Defects in Perinatal Infants in Guangxi, China.
International journal of general medicineLeft-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 surgerySinus node dysfunction in children: different aetiologies, similar clinical course in two-centre experience.
Cardiology in the youngBerlin Heart EXCOR sVAD upsizing and exchange technique.
Multimedia manual of cardiothoracic surgery : MMCTSIdentification 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 biologyHemi-Commando with aortic homograft and atrioventricular septal defect repair for recurrent triple valve endocarditis.
JTCVS techniquesDiagnosis and surgical treatment for complete atrioventricular septal defect with intact atrial septum.
Cardiology in the youngEvaluating Long-Term Outcomes of Children Undergoing Surgical Treatment for Congenital Heart Disease for National Audit in England and Wales.
Journal of the American Heart AssociationA case report: pitfalls in antibacterial therapy with rifampicin for mechanical valve endocarditis-the king of drug interactions.
European heart journal. Case reportsStaged ventricular recruitment following single-ventricular palliation in unbalanced atrioventricular septal defect with heterotaxy syndrome.
Cardiology in the youngEvaluation of mid-term outcomes of partial and intermediate atrioventricular canal defect surgery.
Cardiology in the youngOutcomes of left atrioventricular valve operation following atrioventricular septal defect repair.
The Journal of thoracic and cardiovascular surgeryLeft atrioventricular valve regurgitation repair with concomitant outflow obstruction release on repaired atrioventricular septal defect case.
Journal of cardiothoracic surgeryMissense mutations in the CITED2 gene may contribute to congenital heart disease.
BMC cardiovascular disordersFetal 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 ResonancePartial atrioventricular septal defect: An unexpected anomaly in a 68-year-old patient.
Kardiologia polskaCompound heterozygosity for two variants in BMP5 in human skeletal dysostosis with atrioventricular septal defect.
Clinical geneticsDouble-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 surgeryArrhythmias in congenital heart disease: A nationwide cohort study.
American heart journalImpact 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.)Nationwide Birth Prevalence of Crucial Congenital Heart Defects From 2014 to 2018 in Korea.
Korean circulation journalExtracorporeal Membrane Oxygenation after Pediatric Cardiac Surgery: A Single-Center Experience.
Turkish archives of pediatricsRacial disparities in maternal exposure to ambient air pollution during pregnancy and prevalence of congenital heart defects.
American journal of epidemiologyDown syndrome and associated atrioventricular septal defects in a nationwide Norwegian cohort: Prevalence, time trends, and outcomes.
Acta obstetricia et gynecologica ScandinavicaPulmonary Artery Banding and Arch Repair vs Norwood for Unbalanced Atrioventricular Canal Defect.
The Annals of thoracic surgerySpectrum of Heart Diseases in Children in a National Cardiac Referral Center Tanzania, Eastern Africa: A Six-Year Overview.
Global heartLeft Atrioventricular Valve Regurgitation After Atrioventricular Septal Defect Repair.
The Annals of thoracic surgeryPartial atrioventricular canal defect and aortic coarctation associated with variants in GDF1 and NOTCH1 genes: A case report.
Birth defects researchCongenital heart disease: types, pathophysiology, diagnosis, and treatment options.
MedCommDihydropyrimidinase deficiency with atrioventricular septal defect: a case report.
Journal of pediatric endocrinology & metabolism : JPEMIntra-aortic balloon pump treatment in an adult patient with a Fontan circulation and acute heart failure: a case report.
European heart journal. Case reportsMolecular Pathways and Animal Models of Atrioventricular Septal Defect.
Advances in experimental medicine and biologyHuman Genetics of Atrioventricular Septal Defect.
Advances in experimental medicine and biologyClinical Presentation and Therapy of Atrioventricular Septal Defect.
Advances in experimental medicine and biologyMolecular Pathways and Animal Models of Atrial Septal Defect.
Advances in experimental medicine and biologyCardiac Development and Animal Models of Congenital Heart Defects.
