A Atresia Pulmonar com Defeito do Septo Ventricular (PA-VSD) é uma malformação cardíaca congênita rara que causa cianose (coloração azulada na pele por falta de oxigênio). Ela se caracteriza por: um subdesenvolvimento da "saída" do ventrículo direito (a parte do coração que bombeia o sangue para os pulmões); atresia da válvula pulmonar (ou seja, a válvula que leva o sangue aos pulmões não se desenvolveu ou está completamente fechada); um defeito do septo ventricular (um buraco na parede que divide os dois lados do coração); e vasos sanguíneos colaterais pulmonares (vasos extras que surgem para tentar levar sangue aos pulmões). Os sintomas dependem da forma como a malformação se apresenta em cada paciente. As pessoas podem ter poucos sintomas, ficar muito azuladas (cianose grave) ou desenvolver insuficiência cardíaca (quando o coração não consegue bombear sangue suficiente para o corpo). A PA-VSD pode ser considerada uma forma mais grave da Tetralogia de Fallot.
Introdução
O que você precisa saber de cara
A Atresia Pulmonar com Defeito do Septo Ventricular (PA-VSD) é uma malformação cardíaca congênita rara que causa cianose (coloração azulada na pele por falta de oxigênio). Ela se caracteriza por: um subdesenvolvimento da "saída" do ventrículo direito (a parte do coração que bombeia o sangue para os pulmões); atresia da válvula pulmonar (ou seja, a válvula que leva o sangue aos pulmões não se desenvolveu ou está completamente fechada); um defeito do septo ventricular (um buraco na parede que divide os dois lados do coração); e vasos sanguíneos colaterais pulmonares (vasos extras que surgem para tentar levar sangue aos pulmões). Os sintomas dependem da forma como a malformação se apresenta em cada paciente. As pessoas podem ter poucos sintomas, ficar muito azuladas (cianose grave) ou desenvolver insuficiência cardíaca (quando o coração não consegue bombear sangue suficiente para o corpo). A PA-VSD pode ser considerada uma forma mais grave da Tetralogia de Fallot.
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
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 3 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
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
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 — Atresia pulmonar com defeito do septo ventricular
Centros de Referência SUS
24 centros habilitados pelo SUS para Atresia pulmonar com defeito do septo ventricular
Centros para Atresia pulmonar com defeito do septo ventricular
Detalhes dos centros
Hospital Universitário Prof. Edgard Santos (HUPES)
R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808
Serviço de Referência
Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
Serviço de Referência
Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
Serviço de Referência
Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)
Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207
Serviço de Referência
Hospital das Clínicas da UFG
Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424
Serviço de Referência
Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
Atenção Especializada
Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
Serviço de Referência
Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
Atenção Especializada
Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
Serviço de Referência
Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
Atenção Especializada
Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
Serviço de Referência
Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
Serviço de Referência
Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
Atenção Especializada
Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
Serviço de Referência
Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
Serviço de Referência
Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)
Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988
Serviço de Referência
Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
Serviço de Referência
Hospital de Clínicas de Porto Alegre (HCPA)
Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601
Serviço de Referência
Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
Serviço de Referência
Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
Serviço de Referência
Hospital de Base de São José do Rio Preto
Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798
Atenção Especializada
Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
Serviço de Referência
Hospital de Clínicas de Ribeirão Preto (HCRP-USP)
R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187
Serviço de Referência
UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
Serviço de Referência
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
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2 ensaios clínicos encontrados.
Publicações mais relevantes
Trans-Collateral Retrograde Perforation of the RVOT in Pulmonary Atresia/Ventricular Septal Defect: A Feasible Catheter-Based Approach.
