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Abstract This consensus of nomenclature and classification for congenital bicuspid aortic valve and its aortopathy is evidence-based and intended for universal use by physicians (both pediatricians and adults), echocardiographers, advanced cardiovascular imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists, and researchers spanning these areas of clinical and basic research. In addition, as long as new key and reference research is available, this international consensus may be subject to change based on evidence-based data1.
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AIM: The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes). METHODS: A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate. STRUCTURE: Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.
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Enfermedades de la Aorta , Enfermedad de la Válvula Aórtica Bicúspide , Cardiología , Femenino , Embarazo , Estados Unidos , Humanos , American Heart Association , Enfermedades de la Aorta/diagnóstico , Enfermedades de la Aorta/terapia , AortaRESUMEN
PURPOSE OF REVIEW: Embolic stroke of undetermined source is a challenging clinical entity. While less common than atrial fibrillation and endocarditis, many noninfective heart valve lesions have been associated with stroke and may be considered as culprits for cerebral infarcts when other more common causes are excluded. This review discusses the epidemiology, pathophysiology, and management of noninfective valvular diseases that are commonly associated with stroke. RECENT FINDINGS: Calcific debris from degenerating aortic and mitral valves may embolize to the cerebral vasculature causing small- or large-vessel ischemia. Thrombus which may be adherent to calcified valvular structures or left-sided cardiac tumors may also embolize resulting in stroke. Tumors themselves, most commonly myxomas and papillary fibroelastomas, may fragment and travel to the cerebral vasculature. Despite this broad differential, many types of valve diseases are highly comorbid with atrial fibrillation and vascular atheromatous disease. Thus, a high index of suspicion for more common causes of stroke is needed, especially given that treatment for valvular lesions typically involves cardiac surgery whereas secondary prevention of stroke due to occult atrial fibrillation is readily accomplished with anticoagulation.
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Fibrilación Atrial , Enfermedades de las Válvulas Cardíacas , Accidente Cerebrovascular , Trombosis , Humanos , Enfermedades de las Válvulas Cardíacas/complicaciones , Enfermedades de las Válvulas Cardíacas/epidemiología , Enfermedades de las Válvulas Cardíacas/terapia , Fibrilación Atrial/complicaciones , Fibrilación Atrial/epidemiología , Fibrilación Atrial/terapia , Accidente Cerebrovascular/epidemiología , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/prevención & control , Válvula Mitral , Trombosis/complicacionesRESUMEN
Despite numerous promising therapeutic targets, there are no proven medical treatments for calcific aortic stenosis (AS). Multiple stakeholders need to come together and several scientific, operational, and trial design challenges must be addressed to capitalize on the recent and emerging mechanistic insights into this prevalent heart valve disease. This review briefly discusses the pathobiology and most promising pharmacologic targets, screening, diagnosis and progression of AS, identification of subgroups that should be targeted in clinical trials, and the need to elicit the patient voice earlier rather than later in clinical trial design and implementation. Potential trial end points and tools for assessment and approaches to implementation and design of clinical trials are reviewed. The efficiencies and advantages offered by a clinical trial network and platform trial approach are highlighted. The objective is to provide practical guidance that will facilitate a series of trials to identify effective medical therapies for AS resulting in expansion of therapeutic options to complement mechanical solutions for late-stage disease.
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Estenosis de la Válvula Aórtica/tratamiento farmacológico , Válvula Aórtica/patología , Calcinosis/tratamiento farmacológico , Hipolipemiantes/uso terapéutico , Estenosis de la Válvula Aórtica/complicaciones , Estenosis de la Válvula Aórtica/etiología , Calcinosis/complicaciones , Progresión de la Enfermedad , HumanosRESUMEN
This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes. © 2021 Jointly between the RSNA, the European Association for Cardio-Thoracic Surgery, The Society of Thoracic Surgeons, and the American Association for Thoracic Surgery. The articles are identical except for minor stylistic and spelling differences in keeping with each journal's style. All rights reserved. Keywords: Bicuspid Aortic Valve, Aortopathy, Nomenclature, Classification.
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This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3. Partial-fusion or forme fruste. This consensus recognizes 3 bicuspid-aortopathy types: 1. Ascending phenotype; root phenotype; and 3. extended phenotypes.
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Estenosis de la Válvula Aórtica , Enfermedad de la Válvula Aórtica Bicúspide , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugía , Consenso , Humanos , FenotipoRESUMEN
This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes.
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Estenosis de la Válvula Aórtica , Enfermedad de la Válvula Aórtica Bicúspide , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugía , Consenso , Humanos , FenotipoRESUMEN
This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3. Partial-fusion or forme fruste. This consensus recognizes 3 bicuspid-aortopathy types: 1. Ascending phenotype; root phenotype; and 3. extended phenotypes.
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Enfermedad de la Válvula Aórtica Bicúspide/clasificación , Enfermedad de la Válvula Aórtica Bicúspide/cirugía , Investigación Biomédica , Humanos , Systematized Nomenclature of MedicineRESUMEN
This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes.
