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Honoring a Pioneer

Dr. M. E. Abbott

Dr. M. E. Abbott devoted much of her work to congenital heart disease, including bicuspid aortic valve.
BAV - History Lessons from the Medical Literature Bicuspid Aortic Valve History What does it mean to be born with an aortic valve that has only two leaflets instead of three? On the surface, it seems deceptively simple. But the simplicity fades, the seriousness deepens when at some point, either before or after the bicuspid aortic valve has been found, there is aortic aneurysm or dissection in the chest. Sometimes the aorta also has another problem - there is a narrowing called coarctation. Today many individuals recognize other connective tissue abnormalities elsewhere in their bodies. And there is an increased risk of brain aneurysms also. Blood pressure may soar dangerously high, and cardiac arrhythmias sometimes develop. All this is in addition to the commonly recognized risks of infection or failure of the valve itself. No, it is not simple after all. And no, it is not new. The bicuspid aortic valve and its complications are not a phenomenon of modern life.  Many will be surprised that the first image of a BAV was recorded not by sophisticated technology but drawn by the hand of Leonardo di Vinci several hundred years ago. The very earliest works published are not on line in PubMed, but listings of publications about BAV from the later 1900's are there. Searching PubMed recently for "Bicuspid Aortic Valve"  produced a list of 1,419 papers from 1946 until the present.  Over 400 years ago - It is probably approaching 500 years since a BAV was included in a drawing by Leonardo di Vinci. The sketch is reproduced in Figure 1 of the following paper.  "The Bicuspid Aortic Valve", by Braverman, et.al., Figure 1, Current Problems in Cardiology, September, 2005  1865 - BAV associated with stenosis, regurgitation, endocarditis "Valvular disease of the heart", by TB Peacock, London: Churchill, 1865;2-33 1928 - M. Abbott's paper cited an association between BAV and rupture of the ascending aorta, suggesting thinning of the aortic wall might be part of the same abnormality as the BAV. "Coarctation of the aorta of the adult type. II. A statistical study and historical retrospect of 200 recorded cases, with autopsy, of stenosis or obliteration of the descending arch in subjects above the age of two years", by M. Abbott, American Heart Journal, 1928 1952 - R. Reid published a paper entitled "Bicuspid aortic valve associated with aneurysmal dilatation of the ascending aorta". "Bicuspid aortic valve associated with aneurysmal dilatation of the ascending aorta", The Medical Journal of Australia, November, 1952 1972 - V. McKusick described BAV and medial necrosis of the aorta in connection with a father and son who were both bicuspid and died of aortic dissection. "Association of congenital bicuspid aortic valve and erdheim's cystic medial necrosis", Lancet, 1972 1974 - In the earliest of days, physicians could only listen to the chest and learn though autopsies. The statistics we have today about the incidence of BAVs are from autopsy studies published in 1886, 1970, 1972, and 1997 (from Clinical Significance of Bicuspid Aortic Valve by C Ward ). Technology now has the ability to "see" inside the chest, through x-rays,  sound waves, and creation of a magnetic field. Echocardiography and BAV is first mentioned in 1974 in a paper by N. Nanda and co-authors. "Echocardiographic recognition of the congenital bicuspid aortic valve", by NC Nanda, et.al., Circulation, May, 1974 1975 - Coarctation of the aorta is mentioned in early papers regarding BAV. However,  some other unusual things were being found  in those with BAV. Among the papers published that  mention this are those by CD Higgins, RB Jackson, and SJ Schang (and their co-authors) in 1975. "Reversal of dominance of the coronary arterial system in isolated aortic stenosis and bicuspid aortic valve", by CD Higgins, et.al., Circulation, August, 1975 "Subaortic stenosis, bicuspid aortic valve, single coronary artery and coarctation of the aorta", by RD Jackson, et.al., Collected Works on Cardio-Pulmonary