Pulmonary atresia with ventricular septal defect is a rare and complex congenital heart disease. In cases where pulmonary blood flow is supplied exclusively by major aortopulmonary collateral arteries, traditional surgical interventions may be challenging or delayed, especially in resource-limited settings. This study evaluated the feasibility, safety, and outcomes of the right ventricular outflow tract perforation through the retrograde trans-collateral approach in patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries dependent pulmonary circulation. The study cohort comprised 10 patients with pulmonary atresia and ventricular septal defect who underwent attempted retrograde trans-collateral right ventricular outflow tract perforation via major aortopulmonary collateral arteries from October 2021 to February 2025, including 1 unsuccessful procedure. The median age at intervention was 4.1 years, and the median weight was 17 kg. Post-procedure, systemic oxygen saturation increased significantly (P<0.01). Follow-up imaging demonstrated substantial growth of the pulmonary arteries following retrograde trans-collateral right ventricular outflow tract recanalization, with significant improvements in both right and left pulmonary artery Z scores (P<0.01) and a significant increase in the Nakata index from a median of 49 to 111.7 mm2/m2 (P<0.01). Retrograde trans-collateral right ventricular outflow tract perforation is a feasible and safe catheter-based strategy for selected patients with pulmonary atresia with ventricular septal defect, promoting central pulmonary artery growth and serving as a bridge to future surgical repair.
Marked clinical and haemodynamic improvement with sotatercept in severe refractory pulmonary arterial hypertension associated with corrected complex congenital heart disease.
Pulmonary arterial hypertension (PAH) is a progressive disease associated with high morbidity despite combination therapy. Sotatercept, a recombinant fusion protein that acts as an activin signaling inhibitor, has shown significant clinical and haemodynamic benefit in phase 3 trials. However, evidence in PAH associated with congenital heart disease (PAH-CHD) is very limited. 37-year-old woman with repaired pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, who developed severe PAH years later. Despite treatment with tadalafil, ambrisentan, and high dose subcutaneous treprostinil, she remained at intermediate-high risk, with WHO-FC III, NT-proBNP 3773 pg/mL and a 6-min walk distance (6MWD) of 480 m. Sotatercept was initiated through an expanded access program. Over the following months, she markedly improved to WHO-FC II, NT-proBNP 230 pg/mL and 6MWD 540 m. Right heart catheterization showed a reduction in mean pulmonary artery pressure from 48 to 26 mmHg, PVR from 14 to 5.4 Wood units, and an increase in cardiac index from 1.17 to 2.91 L/min/m2. A significant and rapid improvement in haemodynamics and functional capacity was observed after starting sotatercept, in a patient with severe PAH-CHD refractory to maximal therapy. Sotatercept may represent an effective rescue therapy and a potential therapeutic option for complex and severe PAH patients.
Pulmonary vasomodulators in adults with repaired pulmonary atresia with ventricular septal defect: Single-center experience and review of literature.
Despite repair, many patients with pulmonary atresia with ventricular septal defect (PA-VSD) develop pulmonary hypertension (PH). We aimed to evaluate the effectiveness and safety of pulmonary vasomodulators in patients with PH and PA-VSD. Retrospective analysis of adults with repaired PA-VSD and PH treated with pulmonary vasomodulator therapies from 2003 to 2022. Clinical improvement was defined as a decrease in New York Heart Association Functional Class by at least one point. Adverse effects were defined by therapy discontinuation. In total, 12 adults (47 [29-67] years; 75 % female), with pre-treatment functional class of 3 (2-4) were included. Cardiac anatomy and surgical history was heterogeneous though most patients had non-confluent pulmonary arteries at birth and residual lesions post repair. Pre-treatment mean values for pulmonary artery pressure, pulmonary artery wedge pressure, and transpulmonary gradient were 41 (29-838), 17 (6-34), and 25 (16-55) mmHg, respectively. Pre-treatment cardiac index was 1.9 (1.1-3.1) L/min/m2. Phosphodiesterase-5 inhibitors were the most frequently prescribed therapy, and 50 % of patients were treated with at least 2 classes of medications. During the 3 (0.2-15.5) years of follow-up, 4 patients died, 1 was status post heart/lung transplant, and 7 survived. Adverse effects occurred in 5 patients, with 2 experiencing pulmonary edema potentially related to therapy. For patients who survived without transplant, post-treatment functional class was 2 (2-3), and 5 experienced clinical improvement. Patients who survived without transplant were treated for 7.5 (3-15.5) years. This study suggests relative safety and potential benefit of pulmonary vasomodulator therapies in adults with PH and PA-VSD.
