Artéria pulmonar proveniente da aorta (PACA) é uma malformação cardíaca caracterizada pela origem anômala de um ramo das artérias pulmonares da aorta ascendente e origem normal da outra artéria pulmonar da via de saída do ventrículo direito, apresentando desconforto respiratório, insuficiência cardíaca congestiva e retardo de crescimento nos primeiros dias/meses de vida.
Introdução
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Artéria pulmonar proveniente da aorta (PACA) é uma malformação cardíaca caracterizada pela origem anômala de um ramo das artérias pulmonares da aorta ascendente e origem normal da outra artéria pulmonar da via de saída do ventrículo direito, apresentando desconforto respiratório, insuficiência cardíaca congestiva e retardo de crescimento nos primeiros dias/meses de vida.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 6 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 26 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 — Artéria pulmonar proveniente da aorta
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Inferior wall ST-elevation myocardial infarction in a patient with a single coronary artery from the right coronary cusp trifurcating into the left anterior descending, left circumflex, and right coronary arteries: a rare coronary anomaly, case report.
Abnormal origin of a coronary artery is a rare congenital condition that can significantly affect clinical outcomes especially when associated with acute coronary syndromes. Among these, the presence of a single coronary artery trifurcating from the right coronary cusp into all major coronary branches is exceptionally rare and poorly represented in the literature. A 35-year-old man presented with an inferior ST-elevation myocardial infarction. Emergency angiography revealed a single coronary artery arising from the right coronary cusp. The culprit was the right coronary artery. It was effectively treated with intravascular-guided percutaneous coronary intervention, and the remaining coronaries demonstrated normal flow, as shown in subsequent imaging. The patient recovered uneventfully, rehabilitated without complications. He was discharged on guideline directed medical therapy. Although the anomalous coronary anatomy was not the direct cause of infarction, it introduced significant procedural challenges that could have delayed or compromised revascularization. Our case highlights the importance of recognizing and anticipating coronary anomalies in acute settings. Multimodality imaging and anatomical classification systems help provide timely diagnosis, procedural planning, and risk assessment from a long-term perspective.
Case report of mitral valve replacement in a single coronary artery patient: devil is hidden in the details.
Single coronary artery (SCA) is a rare congenital anomaly that may have serious clinical consequences in different clinical settings despite often being asymptomatic. We report a case of a 75-year-old patient with a SCA originating from the right sinus of Valsalva presenting for pre-operative assessment for mitral valve (MV) stenosis. The patient developed cardiogenic shock post MV replacement surgery on grounds of iatrogenic occlusion of the ectopic left main coronary artery which was treated with a percutaneous revascularization procedure. SCA represents a major anatomical variant that can significantly impact clinical outcomes, particularly during cardiac surgery. An abnormal coronary anatomy poses technical challenges, due to potential inadvertent injury or compression of the anomalous artery relative to the extent of the myocardium at risk.
A case report of J wave syndrome with abnormal potentials in both right and left ventricles and reversed J wave in lead V1.
Abnormal epicardial potentials in J wave syndrome predominantly involve the right ventricular outflow tract (RVOT), while left ventricular (LV) involvement remains less characterized and associates with increased ventricular fibrillation (VF) risk. We report a case demonstrating an inverted J wave in lead V1, suggesting LV posterior wall substrate rather than typical RVOT involvement. A 19-year-old man with resuscitated VF received a subcutaneous implantable cardioverter-defibrillator (S-ICD). Despite cilostazol and quinidine therapy, he experienced five appropriate shocks within 6 months. Twelve-lead electrocardiography showed inferior J waves and a negative deflection in V1, suggesting an inverted J wave. Epicardial mapping showed fractionated potentials in both RVOT and LV posterior wall. Pilsicainide administration augmented RVOT potentials while attenuating those in the LV posterior wall. Spontaneous VF was triggered by premature ventricular contractions (PVCs) originating from the LV posterior wall, where prepotentials preceded QRS onset by 50 ms. Radiofrequency applications eliminated the PVCs, followed by anatomical ablation of RVOT fractionated regions. Subsequently, VF was never induced by program stimulation of up to triple extra stimuli. In the post-operative electrocardiogram, inverted J waves in the V1 lead disappeared. At 8 months post-ablation, the patient remained free from VF recurrence without antiarrhythmic medications, and no S-ICD therapies occurred. This case demonstrates complex electrophysiological manifestations of J wave syndrome, with the inverted J wave in V1 potentially reflecting LV posterior wall substrate. Although the overlapping ablation procedure limited definitive attribution, these findings contribute to understanding the heterogeneous substrates in J wave syndrome.
