Tuesday, December 15, 2015
Thursday, December 10, 2015
Monday, July 27, 2015
Summary 1. Left ventricle: Size was normal. Systolic function was normal by visual assessment. Ejection fraction was estimated in the range of 55 % to 60 %. There were no regional wall motion abnormalities. There was mild concentric hypertrophy. Left ventricular diastolic function parameters were normal. 2. Aortic valve: A bioprosthesis was present. It exhibited normal function and normal motion. The leaflets appeared normal. There was a normal-appearing sewing ring and no rocking motion of the sewing ring. There was trivial aortic regurgitation. There was no significant perivalvular aortic regurgitation. Valve peak gradient was 12 mmHg. Valve mean gradient was 7 mmHg. The aortic valve obstructive index (by VTI) was 0.68. 3. Aorta, systemic arteries: The root exhibited mild dilatation, but represents proximal aortic graft after reconstruction of the proximal ascending aorta.
Tuesday, July 21, 2015
Monday, July 20, 2015
I attended 10 cardiac rehab sessions. I think they were useful for a couple of reasons. First, you are monitored with a running EKG in progress while you exercise. This is reassuring as you exercise and begin to test your exercise tolerance limits. Second, attending rehab gets you into a regular routine of cardiovascular exercise designed to improve your fitness level. This is particularly important for individuals who would not otherwise exercise. Since I already exercise daily, and the trek and expense to rehab was significant, I decided to withdraw from the program after 10 sessions. I do plan to resume workouts that elevate my heart rate into the target range for me. This will be in addition to my walking and my workouts with weights combined with stretching. It's important to keep active as we age to avoid the trap of becoming frail prematurely.
Sunday, July 12, 2015
Friday, July 10, 2015
I was in Philadelphia last week for my first post-operation medical evaluation. My first post-op echocardiogram since discharge was completed and I met with my cardiologist. Everything looks very good. My new aortic tissue valve is performing beautifully with no leakage and low gradients. This is good news. MY next appointment with my cardiologist is set for January, 2017. I am continuing cardiac rehabilitation, walking, and working with weights combined with stretching. The idea is to ward off becoming frail with age.
Tuesday, June 23, 2015
Friday, June 19, 2015
Photo: The Cardiac Rehabilitation Center at Brookhaven Hospital on Long Island
I'm keeping busy with summertime activities while working on my recovery from surgery. I've started monitored cardiac rehabilitation sessions at Brookhaven Hospital here as well as an exercise program on my own with light weightlifting. I hope to improve my overall fitness level so that I can continue scuba diving and stay healthy generally. At 66 it's getting tougher to stay fit enough for some of what I want to do. But I can't complain. I'm lucky to be alive.
Saturday, June 13, 2015
In addition to my daily walks, I've started an exercise program that incorporates some exercises recommended in a recent issue of the AARP newsletter...stretching, body weight squats, touch your toes, etc. I'm combining that with a couple of exercises recommended by my scuba diving insurance company that are designed to improve upper body strength. I use two 20 lb hexagonal dumbells. The two exercises are pullups and pushups both done from a bent-knee position in the deck. Then I add some overhead lifts with the dumbbell one arm at a time, and some curls. I'm hoping that the body weight squats help improve the muscle tone in my left leg, which is somewhat atrophied due to a long ago bout of sciatica and arthritis in the knee. I'm trying real hard to stick to my low carb diet. My weight is good at about 190, down slightly from where I've been in recent months after my surgery. I stopped Warfarin last Tuesday with the blessing of my cardiologist so now the only pill is a daily aspirin. That made me happy. I have my first post op echo in a couple of weeks, but the valve seems really good. I have no palpitations or other symptoms and it really pounds shut. I know it's working, that's for sure! Finally, Monday I start cardiac rehab. I'm getting a late start due to the move to the summer cottage but I at least want to try some sessions to see if they are helpful.
Wednesday, June 10, 2015
Three and a half months after my surgery I have stopped with the last of my prescription medications...Warfarin. My only pill now is a daily aspirin. I'm feeling physically good,I've started to lift light weights and do exercises and stretching, and I am scheduled to start cardiac rehabilitation next Monday. I am still depressed over the loss of our dog Bradley. The problem is that the death of the dog happened at the same time as my surgery and so the two events became intertwined for me. Therefore I don't feel like I will ever be fully recovered because I can't bring Bradley back to life. This is unfortunate because my recovery has been so successful otherwise. I don't know what the answer is. Getting another dog might help but I can't expect another animal to be like the Bradley.
