Raras
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Atresia da válvula aórtica
ORPHA:95448CID-10 · Q23.0CID-11 · LA8A.23DOENÇA RARA

Ventrículo único é uma malformação cardíaca congénita com uma incidência inferior a 10% de todas as cardiopatias congénitas e 5 em cada 100 000 nascimentos, caracterizada pela presença de um único ventrículo e duas aurículas. A comunicação entre as aurículas e os ventrículos faz-se por uma única válvula - canal atrioventricular. Não faz parte do síndrome de hipoplasia do ventrículo esquerdo.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

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Atresia da válvula aórtica é uma malformação congênita grave onde a válvula aórtica não se desenvolve adequadamente, impedindo o fluxo sanguíneo do ventrículo esquerdo para a aorta. Isso resulta em hipoplasia do ventrículo esquerdo e dependência do canal arterial para a circulação sistêmica.

Publicações científicas
300 artigos
Último publicado: 2026 Feb
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SUS: Cobertura mínimaScore: 35%
Centros em: PA, PE, BA, CE, PB +10CID-10: Q23.0
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Anos de pesquisa3
Total histórico300PubMed
Últimos 10 anos200publicações
Pico202592 papers
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2023Hoje · 2026🧪 2010Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

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Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Atresia da válvula aórtica

Centros de Referência SUS

24 centros habilitados pelo SUS para Atresia da válvula aórtica

Centros para Atresia da válvula aórtica

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

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital Infantil Albert Sabin

R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876

Serviço de Referência

Rota
Anomalias CongênitasDeficiência Intelectual

Hospital de Apoio de Brasília (HAB)

AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

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

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

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

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital Universitário da UFJF

R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442

Atenção Especializada

Rota
Anomalias Congênitas

Hospital das Clínicas da UFMG

Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Julio Müller (HUJM)

R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092

Atenção Especializada

Rota
Anomalias Congênitas

Hospital Universitário João de Barros Barreto

R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Lauro Wanderley (HULW)

R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470

Atenção Especializada

Rota
Anomalias Congênitas

Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)

R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Pequeno Príncipe

R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805

Serviço de Referência

Rota
Anomalias CongênitasDeficiência Intelectual

Hospital Universitário Regional de Maringá (HUM)

Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108

Atenção Especializada

Rota
Anomalias Congênitas

Hospital de Clínicas da UFPR

R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Pedro Ernesto (HUPE-UERJ)

Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

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

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital São Lucas da PUCRS

Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

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

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário da UFSC (HU-UFSC)

R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital das Clínicas da FMUSP

R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

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

Rota
Anomalias Congênitas

Hospital de Clínicas da UNICAMP

R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

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

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

UNIFESP / Hospital São Paulo

R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo
Sobre os centros SUS: Estes centros são habilitados pelo Ministério da Saúde como Serviços de Referência em Doenças Raras ou Serviços de Atenção Especializada. O atendimento é pelo SUS, com encaminhamento da rede de atenção básica.

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

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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

Molecular Calcification Imaging and Ascending Aortic Disease in Patients With a Bicuspid Aortic Valve.

JAMA network open2026 Feb 02

Selection of patients with a bicuspid aortic valve and aortopathy for prophylactic aortic surgery remains challenging. In thoracic aortopathy, aortic medial elastin fiber fragmentation initially leads to microcalcification but later declines with progressive loss of elastin content and reduced structural integrity. To determine whether aortic microcalcification detected using fluorine F 18-labeled [18F]-sodium fluoride positron emission tomography (PET) is associated with future aortic diameter expansion. This prospective longitudinal cohort study was conducted in tertiary care centers across Scotland from April 4, 2019, to September 15, 2023. Participants included patients with a bicuspid aortic valve. Data analysis was performed from May 21, 2024, to March 4, 2025. Hybrid [18F]-sodium fluoride PET and computed tomography. Baseline ascending aortic [18F]-sodium fluoride uptake was measured as mean tissue to background ratio. The primary outcome was ascending aortic diameter expansion during 24 months on cardiac magnetic resonance imaging (MRI). Seventy-six patients with a bicuspid aortic valve (mean [SD] age, 52.6 [7.5] years; 57 [75.0%] male) underwent baseline [18F]-sodium fluoride PET and MRI. Fifty-six patients underwent follow-up MRI after a median of 723 (IQR, 515-787) days. There was an inverse correlation between baseline ascending aortic [18F]-sodium fluoride uptake and annual change in diameter (Pearson r = -0.37; P = .005), which remained after adjustment for confounders in multivariable regression analysis. Ascending aortic [18F]-sodium fluoride was not correlated with baseline diameter (Pearson r = 0.08; P = .50) but was moderately correlated with baseline ascending aortic stiffness index (Pearson r = 0.38; P < .001). In this cohort study of patients with a bicuspid aortic valve, the most rapid aortic growth was seen in those with low [18F]-sodium fluoride ascending aortic uptake, indicating reduced aortic wall integrity. High ascending aortic [18F]-sodium fluoride uptake was associated with a stiffer and slow-growing ascending aortic phenotype. These findings suggest that [18F]-sodium fluoride PET imaging represents a promising new noninvasive approach to identify a microcalcified disease phenotype in thoracic aortopathy among patients with a bicuspid aortic valve.

#2

Congenital Coarctation of the Aorta in a Patient With ROBO4 c.695C>T (p.Thr232Met) Germline Variant.

Annals of internal medicine. Clinical cases2026 Jan

Roundabout guidance receptor 4 (ROBO4) germline variants [including c.695C>T (p.Thr232Met)] have been linked with bicuspid aortic valve and thoracic aortic aneurysm. However, the precise nature of ROBO4's role in aortopathy is yet to be fully defined. We studied a 64-year-old woman with a history of surgically corrected congenital aortic coarctation in childhood, bicuspid aortic valve, and mild mitral regurgitation. She had genome sequencing, which revealed a heterozygous germline ROBO4 variant [c.695C>T (p.Thr232Met)]. Our findings suggest that the spectrum of aortic abnormalities that are associated with ROBO4 germline variants may include congenital coarctation of the aorta.

