The Ross Procedure FAQ

The Ross Procedure FAQ (Frequently Asked Questions)

Overview

FAQ Version 1.1. Last Updated 7/17/99.
This FAQ is maintained by Cary Mader.
Email: info@myrossprocedure.com or myrossprocedure@home.com
URL: http://members.home.net/myrossprocedure/RossFAQ.html

I have listed references to material that was largely written by others in sections, where appropriate, in order to give the sources due credit. The references use the notation "[n]" as noted below.

This FAQ is a compilation of information from a variety of sources:

Table of Contents


1. Aortic Valve Replacement Options

1.1. Pulmonary Autograft (The Ross Procedure)

This is the patients own pulmonary valve. The first pulmonary autotransplant was performed by Dr. Donald Ross at Guy's Hospital on June 8, 1967, (thus the name the Ross Procedure) and the patient was still doing well 28 years later.

The Ross Procedure considers the patients own pulmonary valve to be the ideal replacement valve for several reasons:

Results: The pulmonary autograft transplanted to the aortic position was not calcifying, leaking, or showing signs of degeneration. At 20 years, only 15% of patients required another valve operation, usually replacement of the homograft reconstruction of the right ventricle [Ross 1996]. Patient survival at 20 years is an impressive 80%.

[1]

1.2. Mechanical (prosthetic) Valves

A mechanical valve is made from artificial materials. Most commonly, the mechanical valves are made from a material called pyrolitic carbon. This is pure carbon heated and compressed in a trade secret process. The final smooth surface is obtained by layering carbon formed into a gas onto the surface of the compressed carbon. The resulting leaflets are very smooth and resistant to thrombus formation (i.e. clot). Some components of modern mechanical valves are made from titanium or a multi-metal compound called Elgiloy.

The first of these was the Starr-Edwards design, a functioning heart valve made from a steel cage enclosing a silicone rubber ball. Developed in 1961, it was first used to replace the human mitral valve, but had limited success when used as an Aortic valve replacement. One of the more commonly used valves today is the St. Jude bi-leaflet valve.

The major disadvantages of these valves are:

Results: The patient implanted with a mechanical aortic valve continues to run some long term risks of either a stroke or major bleeding episode in the range of 4 to 8% (combined) per year. In addition, the long term survival is 60% at 15 years, with most deaths related to non-cardiac causes.

[1]

1.3. Animal Tissue (xenograft) Valves

These first appeared in 1972. The porcine (pig) aortic valve is the most widely used. The pig valves are made from animal heart valve tissue and thus are relatively free from the risk of clotting. This is an advantage in some patients who cannot take anticoagulation medication (pregnant women, patients with brain aneurysms, bleeding intestinal sources, etc.)

The porcine valve is tanned with a stringent chemical called glutaraldehyde, an agent similar to, but stronger than, formaldehyde. This processing toughens the leaflets and cross-links many of the proteins. In doing so, the valve is also rendered less likely to be rejected. However, the tanned leaflets are no longer living tissue.

Results: Calcification of the valve is a problem, and animal tissue valves usually begin to degenerate within 8 to 10 years after implantation. In patients less than 40 years of age, animal tissues valves may degenerate within 5 years of implantation.

[1]

1.4. Aortic Homografts

A homograft valve is a human cadaver valve (also called allograft). These valves historically have had problems with sizing and suturing of the valve, along with the expected problems of procurement and long term storage.

Although methods of preservation and long-term storage have improved, these valves have suffered from unique problems when used as an Aortic replacement, such as structural deterioration, difficult implant surgery, and occasional size mismatching.

Results: These valves will last an average of 15 years. Over time, the wall of the homograft aorta surrounding the valve becomes severely calcified, creating increased surgical difficulty at the time of subsequent replacement. Rejection of the aortic tissue may play a role in this form of degeneration.

[1]

2. General questions on The Ross Procedure

2.1. What are the disadvantages of the Ross Procedure?

No matter how much improvement has been made in the surgical techniques for performing the pulmonary autotransplant procedure, there are still some drawbacks.

One disadvantage is the magnitude of the surgical procedure. It is a very technicaly demanding surgery.

Some critics are concerned that transferring of the pulmonary valve converts a patient with only aortic valve problems to a patient with 2 heart valves now in jeopardy. The potential for premature degeneration of the pulmonary substitute has also been voiced. Fortunately, the long term results now available tend to refute both of these concerns. Results have shown that premature failure of the homograft valve used to replace the pulmonary valve is much more common when human aortic tissue was used in the right side reconstruction, as opposed to human pulmonary tissue. There was only a 74% freedom from reoperation at 5 years when aortic homografts were used for the right ventricular reconstruction. However, there is a 94% freedom from reoperation at 5 years (and 83% at 20 years) when a pulmonary homograft is implanted into the right ventricular outflow tract. The reasons for this dramatic difference are not known.

In summary, the major disadvantages of this operation for the surgical treatment of aortic valve disease are outlined below.

[1]

2.2. What is the ideal candidate for the Ross Procedure?

Patients ...