Advances in experimental medicine and biologyRisk factors for the recurrence of left atrioventricular valvular regurgitation after surgical repair of partial and transitional atrioventricular septal defect.
Journal of thoracic diseasePredicting surgical outcome by echocardiography in complete atrioventricular canal defect repair.
Echocardiography (Mount Kisco, N.Y.)Staged repair of borderline hypoplastic heart disease with early biventricular conversion.
JTCVS techniquesCongenital heart "Challenges" in Down syndrome.
World journal of cardiologyOutcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus.
The Journal of pediatricsComparison of post-operative transesophageal and transthoracic echocardiogram findings following atrioventricular septal defect repair.
Cardiology in the youngPredictors of post-operative left atrioventricular valve regurgitation in pediatric patients with complete atrioventricular canal defects.
Echocardiography (Mount Kisco, N.Y.)A novel MED12 pathogenic variant in a female fetus with facial cleft and cardiac defects identified in the first trimester.
Prenatal diagnosisImpact of trisomy 21 on late surgical management results in patients with common atrioventricular septal defect: single-center experience.
Polish archives of internal medicineCardiac Complications in Trisomy 21 Patients in a Secondary Hospital: A Descriptive Study.
CureusTumour lysis syndrome in a neonate with transient abnormal myelopoiesis.
Journal of neonatal-perinatal medicinePRKD1-related telangiectasia-ectodermal dysplasia-brachydactyly-cardiac anomaly syndrome: Case report and review of the literature.
European journal of medical geneticsFetal left and right ventricular strain parameters using speckle tracking in congenital heart diseases.
The international journal of cardiovascular imagingManagement 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)Revisiting Atrioventricular Septal Defects: Exploring Chromosomal Abnormalities, Cardiac and Extracardiac Anomalies in a Contemporary Prenatal Cohort.
Pediatric cardiologyRepair of the complete atrioventricular septal defect-impact of postoperative moderate or more regurgitation.
Interdisciplinary cardiovascular and thoracic surgerySuccessful surgical correction of an incomplete atrioventricular septal defect in a 76-year-old female patient.
Journal of surgical case reportsCleft closure and other predictors of contemporary outcomes after atrioventricular canal repair in patients with parachute left atrioventricular valve.
Interdisciplinary cardiovascular and thoracic surgeryRisk of infective endocarditis after hybrid melody mitral valve replacement in infants: the French experience.
Interdisciplinary cardiovascular and thoracic surgeryTwo-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 onePreoperative 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 cardiologyLeft ventricular outflow tract obstruction after repair of atrioventricular septal defect.
JTCVS openRole of ECG in the Accidental Finding of an Atrioventricular Septal Defect in an Asymptomatic Patient Undergoing Cosmetic Surgery.
CureusCanonical Wnt signaling directs the generation of functional human PSC-derived atrioventricular canal cardiomyocytes in bioprinted cardiac tissues.
Cell stem cellEfficacy of atrioventricular valve regurgitation in the first trimester for the diagnosis of atrioventricular septal defect.
Journal of clinical ultrasound : JCUAntifungal medication use during early pregnancy and the risk of congenital heart defects in the National Birth Defects Prevention Study, 1997-2011.
Birth defects researchTricuspid 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 surgeryApplication of neural networks in prenatal diagnosis of atrioventricular septal defect.
Translational pediatricsUse 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 SurgeryNational population-based estimates for major birth defects, 2016-2020.
Birth defects researchSimultaneous 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 cardiologyInteraction 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 GynecologyPercutaneous Closure of Hemodynamically Significant Postoperative Residual Ventricular Septal Defects.
Pediatric cardiology[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 geneticsOutcomes and characteristics in term infants with necrotising enterocolitis and CHD.
Cardiology in the youngPartial heart transplantation of atrioventricular valves in complete atrioventricular septal defect-simulation of techniques using silicone-molded heart models.
JTCVS techniquesIntroduction 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 & InterventionsMulticentre 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 SurgeryFetal single ventricle journey to first postnatal procedure: a multicentre UK cohort study.