Monday, May 25, 2015
Friday, May 8, 2015
Photo: Spring on Fire Island: Beach Plum in Bloom
I am doing a lot of walking. Next week I wear a Holter for a day (three months post-surgery. Hopefully I can get off the warfarin and amiodarone if there are no abnormalities. We'll see. Feeling good with no medical issues. Battling delayed grief and depression over the loss of my dog companion. For two months I concentrated on getting myself better, but now that I'm feeling recovered, the pain of loosing him is overwhelming at times. He was put down while I was in the hospital so closure is difficult. If I had it to do over again I would have tried to find a way to keep him alive longer. He was old and failing, but I didn't realize how much delayed mental pain over him I would have later on. But what's done is done and I'll have to cope.
Tuesday, April 14, 2015
Wednesday, March 25, 2015
Saturday, March 21, 2015
Friday, March 20, 2015
Sunday, March 8, 2015
It's been two weeks since my second of two trips within five days to the OR. Medically things seem OK, no issues with my new valve or all of my surgical repairs, heart rate or BP, etc. I just had no idea how weak I would be and how painfully slow and difficult getting any strength back at all would be. I haven't felt quite strong enough to go out of the house yet, but with all of the snow and ice and cold here in Philadelphia I couldn't go anyway, so I have a major dose of cabin fever. It's supposed to warm up this week and I have my first follow-up appointment on Tuesday so hopefully I'll feel better after i finally get out and get some fresh air.
Wednesday, March 4, 2015
Day 1: Surgery Day - February 16, 2015: After 11 years of waiting and watching the big day arrived. The night before I had to wipe myself down with special anti-bacterial wipes. I repeated the process in the morning before leaving for the hospital to check in for surgery.
When we got the the hospital at around 6 AM, Dan and I were placed in a cubicle in the pre-surgery area. Then it was time for yet another body wipe. There was the possibility that I would need to undergo DHCA (deep hypothermic circulatory arrest) with cerebral perfusion (they basically cool the body down and shut it down but keep the brain supplied with oxygen through arterial connections.) Therefore, I had electro-encephalagram contacts glued to my head. I really looked like a Franmkenstein creation.
Finally, I was ready, said godbye to Dan and I was wheeled into the OR for my procedure.
Day 2: Tuesday, February 17, 2015: I woke up in the ICU at about 2 AM, again at 4 AM and yet again at 6 AM. I was still on the ventilator. I would remain on the ventilator for 17 hours after waking up, until 7 PM in the evening.
Dan and our friend Stephen were bedside. I was awake much of the time and when I was I asked for pen and paper so that I could scribble questions and make requests. I was not in a great deal of pain, and my breathing was not encumbered by the ventilator tubes that were still deep in my throat.
My surgeon Dr. Grayson Wheatley at my bedside.
I had read somewhere that if you wake up post surgery and the vent tubes are still in, think of it like breathing through a scuba regulator. Since I am a scuba diver this worked well for me and I did not fight it or try to yank out the tubes. I couldn't have done that anyway since my arms were restrained. I was on the ventilator that long (17 houus) due to the length of my surgery (7 hours,) from 9 AM until 4 PM, and the complexity of the procedures performed resulted in the need for me to have an inter-aortic balloon pump installed to augment heart function post-surgery.
I felt generally pretty well for just having awaken from surgery, and I was finally taken off of the ventilator and the tubes were removed at about 7 PM.
Day 3 and Day 4, February 18 & 19th, 2015: I remained in the cardiac intensive care unit. Whie I felt good the day after surgery, I began to feel worse during this time. I was unable to get out of bed. I had zero energy. I felt like I was in declinbe and I frequently voiced my concerns about exhaustion to my caregivers.