#3

Evolution of the Aorta After Valve Replacement in Bicuspid Aortic Valves: Results at Mid-Term Follow-Up.

Heart, lung &amp; circulation2026 Mar

Bicuspid aortic valve (BAV) is a common congenital heart defect often associated with ascending aorta dilation. Current guidelines suggest surgical intervention for diameters ≥45 mm in conjunction with surgical aortic valve replacement (SAVR). However, the optimal management of ascending aortas measuring 40-44 mm remains under debate. This study aims to evaluate the evolution of aortic diameter in patients with BAV and ascending aorta diameters between 40 and 44 mm undergoing aortic valve replacement, assessing whether a conservative approach is justified. We conducted a retrospective observational study, analysing 88 patients with BAV who underwent SAVR between January 2012 and December 2018. Patients were divided into two groups based on preoperative ascending aorta diameters: <40 mm (Group 1, n=15) and the ≥40 and <45 mm (Group 2, n=11). Follow-up of at least 5 years included aortic dilation rates, survival, and reoperation rates. No significant differences were observed in aortic dilation between the two groups during follow-up, with overall annual dilation rates of 0.2±0.07 mm/year. Survival rates were 86.3% for Group 1 and 81.9% for Group 2, with no reoperations required in either group. Patients with BAV and ascending aorta diameters of 40-44 mm do not require prophylactic aortic replacement during AVR, provided they undergo regular follow-up. These findings support current guidelines advocating conservative management for ascending aorta diameters in this range. However, in younger patients or those nearing the 45 mm threshold, surgical replacement may still be considered to pre-empt future complications.

#4

[Analysis of optimizing indications for fetal pulmonary valvuloplasty based on a multi-parameter scoring system].

Zhonghua er ke za zhi = Chinese journal of pediatrics2026 Feb 02

Objective: To establish a multi-parametric scoring system for predicting postnatal circulatory outcomes in fetuses with critical pulmonary stenosis (CPS) and pulmonary atresia (PA) with intact ventricular septum (IVS), in order to optimize the indications for fetal pulmonary valvuloplasty (FPV). Methods: This was a retrospective cohort study. A total of 96 fetuses diagnosed with CPS-IVS or PA-IVS at Women and Children's Hospital, Qingdao University between July 2018 and June 2021 were included. Fetuses who did not undergo FPV constituted the modeling cohort, while those who underwent FPV constituted the validation cohort. Fetuses were classified into biventricular and non-biventricular circulation groups based on postnatal circulatory outcomes. Prenatal fetal echocardiographic parameters were compared between groups using independent-samples t test, Mann-Whitney U test, χ2 test or Fisher exact test. Based on inter-group differences, parameters associated with postnatal circulatory outcomes were screened in the modeling cohort. For continuous variables, optimal cut-off values were determined using receiver operating characteristic (ROC) curve and the Youden index and then dichotomized. Right ventricular endocardial fibroelastosis (EFE) was graded as 0, 1 or 2 according to severity. OR of each parameter were calculated, and a multiparametric scoring system was constructed. ROC curve was used to evaluate its predictive performance. Post-FPV outcome improvement was assessed in the validation cohort. Results: Among the 96 fetuses, 78 were assigned to the modeling cohort and 18 to the validation cohort. Postnatally, biventricular circulation was achieved in 56 cases (72%) in the modeling cohort and 13 cases in the validation cohort. In the modeling cohort, the right ventricular long-axis/left ventricular long-axis (RV/LV), tricuspid annulus diameter/mitral annulus diameter (TV/MV) and pulmonary valve annulus/aortic valve annulus (PV/AV) in the non-biventricular group were lower than that in the biventricular group (all P<0.05). The optimal cut-off values for predicting postnatal circulatory outcomes were 0.65, 0.82 and 0.78, respectively. There was statistically significant difference in the distribution of circulatory outcomes between fetuses with grade 2 EFE and those with no or grade 1 EFE (χ2=21.28, P<0.001). Therefore, right ventricular EFE was dichotomized in the model as "none or grade 1" versus "grade 2". Each parameter was assigned a corresponding weight based on its OR value, and an integer score was obtained to construct the scoring system: grade 2 right ventricular EFE was assigned 5 points, RV/LV≤0.65 was assigned 2 points, TV/MV≤0.82 was assigned 2 points, and PV/AV≤0.78 was assigned 1 point, with a maximum total score of 10 points. At cut-off scores of 3, 4, and 5 points, the areas under the curve were 0.88, 0.91, and 0.88, respectively, with the highest specificity (0.95, 95% CI 0.84-0.99) observed at a score threshold of 5 points. In the validation cohort, 15 fetuses had scores ≥5 points, among whom 10 fetuses achieved biventricular circulation after birth, whereas in the modeling cohort, only 1 of 18 fetuses achieved biventricular circulation after birth. Conclusions: A multiparametric scoring system based on prenatal fetal echocardiographic parameters can effectively predict non-biventricular postnatal outcomes in fetuses with CPS-IVS and PA-IVS. By identifying high-risk patients, the indications for FPV can be optimized. 目的: 建立预测胎儿危重型肺动脉狭窄(CPS)和肺动脉闭锁(PA)伴完整室间隔(IVS)出生后循环结局的多参数评分系统,以优化胎儿肺动脉球囊扩张术(FPV)手术指征。 方法: 回顾性队列研究。纳入2018年7月至2021年6月青岛大学附属妇女儿童医院诊断为CPS-IVS和PA-IVS的96例胎儿为研究对象。未接受FPV的胎儿作为建模组,接受FPV的胎儿为验证组。按生后是否实现双心室循环分为双心室组和非双心室组,比较出生前胎儿超声心动图指标,组间比较采用独立样本t检验、Mann-Whitney U检验、χ2检验或Fisher 确切概率法。依据组间差异筛选建模组中与生后循环结局相关的指标,其中计量资料通过受试者工作特征曲线及Youden指数确定最佳截断值并二分类化,右心室心内膜纤维弹力增生(EFE)按程度分为0、1、2级。计算各参数OR并构建多参数评分系统。应用受试者工作特征曲线评价其预测能力。观察验证组FPV后的结局改善情况。 结果: 96例胎儿中建模组78例、验证组18例,分别56例(72%)、13例生后实现双心室循环。建模组中非双心室患儿右心室长轴径/左心室长轴径(RV/LV)、三尖瓣环直径/二尖瓣环直径(TV/MV)、肺动脉瓣环直径/主动脉瓣环直径(PV/AV)均低于双心室患儿(均P<0.05),其预测出生后循环结局的最佳截断值分别为0.65、0.82、0.78。右心室2级EFE与无或1级EFE胎儿在循环结局的分布上差异有统计学意义(χ2=21.28,P<0.001),因此在模型中将右心室EFE二分类为“无或1级”与“2级”。各项参数按其OR值分配相应权重并取整得分后构建评分系统:右心室EFE为2级记5分,RV/LV≤0.65记2分,TV/MV≤0.82记2分,PV/AV≤0.78记1分,总分最高为10分。评分系统在3、4和5分的界值时,曲线下面积分别为0.88、0.91和0.88,评分界值为5分时特异度最高(0.95,95%CI 0.84~0.99)。验证组中,15例评分≥5分,其中10例生后实现双心室循环,而建模组18例中仅1例实现双心室循环。 结论: 基于胎儿期超声心动图多参数构建的评分系统可有效预测CPS-IVS和PA-IVS胎儿出生后的非双心室结局。通过识别高风险者,可优化FPV适应证。.