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Enfermedad de la Válvula Aórtica Bicúspide/clasificación , Enfermedad de la Válvula Aórtica Bicúspide/genética , Humanos , Fenotipo , Systematized Nomenclature of MedicineRESUMEN
This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3. Partial-fusion or forme fruste. This consensus recognizes 3 bicuspid-aortopathy types: 1. Ascending phenotype; root phenotype; and 3. extended phenotypes.
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Aorta , Enfermedades de la Aorta/clasificación , Válvula Aórtica/anomalías , Enfermedad de la Válvula Aórtica Bicúspide/clasificación , Terminología como Asunto , Aorta/diagnóstico por imagen , Aorta/cirugía , Enfermedades de la Aorta/diagnóstico por imagen , Enfermedades de la Aorta/cirugía , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugía , Aortografía , Enfermedad de la Válvula Aórtica Bicúspide/diagnóstico por imagen , Enfermedad de la Válvula Aórtica Bicúspide/cirugía , Técnicas de Imagen Cardíaca , Consenso , Humanos , Fenotipo , Valor Predictivo de las Pruebas , PronósticoRESUMEN
This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3. The partial-fusion (forme fruste) type. The presence of raphe and the symmetry of the fused type phenotypes are critical aspects to describe. The International Consensus also recognizes 3 types of bicuspid valve-associated aortopathy: 1. The ascending phenotype; 2. The root phenotype; and 3. Extended phenotypes.
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Aorta , Enfermedades de la Aorta/clasificación , Válvula Aórtica/anomalías , Enfermedad de la Válvula Aórtica Bicúspide/clasificación , Terminología como Asunto , Aorta/diagnóstico por imagen , Aorta/cirugía , Enfermedades de la Aorta/diagnóstico por imagen , Enfermedades de la Aorta/cirugía , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugía , Aortografía , Enfermedad de la Válvula Aórtica Bicúspide/diagnóstico por imagen , Enfermedad de la Válvula Aórtica Bicúspide/cirugía , Técnicas de Imagen Cardíaca , Consenso , Humanos , Fenotipo , Valor Predictivo de las Pruebas , PronósticoAsunto(s)
Enfermedades de las Válvulas Cardíacas/cirugía , Implantación de Prótesis de Válvulas Cardíacas/normas , Válvulas Cardíacas/cirugía , Consenso , Medicina Basada en la Evidencia/normas , Enfermedades de las Válvulas Cardíacas/diagnóstico por imagen , Enfermedades de las Válvulas Cardíacas/fisiopatología , Prótesis Valvulares Cardíacas/normas , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Implantación de Prótesis de Válvulas Cardíacas/instrumentación , Válvulas Cardíacas/diagnóstico por imagen , Válvulas Cardíacas/fisiopatología , Hemodinámica , Humanos , Diseño de Prótesis , Recuperación de la Función , Factores de Riesgo , Resultado del TratamientoAsunto(s)
Aterosclerosis , Calcinosis , Enfermedades Cardiovasculares , Enfermedades de las Válvulas Cardíacas , Aterosclerosis/diagnóstico por imagen , Calcinosis/diagnóstico por imagen , Enfermedades de las Válvulas Cardíacas/diagnóstico por imagen , Humanos , Válvula Mitral/diagnóstico por imagenRESUMEN
AIM: This executive summary of the valvular heart disease guideline provides recommendations for clinicians to diagnose and manage valvular heart disease as well as supporting documentation to encourage their use. METHODS: A comprehensive literature search was conducted from January 1, 2010, to March 1, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, Cochrane, Agency for Healthcare Research and Quality Reports, and other selected database relevant to this guideline. Structure: Many recommendations from the earlier valvular heart disease guidelines have been updated with new evidence and provides newer options for diagnosis and treatment of valvular heart disease. This summary includes only the recommendations from the full guideline which focus on diagnostic work-up, the timing and choice of surgical and catheter interventions, and recommendations for medical therapy. The reader is referred to the full guideline for graphical flow charts, text, and tables with additional details about the rationale for and implementation of each recommendation, and the evidence tables detailing the data considered in developing these guidelines.
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Cardiología , Enfermedades de las Válvulas Cardíacas , Humanos , American Heart Association , Cardiología/organización & administración , Enfermedades de las Válvulas Cardíacas/terapia , Estados UnidosRESUMEN
AIM: This executive summary of the valvular heart disease guideline provides recommendations for clinicians to diagnose and manage valvular heart disease as well as supporting documentation to encourage their use. METHODS: A comprehensive literature search was conducted from January 1, 2010, to March 1, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, Cochrane, Agency for Healthcare Research and Quality Reports, and other selected database relevant to this guideline. STRUCTURE: Many recommendations from the earlier valvular heart disease guidelines have been updated with new evidence and provides newer options for diagnosis and treatment of valvular heart disease. This summary includes only the recommendations from the full guideline which focus on diagnostic work-up, the timing and choice of surgical and catheter interventions, and recommendations for medical therapy. The reader is referred to the full guideline for graphical flow charts, text, and tables with additional details about the rationale for and implementation of each recommendation, and the evidence tables detailing the data considered in developing these guidelines.