Isolated left ventricular apical hypoplasia: a case report.
Isolated left ventricular apical hypoplasia is a rare and lesser-known form of cardiomyopathy characterized by specific findings on cardiac imaging. It is thought to be congenital in origin, with clinical features that can range from being asymptomatic to presenting with heart failure and arrhythmias. A 43-year-old woman presented with chest pain and had previously undergone evaluation at another facility, including a 2D echocardiogram and computed tomography (CT) coronary angiography. These tests revealed a dilated left atrium and ventricle and reduced left ventricular function alongside a Type I left anterior descending artery. A cardiac magnetic resonance image (MRI) and review of the CT scan confirmed a diagnosis of isolated left ventricular apical hypoplasia. Guideline-directed medical therapy was initiated due to the initial presentation of left ventricular dysfunction. Left ventricular apical hypoplasia is characterized by (i) a truncated, spherical, and impaired left ventricle (LV) with bulging of the interventricular septum towards the right ventricle (RV), (ii) fatty material in the apical region of the LV, (iii) abnormalities in the papillary muscles and trabecular structures, and (iv) elongation of the RV wrapping around the underdeveloped LV. Limited data is available on this condition, with only a few reported cases. Currently, no definitive guidelines exist, and management is tailored to the patient's specific presentation, including treating heart failure and any arrhythmias that may arise. While the aetiology of this condition remains poorly understood, it is crucial to recognize it to prevent misdiagnosis and to encourage further research into its management.
Fusiform dilatation of the ascending aorta and outpouching of the left A2 anterior cerebral artery segment in COL4A1 -related disorder.
COL4A1 encodes the alpha-1 chain of type IV collagen, which plays a crucial role in vascular basement membranes. The clinical manifestations of COL4A1-related disorders are yet to be fully defined. There is increasing evidence that COL4A1 may play crucial roles across the vascular system. We studied a 71-year-old man with a novel splice acceptor variant, COL4A1 [NM_001845.6] c.1466-1G > C. Clinical, radiological (including comprehensive imaging of the brain as well as chest and abdomen), and genetic assessments were performed. His medical history included fusiform dilatation (4.2 cm) of the ascending thoracic aorta, hearing loss, adrenal and pulmonary nodules, mild splenomegaly, and gallstones. Imaging showed fusiform dilatation (4.2 cm) of the ascending thoracic aorta and concomitant outpouching of the left A2 anterior cerebral artery in the patient in the setting of the heterozygous likely pathogenic splice site variant, COL4A1 c.1466-1G > C. Consistent with a diagnosis of COL4A1-related disorder, multiple cystic bilateral renal lesions were noticed on abdominal imaging. Magnetic resonance imaging of the brain also showed findings consistent with cerebral vasculopathy with scattered bilateral punctate T2/FLAIR hyperintensities within the supratentorial and pontine white matter. Our observations support the growing body of evidence suggesting crucial roles for COL4A1 in vascular homeostasis including medium sized vessel cerebral vasculopathy and aortopathy.
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Single Coronary Artery Arising from the Right Sinus of Valsalva and the Role of Coronary Computed Tomography Angiography.
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📚 EuropePMCmostrando 200
Inferior wall ST-elevation myocardial infarction in a patient with a single coronary artery from the right coronary cusp trifurcating into the left anterior descending, left circumflex, and right coronary arteries: a rare coronary anomaly, case report.
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The American journal of case reportsScreening for Pulmonary Hypertension in Interstitial Lung Disease: Preliminary Results from the PHINDER Study.
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The American journal of case reports[MINOCA revealing an anomalous origin of right coronary artery and a pheochromocytoma : A case report with literature review].
Annales de cardiologie et d'angeiologieIntegrated Cardio-Cerebral Assessment Predicts Adverse Outcomes in Aortic Stenosis: A Preliminary Study.
Echocardiography (Mount Kisco, N.Y.)Application of 3D printing in the evaluation of a clinically significant congenital uterine anomaly.
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Surgical and radiologic anatomy : SRAAnomalous Aortic Origin of the Right Coronary Artery Between the Right and Left Coronary Sinuses: A Case Report.
Cureus[Anomalous origin of the left anterior descending artery from the right coronary artery: A case report].
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European journal of preventive cardiologySalmonella Aortitis-Induced Aortocaval Fistula Masquerading as Tricuspid Disease.
JACC. Case reports[Prognostic analysis of imaging features of coronary artery anomalous origin from the opposite sinus in middle-aged and elderly patients].
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CureusCase Report: A case of intrahepatic papillary neoplasm of the bile duct with invasive carcinoma in a young patient and review of the literature.