#5

Outcomes of Heart Transplantation in Single-Ventricle Physiology: A Retrospective Single-Center Experience with Emphasis on Surgical Complexity.

Journal of clinical medicine2026 Feb 24

Background: Patients with single-ventricle physiology represent a high-risk group for heart transplantation. Due to complex anatomical and physiological challenges, including multiple prior sternotomies, pulmonary artery abnormalities, and systemic consequences of altered circulation, they represent both a surgical and a clinical challenge. We aimed to analyze perioperative challenges, as well as early and long-term complications, in this specific group of patients. Methods: We performed a retrospective data analysis of a high-volume heart transplant center, focusing on patients with single-ventricle physiology who were scheduled for heart transplantation due to end-stage heart failure. We retrospectively analyzed the period from the beginning of the transplant program in November 1985 to the end of November 2024. Results: Among 1553 transplanted patients (adults and children), 29 were transplanted due to congenital heart disease (congenital valvular disease not included). In this group, nine patients were transplanted due to end-stage heart failure in the course of single-ventricle physiology. Age at transplantation ranged from 7 to 31 years (median, 17 years), and body weight ranged from 15 to 69 kg (median, 47.9 kg). All nine patients referred for heart transplantation presented with single-ventricle physiology. Their underlying congenital heart defects were heterogeneous and included hypoplastic left heart syndrome (HLHS), double-outlet left ventricle (DOLV), transposition of the great arteries (TGA) with associated ventricular septal defects (VSDs), atrial septal defects (ASDs), valvular abnormalities such as tricuspid and or pulmonary valve atresia or stenosis, systemic or atrioventricular valve regurgitation, and vascular abnormalities, including right-sided aortic arch, aortic coarctation, and pulmonary artery hypoplasia, stenosis, or occlusion, as well as associated pulmonary vascular abnormalities such as left pulmonary artery stenosis and MAPCAs. All patients had previously undergone staged palliative procedures, including Norwood, Hemi-Fontan, Fontan, bidirectional Glenn, modified Blalock-Taussig shunts, Bjork-Fontan, or pulmonary artery banding, often with repeated interventions such as balloon angioplasty, stent placement, or MAPCA closure. Extracardiac comorbidities were common and included coagulopathies, protein-losing enteropathy, hepatic dysfunction, and chronic venous insufficiency. Preoperative functional status was markedly impaired in all patients (NYHA III-IV, INTERMACS 3-4), with severely reduced exercise capacity and thrombotic events in several individuals. Perioperative transplant surgical strategies included femoral cannulation in four cases and standard aortic and caval cannulation in five cases. Pulmonary artery reconstruction was required in all patients. Extended donor pulmonary arteries were applied in eight cases, while a bifurcated Dacron prosthesis was utilized in one patient. Perioperative mortality was 33%, with three deaths attributed to bleeding and hemodynamic instability, while overall mortality was 44% including one late death unrelated to transplantation. Protein-losing enteropathy, although persistent in the immediate postoperative period, resolved in all surviving patients, underscoring the transformative impact of transplantation. Conclusions: These findings emphasize the importance of individualized surgical planning, extended donor pulmonary artery harvesting, and careful preoperative coordination. Heart transplantation remains a viable and life-extending option for selected single-ventricle patients, despite the significant technical and clinical challenges involved.

Publicações recentes

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📚 EuropePMC2 artigos no totalmostrando 200

2026

Outcomes of Heart Transplantation in Single-Ventricle Physiology: A Retrospective Single-Center Experience with Emphasis on Surgical Complexity.

Journal of clinical medicine
2026

Prenatal Diagnosis of Isolated Congenitally Corrected Transposition of the Great Arteries.

Cureus
2026

Molecular Calcification Imaging and Ascending Aortic Disease in Patients With a Bicuspid Aortic Valve.

JAMA network open
2026

The pivotal role of echocardiography in structural heart interventions: from planning to success.

Therapeutic advances in cardiovascular disease
2026

Over 40 Years of Experience With Aortic Valve Surgery in the Paediatric Population-What Is the Current Best Strategy?

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2026

Congenital Coarctation of the Aorta in a Patient With ROBO4 c.695C>T (p.Thr232Met) Germline Variant.

Annals of internal medicine. Clinical cases
2026

Evolution of the Aorta After Valve Replacement in Bicuspid Aortic Valves: Results at Mid-Term Follow-Up.

