Bicuspid Aortic Valve and Ascending Aortic Aneurysm
My husband Jim and I live in southern California, where we share our home with a seven-year-old parrot named Petie. He is an African Grey Timneh, about the size of a dove with soft grey feathers and a maroon tail. We describe him as love in feathers. When his little feet wrap around my finger as he perches on my hand, or he charmingly says "Hi Sweetheart", I can't help but think it must have been some sweet bird like Petie that inspired Emily Dickinson's description of hope!
Hope is a thing with feathers
That perches in the soul
And sings the tune
Without the words
And never stops at all.
~Emily Dickinson~
30 November 2001
Reflecting on my husband's experience, we realize that because of the advancement of medical science there has always been hope. At birth he was slightly blue and had a slight heart murmur. In childhood rheumatic fever again drew attention to a pronounced heart murmur. In his late forties pneumonia and congestive heart failure preceded surgery to replace his aortic valve. We were assured he was "fixed" then! But eleven years later, an aortic aneurysm meant that his chest must be opened again. Thankfully at each critical point there was hope and help. Coming through two heart surgeries successfully, we found that hope turned into joy, almost as though "a thing with feathers" took wing and soared within. But if hope had slipped away during or after those surgeries, I knew that somehow comfort would have come and gently perched where hope had been. Perhaps while reading this narrative a feathered thing called hope will softly perch in the soul and sing to those who need it. May you have hope today, and joy tomorrow. And should it be that the very best of medicine and surgery can no longer bring hope, may comfort gently take its place.
My husband's aneurysm experience began when an echocardiogram intended to check the condition of his artificial aortic valve discovered something else - an ascending aortic aneurysm of about 5.0 cm. The mechanical valve in place since the failure of his bicuspid aortic valve appeared to be fine. There had been no symptoms to indicate the presence of the aneurysm. We realize in retrospect how very fortunate this discovery was, providing an opportunity to deal with the aneurysm in a non-emergency setting.
A CT scan was scheduled at the local hospital, and I turned to the Internet in search of answers and help! It became apparent almost immediately that we needed to go beyond the local medical community with this condition. While not dwelling on the issues we faced locally, I will say that we quickly learned we needed to take responsibility for finding the expertise and help we needed. When the aneurysm was found, my husband's blood pressure was very high, which had not been the case previously. That high pressure on the aneurysm was a concern, and we felt desperate to find the right expertise. The Internet was a wonderful ally in compiling a list of surgeons and major medical centers performing aortic surgery.
From that list, we made an appointment for a consultation with Dr. Sharo Raissi, a cardio-thoracic surgeon at Cedars-Sinai in Los Angeles. We went off to the consultation with medical records, a list of questions based on reading the medical literature, and a lot of apprehension about what would happen. During the consult, it was unnecessary to ask most of the questions on the list -- they were covered in the course of the discussion. The assessment and approach to dealing with the aneurysm made us very comfortable. Dr. Raissi prescribed medication to control the blood pressure, emphasizing that it should be started that very night. We began to feel tremendously relieved and thankful. We had come to the right place! (I had contacted one other medical center prior to this, and knew from the phone experience alone that it was not the right place for us.)
Surgery was projected to occur within six months. Dr. Raissi explained that it was important to lower the blood pressure first. Doing so would make things go much better during and after surgery. Diagnostic tests were done prior to surgery. An adenosine-thallium test to profile the coronary arteries revealed that they were free of blockage. (These noninvasive tests were done in lieu of an angiogram. They do not expose the fragile aortic tissue to any risk of trauma.) An MRI was done three months after the CT scan, and surgery to remove the aneurysm was performed one month later.
In facing a second heart surgery, there are both advantages and disadvantages in knowing what to expect. This second surgery was an excellent experience, far exceeding our expectations. If we could have designed the ideal surgery, this would have been it. Everything went perfectly. During surgery, the decision was made to leave the existing artificial valve in place. The aneurysm began above the coronary arteries, so they were not involved. The dilated aortic tissue was replaced with a Dacron graft. My husband awoke from the anesthesia quickly and was fully alert, did amazingly well even in ICU, and remarkably was discharged from the hospital on the third morning after surgery. Healing went well at home, and he has returned to work and a normal life. Coumadin and PT INR measurements had become part of life along with the artificial valve. Now in addition, he continues on blood pressure medication, keeping the systolic average below 120. A baseline MRI was taken after surgery and will be repeated in one year.
What were some of the key contributors to this experience?