Genetic Syndromes and Multimorbidity in Adults with Congenital Heart Disease and Heart Failure: Insights from the PATHFINDER-CHD Registry.
Background/Objectives: Progress in diagnostic and therapeutic strategies has resulted in an increasing prevalence of adults with congenital heart disease (ACHD), including those involving genetically determined syndromes. This study aimed to characterize prevalence, congenital phenotypes, heart failure (HF) stages, comorbidity burden, and current medical management of ACHD and concomitant genetically determined syndromes enrolled in a prospective HF-focused registry. Methods: The PATHFINDER-CHD Registry is a German-based (est. 2022) multicenter observational registry. This web-based platform consecutively tracks ACHD patients across the heart failure spectrum, including those with current or prior HF, as well as those at high structural or functional risk. HF stage was classified using a modified ACC/AHA scheme adapted for CHD; functional capacity was graded according to the Perloff classification. Baseline demographics, CHD anatomy, prior surgical/interventional treatment, cardiac and extracardiac comorbidities, and medication were collected from medical records. Results: Among 1987 enrolled ACHD, 107 (5.4%) had a genetic syndrome (n = 65, 60.7% women; mean age 33.5 ± 9.4 years; range 18-68). Most common syndromes were trisomy 21 (n = 49; 45.8%) and 22q11.2 deletion (n = 27; 25.2%); 31 patients (30.0) had rarer syndromes. Predominant CHD diagnoses were atrioventricular septal defect (n = 42, 39.3%), tetralogy of Fallot (n = 19, 17.8%), and pulmonary atresia with ventricular septal defect (n = 7, 6.5%). A systemic left ventricle was present in 102 (95.3%); 40 (37.4%) had primarily cyanotic CHD, and 7 (6.5%) an Eisenmenger physiology. Most patients (n = 71; 66.4%) had undergone definite surgical repair; 25 patients (23.3%) had at least one catheter intervention, including transcatheter valve implantation in 17 cases (15.9%). HF stage was mainly B (n = 30, 28.0%) or C (n = 75, 70.1%). Perloff functional class I/II was present in 97 (90.7%). Leading cardiac comorbidities included intrinsic aortopathy (n = 49, 45.8%), pulmonary arterial hypertension (n = 12, 11.2%), and arrhythmias (n = 10, 9.3%). Frequent extracardiac comorbidities were thyroid dysfunction (n = 34, 31.8%), kidney disease (n = 16, 15.0%), hyperuricemia (n = 13, 12.1%), and depression (n = 15, 14.0%). Pharmacotherapy was used in 66 patients (61.7%). Beta-blockers (n = 25, 23.4%) were common, while ACEi/ARB (n = 9, 8.4%), diuretics (n = 10, 9.3%), MRAs (n = 8, 7.5%), and SGLT2 inhibitors (n = 3; 2.8%) were infrequently prescribed; no patient received ARNI or digitalis. For targeted treatment of pulmonary arterial hypertension, phosphodiesterase-5 inhibitors (n = 7, 6.5%), endothelin receptor antagonists (n = 6, 5.6%), or prostacyclin analogues (n = 1, 0.9%) were used. As oral anticoagulants, vitamin K antagonists or direct oral anticoagulants (DOACs) were prescribed in 17 cases (15.9%). Forty-one patients (38.3%) received thyroid hormone replacement. Conclusions: Syndromic ACHD constitute a small but clinically high-risk subgroup within an HF-oriented registry, marked by complex CHD, substantial cardio-extracardiac multimorbidity (notably aortopathy, PAH, thyroid disease, renal dysfunction, depression), and low utilization of contemporary HF therapies. These data support specialized, interdisciplinary, longitudinal care pathways and prospective studies addressing outcomes and evidence-based HF management in syndromic ACHD.