Heart, lung &amp; circulation
2026

[Analysis of optimizing indications for fetal pulmonary valvuloplasty based on a multi-parameter scoring system].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2026

Robotic aortic valve replacement with a mechanical prosthesis: procedural steps.

Multimedia manual of cardiothoracic surgery : MMCTS
2025

Prevalence of cardiac abnormalities in patients with idiopathic scoliosis: a systematic review and meta-analysis.

Spine deformity
2025

Prevalence and Spectrum of Congenital Heart Disease in Individuals With Distal Chromosome 22q11.22-23 Deletions.

Clinical genetics
2025

Ambulatory General Anesthesia for Dental Treatment in a Patient With Williams Syndrome and Supravalvular Aortic Restenosis: A Case Report.

Anesthesia progress
2025

A Rare Case of Giant Cell Arteritis Affecting Two Giants: Pulmonary and Root of Aorta Aneurysm.

Clinical case reports
2026

Bicuspid and Unicuspid Aortic Valves: Development, Genetics, and Lifelong Management.

The American journal of cardiology
2025

Structure and Function of the Extracellular Matrix in Normal and Pathological Conditions: Looking at the Bicuspid Aortic Valve.

International journal of molecular sciences
2026

Patient-Informed Fluid-Structure Interaction Simulations of Bicuspid Aortic Valve in Young Adults Reveal Regionalized Differences in Mechanical Stress.

Annals of biomedical engineering
2026

Development of a Polymeric TAVR Device Tailored to Bicuspid Aortic Valve Patients Using In Silico Design Optimization and Evaluation.

Annals of biomedical engineering
2026

Bicuspidization of the unicuspid aortic valve using the pericardial advancement technique.

Asian cardiovascular &amp; thoracic annals
2025

Transcatheter treatment of bicuspid aortic valve stenosis.

EuroIntervention : journal of EuroPCR in collaboration with the Working Group on Interventional Cardiology of the European Society of Cardiology
2026

Study on the Correlation Between Ventricular Function Evaluated by Z-Score of Atrioventricular Annular Plane Systolic Excursion and Pulmonary Artery Abnormality and Retrograde Ductus Arteriosus Flow in Fetuses With Ebstein Anomaly.

Journal of clinical ultrasound : JCU
2025

Complex Reimplantation of Aneurysmal Left Main Coronary Artery During Reoperative Aortic Root Replacement.

Cureus
2025

Shear Stress-Induced Microstructure Remodeling of Human Bicuspid Aortopathy Revealed by Nonlinear Optical Microscopy.

Analytical chemistry
2025

Machine learning and multi-omics integration reveal TRPV2 as a central regulator in bicuspid aortic valve calcification.

Biochemical and biophysical research communications
2025

Beyond the usual spectrum: Atrial septal defect in a Marfan syndrome patient with severe aortic pathologies.

Journal of family medicine and primary care
2025

Successful Cardiac Resynchronization Therapy Defibrillator Implantation via the Vein of Marshall in a Patient with Coronary Sinus Ostial Occlusion.

The Journal of innovations in cardiac rhythm management
2025

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

Pediatric cardiology
2025

A novel NOTCH1 nonsense variant in a bicuspid aortic valve family with intrafamilial clinical heterogeneity.

BMC cardiovascular disorders
2026

An Expanding Perspective: Extreme Aortic Dilation in a Patient With Single Ventricle Palliation.

World journal for pediatric &amp; congenital heart surgery
2025

Cardiac manifestations in children with osteogenesis imperfecta: A single-center observational study.

International journal of cardiology
2025

Multivalvular infective endocarditis due to streptococcus pluranimalium in a young patient with bicuspid aortic valve.

Oxford medical case reports
2025

Balloon vs. self-expanding valves for transcatheter aortic valve implantation in bicuspid aortic stenosis: a meta-analysis.

Journal of cardiovascular medicine (Hagerstown, Md.)
2026

Aortic valve morphology rather than aortic valve function, aortic dilatation, and age interferes with ascending aortic structural and biomechanical properties.

Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology
2026

Aortic regurgitation as a cause of sudden cardiac death with aortic and left ventricular remodelling - the role of the bicuspid valve.

Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology
2026

FOXO1-driven endothelial senescence in bicuspid aortic valve-associated thoracic aortic aneurysm.

Biochimica et biophysica acta. Molecular basis of disease
2025

Aortic Valve Function and Aortic Dilatation Associated with Bicuspid Aortic Valve in Early Childhood.

Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography
2025

Thinking Outside the Box: Case Report of a Rare Quadricuspid Aortic Valve as an Underrecognized Cause of Heart Failure and Atrial Fibrillation.

Journal of medical cases
2025

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

Circulation. Cardiovascular interventions
2025

Long-term clinical outcomes in adult congenital aortic stenosis: results from a national study.

Open heart
2025

Histological examination of the pulmonary artery and aorta in an adolescent undergoing the Ross procedure.

Cardiology in the young
2025

Diagnostic value of cardiac magnetic resonance imaging during transition care in adolescents with Turner syndrome.

Frontiers in pediatrics
2026

Assessing the Utility of Targeted Neonatal Echocardiography for Congenital Heart Disease Detection: Retrospective Cohort Analysis.

Journal of clinical ultrasound : JCU
2025

Clinical Outcomes in Patients With Quadricuspid vs Bicuspid Aortic Valve.

JAMA network open
2026

A Rare Case of Posterior Malalignment VSD with Interrupted Right Arch and Isolated Left Common Carotid Artery.

Pediatric cardiology
2025

Case Report: A novel compound heterozygosity of the EVC2 gene identified in a Chinese pedigree with congenital heart defect.

Frontiers in pediatrics
2025

Left circumflex artery occlusion during aortic valvuloplasty in a young patient with bicuspid aortic valve: a case report.

JA clinical reports
2025

A case of heart failure due to multiple late complications after repair of tetralogy of Fallot in adulthood.

