Last New York Trip Pre-Surgery

I'll be walking to the train station along the beautiful Schuylkill Banks walkway this morning. Then it's off to New York City via Amtrak for a performance of the New York City Ballet. I'm still walking outdoors and enjoying each walk very much. I'll miss them post surgery.

The Waiting For Surgery Is Tough

My date for surgery, February 16, was set on January 7. I wanted an earlier date but my surgeon felt the need for me to wait to insure any brain injury from my incident on December 9 is fully healed. But the waiting is tough on me and on Dan. We can't have a normal life with the surgery hanging over us for so many weeks. I sure hope there is no further delay or postponement.

Medical Coverage Approved For Surgery

Got my approval letter from Independence Blue Cross yesterday submitted by my surgeon, Dr. Wheatley. Things are moving inexorably forward.

Trans-catheter Re-Ops: The Wave Of The Future

According to my surgeon, Dr. Wheatley:

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.

Committed To A Tissue Valve

I had a valuable exchange of emails with my surgeon, Dr. Wheatley in regard to the choice of valve prosthesis for my aortic valve replacement. Dr. Wheatley is confident that, if I were to need another replacement in the future, it will be possible to to have it done via the new trans-catheter (TAVR) method. So, while it remains a gamble on the future against the possibility of having to undergo another open heart procedure, this seems to be a reasonable course for me to follow. The other option, selecting a mechanical valve combined with the need for lifelong anti-coagulation therapy, has faded as a viable option in my mind. With about two and a half weeks until my surgery date, I am becoming sanguine about my situation.

Daily Medications Organizer

I'm anticipating being on more than a few medications immediately post-surgery. This organizer ought to help keep everything on schedule.

Modified Bentall: Low Incidence Of Complications

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.

Aortic Valve Replacement: St. Jude "Trifecta"

So this is the valve prostheses my surgeon recommends. It looks good to me. Thank you Dr. Wheatley! I especially like the low pressure gradients that this valve can deliver to the patient. From the St. Jude web site:

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.

Bears Repeating!

From the ACC/AHA 2014 Guidelines:

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.

Age And Reoperation Risk Graphic

From the 2010 study referenced in my post the other day:

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.

The Senseless Death Of A Fine Doctor

This fine surgeon was gunned down yesterday inside his Boston hospital by the son of one of his elderly patients. The gun lobby, the NRA and anyone who champions guns all have his blood on their hands.

Tissue Valve At Age >60: The Excellent Outlook

A 2010 study contains the following statement:

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.

Surgical Preparation for Cardiac Surgery

Well, if you have any shyness about your body you'd better leave it at the door when you go in for open heart surgery!

Unique Aorta Procedure

I suppose this procedure is used if the aortic tissue is too diseased for the Bentall Procedure. Diagram shows the Cabrol procedure, in which a composite aortic graft and a prosthetic conduit that connects the coronary ostia are anastomosed to the aortic graft. Blood flows (arrows) from the aorta into the right and left coronary limbs and, eventually, the coronary arteries.

Surgical Q & A

Here's an interesting Q & A exchange that I had with my surgeon's assistant.

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.

It Could Have Been Much Worse

According to my cardiologist:

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.

Great Explanation From My Cardiologist

I posed a question to my cardiac care team at Temple University Hospital asking why it will be necessary to replace my entire aortic root and ascending aorta. My cardiologist, Dr. Martin Keane responded with this comprehensive explanation:

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."

Responsive Medical Team At Temple Hospital

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.

Excellent Article On BAV Disease

From the Journal of the American College Of Cardiology, February 2013.

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.

Why Is My Surgeon Replacing My Aortic Root?

I forgot to ask a very important question when I met with my surgeon, Dr. Wheatley. His treatment plan calls for the replacement of my aortic valve, root and ascending aorta. This means that I will need a Bentall Procedure, where the coronary arteries are grafted onto the dacron replacement lower aorta. Since the measurements of my lower aorta do not seem to indicate that is is aneurysmal, I'm wondering why it needs to be replaced. One reason could be Annuloaortic ectasia...a dilation of the proximal ascending aorta and aortic annulus. Here are three views of the aorta...the first is a healthy aorta, the second is one with annuloaortic ectasia, and the last is mine. Look at the difference at the level of the annulus (circled in images 1 & 2), where the aorta joins the heart, then compare that to mine in image 3.

