Bicuspid Aortic Valve
2014; Lippincott Williams & Wilkins; Volume: 129; Issue: 25 Linguagem: Catalão
10.1161/circulationaha.113.007851
ISSN1524-4539
AutoresHéctor I. Michelena, Siddharth K. Prakash, Alessandro Della Corte, Malenka M. Bissell, Nandan S. Anavekar, Patrick Mathieu, Yohan Bossé, Giuseppe Limongelli, Eduardo Bossone, D. Woodrow Benson, Patrizio Lancellotti, Eric M. Isselbacher, Maurice Enriquez‐Sarano, Thoralf M. Sundt, Philippe Pîbarot, Artur Evangelista, Dianna M. Milewicz, Simon C. Body,
Tópico(s)Infective Endocarditis Diagnosis and Management
ResumoHomeCirculationVol. 129, No. 25Bicuspid Aortic Valve Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessResearch ArticlePDF/EPUBBicuspid Aortic ValveIdentifying Knowledge Gaps and Rising to the Challenge From the International Bicuspid Aortic Valve Consortium (BAVCon) Hector I. Michelena, MD, Siddharth K. Prakash, MD, PhD, Alessandro Della Corte, MD, PhD, Malenka M. Bissell, MD, BM, MRCPCH, Nandan Anavekar, MB, BCh, Patrick Mathieu, MD, Yohan Bossé, PhD, Giuseppe Limongelli, MD, Eduardo Bossone, MD, D.Woodrow Benson, MD, PhD, Patrizio Lancellotti, MD, PhD, Eric M. Isselbacher, MD, Maurice Enriquez-Sarano, MD, Thoralf M. SundtIII, MD, Philippe Pibarot, DVM, PhD, Artur Evangelista, MD, PhD, Dianna M. Milewicz, MD, PhD and Simon C. Body, MBChB, MPHon behalf of the BAVCon Investigators* Hector I. MichelenaHector I. Michelena From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Siddharth K. PrakashSiddharth K. Prakash From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Alessandro Della CorteAlessandro Della Corte From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Malenka M. BissellMalenka M. Bissell From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Nandan AnavekarNandan Anavekar From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Patrick MathieuPatrick Mathieu From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Yohan BosséYohan Bossé From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Giuseppe LimongelliGiuseppe Limongelli From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Eduardo BossoneEduardo Bossone From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , D.Woodrow BensonD.Woodrow Benson From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Patrizio LancellottiPatrizio Lancellotti From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Eric M. IsselbacherEric M. Isselbacher From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Maurice Enriquez-SaranoMaurice Enriquez-Sarano From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Thoralf M. SundtIIIThoralf M. SundtIII From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Philippe PibarotPhilippe Pibarot From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Artur EvangelistaArtur Evangelista From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). , Dianna M. MilewiczDianna M. Milewicz From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). and Simon C. BodySimon C. Body From the Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (H.I.M., N.A., M.E.-S.); Division of Medical Genetics; Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX (S.K.P., D.M.M.); Department of Cardiothoracic Sciences, Second University of Naples, Naples, Italy (A.D.C.); Department of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, United Kingdom (M.M.B.); Institut Universitaire de Cardiologie et de Pneumologie de Québec, Laval University, Québec, Canada (P.M., Y.B., P.P.); Cardiologia SUN, Monaldi Hospital, Naples, Italy (G.L.); Cardiology Division, "Cava de' Tirreni and Amalfi Coast" Hospital, Heart Department, University of Salerno, Italy (E.B.); Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI (D.W.B.); University Hospital of Liège, GIGA Cardiovascular Sciences, CHU Sart Tilman, Liège, Belgium (P.L.); Heart Center and Thoracic Aortic Center, Massachusetts General Hospital, Boston, MA (E.M.I.); Division of Cardiac Surgery and Corrigan Minehan Heart Center, Massachusetts General Hospital, Boston, MA (T.M.S.); Servei de Cardiologia, Hospital Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (A.E.); and Dept of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA (S.C.B.). and on behalf of the BAVCon Investigators* Originally published24 Jun 2014https://doi.org/10.1161/CIRCULATIONAHA.113.007851Circulation. 2014;129:2691–2704IntroductionEverything should be kept as simple as possible, but no simpler.—Albert Einstein1Since its estimated first description >500 years ago by Leonardo da Vinci,2 the bicuspid aortic valve (BAV) has progressively built a reputation; initially, as a curious valvular phenotype with a tendency to develop obstruction and insufficiency. In more contemporary times, however, the BAV is recognized as underlying almost 50% of isolated severe aortic stenosis cases requiring surgery,3 and has been extensively associated with ominous outcomes such as bacterial endocarditis and aortic dissection.4 These associations, coupled with the high prevalence of BAV in humans,5 have prompted investigative efforts into the condition, which although insightful, have generated more questions than answers. This review describes our current knowledge of BAV, but, more importantly, it highlights knowledge gaps and areas where basic and clinical research is warranted. Our review has 2 sections. The first section outlines the multifaceted challenge of BAV, our current understanding of the condition, and barriers that may hamper the advancement of the science. The second section proposes a roadmap to discovery based on current imaging, molecular biology, and genetic tools, recognizing their advantages and limitations.Bicuspid Aortic Valve: A Multifaceted ChallengeA Condition Characterized by Variable Clinical PresentationThe clinical presentation and consequences of BAV in humans are exceedingly heterogeneous, with few clinical or molecular markers to predict associated complications.4,6 BAV can be diagnosed at any stage during a lifetime, from newborns7 to the elderly,8 and in the setting of variable clinical circumstances. Some are benign circumstances such as auscultatory abnormalities or incidental echocardiographic findings in otherwise healthy patients8; other circumstances are morbid, such as early severe aortic valve dysfunction, premature congestive heart failure, and thoracic aortic aneurysms (TAAs).8,9 Life-threatening circumstances include bacterial endocarditis and acute aortic dissection.8–11 These complications may present within a wide age range and may constitute the clinical debut of an otherwise previously healthy patient with unidentified bicuspid status.10,11 In the community, auscultatory abnormalities account for ≈60% to 70% of diagnostic echocardiograms for BAV.8,10 These diverse presentations hamper coordinated screening efforts for BAV. In addition, the failure of clinicians to recognize a systolic ejection murmur or click, typically best heard at the apex and aortic area,12,13 or to appreciate the significance of aortic valve and aorta disease in the family history, may hinder the prompt diagnosis of BAV.A Valvuloaortopathy With Varied Phenotypes and Unpredictable OutcomesGiven the high incidence of BAV dysfunction requiring surgical intervention and the high incidence of associated TAA formation8–10,14–18 (Table), the BAV condition should be viewed as a valvuloaortopat
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