Day 5, February 20, 2015: At 6 Am my nurses decided to have me get out of bed finally and sit in a chair. I tried as hard as I could with their assistance but I only made a few steps in the direction of the chair when I passed out cold. I awoke surrounded by a team of nurses with one of them using a breathing bag on me. It was clear that something was amiss. I was scheduled for a heart catheterizaton later in the day. After the cath it was decided that I would require a return trip to the operating room. I went into surgery at about 7 PM that evening for a condition known as cardiac tamponade, where fluid a clots develop around the heart post-surgery and prevent it from functioning fuly. Thankfully the procedure was relatively short and it was succesful. I was returned to my room in the cardiac ICU.
March 2, 2015 Just completed a 30 minute walk in the house sans walker. It wasn't that long ago that I couldn't even get out of bed. I have some puffiness in the ankles, but other than that I have no complaints at all. I haven't taken a pain medication in a week. If I can continue to improve a little at a time like this I'm optimistic that I'll be walking outside soon...if Old Man Winter will give us a break here in the Northeast. Day 13: Discharge To Home! Journal posted on February 28, 2015 My stamina all of a sudden leaped to the point where I can walk a lot and even climb steps! I never thought I'd get back to this point. So the insurance denial doesn't matter...the hospital has cleared me to go home later today! Tears of happiness I gotta tell ya. Day 12: Cardiac Floor Journal posted on February 27, 2015 Waited all day today for insurance approval to enter acute rehab only to be denied. So the next option is to enter sub acute rehab at a different facility maybe as early as tomorrow. I am medically stable and feeling good just not strong enough to go home. Day 11: Cardiac Floor Journal posted on February 26, 2015 My Afib spontaneously converted to normal sinus rhythm last night so I didn't need the dc cardioversion. May enter acute rehab tomorrow or Saturday. It's been a long, tough haul. Day 10: Cardiac Care Journal posted on February 25, 2015 Spent some time with physical therapy today. Bowels are working finally. Afib and high heart rate are issues the doctors are working on. Will be making arrangements to go to a rehab facility upon discharge. Sorry I cannot be as actively supportive of everyone here but you are all in my thoughts. Day 9: ICU Journal posted on February 24, 2015 Well,.tubes are out and I am medically stable, I think. The Afib that I developed when they took me off Amiodarone due to liver concerns continues to rock the boat and is very noticeable. I have zero strength and get winded at any activity. I met with physical therapy today and they are recommending discharge to a rehab facility. I know I can't go home in my current state if extreme weakness. Thanks everyone for the continued love and support. I am glad this thing is not ad bad for most of you as it has been for me. Day 8: ICU Journal posted on February 23, 2015 Thanks everyone for all of the support. My numbers are improving. Two chest tubes were removed this morning...two remain. My swan neck arterial line from Fridays second surgery is supposed to be removed today also. No appetite and taking meds to get my bowels to move. But slow progress is being made I think. Patience is necessary. Update Sunday Feb 22 Journal posted on February 22, 2015 Still in ICU day 7...trying to get heart function improved...outlook trying to keep positive...at least one more day then maybe I can try to sit again...thanks for keeping me in your thoughts Journal posted on February 21, 2015 Still in the ICU since Monday. Had a second full steroenotomy Friday to relieve fluid backup behind heart Original surgery was 7 hours valve, root, ascending and two cabg...been a tough week...hope I have turned the corner
Sunday, February 15, 2015
PHOTO: Happy listening to the Wanamaker Organ
My surgery is tomorrow. It's been a long time coming. People often ask me "how do you feel?" Or they ask "how are you handling this?" Or, "how do you keep your spirits up, stay positive?" I usually respond that I feel fine and that having a wonderful husband, pets and a great life keeps me positive.
Dan points out that this whole thing, finally getting to the point of needing surgery, hasn't happened the way we thought it would. We figured I'd get progressively weaker and symptomatic, and that one day I would collapse like a friend did when his aortic valve went bad. But that didn't happen in my case. I think of my experience as more than a little surreal. This is because, with the exception of my serious episode last December 9 and a few lesser episodes over the moths and years, I usually feel just fine with minimal symptoms. I can still lift heavy things, walk five to six miles a day, and in short, do just about everything I want. I get a little winded once in a while climbing steps, but other than that I feel perfectly normal. So yes, it feels like I'm in some sort of surreal dream wherein I'm fine, a dream that never ends ... but I have to get this heavy duty operation anyway, that will rob me, (hopefully only temporarily,) of the strength and conditioning that I've worked so hard to maintain into my senior years.