Bentall Procedure

I was shocked to find out that my surgeon will be replacing my aortic valve, aortic root and ascending aorta. But if it's necessary then I'm fine with that plan.

We Have Game Plan For Surgery!

This afternoon Dan and I met with a surgeon at the Temple University Heart and Cardiovascular Center in Philadelphia. This surgeon was recommended by my cardiologist. This was my second surgical opinion. I did some research on the surgeon I met with today and I was impressed with his credentials. Our meeting was intense, with a bombshell surprise: I would need a Bentall procedure, involving composite graft replacement of the aortic valve, aortic root and ascending aorta, with re-implantation of the coronary arteries into the graft. This complex and long operation is used to treat combined aortic valve and ascending aorta disease, which I have, apparently. This treatment plan was radically different from the first opinion that I got on Monday, where the surgeon suggested that my aneurysm did not need to be replaced, let alone the aortic root. The first surgeon wanted to do an isolated replacement of the aortic valve via a mini-sternotomy, and agreed to address my aortic aneurysm only after I insisted. The second surgeon today indicated that the valve and aorta needed replacement from the outset. The first surgeon suggested the need for circulatory arrest during the procedure, but the surgeon today stated that this would not be necessary. I find it interesting and a bit troubling that such divergent therapies can be suggested for the same pathology. But in fairness the first surgeon stated that he preferred a conservative approach. Today the second opinion surgeon seemed to want to correct as much as possible during open heart surgery. I come down firmly with the latter approach. I want the valve and the aneurysm taken care of, and if the aortic root needs replacement then so be it. Get it done. Therefore, I have opted to go with the approach outlined by today's surgeon and I have scheduled my procedure for February 16.

Mini-Sternotomy for Ascending Aortic Aneurysm and Bicuspid Aortic Valve ...

This is exactly the procedure that I need, with the exception that I need a replacement aortic valve rather than a repair. I would think that replacing the aortic valve would be even easier than repairing the native valve.

Surgical Consultation No. 1

On Monday, January 5, I met with a cardio-thoracic surgeon at Penn Presbyterian Hospital, a branch of the Penn Medicine health care system in Philadelphia. Unfortunately, my husband and I were forced to wait over two hours past our appointment time before we finally got a chance to talk to the doctor himself. While the consultation was valuable in terms of adding to the information we need to make decisions about my surgery, we both felt that this particular surgeon would not be the best choice for me, and we were disappointed in the way we were treated at the facility. Unlike my meeting with another surgeon back in 2009 at a different branch of Penn Medicine, this doctor was at least personable, honest and sincere. He was also willing to listen to our concerns and to try to accommodate my wishes in terms of how I view the procedure I need. In particular, while he initially indicated that his conservative approach would be to not address my 4.6 cm aortic aneurysm at the time of AVR, he agreed to replace it after I indicated that I wanted it addressed. At first he told me "I don't think it will give you any problems." Perhaps not, but who wants to have open heart surgery and not see the surgeon correct an aneurysm that could potentially dissect in the future or require another operation? This captures the difference between the approach of this surgeon and the approach that I need. I don't want a conservative approach. I want a surgeon with a proactive approach willing to fix as much as possible as long as he is in there. So Wednesday we meet with another surgeon at a different facility. Hopefully, the experience will be better and the surgeon will prove to be more in line with my needs and expectations.

Tomorrow It Starts

Photo: Holiday greeting from my friend Wolfgang in Leipzig.

Tomorrow Dan and I meet with the first of two surgeons that we will see this week. We'll see how it goes.

Two Surgical Consultations Next Week

Photo: Philadelphia's Newly Re-designed Dilworth Park at City Hall

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?