Journal of cardiology cases
2025

Assessment of Knowledge, Attitude, and Perception about Secondhand Smoking (SHS). A Questionnaire-based Survey.

Journal of pharmacy &amp; bioallied sciences
2025

Implications of monogenic bicuspid aortic valve (BAV) forms among sporadic BAV patients.

European journal of human genetics : EJHG
2025

The first report of quadricuspid aortic valve in cattle: clinical, anatomical and histological findings in three Holstein cattle.

Journal of comparative pathology
2025

Incidental Pelvic Ectopic Kidney Discovery During Pre-TAVR Imaging: Implications for Surgical Planning.

The American journal of case reports
2025

Bicuspid Aortic Valve and Sudden Cardiac Death.

Life (Basel, Switzerland)
2026

Early predictors of aortic valve surgery in patients with significant bicuspid aortic regurgitation without initial surgical indication.

Heart (British Cardiac Society)
2025

A case-control study of early-life residential exposure to tetrachloroethylene and risks of childhood cancer and birth defects.

Environment international
2025

Accessory mitral valve tissue causing left ventricular outflow tract obstruction associated with subaortic membrane and aortic sinus aneurysm: A case report and literature review.

The Journal of international medical research
2025

Characterization of an induced pluripotent stem cell line (NCHi024-A) from a male infant with bicuspid aortic valve.

Stem cell research
2025

Clinical course and outcomes of supravalvular aortic stenosis in adults.

Open heart
2025

The Living Ross Procedure: A New Paradigm in Partial Heart Transplantation.

The Annals of thoracic surgery
2025

Aortopathy associated with bicuspid aortic valve: advances in clinical and hemodynamics research.

Frontiers in physiology
2025

Surgical Management of Congenital Aortic and Truncal Valve Disease: A Comprehensive Review.

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual
2026

Children are Less Likely Than Adults to Develop Complete Heart Block Following TAVR.

Pediatric cardiology
2024

Aortic atresia with complex aortic arch collateralization through the subclavian arteries.

Annals of pediatric cardiology
2025

Age Differences in Aortic Stenosis.

Reviews in cardiovascular medicine
2025

The Role of First-Trimester Ultrasound in Detecting Aortic and Pulmonary Valve Agenesis: A Rare Case of Trisomy 13.

Journal of clinical ultrasound : JCU
2025

Bicuspid aortic valve disease: advancements and challenges of transcatheter aortic valve implantation.

European heart journal
2025

Surgical Aortic Valve Replacement For Bicuspid And Tricuspid Valve Disease: 7-Year Outcomes In >1100 Patients.

Portuguese journal of cardiac thoracic and vascular surgery
2025

Bicuspid Aortic Valve among Children Presenting in Two Health Institutions in Enugu, South-East Nigeria.

Nigerian journal of clinical practice
2025

Patent Ductus Arteriosus with Left to Right Shunting Exacerbating Aortic Valve Stenosis.

Methodist DeBakey cardiovascular journal
2025

The utility of neocuspidization in the surgical management of congenital aortic valve pathology: mid-term results of single-center experience with AVNeo procedure in children.

General thoracic and cardiovascular surgery
2025

Physical activity characteristics in adults with bicuspid aortic valve versus age- and sex-matched controls.

International journal of cardiology
2025

The Combined Risk of Marfan Syndrome and Bicuspid Aortic Valve in the Elderly.

Cureus
2025

Automated, Standardized, Quantitative Analysis of Cardiovascular Borders on Chest X-Rays Using Deep Learning.

JACC. Advances
2025

Transcatheter aortic valve replacement via direct aortic access for severe aortic stenosis with double aortic arch.

Journal of surgical case reports
2025

Anomalous left coronary artery from the pulmonary artery: A case report of ALCAPA syndrome with bicuspid aortic valve.

Medicine
2025

A Rare Combination: A Right Atrial Myxoma Located at the Superior Margin of the Fossa Ovalis, in Conjunction With a Congenitally Bicuspid Aortic Valve.

Journal of clinical ultrasound : JCU
2025

Surgical Management of a Patient With Non-Fallot-Type Absent Pulmonary Valve Syndrome.

World journal for pediatric &amp; congenital heart surgery
2025

Right Atrial Appendage for Aortic Valve Reconstruction.

The Annals of thoracic surgery
2025

Two-dimensional speckle tracking of the ascending aorta: a novel approach to evaluate arterial stiffness in pediatric patients with repaired conotruncal anomalies using echocardiography.

Frontiers in cardiovascular medicine
2025

Anatomical Left Superior Vena Cava Correction: An Option for Left Ventricular Recruitment?

The Thoracic and cardiovascular surgeon
2025

Catheter for Hemodialysis in Persistent Left Superior Vena Cava in a Patient with Aortic Valve Endocarditis.

Brazilian journal of cardiovascular surgery
2025

Prevalence, clinical characterization, management and evolution of bicuspid aortic valve classified according to the 2021 International Consensus Statement in a tertiary care hospital.

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

Quadricuspid aortic valve in an asymptomatic young adult: a case report.

Annals of medicine and surgery (2012)
2025

Endocardial-to-mesenchymal transition underlies cardiac outflow tract septation and bicuspid aortic valve formation in the Syrian hamster model.

Scientific reports
2025

[Diagnostic characterization and morbi-mortality in pediatric patients with diagnosis of vascular ring].

Archivos de cardiologia de Mexico
2025

Bicuspid Aortic Valves (BAV) Registry (RE-BAV): Clinical and echocardiographic characteristics of patients with BAV according to novel classification of bicuspid aortic valves.

Kardiologia polska
2025

Multimodal imaging in the assessment of quadricuspid aortic valve.

Journal of cardiothoracic surgery
2025

Repair of aorto-left ventricular tunnel type IV with origin above the left main coronary artery.

Multimedia manual of cardiothoracic surgery : MMCTS
2026

Factors Predicting Early Outcome of Aortic Valve Neocuspidization in the Pediatric Population.