There's a hill that I climb every day on my morning walk on my way back to the house. I've always said to myself that the day I can't climb this hill will be the day that I need to have surgery. But that's not the way it worked out. I can still climb the hill today but I won't be able to do it (for a while) after surgery. So now I say to myself, the day that I'm able to once again climb that hill...that will be the day when I'll feel recovered.
Saturday, February 14, 2015
Friday, February 13, 2015
PHOTO: Underwater selfie in St.Barth
I am in a great place (mentally) all set for Monday...no worries, bring it on. I have a busy weekend with a social event Friday evening, and Saturday and Sunday performances to attend. And I can drink my Sake until Saturday evening, how great is that? Finally, my weight...194.2 down 2.0 since last week and I made my target weight for surgery of 195.0. I have asked Dan to take many photos on Monday and Tuesday and to bring me my laptop as soon as I am out of the ICU. But who knows what condition I'll be in or whether I'll want to deal with it. We'll see. It's been quite a ride these past 11 years since my diagnosis. But like Sondhiem wrote ....I'm still here.
Thursday, February 12, 2015
Wednesday, February 11, 2015
PHOTO: "Sternal Lok Blu" Rigid Sternal Fixation
Today was pretty intense. First I met with my surgeon's right hand man, a nurse-practitioner named Bill. Bill has been very helpful since I first met with him. Today he answered the follow-up questions that I had such as about the total number of incisions I would wake up with post surgery (7 or 8 including three or four drainage tubes, two regular IVs, an arterial IV, and a neck incision for a cardiac catheter!), what medications I would be on, probably at least six including a water pill, something for my bowels, a pain killer, a beta blocker for blood pressure medication, aspirin, and coumadin. I also asked Bill about the relative complexity of my procedure. On a scale of 1 to 10 with 10 being highly complex, my procedure a "Button Bentall" is an 8 or a 9. That's why you go to a major university center like Temple or Penn, a heart center of excellence, to get this type of surgery. Bill also explained the timings that my husband Dan can expect for my procedure. The operation will last between six and eight hours including prep time and post surgery housekeeping before I will be transferred to the ICU. If my procedure starts as scheduled early Monday, Dan can expect to get a report from my surgeon Dr. Wheatley sometime between about 1 PM and 3 PM. Bill also commented on the general anesthesia used. Two different agents are administered. One knocks you out and the other paralyzes you.
After meeting with Bill I met with my surgeon Dr. Wheatley. By this point in the process most of my questions had already been answered. I did disucss the possibility that I might require a permanent pacemaker post surgery. Dr. Wheatley told me that there is only about a 10 percent chance of that. Since I blog about BAV disease and aneurysms and my own situation, I might want to write further about it at some point. In this connection i asked Dr. Wheatley if he planned to take photos of my procedure. He indicated that he did not do this routinely but he would try to make some media available for me.
After meeting with Dr. Wheatley it was time for pre-admission testing. This included an examination by a nurse-practitioner, blood work, and chest X-rays. I got anti-bacterial body wipes that I must use the night before my surgery and also on the morning of the surgery. I also asked if I could see the cardiac intensive care facility and the step down unit, but apparently Temple Hospital does not allow casual visitors to these facilities.
Finally, Dr. Wheatley asked me if I would be interested in participating in an evaluation of an alternative sternal closure device, "Sternal Lok Blu," which are metal plates screwed to the sternum instead of wires. Dr. Wheatley feels that this method is superior to the wires. I agreed but since it is a study only half of the participants will receive the rigid sternal fixation devices and the other half will get the usual closure with wires. I'm hoping to be one of the lucky recipients of the Sternal Lok Blu closures. I won't know until after the study ends, though, in about a year.
So now I have only four days before surgery happens. The train has left the station and I'm on board. There's no getting off now.
Tuesday, February 10, 2015
Monday, February 9, 2015
Saturday, February 7, 2015
Friday, February 6, 2015
Thursday, February 5, 2015
Tuesday, February 3, 2015
Photo: no scar...yet!