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Human cellOdontogenic Cutaneous Fistula-Induced Submandibular Abscess in a Dog: A Rare Presentation.
Veterinary sciencesProboscis lateralis with trans-ethmoidal meningocele and congenital nostril hypoplasia: a case report and literature review.
BMC pediatricsAssessment of cardiac function by speckle tracking echocardiography in children with osteogenesis imperfecta.
Pediatric research[Anomalous Origin of the Right Coronary Artery from the Left Sinus of Valsalva Treated with Modified Unroofing Procedure].
Kyobu geka. The Japanese journal of thoracic surgeryAnatomical Variation in the Formation of the Median Nerve With Triple and Quadruple Roots of Origin: A Cadaveric Case Report.
CureusRight lower lobectomy for lung cancer with anterior truncal basal pulmonary artery: A case report.
SAGE open medical case reportsParadoxical embolism: A case of thromboembolic stroke secondary to pulmonary embolism and deep vein thrombosis.
The Journal of international medical researchAnomalous Right Coronary Artery: A Case of Exertional Angina and Sinus Node Ischemia.
JACC. Case reportsDigestion is unaffected by surgical elimination of the right-to-left cardiac shunt in American alligators (Alligator mississippiensis).
The Journal of experimental biologySudden-Onset Platypnea-Orthodeoxia Syndrome in a Patient with Lung Adenocarcinoma and COVID-19 Without a Patent Foramen Ovale.
European journal of case reports in internal medicineA case of hereditary hemorrhagic telangiectasia with hypoxemia onset in the neonatal period.
BMC pulmonary medicineMyocardial Ischemia Secondary to Anomalous Origin of Left Anterior Descending Coronary Artery: A Case Report and Literature Review.
The American journal of case reportsInhaled iloprost and 2D phase-contrast cardiac MRI in pulmonary vascular reversibility: an adolescent case of anomalous right pulmonary artery from the ascending aorta.
Cardiology in the youngUnusual Venous-Origin Intraparenchymal Hematoma in the Context of a Post-traumatic Carotid-Cavernous Fistula.
CureusAnatomical considerations in fixation of sternal fractures and nonunions.
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BMC infectious diseasesPrimary internal Iliac Aneurysm-Rectal Fistula treated with a combined endovascular and endoscopic approach as a palliative strategy: a case report.
Frontiers in cardiovascular medicineOccult breast cancer with CSF TBA positivity and abnormal involuntary movements.
Discover oncologyAortic atresia, interrupted hypoplastic right aortic arch, single ventricle and bilateral arterial duct - a unique anatomy with surgical palliation.
Journal of cardiothoracic surgeryDual Radial and Femoral Arterial Access for Mechanical Thrombectomy in Acute Middle Cerebral Artery Occlusion: A Case Report.
The American journal of case reportsFetal cardiac remodeling in second trimester in pregnancies with pre-eclampsia and/or fetal growth restriction: deep-learning-based approach using population-wide data.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyMalignant Anomalous Aortic Origin of the Left Main Coronary Artery Managed by Ostial Reimplantation: A Case Report.
Clinical case reportsTracheal Compression on Chest X-ray Leading to the Diagnosis of Right Aortic Arch in 22q11.2 Deletion Syndrome.
CureusSymptomatic anomalous right coronary artery in mother and daughter, a case report.
Journal of cardiothoracic surgeryDouble origin of the superior cerebellar artery from the Basilar artery and the P1 segment of the posterior cerebral artery.
Surgical and radiologic anatomy : SRADeveloping a novel deep learning-based model for automatic right ventricular parameters assessment on ctpa in pulmonary embolism.
Emergency radiologyA Case of Follicular Dendritic Cell Sarcoma of the Palatine Tonsil That Developed as a Radiation-Induced Sarcoma.
Case reports in otolaryngologyPulmonary Vascular Features on CT Chest Differentially Associate with Adverse Outcomes in Smokers in COPDGene.
Annals of the American Thoracic SocietySubclavian-to-descending aortic bypass for the treatment of severe late-stage aortic coarctation in a 62-year-old adult: a case report and literature review.
Frontiers in cardiovascular medicineAcute rupture of primary hepatic angiosarcoma with Kasabach-Merritt syndrome: a rare case report and literature review.
World journal of surgical oncologyExtra-anatomical ascending-to-descending aortic bypass in an adult patient with aortic coarctation.
Multimedia manual of cardiothoracic surgery : MMCTSClinical features and prognosis in patients with coronary anomalies: the experiences and follow-up results of a single center.
Postepy w kardiologii interwencyjnej = Advances in interventional cardiologyRare congenital coronary artery anomalies diagnosed by coronary CT angiography: A 4-case series.