Researching the condition -
The Internet was not available to help in preparing for that first
surgery, but this time it made such a difference. The first
publication found that was very enlightening was an abstract of a
paper by Dr. John M. Burks, et al., published in Clinical
Cardiology in 1998. It describes the association between bicuspid
aortic valves and the risk of aortic aneurysm (regardless of
whether or not the valve has been replaced) and the need for
medical management with beta blockers. This explained the reason
for my husband's aneurysm. I later obtained a copy of the entire
paper, which also makes the case for proactively monitoring the
aorta of bicuspid aortic valve patients via echocardiogram.
Everyone with a bicuspid aortic valve should know about this paper
- that knowledge could make a life-saving difference. I found many
papers published by cardiac surgeons and the web sites of various
medical centers to be very helpful. Reading all this initially
facilitated our search for help, and meant a great deal through out
the entire experience.
Expertise of the surgical team -
Many comprised the surgical team along with Dr. Raissi, only some
of whom we are aware. A hospital staff member later told us that
the anesthesiologist had been a cardiologist prior to going into
anesthesiology. What a great combination for this surgery! An
expert was called in to help with the assessment of the artificial
valve. Nurses and others each had their role. I appreciated so much
the nurse who kept me updated during surgery and accompanied me to
ICU for that first glimpse of my husband after surgery. All I can
say is that he looked amazingly good that first time I saw him
(compared to his first surgery), which in itself is a tribute to
the team's excellence. It seemed ironic that on the very day of the
surgical consultation, the local paper carried an article headlined
"Mental Decline Common After Heart Surgery". It described a study
published that day in the New England Journal of Medicine. The
sub-title stated that 40 per cent of those hooked up to a
heart-lung machine experience a 20 per cent decline in brainpower.
Perhaps the greatest fear one has is emerging from surgery with
diminished mental capacity. The heart-lung machine is a necessity
for this surgery, but we felt concern about its affect. What we did
not realize or appreciate until recently is that there is an even
greater need for brain protection in this aortic surgery. While we
did know that aortic arch aneurysm surgery requires specialized
techniques to protect the brain, we did not know that HCA, or
hypothermic circulatory arrest, was also used in this case, while
connecting the ascending graft to the arch without clamping the
aorta. (In my husband's case, a small amount of graft also extended
beneath the arch.) Essentially during this time, while the body and
especially the brain are very cold, and metabolic activity slowed,
the heart lung machine is turned off. This is safe for only a
limited time, according to a clinical study up to 40 minutes.
During my husband's surgery, this crucial HCA time, when the
heart-lung machine was off, was just 23 minutes. He experienced
absolutely no adverse affects mentally. We cannot begin to imagine
all that took place prior to and during those very crucial minutes,
but are very grateful for the judgment and skill that made it
happen.
Overall health of the patient -
My husband was not allowed to participate in athletics as a child
due to his heart murmur. On his own, he began lifting weights - and
continued to do so through out his life. Following the first heart
surgery, with the new artificial valve there were no restrictions
placed on his physical activity, and in the years following he
continued to lift weights. (At that time, of course, there was no
recognition of any risk of aortic aneurysm.) Consequently, he was
in excellent physical condition at the time the aneurysm was found.
From that point on, heavy lifting was not and still is not allowed.
We fortunately found a local internist who assisted in getting
ready for the surgery. He suggested walking an hour seven days a
week. This local doctor focused on strengthening the immune system
and providing overall nutritional support. We planned the surgery
date carefully. My husband continued to work, but just prior to
surgery he took time off in order to be well rested. Very
importantly, by this time his blood pressure was right where Dr.
Raissi wanted it. The net of all this was that my husband went into
surgery in excellent condition apart from the aneurysm.
Relationship with the surgical team
-
In a non-emergency situation, there is sufficient time and
opportunity for a trusting relationship to develop with the
surgeon. Ideally, the surgeon and patient work together toward the
common goal of the best possible surgical result. Positive contacts
with the administrative staff and various other hospital
departments also are very reassuring. In the months prior to
surgery, the trust that began during the consultation with Dr.
Raissi deepened in the course of taking the diagnostic tests at the
hospital, controlling the hypertension, having questions answered
as they arose, and setting the surgery date. Going into heart
surgery with that type of relationship is priceless.
Before I met my husband, the life of a dear friend ended abruptly when a cerebral aneurysm ruptured. It was tragic, particularly for her grieving husband and little boy. Since then the word aneurysm has always been tinged with something akin to terror for me. Perhaps partially because of that, the time interval between receiving the aneurysm diagnosis and finding Dr. Raissi was the most difficult part of the entire experience emotionally for me - a time spent desperately searching for information, uncertain of where to find help. It was only about two weeks but seemed much longer. If that was the worst part, what was the best? The best part is just being where we are today! It is wonderful to again live a normal life - a precious gift given to us by the removal of the aneurysm. We are very thankful for the advances in cardio-thoracic surgery that have made this possible. Our hearts go out to everyone whose lives are touched by aortic disease, and it is our sincere desire that this account may in some way give hope to others.