My current weight: 197.0
From the internet:
Based on the Robinson formula (1983), your ideal weight is 185.8 lbs
Based on the Miller formula (1983), your ideal weight is 176.7 lbs
Based on the Devine formula (1974), your ideal weight is 196.4 lbs
Based on the Hamwi formula (1964), your ideal weight is 207.0 lbs
Based on the healthy BMI recommendation, your recommended weight is 156.0 lbs - 210.8 lbs
Monday, February 2, 2015
Sunday, February 1, 2015
What's a little "kink" amongst friends? (Part 2) http://badaorta.com/
Saturday, January 31, 2015
Friday, January 30, 2015
Thursday, January 29, 2015
Wednesday, January 28, 2015
With regard to the tissue valves, there is growing evidence that a TAVR inside a tissue valve is a very realistic and safe option in the future. New data suggests that you do not decrease the size of the valve with the TAVR valve in valve, and can put multiple TAVRs inside valves over time as needed. This is all preliminary work but the evidence is mounting.
Tuesday, January 27, 2015
Sunday, January 25, 2015
Saturday, January 24, 2015
The Modified or "Button" Bentall is a relatively complex surgical procedure wherein the aortic root is replaced with a graft. This necessitates removing the coronary arteries and re-atttaching them to the aortic graft. Complications can arise particulalry at the sites where the coronary arteries are sewn into the graft, known as the coronary anastamoses. However, this study notes that the incidence of such complications is extremely low.
Ann Thorac Surg. 2003 Jun;75(6):1797-801; discussion 1802. Fate of coronary ostial anastomoses after the modified Bentall procedure. Milano AD1, Pratali S, Mecozzi G, Boraschi P, Braccini G, Magagnini E, Bortolotti U.
CONCLUSIONS: The modified Bentall operation is associated with an extremely low incidence of anastomotic complications particularly at the coronary ostia. More extensive use of new imaging techniques is desirable to assess the true incidence of such complications in patients receiving a composite aortic conduit.
Friday, January 23, 2015
Designed for outstanding performance in all three areas of hemodynamics, durability and implantability, the Trifecta valve demonstrates our passion for putting more control into the hands of physicians.
When the Goal Is Hemodynamics Created exclusively for the aortic position, the Trifecta valve delivers larger EOAs, resulting in single-digit pressure gradients.1 The Trifecta valve is designed to mimic the flow of a natural, healthy heart valve and offers excellent hemodynamic performance, which may provide patients with an improved quality of life.
Thursday, January 22, 2015
Patients who have undergone valve replacement are not cured but still have serious heart disease. Patients have exchanged native valve disease for prosthetic valve disease and must be followed with the same care as those with native valve disease.
Reoperation after bioprosthetic aortic valve replacement (AVR). The freedom from reoperation was determined for patients according to the age at initial AVR. These patients did not receive concomitant mitral valve replacement. A, 294 reoperations for the 3152 patients who underwent bioprosthetic AVR. B, 46 reoperations for the 2158 patients who underwent bioprosthetic AVR with contemporary, stented bioprostheses.
Wednesday, January 21, 2015
Tuesday, January 20, 2015
One of the main observations of this study was that reoperation following bioprosthestic valve replacement is influenced by patient age. Fifteen-year freedom from reoperation was 78% following AVR and 62% following MVR in patients more than 60 years of age.
A tissue valve seems like the only way to go for me at age 66.
Monday, January 19, 2015
Sunday, January 18, 2015
Saturday, January 17, 2015
1. During my surgery, will Dr. Wheatley be performing a "button" Bentall procedure or using some other technique to re-implant the coronary arteries?
A. If the coronaries need to be reimplanted, they are done with the button technique. If your aneurysm does not involve the sinus segment (where the coronaries are located) he’ll be able to replace the aorta above the coronaries – negating the need to reimplant. This final decision is made at the time of surgery.
2. Can you outline the surgical steps during my procedure once my heart is exposed? Since I agree with Dr. Wheatley's recommendation to select a St. Jude Trifecta bio prosthesis, is the valve sized first then sewn into the Dacron ascending aorta graft before being sewn into the aortic annulus all during my time on the pump? Or do these valves come in different sizes already sewn into a conduit?