Disease, December 1975 "Anomalous coronary artery origin and bicuspid aortic valve", by SJ Schang, et.al., Vascular Surgery, March-April, 1975 1978 - In December of this year, the British Heart Journal published a paper with the results of studying the inheritance of BAVD in 41 families. "Congenitally bicuspid aortic valves. Clinicogenetic study of 41 families."), by R Emanuel, et.al., British Heart Journal, December 1978 1986 - Additional papers  were published during the decade of the 1980's. Among them is this pathology paper about 6 young people with varying forms of aortic disease, including BAV. The risk of loss of life in the young, often athletic population remains a great concern to this day. "Aortic root pathology and sudden death in youth: review of anatomical varieties", by G Thiene and SY Ho, Applied Pathology, 1986 1988 - The bicuspid aortic valve continued to appear on the scene with other aortic abnormalities, as noted in a paper by J. Lindsay, Jr. in 1988. "Coarctation of the aorta, bicuspid aortic valve and abnormal ascending aortic wall", J. Lindsay, Jr., American Journal of Cardiology, January, 1988 1990 - A paper from the University of Chicago Hospital reported on a bicuspid aortic valve and dissection of an aortic aneurysm in a 22 year old man who underwent surgery but died on the eighth post operative day. "Dissecting thoracic aortic aneurysm in a 22-year-old man", by KB O'Dell and SN Hakim, Annals of Emergency Medicine, March, 1990 1995, 1996, 2003 - The publications of Dr. Wouter Schievink and co-authors document the finding of BAV along with abnormalities of the head and neck vessels, which originate from a common source, neural crest cells. "A syndrome of spontaneous cerebral and cervical artery dissections with angiolipomatosis. Report of two cases.", by W Schievink et.al,, Journal of Neurosurgery, May, 2003 "Intracranial aneurysms and cervicocephalic arterial dissections associated with congenital heart disease.", by W Schievink, et.al., Neurosurgery, October, 1996 "Familial aorto-cervicocephalic arterial dissections and congenitally bicuspid aortic valve", by W Schievink and B Mokri, Stroke, October 1995) 1998 - Dr. John Burks, et.al., published a paper about bicuspid aortic valves in two young men. "Ascending aortic aneurysm and dissection in young adults with bicuspid aortic valve: implications for echocardiographic surveillance", by JM Burks, et.al., Clinical Cardiology, June, 1998 2002 - At the Aortic Surgery Symposium in 2002, Dr.Claudio Russo presented a review of bicuspid aortic valve patients who had valve replacement surgery between 1975 and 1985 in Milan, Italy.  "Aortic complications after bicuspid aortic valve replacement: long-term results", by CF Russo, et.al., The Annals of Thoracic Surgery, 2002 2002 - Also in 2002, a BAVD paper was published from the University of Toronto. "Clinical and Pathophysiological Implications of a Bicuspid Aortic Valve" by P Fedak, et.al., Circulation 2002 2004 - A paper from Cincinnati Children's Hospital presents a study of the inheritance of BAVD. In the conclusion section, the first sentence states "The high heritability of BAV suggests that in this study population BAV determination is almost entirely genetic." "Bicuspid aortic valve is heritable", by L Cripe, et.al., Journal of the American College of Cardiology, July 7, 2004 2006 - The American College of Cardiology/American Heart Association Guidelines for Management of Valvular Heart Disease include bicuspid aortic valve and ascending aortic aneurysm. This is mentioned in a paper entitled "Approach to the patient with bicuspid aortic valve and ascending aorta aneurysm" from Northwestern University Fineberg School of Medicine in Chicago , published in Current Treatment Options of Cardiovascular Medicine, December 2006  2010 - First Guidelines for Diagnosis and Management of Patients with Thoracic Aortic Disease published. 2010 - Wouter Schievink et.al., publish “Screening for intracranial aneurysms in patients with bicuspid aortic valve”. 2012 - Schievink and Raissi publish “Spontaneous intracranial hypotension in patients with bicuspid aortic valve”.
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