Unifocalization of Major Aortopulmonary Collateral Arteries and Pulmonary Arteries-Lessons Learned Over 20 years.
Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a complex and heterogeneous form of congenital heart disease. The purpose of invited review is to provide a 20-year perspective on the lessons learned and related advancements in unifocalization of MAPCAs and pulmonary arteries. This review provides an unapologetic Stanford-centric perspective on the most salient advances in treatment of PA/VSD/MAPCAs. There have been a multitude of advances in the treatment of PA/VSD/MAPCAs over the past 20 years. Some of these advances have occurred in surgical techniques for performing unifocalization and unifocalization revision. Significant advances have also occurred in the nonsurgical aspects of care. Cumulatively and incrementally, these advances have resulted in better and better results over time. Dissemination of this knowledge and information has been an important step in a more widespread understanding of these techniques and results. Finally, the improvements in results for unifocalization of MAPCAs and pulmonary arteries have further widened the gap compared with the pulmonary artery rehabilitation approach; thus, what was once a controversy, is now largely settled. Unifocalization of MAPCAs and pulmonary arteries has undergone numerous refinements over the past 20 years. It is now clear that this is the procedure of choice for patients with single-supply MAPCAs.
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The Journal of thoracic and cardiovascular surgeryPercutaneous major aortopulmonary collateral artery banding using a covered stent in an infant.
Interactive cardiovascular and thoracic surgeryInitial Clinical Trial of a Novel Pulmonary Valved Conduit.
Seminars in thoracic and cardiovascular surgeryOutcomes After Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries: A Tailored Approach in a Developing Setting.
Frontiers in cardiovascular medicineAnesthetic and intensive care management of left main coronary artery to main pulmonary artery fistula diagnosed in postoperative case of tetralogy of fallot.
Annals of cardiac anaesthesiaA rare combination of pulmonary atresia with ventricular septal defect and congenital aortic stenosis with rheumatic mitral disease in an adolescent.
Journal of echocardiographyPulmonary atresia with a ventricular septal defect and left pulmonary artery discontinuity: a case report.
Journal of medical case reportsPerforated Acute Appendicitis in a Six-Day-Old Neonate: A Rare Differential Diagnosis of Neonatal Peritonitis.
Case reports in gastroenterologyEarly extubation is associated with improved outcomes after complete surgical repair of pulmonary atresia with ventricular septal defect and hypoplastic pulmonary arteries in pediatric patients.
Journal of cardiothoracic surgeryOutcomes Following Heterotopic Placement of Right Ventricle to Pulmonary Artery Conduits.
World journal for pediatric & congenital heart surgeryFate of the Arterial Origin of Major Aortopulmonary Collateral Arteries After Unifocalization.
World journal for pediatric & congenital heart surgeryAccuracy of Fetal Echocardiography in Defining Pulmonary Artery Anatomy and Source of Pulmonary Blood flow in Pulmonary Atresia with Ventricular Septal Defect (PA/VSD).
Pediatric cardiologyBilateral coronary ostial atresia in a patient with pulmonary atresia and ventricular septal defect.
Cardiology in the youngPrenatal diagnosis of major aortopulmonary collateral arteries (MAPCA) in fetuses with pulmonary atresia with ventricular septal defect and agenesis of ductus arteriosus.
The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal ObstetriciansAnterograde blood flow associated with modified Blalock-Taussig shunt does not modify pulmonary artery growth compared with modified Blalock-Taussig shunt alone.
Archives of cardiovascular diseasesPredictors of death after receiving a modified Blalock-Taussig shunt in cyanotic heart children: A competing risk analysis.
PloS oneOutcome after surgery for pulmonary atresia with ventricular septal defect, a long-term follow-up study.
Acta paediatrica (Oslo, Norway : 1992)A loss-of-function mutation p.T256M in NDRG4 is implicated in the pathogenesis of pulmonary atresia with ventricular septal defect (PA/VSD) and tetralogy of Fallot (TOF).