Pediatric cardiology
2025

Reinterventions in patients with congenital aortic stenosis and a commissurotomy.

Open heart
2025

Aorto-Left Ventricular Tunnel: The First Systematic Review of An Uncommon Entity (177 Worldwide Cases from 1965 to 2024).

Reviews in cardiovascular medicine
2025

Surgical Ligation of Isolated Type I Aortopulmonary Window Without Cardiopulmonary Bypass is a Safe and Cost-Effective Surgical Technique.

World journal for pediatric &amp; congenital heart surgery
2025

Staged biventricular repair in aortic hypoplasia: the value of intraoperative myocardial perfusion.

Multimedia manual of cardiothoracic surgery : MMCTS
2025

The hemi-Yacoub procedure and valve repair in a patient with Turner syndrome and bicuspid aortic valve.

Multimedia manual of cardiothoracic surgery : MMCTS
2025

Cobrahead reimplantation of anomalous coronary arteries arising from the left coronary sinus.

Multimedia manual of cardiothoracic surgery : MMCTS
2025

Complex congenital heart and lung defects as a cause of hydrops fetalis in French bulldogs -micro-CT with contrast study.

Scientific reports
2025

Impact of Atretic Aortic Valve on Systemic Ventricular Function After the Norwood Procedure.

The Annals of thoracic surgery
2025

Suspected Transient Ischemic Attack Related to Dysfunctional Quadricuspid Aortic Valve.

Journal of investigative medicine high impact case reports
2025

Hypertension at diagnosis of coarctation of the aorta as a risk factor for recoarctation.

Pediatric research
2024

A Case of a Newborn Presenting With a VACTERL-Like Association.

Cureus
2024

Aortopathy in repaired tetralogy of Fallot and David procedure.

Cardiovascular diagnosis and therapy
2025

Unicuspid unicommissural aortic valves: A surgical pathology analysis.

Indian journal of pathology &amp; microbiology
2024

Surgical treatment experience of seven cases of Berry syndrome.

Journal of cardiothoracic surgery
2024

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

Cardiology in the young
2024

Bicuspid aortic valve repair with autologous aortic wall patches in an adolescent with acute severe regurgitation after balloon dilatation.

Multimedia manual of cardiothoracic surgery : MMCTS
2025

Contribution of rare chromosome 22q11.2 copy number variants to non-syndromic bicuspid aortic valve.

Heart (British Cardiac Society)
2024

[An unusual case of bicuspid aortic valve in adult age].

Giornale italiano di cardiologia (2006)
2025

The Congenitally Malformed Aortic Valve in Type A Aortic Dissection: Its Frequency and Consequences.

The American journal of cardiology
2024

Severe acute recoil following transcatheter aortic valve replacement with a self-expanding prosthesis in a heavily calcified bicuspid aortic valve.

Future cardiology
2024

Do anomalous aortic origin of a coronary artery patients have higher risk of myocardial ischaemia and adverse cardiac events during aortic and mitral valve replacements?

Cardiology in the young
2024

Quadricuspid aortic valve with complete heart block: a double whammy.

The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology
2024

Multiple cell types including melanocytes contribute to elastogenesis in the developing murine aortic valve.

Scientific reports
2025

Zebrafish arterial valve development occurs through direct differentiation of second heart field progenitors.

Cardiovascular research
2025

A Concise Review of Marfan Syndrome with a Congenital Cardiac Surgery Focus.

Cardiology in review
2024

Pacemaker Implantation for Low-Grade Conduction Abnormalities After Balloon-Expandable Transcatheter Aortic Valve Implantation.

Clinical cardiology
2024

Unicuspid aortic valve repair in a neonate.

Multimedia manual of cardiothoracic surgery : MMCTS
2025

Current evidence of unicuspid aortic valve in young adults: A systematic review and metanalysis.

Current problems in cardiology
2024

Mitral Annular Disjunction in Heritable Thoracic Aortic Disease: Insights From the Montalcino Aortic Consortium.

Journal of the American Heart Association
2024

Effect of aortic valve phenotype and sex on aorta dilation in patients with aortic stenosis.

Open heart
2024

Surgical outcome of the borderline hypoplastic left ventricle: impact of the left ventricle rehabilitation strategy.

Cardiology in the young
2025

Bullous Lung Disease in Turner Syndrome: An Underrecognized Comorbidity?

American journal of medical genetics. Part A
2025

Unraveling aortic hemodynamics using fluid structure interaction: biomechanical insights into bicuspid aortic valve dynamics with multiple aortic lesions.

Biomechanics and modeling in mechanobiology
2024

Aortic root replacement for bicuspid aortic valve dysfunction does not impair survival rates.

Journal of cardiothoracic surgery
2024

[Genetic analysis of a child with 18q terminal deletion and aortic regurgitation and a literature review].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2025

Prevalence of cardiomyopathy and cardiac mortality in a colony of non-purebred cats in New Zealand.

New Zealand veterinary journal
2024

Pathogenesis and Surgical Treatment of Congenitally Corrected Transposition of the Great Arteries (ccTGA): Part III.

Journal of clinical medicine
2024

Assessment of abnormal transvalvular flow and wall shear stress direction for pediatric/young adults with bicuspid aortic valve: A cross-sectional four-dimensional flow study.

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance
2024

Quadricuspid aortic valve in a 16-year-old Quarter horse.

Journal of equine veterinary science
2025

Study on ultrasound diagnosis and pathological anatomy of fetal complex congenital heart disease in the first trimester.

Journal of clinical ultrasound : JCU
2025

Single ventricle palliation in congenitally corrected transposition of the great arteries: An international multicenter study.

The Journal of thoracic and cardiovascular surgery
2024

Quadricuspid aortic valve and its rare association with left main obstruction: case report and literature review.

Future cardiology
2024

"One-stop" interventional therapy for quadricuspid aortic valve combined with severe coronary artery disease: a case report.

Journal of cardiothoracic surgery
2025

Further Delineation of the Proximal 16p11.2 Microdeletion Syndrome: Novel Findings Among 22 New Individuals.

