Bicuspid Aortic Valve Disease - What is it? Something simple? Something
new?
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.
Recently someone asked when the first papers about BAVD were published. The
bicuspid aortic valve and its complications are not a phenomenon of modern life.
Many of us 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
online in
PubMed, but listings of publications about BAVD from the later 1900's are there. Searching
PubMed recently for "Bicuspid Aortic Valve" produced a list of 716 papers
from 1952 until the present. The vast majority, 659 papers, from that list
have been published since 1980. Since 1990, 545 of the listed papers were published,
79 of them between January and November of 2006 .
Following
are a few papers that I have listed as milestones in the history of bicuspid
aortic valve disease, as recorded in the medical literature.
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 cites 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 adut 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)
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.
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 has brought the ability to "see" into 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.
1975 - Coractation 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.
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.
1998 - I will always be very grateful to someone I have never met. His name is Dr. John
Burks, and along with others he published a paper in 1998 about bicuspid aortic
valves in two young men who were his patients. In 2001, I was searching for
information following the diagnosis of my husband's ascending aortic aneurysm.
When I searched the Internet for "bicuspid aortic valve" and "aneurysm", the abstract
of Dr. Burks' paper came up. From the abstract alone, I realized that my
husband's aneurysm was related to being born with a bicuspid aortic valve, even
though the BAV had been replaced in 1990. Following my husband's aneurysm surgery,
I wrote to thank Dr. Burks and requested a copy of the entire paper. I count
this paper among my treasures. ("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 at his hospital in Milan, Italy. The fate of these individuals was followed
through 2001. Of the 50 BAV patients studied, 5 had acute aortic dissection
and there were 7 sudden deaths. Very large (greater than 6 cm) ascending aneurysms
were discovered in 3, who underwent surgery. It also mentions that many
of the acute aortic syndrome events or unexplained deaths occured 10 to 15 years after the valve was replaced.
This paper recommends dealing with the ascending aorta when AVR is done for BAV,
regardless of the size of the ascending aorta. Very importantly, it establishes
the need for careful monitoring following replacement of the bicuspid aortic
valve. ("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.
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 disease continues to adversely affect individuals and families.
It apparently has done so for centuries. We cannot change the past - the lives
lost, the injury suffered - but we can change the future, beginning from today!
As 2006 draws to a close and you consider where your charitable dollars
can make a difference, I invite you to join in our mission. Educational events
and materials for patients, families, and physicians are being planned for
2007. A donation in any amount will help make them a reality.
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For families and medical professionals,
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Creating a climate of hope, an atmosphere of caring, and a source of information for
all who are touched by thoracic aortic disease and the medical professionals
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