Radiology case reportsThe Silent Stranglehold: Multimodal Imaging in Postoperative Pulmonary Artery Stenosis.
Echocardiography (Mount Kisco, N.Y.)Untreated incomplete ALCAPA in an adult resulting in mitral regurgitation and reduced left ventricular ejection fraction: case report.
European heart journal. Case reportsY-Incision Aortic Annular Enlargement in Patients With Anomalous Left Circumflex Artery.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryFemorocele: A Rare Form of Femoral Hernia.
Surgical case reportsA case of paracentral acute middle maculopathy and retinal vein occlusion: idiopathic occlusive retinal vasculitis or incomplete Behçet's disease?
BMC ophthalmologyWeight-based flow rate triphasic contrast injection protocol for pediatric cardiac computed tomography.
Quantitative imaging in medicine and surgeryThe diagnostic efficacy of unenhanced CT for detecting acute central pulmonary embolism.
BMC cardiovascular disordersPediatric photon-counting chest CT enables iodinated contrast dose reduction with preserved image quality.
Pediatric radiologyAnomalous origin of right coronary artery with interarterial and intramural course associated with bicuspid aortic valve and dilation of ascending aorta.
BMJ case reportsAnomalous Systemic Arterial Supply to the Left Basal Segment With Arterial Dissection of the Feeding Artery: A Case Report and Literature Review.
Clinical case reportsClarifying the Cardiovascular Morphology and Associated Abnormalities in Patients with Double Inlet Left Ventricle Using Multidetector CT Angiography.
Pediatric cardiologyTechnical Success of Stent Placement via Transradial Approach for Aberrant Right Subclavian Artery Stenosis.
Vascular and endovascular surgeryPerforated Meckel Diverticulum Caused by Vegetal Food Entrapment: A Rare Case Report and Literature Review.
The American journal of case reportsLeft Pulmonary Artery Sling: Anatomy, Associations, and Outcome of Surgical Repair as Demonstrated by Autopsies.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyPregnancy in Takayasu Arteritis: A Case Report of Successful Pregnancy in a Large Aortic Aneurysm.
Clinical case reportsDiagnosis of pleural aspergillosis caused by Aspergillus infection via metagenomic next-generation sequencing from a patient with unexplained pleural effusion: a case report.
BMC pulmonary medicineTreatment of internal carotid artery agenesis with intercavernous anastomosis (Lie's type D anastomosis) by simple balloon dilatation.
BMC neurologyAtypical presentation of ALCAPA in a young boy: a case report.
Cardiology in the youngMyocardial Infarction in a Young Adult: A Rare Case of Left Coronary Artery Arising from the Pulmonary Artery.
Life (Basel, Switzerland)Longitudinal Magnetic Resonance Imaging Study of Hemodynamic Changes in the Third Trimester in Fetuses With Major Congenital Heart Defects and Healthy Fetuses.
Prenatal diagnosisNeonatal transposition of the great arteries associated with abnormal right pulmonary artery.
Cardiology in the youngRight coronary artery-right ventricle fistula with a rare giant thrombotic aneurysm: a case report and literature review.
Cardiovascular diagnosis and therapyWhen Neurological Symptoms Hide a Life-Threatening Hemorrhage: A Case Report of Diagnostic Challenges and Management of Hemorrhagic Shock From an Aberrant Radial Artery.
The American journal of case reportsContinuous right-to-left ductal flow in a newborn.
Annals of pediatric cardiologyLeft ventricular pressure-loading improves pressure-induced right ventricular remodeling by redistributing mechanical load and reducing mechanosignaling.
Physiological reportsTrigeminal neuralgia caused by dolichoectatic vertebral artery: Reports of two cases.
Scandinavian journal of painDual Roots of Origin of Inferior Alveolar Nerve and "Vagal Ansa" Cervicalis: Surgical and Anaesthetic Implications.
Prague medical reportPrenatal Diagnosis of Fetal Heart Malformation With Abnormal Number of Pulmonary Artery Branches as the Initial Clue.
Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in MedicineLeft Ventricular Myocardial Perfusion before Contrast Pool in Ventricular Cavity in Cardiac MRI in Adult Type Anomalous Left Coronary Artery from Pulmonary Artery with Pulmonary Hypertension.
The Indian journal of radiology & imagingContrast-Enhanced Ultrasonography for Clinical Assessment of Vertebral Artery Origin Pathologies.
Ultrasound in medicine & biologySurgical Results for Anomalous Aortic Origin of Coronary Artery - Is Right Side Prompt Surgery Necessary?