American journal of medical genetics. Part A
2024

Deficient GATA6-CXCR7 signaling leads to bicuspid aortic valve.

Disease models &amp; mechanisms
2024

Short-Term Results With Ozaki Valved Conduit-A Simple Solution for Patients Needing Right Ventricle to Pulmonary Artery Conduit in a Low-Resource Setting.

World journal for pediatric &amp; congenital heart surgery
2024

Rare genomic copy number variants implicate new candidate genes for bicuspid aortic valve.

PloS one
2024

Whole-exome sequencing uncovers the genetic complexity of bicuspid aortic valve in families with early-onset complications.

American journal of human genetics
2024

Double-orifice mitral valve associated with mild mitral stenosis and coarctation of the aortic isthmus: A rare case of incomplete form of Shone's syndrome.

Echocardiography (Mount Kisco, N.Y.)
2025

State of the Art Review: Aortic Valve Repair in Infants and Children.

The Annals of thoracic surgery
2024

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

Korean circulation journal
2024

Durability of Aortic Homografts in Pulmonary Atresia and Major Aortopulmonary Collateral Arteries.

World journal for pediatric &amp; congenital heart surgery
2024

Calcific Aortic Stenosis - Inflammatory Disease.

Ceskoslovenska patologie
2024

Impact of training data composition on the generalizability of convolutional neural network aortic cross-section segmentation in four-dimensional magnetic resonance flow imaging.

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance
2024

The impact of congenital heart disease on treatment and survival of patients with hepatoblastoma: A single-center experience.

Pediatric blood &amp; cancer
2024

Management of bicuspid aortic valve disease in the transcatheter aortic valve implantation era.

Heart (British Cardiac Society)
2024

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

The Annals of thoracic surgery
2024

Clinical presentation and surgical outcomes in patients with Shone's complex: a systematic review.

General thoracic and cardiovascular surgery
2024

"Unkinking" the "Kink" Normalizes the Doppler Pattern.

Diagnostics (Basel, Switzerland)
2024

Pediatric Quadricuspid Aortic Valve: Morphology, Characteristics, Clinical Outcomes, and Literature Review.

World journal for pediatric &amp; congenital heart surgery
2024

Patients with a Bicuspid Aortic Valve (BAV) Diagnosed with ECG-Gated Cardiac Multislice Computed Tomography-Analysis of the Reasons for Referral, Classification of Morphological Phenotypes, Co-Occurring Cardiovascular Abnormalities, and Coronary Artery Stenosis.

Journal of clinical medicine
2025

Bicuspid Aortic Valve Function and Aortopathy on Presentation and Progression in Children: Does Sex Difference Have Any Implications?

Pediatric cardiology
2024

Rationale and design of the SPREAD study: Sport Practice and its Effects on Aortic Size and Valve Function in Bicuspid Aortic Valve Disease.

The Journal of sports medicine and physical fitness
2024

Atrial and Ventricular Arrhythmia in Adults With Shone Complex: A Single-Center Cohort Study.

JACC. Advances
2024

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa in children diagnosed by echocardiography: Two case reports.

Journal of clinical ultrasound : JCU
2024

Clinical Presentation and Therapy of Hypoplastic Left Heart Syndrome.

Advances in experimental medicine and biology
2024

Molecular Pathways and Animal Models of Semilunar Valve and Aortic Arch Anomalies.

Advances in experimental medicine and biology
2024

Human Genetics of Semilunar Valve and Aortic Arch Anomalies.

Advances in experimental medicine and biology
2024

Clinical Presentation and Therapy of Semilunar Valve and Aortic Arch Anomalies.

Advances in experimental medicine and biology
2024

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

Advances in experimental medicine and biology
2025

Lifetime Management of Adolescents and Young Adults with Congenital Aortic Valve Disease.

The Annals of thoracic surgery
2024

Parachute Mitral Valve and Mid-Aortic Syndrome - Unusual Associations of Alagille Syndrome.

European journal of case reports in internal medicine
2024

Aortic disease and cardiomyopathy in patients with a novel DNMT3A gene variant causing Tatton-Brown-Rahman syndrome.

Clinical epigenetics
2025

Prevalence and incidence of chest wall deformities in children below 18 years old: the first systematic review and meta-analysis.

Archives of medical science : AMS
2024

Myosin Heavy Chain 7 (MYH7) Variant Associated Cardiovascular Disease: An Unusual Case of Heart Failure in a Young Male.

Cureus
2024

Uncommon presentations of type A quadricuspid aortic valve in the Septuagenarian.

Journal of cardiothoracic surgery
2024

Identification of congenital aortic valve malformations in juvenile natriuretic peptide receptor 2-deficient mice using high-frequency ultrasound.

American journal of physiology. Heart and circulatory physiology
2024

Hydrops fetalis caused by a complex congenital heart defect with concurrent hypoplasia of pulmonary blood vessels and lungs visualized by micro-CT in a French Bulldog.

BMC veterinary research
2024

Bilateral pulmonary artery banding facilitates the systemic ventricular outflow tract growth for biventricular and univentricular repair candidates of complex arch anomaly.

Heart and vessels
2024

Quadricuspid Aortic Valve: Interesting Images.

Annals of cardiac anaesthesia
2024

Outcomes of Bicuspid Aortic Valve in Pregnancy.

The American journal of cardiology
2025

Long-term outcomes after aortic root replacement for bicuspid aortic valve-associated aneurysm.

The Journal of thoracic and cardiovascular surgery
2024

Quadricuspid Aortic Valve: Imaging, Diagnosis, and Prognosis.

Texas Heart Institute journal
2024

Illuminating the Genetic Basis of Congenital Heart Disease in Patients with Kabuki Syndrome.

Diagnostics (Basel, Switzerland)
2024

Unicommissural unicuspid aortic valve (UAV) presenting as ascending aortic aneurysm with aortic dissection.

Echocardiography (Mount Kisco, N.Y.)
2024

Quantifying aortic valve regurgitation in patients with congenital aortic valve disease by 2D and 4D flow magnetic resonance analysis.