Update: 3 Sep 2002
Perhaps it would be helpful to share the outcome of my husband's last MRI. Shortly after the first anniversary of his aortic surgery it was time for a follow up MRI. Having once received a diagnosis of an aortic aneurysm, one is no longer innocent; there will always be distrust of the remaining aortic tissue. And so despite having many reasons to believe that the MRI results should be good, it was still a relief to actually be told that all is well. Everything inside - the graft, aorta, and heart itself - all were described as "beautiful" - so much so that it is safe to wait two years until the next MRI. The opportunity to prepare for and have surgery electively, the judgment and skill of the surgeon, ongoing me dical management (beta blocker and other hypertension medications), and giving up heavy weight lifting all have contributed to this first post surgical year being such a good one, for which we are very thankful.
Reading a little of the history of aortic surgery has given me an appreciation for how far thoracic aortic surgery has come. Dr. Larry Stephenson's book, State of the Heart, begins with a chapter on the history of heart surgery. Then in the chapter on aneurysms there is the story of Dr. Michael DeBakey, whose mother had taught him to sew, buying a new fabric called Dacron at a department store and creating tubes from it on his wife's sewing machine. These first Dacron grafts, used for experiments in animals, were the beginnings of the Dacron graft in my husband's chest today. He would have been a child at the time of Dr. DeBakey's experiments. Some who have Dacron grafts today were not yet born when Dr. DeBakey placed the first one in a human being in 1954. Everything learned from all the subsequent aortic surgeries has contributed to my husband's successful treatment and recovery. Much of the progress is fairly recent. At the end of the chapter, Dr. Kouchoukos writes of the "extraordinary progress" made in just the last decade in the treatment of thoracic aneurysms. The successes in aortic surgery today stand in tribute to all the dedicated surgeons and their patients who have gone before.
And yet there remains more to be done. The hope is that someday others will reap the benefit of advancements still to be made, beyond what is possible today. Those with aortic disease are conscripted into the fight not only for their own lives, but the struggle for a better future for the generations still to come. The narratives on this web site, the emails we receive, additional research and new publications in the medical literature all assure me that there are those still rising to the challenge.
Update: 05 Mar 2004
There is a second verse to Emily Dickinson's poem about hope:
And sweetest in the gale is heard
And sore must be the storm
That could abash the little bird
That kept so many warm."
Hope may be a little bird, but it cannot be a timid thing... it is called upon to sing to us in the most dire moments of our lives. Those diagnosed with aneurysms and their families need this strong but gentle little bird with them when they receive that diagnosis. There is hope in the skilled hands of expert aneurysm surgeons. Recently I have learned that includes the hands of the neurosurgeon.
In January I walked into recovery for a glimpse of my dear friend following cerebral aneurysm surgery. (Yes, she also has a bicuspid valve and an ascending aortic aneurysm that are stable and being "watched".) Roughly a month earlier I was with her when the neurosurgeon told her a cerebral CT angio had revealed an aneurysm in her brain. (We felt instant denial. How could it be? She has an aneurysm in her chest, surely not in her brain too!) She bravely set the date for surgery as soon as possible, a wise and courageous decision. When I later saw the picture of her aneurysm and her neurosurgeon pointed out the thinness of that bulging vessel wall, I was very thankful she had not delayed.
It may be hard to fathom that bicuspid aortic disease also may involve brain aneurysm and dissection. Seeing the before and after pictures of my friend's cerebral aneurysm surgery was very convincing. The CT angio had revealed one aneurysm, but in surgery another tiny one was found right next to the larger one. Proactive cerebral screening saved her life! Dr. Wouter Schievink, of Cedars-Sinai's Neurosurgical Institute, who specializes in brain aneurysms related to familial and connective tissue disorders, performed her surgery. Yes, there is hope and help in expert hands. For those interested in the technical aspects, Dr. Schievink has published his work regarding the relationship between bicuspid aortic valve disease, as well as other connective tissue disorders, and cerebral aneurysm/dissection in the medical literature.