Archives of disease in childhood. Fetal and neonatal editionUse of glutaraldehyde-treated autologous pericardial patch in complete atrioventricular septal defect repair.
European review for medical and pharmacological sciencesDouble-orifice mitral valve and partial atrioventricular septal defect: A rare combination.
Asian journal of surgeryLong-Term Results Following Combined Repair of Atrioventricular Septal Defect with Tetralogy of Fallot.
Pediatric cardiologyMinimally Invasive Surgery for Simple Congenital Heart Defects: Preserving Aesthetics without Jeopardizing Patient Safety.
Journal of cardiovascular development and diseaseSuccessful Treatment of Pulmonary Artery Aneurysm With Atrioventricular Septal Defect and Pulmonary Hypertension: Clinical and Pathologic Features.
CJC pediatric and congenital heart diseasePrimary 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 surgeryEarly and Mid-Term Outcomes of Primary Repair After Atrioventricular Canal Defect: A Single-Center Eight-Year Experience.
CureusA retrospective audit of young adults who received permanent pacemakers at a teaching hospital in the Western Cape, South Africa.
Frontiers in cardiovascular medicineDouble outlet right ventricle.
Frontiers in pediatricsAnatomic considerations in the management of complete atrioventricular canal.
Cardiology in the youngPlasma proteoglycan 4: a novel biomarker for acute lung injury after pediatric cardiac surgery.
Translational pediatricsRedo 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 surgeryRole of autopsy in diagnosing asplenia and right bilobed lung in a fetus with dextrocardia.
BMJ case reportsLecompte maneuver for compressed left coronary artery after pulmonary artery banding.
Asian cardiovascular & thoracic annalsTranscatheter aortic valve-in-valve implantation in right ventricle-aorta conduit in an adult patient with Fontan circulation.
International journal of cardiology. Congenital heart diseaseAtrioventricular Septal Defect With Tetralogy of Fallot and Exclusive Shunting at the Ventricular Level.
World journal for pediatric & congenital heart surgeryCatheter 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 CardiologyClinical profile, prenatal detection and predictors of outcome of heterotaxy syndromes in Western Australia.
International journal of cardiology. Congenital heart diseaseOutcomes of atrioventricular septal defects with and without down syndrome: analysis of the national inpatient database.
Cardiology in the youngDetection of severe congenital heart defects in live-born infants in Norway 2017-2020.
Acta paediatrica (Oslo, Norway : 1992)Combined tricuspid atresia- AV septal defect-a rare congenital cardiac abnormality.
Echocardiography (Mount Kisco, N.Y.)A Case of Successful Biventricular Repair of the Transposition of the Great Arteries with a Coronary Anomaly Associated with an Atrioventricular Septal Defect.
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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.
- 'Double-ring'-like dysplastic mitral leaflet associated with atrioventricular septal defect mimicking endocarditis: computed tomography as key to diagnosis.
- Case Report: Anaesthetic management in a canine patient with severe atrioventricular septal defect and pulmonary hypertension undergoing non-cardiac surgery.
- Transcatheter Occlusion of Hepatic Vein-Atrial Communication After Modified Fontan in a Child Post Kawashima Surgery.
- Development of 3D-Printed Congenital Heart Disease Models Using Feasible Low-Cost Workflow - A Potential Tool to Improve Pediatric Cardiology Education.
- 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.
- 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.
- Left Atrial Isomerism in a Four-Year-Old Girl Diagnosed due to Gastrointestinal Bleeding: A Case Report.
- Eccentricity Index Is Associated With Pulmonary Arterial Hypertension in Children After Repair of Complete Atrioventricular Septal Defects.
- Pulmonary Hypertension in Down Syndrome Versus Non-syndromic Pediatric Populations With Congenital Heart Disease: A Comparative Study.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:98722(Orphanet)
- MONDO:0020290(MONDO)
- GARD:802(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- 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