FEBS open bio[Surgical Strategy for Pulmonary Atresia with Ventricular Septal Defect with Major Aortopulmonary Collateral Artery].
Kyobu geka. The Japanese journal of thoracic surgeryReversible Hypoxia-Induced Thrombocytopenia in an Infant of Pulmonary Atresia with Ventricular Septal Defect.
International medical case reports journalSegmental Pulmonary Hypertension in Children with Congenital Heart Disease.
Medicina (Kaunas, Lithuania)Surgical Strategies and Results for Repair of Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals: Experience of a Single Tertiary Center.
Brazilian journal of cardiovascular surgeryMyocardial ischemia from previously placed autologous pericardial roll conduits.
Asian cardiovascular & thoracic annalsPulmonary atresia with ventricular septal defect and tetralogy of Fallot: transannular path augmentation versus systemic to pulmonary artery shunt for first-stage palliation.
Cardiology in the youngAssociation of Preoperative Mixed Venous Oxygen Saturation with Postoperative Segmental Pulmonary Hypertension in Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals.
Pediatric cardiologyComparison of computed tomography angiography versus cardiac catheterization for preoperative evaluation of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect.
Annals of pediatric cardiologyTetralogy of Fallot With Pulmonary Atresia: Anatomy, Physiology, Imaging, and Perioperative Management.
Seminars in cardiothoracic and vascular anesthesiaTetralogy of Fallot or Pulmonary Atresia with Ventricular Septal Defect after the Age of 40 Years: A Single Center Study.
Journal of clinical medicineSurgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants.
Pediatric cardiologyThe prevalence of genetic diagnoses in fetuses with severe congenital heart defects.
Genetics in medicine : official journal of the American College of Medical GeneticsMidline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
Journal of thoracic diseaseCurrent era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand.
Scientific reportsEvaluation of personalized right ventricle to pulmonary artery conduits using in silico design and computational analysis of flow.
JTCVS open22q11.2 deletion syndrome is associated with increased mortality in adults with tetralogy of Fallot and pulmonary atresia with ventricular septal defect.
International journal of cardiologyIdentification of rare variants in novel candidate genes in pulmonary atresia patients by next generation sequencing.
Computational and structural biotechnology journalNovel JAG1 Deletion Variant in Patient with Atypical Alagille Syndrome.
International journal of molecular sciencesImaging of Pulmonary Atresia With Ventricular Septal Defect.
Journal of computer assisted tomographyPrediction of pulmonary pressure after Glenn shunts by computed tomography-based machine learning models.
European radiologyPulmonary Atresia With Ventriculocoronary Arterial Connections and a Large Conoventricular Septal Defect.
JACC. Case reportsPerfusion Methods and Modifications to the Cardiopulmonary Bypass Circuit for Midline Unifocalization Procedures.
The journal of extra-corporeal technologyLecompte Procedure for Relief of Severe Airway Compression in Children With Congenital Heart Disease.
World journal for pediatric & congenital heart surgeryMidline one-stage complete unifocalization early outcomes from a single center.
Anatolian journal of cardiologyFetal pulmonary atresia with ventricular septal defect: Features, associations, and outcome in fetuses with different pulmonary circulation supply types.
Prenatal diagnosis[Joint Cardiovascular Surgeries of Two Hospitals in Shizuoka for Adult Congenital Heart Disease (ACHD) Patients].
Kyobu geka. The Japanese journal of thoracic surgerySurgical results of unifocalization revision.
The Journal of thoracic and cardiovascular surgeryMeasurement of Residual Collateral Flow in Pulmonary Atresia With Major Aortopulmonary Collaterals.
The Annals of thoracic surgeryPulmonary Atresia with Ventricular Septal Defect: Rare Presentation with Coronary-to-Pulmonary Artery Collaterals from Both Right and Left Coronaries.