In early January, the brain scan done on a young wife and mother (who had bicuspid aortic valve and aortic aneurysm surgery fourteen months earlier) revealed a tumor. She was scanned after hearing of our friend's cerebral aneurysm diagnosis. Hearing the news from her over the phone was such a shock. Somehow as we continued to talk we heard that little bird singing, telling us it had been found early and it would be all right. Thankfully it was later determined to be a more benign form than originally thought, and can be monitored, at least for now. Whether or not her brain tumor is in any way related to her connective tissue condition is unclear. We find a great deal of hope and comfort in knowing she is under the care of an expert neurosurgeon who is amazingly located near her home.
This narrative began with my husband's bicuspid aortic valve and ascending aortic aneurysm. From the above you would understand why he has also been screened for brain aneurysms. Having come this far with his valve and his aorta, we certainly do not want to be surprised by an aneurysm silently lurking in his brain. My husband's cerebral MRA came back just fine...there were no aneurysms in his head. He continues to feel well, maintains control of his blood pressure, and keeps busy both at work and home. He will continue to be monitored for aneurysms. He is due for an MRI of his chest this summer. Thanks to expert care, he lives a normal, active life. Medical innovation first gave him a new valve in place of the defective one he had at birth and later a Dacron graft in exchange for his aneurysmal aorta.
Not everyone with aortic disease will develop a cerebral aneurysm, but just as with any aneurysm, detecting it gives you the opportunity to do something about it. While an aneurysm may develop in any blood vessel, they occur either in the brain or the aorta most commonly. It may seem unthinkable to have them in multiple places in the body, but it is certainly possible. It is also possible to proactively detect and successfully treat them. Aneurysms form over time, and one might speculate that in successfully removing an aneurysm, life is extended beyond what it has been in prior generations. And with additional time, aneurysms may develop else where. This is not meant to frighten, but to encourage proactive screening of the brain and the aorta for those at risk.
Three years ago when my husband's aortic aneurysm was diagnosed, I could certainly not see ahead to where we are today. At that time it was a major revelation that bicuspid aortic valves could be more than just a valve problem. Today we understand the wisdom in continuing to monitor both his aorta and his brain. When I think back to the time prior to his diagnosis now, I marvel that he was spared aortic dissection or rupture. Why do some aortas dissect and others remain intact at the same size? For now at least, it is a question that is difficult to answer, and those with the opportunity for elective surgery today are fortunate that in expert centers it can be done safely at smaller sizes than ever before.
Not long ago I held the hand of an aortic dissection survivor, still recovering in intensive care, whose aneurysmal aorta tore when just slightly larger than my husband's had been. The bicuspid valve and enlarged aorta had been silently there, never diagnosed. There were no prior symptoms, no warning. Just sudden excruciating pain, paramedics, a rush to the hospital, transfer to another hospital, and life saving surgery. Words like miracle come to mind, because we know it does not always end this way. For those in the wrong place at the wrong time, there is no time, there are no expert hands, and there is no hope.
It should not take a miracle for those with aneurysmal disease to live safely. Careful medical histories can proactively identify the risk factors that now too often are recognized after the fact. And when there is any hint of risk, diagnostic testing and medical treatment can be used to monitor and treat aneurysm development, followed by properly timed, expert aortic surgery. Those with a known aortic condition should never need to visit the emergency room.
I am very privileged to be involved with Dr. Raissi's Aortic Surgery Program. I appreciate so much Bill Maples' kindness in sharing the link to the Cedars-Sinai Thoracic Aortic Surgery web pages here. These pages have sprung from the conviction that information is vital to those dealing with aortic disease. Finding accurate information gave me the first glimmer of hope in a very bleak picture, colored with terrifying memories of the death of my friend years before. Hope grounded in knowledge accompanied us as we put my husband's life into Dr. Raissi's expert hands.
I've heard it in the chillest land,
And on the strangest sea;
Yet, never, in extremity,
It asked a crumb of me."
This last verse of Emily's poem refers to the "chillest land" and "strangest sea". Those who receive an aneurysm diagnosis find themselves there, where fear, panic, disbelief, anger, and shock live. Those words "you have an aneurysm" bring these negative emotions flooding in to rob and deplete emotionally. It is so difficult to think and plan. That is why it is important to find hope as soon as possible. Hope never asks anything of us. Hope only gives; it never takes. May you always hear a little bird singing; may you find the expert help you need.
Editorial note: Arlys and her husband's surgeon, Dr. Sharo Raissi, have erected a very professional addition, "Thoracic Aortic Surgery Program" to the "Cedars-Sinai Health System" website. It is well worth visiting.
Arlys is founder and Chair of the Los Angeles, CA based "Bicuspid Aortic Foundation"
Discussion, comments, or questions: Arlys Velebir
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