International journal of cardiology
2024

Clinical features of quadricuspid aortic valve in middle-aged and elderly patients: Insights from a regional study.

Echocardiography (Mount Kisco, N.Y.)
2024

Ventricular arrhythmias in patients with bicuspid aortic valves.

Journal of cardiovascular electrophysiology
2025

Asymptomatic giant ascending aortic aneurysm: a challenging surgical strategy for a silent bicuspid aortopathy.

Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace
2024

Evaluating the relationship between the proportion of X-chromosome deletions and clinical manifestations in children with turner syndrome.

Frontiers in endocrinology
2025

Morphology, Clinical Associations, and Pathologic Follow-up of Quadricuspid Aortic Valves in Children.

Pediatric cardiology
2024

Echocardiographic abnormalities and joint hypermobility in Chinese patients with Osteogenesis imperfecta.

Orphanet journal of rare diseases
2024

Risk stratification by systemic manifestations secondary to hemodynamic disorders of patients with severe tricuspid regurgitation.

BMC cardiovascular disorders
2024

Patient-specific analysis of bicuspid aortic valve hemodynamics using a fully coupled fluid-structure interaction model.

Computers in biology and medicine
2024

Hypoplastic Left Heart Syndrome with Mitral Stenosis and Aortic Atresia-Echocardiographic Findings and Early Outcomes.

Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography
2023

Navigating the challenges of bicuspid aortic valve-aortopathy.

Global cardiology science &amp; practice
2024

Association of Prenatally Diagnosed Isolated Single Left Superior Vena Cava and Postnatal Development of Coarctation of the Aorta.

Pediatric cardiology
2024

The Ross Procedure in a Case of Baraitser-Winter Syndrome: A Case Report.

Cureus
2024

An Unlucky Four-Leaf Clover Stenosis With a Single Coronary Artery.

Cureus
2024

Risk Factors for Coronary Artery Complications After Prosthetic Pulmonary Valve Implantation in Patients With Congenital Heart Disease.

Circulation journal : official journal of the Japanese Circulation Society
2024

[Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes].

Archivos de cardiologia de Mexico
2024

A Y-incision to enlarge the aortic root for aortic valve stenosis with anomalous aortic origin of the right coronary artery.

Journal of cardiothoracic surgery
2024

[Diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery].

Zhonghua wai ke za zhi [Chinese journal of surgery]
2024

An Undiagnosed Shone Complex in a 52-Year-Old Female: A Case Report.

Journal of investigative medicine high impact case reports
2024

Use of transillumination echocardiography in the assessment and diagnosis of bicuspid aortic valve.

Echocardiography (Mount Kisco, N.Y.)
2024

Hemodynamics, anatomy, and outcomes of quadricuspid aortic valves: Multimodality imaging assessment.

Journal of cardiovascular computed tomography
2024

Bicuspid aortic valve: The most frequent and not so benign congenital heart disease.

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

Simulation-based design of bicuspidization of the aortic valve.

The Journal of thoracic and cardiovascular surgery
2023

Aortic valve leaflets are asymmetric and correlated with the origin of coronary arteries.

Translational pediatrics
2024

Heart transplantation using a donor heart with repaired tetralogy of Fallot: a case report.

European heart journal. Case reports
2024

A rare and unique case: aortic valve replacement in a young adult with a stenotic unicommissural unicuspid aortic valve.

Future cardiology
2024

Aortic valvar atresia with a normal-sized left ventricle in an adolescent.

Cardiology in the young
2024

Partial Heart Transplant in a Neonate With Irreparable Truncal Valve Dysfunction.

JAMA

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

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

  1. Molecular Calcification Imaging and Ascending Aortic Disease in Patients With a Bicuspid Aortic Valve.
    JAMA network open· 2026· PMID 41729522mais citado
  2. Congenital Coarctation of the Aorta in a Patient With ROBO4 c.695C&gt;T (p.Thr232Met) Germline Variant.
    Annals of internal medicine. Clinical cases· 2026· PMID 41613935mais citado
  3. Evolution of the Aorta After Valve Replacement in Bicuspid Aortic Valves: Results at Mid-Term Follow-Up.
    Heart, lung &amp; circulation· 2026· PMID 41558910mais citado
  4. [Analysis of optimizing indications for fetal pulmonary valvuloplasty based on a multi-parameter scoring system].
    Zhonghua er ke za zhi = Chinese journal of pediatrics· 2026· PMID 41539952mais citado
  5. Outcomes of Heart Transplantation in Single-Ventricle Physiology: A Retrospective Single-Center Experience with Emphasis on Surgical Complexity.
    Journal of clinical medicine· 2026· PMID 41827132mais citado
  6. Aortic valve neocuspidization in children: A systematic review and meta-analysis.
    JTCVS Open· 2026· PMID 41960115recente
  7. Modification of Nicks Technique for Aortic Annular Enlargement in Children.
    Pediatr Cardiol· 2026· PMID 41843014recente
  8. Using preoperative cardiac computed tomographic conduction axis prediction to avoid damage in congenital left ventricular outflow tract and aortic valve surgery.
    J Thorac Cardiovasc Surg· 2026· PMID 41780715recente
  9. Long-term outcomes of aortic valve repair in children after infancy.
    J Thorac Cardiovasc Surg· 2026· PMID 41707953recente
  10. Over 40 Years of Experience With Aortic Valve Surgery in the Paediatric Population-What Is the Current Best Strategy?
    Eur J Cardiothorac Surg· 2026· PMID 41666304recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:95448(Orphanet)
  2. MONDO:0019808(MONDO)
  3. GARD:19260(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55788885(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

Atresia da válvula aórtica
Compêndio · Raras BR

Atresia da válvula aórtica

ORPHA:95448 · MONDO:0019808
CID-10
Q23.0 · Estenose congênita da valva aórtica
CID-11
MedGen
UMLS
C0265843
EuropePMC
Wikidata
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