World journal for pediatric & congenital heart surgeryComplete repair with unifocalization of the only unilateral lung.
General thoracic and cardiovascular surgeryRare Copy Number Variations Might Not be Involved in the Molecular Pathogenesis of PA-IVS in an Unselected Chinese Cohort.
Pediatric cardiologyA Review of the Management of Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.
The Annals of thoracic surgeryCatheterization Performed in the Early Postoperative Period After Congenital Heart Surgery in Children.
Pediatric cardiologyUtilisation of a three-dimensional printed model for the management of coronary-pulmonary artery fistula from left main coronary artery.
Cardiology in the youngIdentification of Rare Copy Number Variants Associated With Pulmonary Atresia With Ventricular Septal Defect.
Frontiers in geneticsPrenatal Diagnosis and Postnatal Outcome of Fetuses with Pulmonary Atresia and Ventricular Septal Defect.
Ultraschall in der Medizin (Stuttgart, Germany : 1980)Analysis of achieving an "ideal" outcome following midline unifocalization.
Asian cardiovascular & thoracic annalsTetralogy of fallot with pulmonary atresia and aorto-pulmonary window: Or is it truncus arteriosus?
Journal of cardiovascular computed tomographyPatent ductus arteriosus: The rare cause of left bronchial obstruction in neonate with pulmonary atresia.
Journal of cardiovascular computed tomographyEarly Childhood Inpatient Costs of Critical Congenital Heart Disease.
The Journal of pediatricsOutcome of neonatal palliative procedure for pulmonary atresia with ventricular septal defect or tetralogy of Fallot with severe pulmonary stenosis: experience in a single tertiary center.
Korean journal of pediatricsLive-Born Major Congenital Heart Disease in Denmark: Incidence, Detection Rate, and Termination of Pregnancy Rate From 1996 to 2013.
JAMA cardiologyTei Index Is the Best Echocardiographic Parameter for Assessing Right Ventricle Function in Patients With Unrepaired Congenital Heart Diseases With Outflow Tract Obstruction.
Frontiers in pediatricsPulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: collateral vessel disease burden and unifocalisation strategies.
Cardiology in the youngC3, C4, and C5 Keeps the Diaphragm Alive… and Reduces Morbidity and Hospital Costs.
Seminars in thoracic and cardiovascular surgeryAortopulmonary window with pumonary atresia with ventricular septal defect with D-transposition of great arteries: extremely rare anomaly.
BMJ case reportsSurgical strategies for pulmonary atresia with ventricular septal defect associated with major aortopulmonary collateral arteries.
General thoracic and cardiovascular surgerySurgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals.
The Journal of thoracic and cardiovascular surgeryCollateral benefit in pulmonary atresia with ventricular septal defect?
The Journal of thoracic and cardiovascular surgeryCatheter, MRI and CT Imaging in Newborns with Pulmonary Atresia with Ventricular Septal Defect and Aortopulmonary Collaterals: Quantifying the Risks of Radiation Dose and Anaesthetic Time.
Pediatric cardiologyRepair of Ductus or Hemi-Truncus to One Lung and Major Aortopulmonary Collaterals to the Other Lung.
The Annals of thoracic surgeryImpact of Phrenic Nerve Palsy and Need for Diaphragm Plication Following Surgery for Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals.
Seminars in thoracic and cardiovascular surgeryAnesthetic Management During Surgery for Tetralogy of Fallot With Pulmonary Atresia and Major Aortopulmonary Collateral Arteries.
World journal for pediatric & congenital heart surgeryAn analysis of patients requiring unifocalization revision following midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryAnatomy of the retro-oesophageal major aortopulmonary collateral arteries in patients with pulmonary atresia with ventricular septal defect: results from preoperative CTA.
European radiologyStenting the complex patent ductus arteriosus in tetralogy of Fallot with pulmonary atresia: challenges and outcomes.
Future cardiologyRole of computed tomography angiography in the differentiation of feline truncus arteriosus communis from pulmonary atresia with ventricular septal defect.