A. You’ve done some good homework. This is an excellent question. Currently a “biologic composite graft” does not exist. This is a device where the valve is pre-sewn into the aortic conduit. The answer to this question depends upon the extent of the repair. Regardless of the method, the aorta is first transected (cut open) to expose the aortic valve. The damaged aortic valve is removed and then sized at this time. If the aortic root (sinus segment) is enlarged enough necessitating replacement, then this is done with a composite graft. Dr. Wheatley will sew the valve into the Dacron aortic conduit (replacement aorta) and then implant this unit into the aortic root and then reimplant the coronary buttons as in question #1. If the sinus segment is not dilated, then the valve will be sewn in the aortic annulus (into the heart) first, followed by replacement of the ascending aorta. There are three general techniques used to complete this procedure – revolving around the extent of aortic root involvement – Full root (with a composite graft), Wheat procedure, AVR/ascending repair with retention if the native sinus segment.
3. Can you estimate the total time on the pump and total time in the OR for me? I know I asked this of Dr. Wheatley but I'm a bit confused about it.
A. There are various times we record for cardiac procedures such as this – Cross clamp time, bypass time, operative time. as with any “plumbing repair” (and valve surgery is essentially a plumbing job), the water (in this case blood) needs to be turned off. To “turn off the water” we clamp the aorta (ie cross clamp). We then need to stop the heart in an effort to fix it. Once your pump (your heart) stops, we need to substitute in our pump (the heart bypass machine – also known at the heart lung machine or simply “the pump”). The length of time you are on the pump is recorded. I suspect you’ll be on the pump 2-3 hours. I usually quote the total length of surgery as 4-5 hours and total time out of Dan’s sight about 6-8 hours. I always build into this estimate an hour at the beginning to set you up and an hour at the end to dismantle the equipment: (1 hour set up) + (4-5 hours surgery) + (1 hour dismantle) = 6-8 hours.
Friday, January 16, 2015
The good news is that unlike other aortic aneurysmal pathologies (Marfan Syndrome, Ehlers-Dahnlos Syndrome, etc), where aneurysm formation THROUGHOUT the aorta is a risk at all times… the bicuspid aortopathy is limited to the root and proximal 1/3rd of the ascending aorta only. The rest of your aorta is normal, and is not at any increased risk of aneurysm formation beyond that of the general population.
Thursday, January 15, 2015
You have a bicuspid aortic valve (“congenital anomaly” - most common one - present in about 2% of human population). It turns out that not only does having a bicuspid aortic valve result in early valve disease (stenosis, regurgitation or both), but it is also associated with an abnormal weakness of the most proximal portions of the aortic “root” and ascending aorta. It’s known as “bicuspid aortopathy”. It is a form of annuloaortic ectasia, and any dilatation of the proximal aorta is usually associated with sinotubular effacement (that’s just a descriptor, not a pathology). After much population-based research, it has been determined that an aortic root or proximal ascending aortic diameter of ≥4.5 cm portends elevated risk of further dilatation and aneurysm formation over the long term. Thus, it is recommended that in bicuspid aortic valve patients whose aortas are larger than that, the proximal aortic root be replaced as well. It’s more of a gray zone below 4.5, but I’ve cared for patients who’d had smaller aortas at the time of bicuspid aortic valve replacement, and then a few years later, needed to go BACK to the OR for replacement of an ascending aortic aneurysm. That is clearly a sub-optimal situation. With you proximal ascending aortic measurement of 4.6 on your recent CTA, it is most prudent to replace both the valve, root and proximal-most portion of the ascending aorta."
Tuesday, January 13, 2015
Dr. Grayson Wheatley
After I thought about the discussion I had last week with my surgeon, Dr. Wheatley, I had a few questions so I emailed Dr. Wheatley and my cardiologist, Dr. Keane. Dr. Keane responded immediately with answers to my questions, and Dr. Wheatley confirmed the information. Later in the day I posed a few more questions to Dr. Wheatley's assistant and once again I got an immediate comprehensive response. I am very with this team that I have selected for my cardiac procedure and care.
Monday, January 12, 2015
Bicuspid Aortic Valve and Aortopathy: See the First, Then Look at the Second
Rosario V. Freeman, MD, MS; Catherine M. Otto, MD
Bicuspid aortic valve (BAV) disease is the most common congenital cardiac anomaly, with a prevalence in the general population between 0.5% and 2% (1). There is significant cardiac morbidity associated with BAV disease, predominantly due to progressive valve dysfunction (stenosis or regurgitation) that requires surgical intervention for symptom relief or prevention of left ventricular dysfunction, or less commonly, for complications of endocarditis (2,3). We now understand that BAV disease is more than simply having 2, instead of 3, aortic valve leaflets. BAV disease encompasses a spectrum of phenotypic manifestations that not only includes valve dysfunction, but also abnormalities of the ascending aorta. Less common cardiovascular abnormalities may also occur, such as aortic coarctation, atrial septal defects, and ventricular septal defects.