Circulation journal : official journal of the Japanese Circulation SocietyRecognition of a cardiac differential diagnosis for bronchiolitis: coronary steal phenomenon in a baby with anomalous right pulmonary artery arising from the aorta.
BMJ case reportsUnusual Revelation of an Anomalous Right Coronary Artery: Non-ST Segment Elevation Myocardial Infarction (NSTEMI) in the Context of Drug Abuse.
CureusSTEMI From Left Main Compression by Pulmonary Artery Aneurysm Diagnosed With IVUS.
JACC. Case reportsA rare bilateral renal arterial configuration featuring a right-sided crossed trajectory and left-sided type IIa branching: morphometric and embryological insights from cadaveric dissection.
Surgical and radiologic anatomy : SRANoninvasive Anatomical and Functional Imaging for Hemodynamic Relevance in Right Coronary Artery Anomalies.
JAMA cardiologyFetal 4D Flow CMR for Advanced Diagnostics of Congenital Heart Disease: A Prospective Cohort Study.
European heart journal. Cardiovascular ImagingFirst evaluation of a novel pulsatile LVAD: Feasibility and haemodynamic impact in acute heart failure.
ESC heart failureCase Report: Rare multisystem metastasis in head and neck paraganglioma with SDHB pathogenic variant and KIF1B VUS manifested as FUO.
Frontiers in endocrinologyMarcus Gunn jaw winking associated with Duane's retraction syndrome.
StrabismusPrevalence of Neural Axis Abnormalities in Typical and Atypical Laterality Idiopathic Scoliosis: A Systematic Review and Meta-Analysis of Observational Studies.
SpineCabergoline-induced prolactinoma treatment and subsequent estrogen-driven vascular smooth muscle tumorigenesis in a middle-aged female: A case report.
International journal of surgery case reportsA Rare Case of Femur Metastasis from Brain Meningioma.
Cancer diagnosis & prognosisInfundibular origin of a duplicated anterior communicating artery mimicking an aneurysm.
Surgical and radiologic anatomy : SRACase Report: Rare Bilateral Double Profunda Femoris Artery Variation: Clinical Implications and Considerations.
F1000ResearchBilateral anterior cerebral artery-anterior communicating artery junction arterial ring (duplicate origin of the A2) associated with unilateral A1 aplasia diagnosed by magnetic resonance angiography.
Surgical and radiologic anatomy : SRASevere Diffuse Takayasu Arteritis Manifestation in Childhood.
JACC. Case reportsProgressive gray matter reduction in schizophrenia patients with persistent auditory hallucinations by causal structural covariance network analysis.
Psychological medicine[When rarity duplicates: myocardial non-compaction and single coronary artery, an association not to be underestimated].
Giornale italiano di cardiologia (2006)An Adult-Onset Case of Porokeratotic Eccrine Ostial and Dermal Duct Nevus (PEODDN) in a Previously Healthy 28-Year-Old Indian Male: A Case Report.
CureusKommerell's Diverticulum Masquerading in a Right Aortic Arch: A Vascular Surprise.
Methodist DeBakey cardiovascular journalCase Report: Anomalous origin of the left main coronary artery arising from the left ventricular outflow tract.
Frontiers in cardiovascular medicinePercutaneous Closure of Aortic-Pulmonary Artery Fistula in Cardiogenic Shock: The Modified Tootsie Roll Technique.
JACC. Case reportsAssociações
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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.
- Inferior wall ST-elevation myocardial infarction in a patient with a single coronary artery from the right coronary cusp trifurcating into the left anterior descending, left circumflex, and right coronary arteries: a rare coronary anomaly, case report.
- Case report of mitral valve replacement in a single coronary artery patient: devil is hidden in the details.
- A case report of J wave syndrome with abnormal potentials in both right and left ventricles and reversed J wave in lead V1.
- Isolated left ventricular apical hypoplasia: a case report.
- Fusiform dilatation of the ascending aorta and outpouching of the left A2 anterior cerebral artery segment in COL4A1 -related disorder.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology· 2026· PMID 41514066mais citado
- Valve-sparing aortic root replacement after neonatal arterial switch operation.
- Single Coronary Artery Arising from the Right Sinus of Valsalva and the Role of Coronary Computed Tomography Angiography.
- [Assessment of congenital vascular rings with MDCT on children].
- Innovative hybrid procedure for dissection thoracic aorta in a patient with a previous bentall operation.
- The 'aortic rim' recount: embolization of interatrial septal occluder into the main pulmonary artery bifurcation after atrial septal defect closure.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:99050(Orphanet)
- MONDO:0020391(MONDO)
- GARD:4586(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55789325(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