Journal of veterinary cardiology : the official journal of the European Society of Veterinary CardiologyOne-stage definitive repair of complete atrioventricular septal defect and pulmonary atresia with major aortopulmonary collateral arteries.
Interactive cardiovascular and thoracic surgeryCoronary-pulmonary arterial fistula in a neonate with pulmonary atresia-ventricular septal defect and single coronary artery.
Echocardiography (Mount Kisco, N.Y.)Determinants of Adverse Outcomes After Systemic-To-Pulmonary Shunts in Biventricular Circulation.
The Annals of thoracic surgeryImpact of Pulmonary Flow Study Pressure on Outcomes After One-Stage Unifocalization.
The Annals of thoracic surgeryPrenatal diagnosis of pulmonary atresia with ventricular septal defect.
Journal of medical ultrasonics (2001)The value of dual-source multidetector-row computed tomography in determining pulmonary blood supply in patients with pulmonary atresia with ventricular septal defect.
Folia morphologicaThe unexpected in grown-up congenital heart disease: Takotsubo syndrome.
The Journal of thoracic and cardiovascular surgeryOutcomes of palliative right ventricle to pulmonary artery connection for pulmonary atresia with ventricular septal defect.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryDental management of pediatric patients affected by pulmonary atresia with ventricular septal defect: A scoping review.
Medicina oral, patologia oral y cirugia bucalMajor Aortopulmonary Collateral Arteries With Anatomy Other Than Pulmonary Atresia/Ventricular Septal Defect.
The Annals of thoracic surgeryBilateral Coronary-Pulmonary Artery Fistulas in Pulmonary Atresia With Ventricular Septal Defect.
International heart journalSignificant survival advantage of high pulmonary vein index and the presence of native pulmonary artery in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: results from preoperative computed tomography angiography.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryIntraoperative Stenting of Pulmonary Artery Stenosis in Children With Congenital Heart Disease.
The Annals of thoracic surgeryImportance of multidisciplinary management for pulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries and completely absent central pulmonary arteries.
General thoracic and cardiovascular surgeryReconstruction of the pulmonary posterior wall using in situ autologous tissue for the treatment of pulmonary atresia with ventricular septal defect.
Journal of cardiothoracic surgeryThree-dimensional printing technology supports surgery planning in patients with complex congenital heart defects.
Kardiologia polskaRight Ventricle-to-Pulmonary Artery Shunt in Pulmonary Atresia with a Ventricular Septal Defect: A Word of Caution.
Pediatric cardiologyBranch pulmonary artery peel operation in a patient without a native intrapericardial pulmonary artery.
Journal of cardiac surgeryRight Ventricular Outflow Tract Reconstruction With a Polytetrafluoroethylene Monocusp Valve: A 20-Year Experience.
Seminars in thoracic and cardiovascular surgeryCoronary-Pulmonary Artery Fistulas: A Systematic Review.
Journal of thoracic imagingPulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals Associated with Left Pulmonary Artery Interruption.
The Korean journal of thoracic and cardiovascular surgeryReverse Szabo technique for stenting a single major aorto-pulmonary collateral vessel in pulmonary atresia with ventricular septal defect.
Translational pediatricsPulmonary flow study predicts survival in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
The Journal of thoracic and cardiovascular surgeryAdvances in molecular genetics for pulmonary atresia.
Cardiology in the youngFirst Successful Clinical Application of the In Vivo Tissue-Engineered Autologous Vascular Graft.
The Annals of thoracic surgeryBanding of Nonrestrictive Large Hypertensive Aortopulmonary Collaterals in Patients With Ventricular Septal Defect and Pulmonary Atresia Presenting in Suboptimal Condition.
World journal for pediatric & congenital heart surgeryManagement of a dissection of matrix P right ventricular-to-pulmonary artery conduit by implanting two pre-stents and a melody valve.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & InterventionsFungal infections in children in the early postoperative period after cardiac surgery for congenital heart disease: a single-centre experience.