. Long-term cardiovascular outcomes in adults with a BAV were defined in 2 recent clinical studies. In a series of 642 asymptomatic adults with a BAV, most (63%) had normal or mildly abnormal valve function at baseline. Over an average 9 years of follow-up, about 25% required surgery for symptomatic valve disease, left ventricular dysfunction, ascending aortic dilation, or endocarditis. Independent predictors of adverse cardiovascular events were age >30 years and at least moderate aortic valve dysfunction at baseline (3). Similarly, in another series of 212 asymptomatic adults with a BAV and at most mild valve dysfunction, primary cardiac outcomes were frequent over follow-up, occurring in 42% of participants. In this study, independent predictors for primary outcomes were older age (>50 years), presence of valve degeneration at baseline, and a baseline aortic dimension of >40 mm (2). Importantly, these studies demonstrated that although the cardiac morbidity associated with a BAV is significant, overall life expectancy is not shortened relative to general population estimates. In the Olmstead County study, survival was 97% and 90% at 10 and 20 years, respectively, from diagnosis (2). Similarly, in the Toronto cohort, 10-year survival was 97% (3).
. BAV disease is not confined to the valve leaflets; the aorta also is abnormal. Compared with normal adults with a trileaflet aortic valve, BAV patients have larger dimensions of the aortic sinuses and ascending aorta, abnormal aortic elasticity, and are at risk for progressive aortic dilation and dissection (4). In the past, aortic dilation was thought to be primarily a hemodynamic consequence of the eccentric ejection jet created by the bicuspid valve. However, histopathologic studies now support an underlying connective tissue disease process with elastin fragmentation, irregularities in smooth muscle integrity, and increased collagen deposition (5). Dilation is often progressive, with an average annual change in diameter ranging from 0.2 to 1.2 cm/year (6). Risk factors for more rapid progression of aortic dilation include hypertension, male sex, concurrent valve disease, and older age. In the study by Tzemos et al. (3), 280 patients (45%) developed dilation of the aortic sinus, ascending aorta, or both at follow-up. In a subsequent publication from the Olmstead County cohort, which included 416 patients, although 53% of patients eventually required aortic valve replacement, a significant portion of patients (25%) also ultimately required surgical intervention for aortopathy (6). Because not all patients are at risk for progressive aortic dilation, the clinical challenge is in identifying which patients are at highest risk for aortic complications and might therefore require more frequent imaging evaluation.
. In this issue of iJACC, a study by Kang et al. (7) focuses on the potential value of computed tomographic angiography (CTA) to more precisely define BAV phenotypes and to characterize the associated aortopathy. Typically, bicuspid valve cusps are asymmetric with fusion along a commissural line, which creates 2 cusps of unequal size. Similar to other series, this study found that fusion of the right and left coronary cusps (anterior–posterior [AP] leaflet type) was the most common pattern, occurring in 56% of patients, with fusion of the right and noncoronary cusps (right–left [RL] leaflet type) seen in the remaining 44% of patients. Although the study suggests that the RL phenotype is associated with valve stenosis and the AP phenotype with regurgitation, this should be considered a hypothesis, not a conclusion. All the subjects in this study were referred for preoperative CTA; thus, all had significant valve dysfunction and/or aortic dilation and should not be considered representative of an unselected group of BAV patients. Kang et al. (7) also found that leaflet phenotype was associated with different patterns of aortic dilation. Normal aortic shape and dimensions were seen more often in BAV patients with an AP leaflet phenotype, whereas those with a RL leaflet phenotype more often had aortic dilation extending to the arch. These findings parallel a study from our group that demonstrated larger and stiffer aortic sinuses with the AP phenotype and larger aortic arch dimensions with the RL leaflet phenotype (8,9). These differences in aortic anatomy and valve function associated with different valve phenotypes support the possibility that BAV disease is not a uniform disease process.