Interactive cardiovascular and thoracic surgeryPolytetrafluoroethylene Bicuspid Pulmonary Valve Replacement: A 5-Year Experience in 119 Patients With Congenital Heart Disease.
The Annals of thoracic surgeryDouble Aortic Arch Associated with Pulmonary Atresia with Ventricular Septal Defect.
Brazilian journal of cardiovascular surgeryPulmonary Valve Replacement With Balloon-Expandable Prosthesis Under Direct Vision: A Novel Therapeutic Approach.
The Annals of thoracic surgeryOutcomes of redo pulmonary valve replacement for bioprosthetic pulmonary valve failure in 61 patients with congenital heart disease.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryTetralogy of Fallot With Left Pulmonary Artery Discontinuity and Totally Anomalous Pulmonary Venous Drainage to the Azygos Vein.
World journal for pediatric & congenital heart surgeryPulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: primary repair.
Multimedia manual of cardiothoracic surgery : MMCTSAn unusual case of left bronchial compression caused by a large patent arterial duct in a child with pulmonary atresia with ventricular septal defect.
European heart journal. Cardiovascular ImagingComplete Preoperative Evaluation of Pulmonary Atresia with Ventricular Septal Defect with Multi-Detector Computed Tomography.
PloS oneChallenging Lead Implantation Guided by Voltage Map.
Revista espanola de cardiologia (English ed.)Long-term outcome after treatment of pulmonary atresia with ventricular septal defect: nationwide study of 109 patients born in 1970-2007.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryAn Overview of Pulmonary Atresia and Major Aortopulmonary Collateral Arteries.
World journal for pediatric & congenital heart surgeryReoperative double ventricular outflow tract reconstruction in grown-up congenital heart disease patients with conotruncal anomalies.
General thoracic and cardiovascular surgeryPulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: single-stage complete unifocalization.
Multimedia manual of cardiothoracic surgery : MMCTS[Initial experience with the new Pul-Stent in treating postoperative branch pulmonary artery stenosis].
Zhonghua er ke za zhi = Chinese journal of pediatricsResults of Primary Repair Versus Shunt Palliation in Ductal Dependent Infants With Pulmonary Atresia and Ventricular Septal Defect.
The Annals of thoracic surgerySurgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries.
The Annals of thoracic surgeryAccuracy of Fetal Echocardiography in the Differential Diagnosis between Truncus Arteriosus and Pulmonary Atresia with Ventricular Septal Defect.
Fetal diagnosis and therapyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Atresia pulmonar com defeito do septo ventricular.
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Atresia pulmonar com defeito do septo ventricular
Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.
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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.
- Trans-Collateral Retrograde Perforation of the RVOT in Pulmonary Atresia/Ventricular Septal Defect: A Feasible Catheter-Based Approach.
- Marked clinical and haemodynamic improvement with sotatercept in severe refractory pulmonary arterial hypertension associated with corrected complex congenital heart disease.
- Pulmonary vasomodulators in adults with repaired pulmonary atresia with ventricular septal defect: Single-center experience and review of literature.
- Genetic Syndromes and Multimorbidity in Adults with Congenital Heart Disease and Heart Failure: Insights from the PATHFINDER-CHD Registry.
- Unifocalization of Major Aortopulmonary Collateral Arteries and Pulmonary Arteries-Lessons Learned Over 20 years.
- Outcomes of Staged Repair Following Systemic-to-Pulmonary Artery Shunt in Pulmonary Atresia with Ventricular Septal Defect.
- Single Ventricle: Radiology Primer for Preprocedural Evaluation.
- Case Report: De novo USP9X missense mutation in a male fetus with pulmonary atresia and ventricular septal defect: expanding the genotype-phenotype spectrum of USP9X-related disorders.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:1207(Orphanet)
- OMIM OMIM:178370(OMIM)
- MONDO:0008343(MONDO)
- GARD:4588(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q2869826(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