. Despite the insights into the disease process provided by this study, which was performed with computed tomography, from a practical point of view, echocardiography remains the key imaging approach in adults with BAV disease. CTA is only an adjunct in selected patients. BAV disease is usually asymptomatic, often incidentally diagnosed on echocardiography obtained for other indications or suspected on physical examination with auscultation of a murmur or a mid-systolic “click.” Transthoracic echocardiography has a high sensitivity and specificity for the diagnosis of a BAV. Characteristic findings include an eccentric diastolic leaflet closure plane and systolic doming of the leaflets in long-axis views. The number of valve leaflets, the type of leaflet fusion, and the presence or absence of a raphe can be reliably determined in short-axis views. Doppler echocardiography allows accurate measurement of the severity of valve stenosis and regurgitation. If transthoracic image quality is not adequate, transesophageal echocardiography often provides improved visualization of aortic leaflet morphology. Three-dimensional imaging of the aortic valve may further improve the accuracy of echocardiography for diagnosis of BAV disease.
. Echocardiography allows evaluation of the aortic sinuses and, by moving the transducer up 1 intercostal space, the proximal ascending aorta. Because of the low cost, lack of ionizing radiation, and wide availability, echocardiography is often used to evaluate and follow the aortopathy associated with BAV disease. However, for a more comprehensive evaluation of aortic anatomy, CTA or magnetic resonance angiography (MRA) both provide comprehensive tomographic evaluation of the entire aorta, and are particularly helpful when visualization of the ascending aorta by echocardiography is limited. Our approach in patients newly diagnosed with BAV is to obtain an index tomographic (CTA or MRA) imaging study of the aorta to determine the pattern and severity of aortic dilation. If there is no significant dilation or if echocardiography adequately visualizes the aorta, then serial routine CTA or MRA imaging is not indicated. In patients who have disease progression necessitating aortic valve replacement, aortic surgery, or both, pre-surgical CTA or MRA imaging provides a better understanding of the extent of aortic involvement to aid in surgical planning and graft choice.
. A majority of patients with BAV disease will have disease progression requiring surgery over the course of their lifetime, most often for valve stenosis or regurgitation, with established clinical guidelines providing recommendations on optimal timing of intervention (10). Clinical recommendations for surgical intervention for the aortopathy associated with BAV disease are less well defined, but most centers recommend intervention at an aortic diameter greater than ∼50 to 55 mm, independent of valve disease. Aortic graft replacement may also be considered at a smaller aortic diameter (45 to 50 mm) in patients who are otherwise undergoing aortic valve surgery or if there is evidence of rapid disease progression (interval increase in aortic diameter >5 mm over 6 months.)
. Although adults with BAV disease can have excellent clinical outcomes with appropriate disease monitoring and with correctly timed intervention to prevent adverse events, our current approach is largely pragmatic with little understanding of the underlying disease process. Several studies, such as the one by Kang et al. (7), suggest that there is more than 1 BAV phenotype with different clinical associations. Do these different phenotypes also have different genetics and different clinical outcomes? Until we can identify which patients with BAV disease are at risk for aortic dissection and would benefit from prophylactic aortic intervention, we will need to continue serial imaging of all BAV patients to detect the few who have progressed to surgical disease.
Saturday, January 10, 2015
Thursday, January 8, 2015
Wednesday, January 7, 2015
Tuesday, January 6, 2015
Sunday, January 4, 2015
Saturday, January 3, 2015
I'm meeting with two different surgeons next week. One is associated with the University of Pennsylvania hospital and the other with Temple University hospital, where my cardiologist works. I'm compiling a list of questions to ask. But first I will give each physician a chance to propose a course of treatment. I think I have to listen closely first before peppering them with a lot of questions. I've sent my records to both so they should have a pretty good idea of what they would do before I get there. I'm taking my husband Dan with me so we'll have four ears and two heads to process the information. The obvious questions are about AVR, (prosthesis type, etc.) and what to do about my aneurysm. Also, how quickly can surgery be scheduled? Other questions have to do with the surgery itself, but I don't want to get too far out ahead of things, like I was six years ago when I first met with a surgeon. It wasn't time then I was told, but I think it is time now. How many more heart attacks and strokes do I have to have?