The accepted age range for patients having the pulmonary autograft procedure has expanded significantly. Many surgeons view the procedure as a solution for congenital aortic stenosis that can be performed early in life, and quite possibly eliminate the need for multiple aortic valve replacements. As increasing durability of the operation is evidenced, older patients with more active lifestyles become candidates for the procedure. The pulmonary autograft procedure is certainly indicated for young women of child-bearing potential, due to the problems associated with the use of blood thinning agents (Coumadin) during pregnancy. [1],[2]

2.3. When would the Ross not be the best choice?

In the older adult, coronary artery disease (CAD) is a additional finding commonly discovered during the preoperative evaluation of patients with heart valve disease. Significant CAD weighs against doing a Ross procedure, since the combined complexity of simultaneous coronary artery bypass grafting (CABG) and the Ross is far too much surgery at one time. Thus patients with CAD are usually selected for a mechanical valve.

Also, there are some patients who will be required to take anticoagulation for life regardless of the issue of valve substitute. Such patients are individuals in whom chronic atrial fibrillation, left atrial thrombosis (clot), or stroke have already occurred as a consequence of their heart or valve disease. In these patients, it is not wise to place a substitute that will avoid Coumadin® since there are already strong indications to use long term anticoagulation.

Some doctors believe that the pulmonary autograft will age in synchrony with the aging process of the patient as a whole. Thus they do not recommend this operation in patients over 45 years of age. However, there is no current data to support any such concern.

The following conditions are considered factors against using the pulmonary autotransplant procedure...

[1], [2]

2.4. Is the Ross Procedure an option for a woman wanting to have children in the future?

women of child bearing age with aortic valve disease represent a special challenge. Any valve substitute chosen for a female patient must be durable enough to provide many decades of uninterrupted performance. At the same time, mechanical valves require Coumadin® to prevent thromboembolism (clot formation leading to stroke or other tragic consequences). Coumadin® taken by the pregnant mother can cause birth defects in the fetus. Thus any heart valve replacement in a woman of child bearing years must permit her to exist safely without the burden of anticoagulation. Unfortunately, porcine or bovine tissue valves, and human homograft valves do not last long enough to be an effectively curative operation in a young woman. [1]

All of these surgical and clinical issues are avoided by using the Ross procedure on women of child bearing age.

2.5. What is the youngest age at which the Ross can be done?

Although initial results from the Ross Registry indicate successful application of this procedure in infants, more follow-up is needed to validate the autograft growth potential and long term clinical outcomes [2].

Dr. John Calhoun of the University of Texas, San Antonio was the first to use a Ross reconstruction in a newborn infant. In newborns, the potential for growth of the aortic reconstruction is a vital advantage of the Ross procedure over any other form of replacement.

Dr Willem Daenen of Gasthuisberg University Hospital in Belgium was the first to publish results (1993)of a modified Ross autotransplant done in concert with annular enlargement as first described by Konno, et. al. In the U.S., the Dr. Steven Gundry at Loma Linda University has reportedly performed the operation as well.[1]

2.6. What if there is a problem with my Pulmonary valve?

This usually is not a problem. Typically, even if there is a congenital deformity of the aortic valve, the pulmonary valve will be perfectly normal. The doctors should be able to determine the viability of the pulmonary valve using an echocardiogram, though it is possible for a problem to not be detected until the time of surgery. If the pulmonary valve were found unsuitable, the surgeon would make a decision as to the best valve replacement option for the patient, if he has not already discussed options with the patient.

See section 4.12. also.

2.7. How can I contact others who have had the Ross Procedure?<

The ROSS-PROCEDURE mailing list is an unmoderated discussion list for patients, family, friends, and others to discuss issues relating to the open heart surgical procedure known as the Ross Procedure. The primary purpose of this list is to offer support for those that may be undergoing this surgery, and to provide a way for people who have already had the surgery to communicate and offer support to others. There are over 100 subsribers on the list, many of whom became members prior to surgery, and have stayed on to share their experiences after surgery.

A mailing list is simply a collection of mail addresses (the list subscribers) that typically is managed by a software program. Mail messages are sent to the list server, who in turns bounces the message to all of the subscribers.

To subscribe to the list, send an email message to:
listserv@maelstrom.stjohns.edu

The body of the message must contain:
"SUBSCRIBE ROSS-PROCEDURE first-name last-name"

The subject line can be anything you wish, or preferably, just leave it blank and ListServ will fill in a subject on its replies.

You will receive a welcome message once you have joined that provides more information on how to send messages to the list, access archives, etc.

3. Finding a Surgeon

3.1. How do I obtain information on the Surgeon?

The most important thing to do is check your surgeon out thoroughly. If you are doing this as an elective procedure, it is best to ask questions and do some research. If you have a good relationship with you Primary care M.D. or cardiologist, they can help you with some of the technical research.

Most surgeons will be up front and honest in answering questions. If you feel the surgeon is not, then it's probably best to find another.

Some of the questions you should ask are:


Have your cardiologist check with another physician (preferably a heart surgeon who does not do ross procedures) who they recommend, and who is well thought of in the cardiac surgery community. Remember, often a surgeon is well thought of because he publishes a lot and is not necessarily the best technician in the Operating Room.

Be careful of other patient's recommendations. If they had a positive outcome they tend to put their surgeon on a pedestal and may not have the best information on the statistics and actual performance and technical ability of a particular surgeon.

In summary, try and do a lot of research, as the ross procedure is a technically very difficult procedure. Ideally, you should try to choose a surgeon who has done at least 75 to 100 Ross procedures, and a hospital that does at least 500-700 total heart procedures per year, with a mortality rate for ross procedures that is under 5%.

[3]

3.2. What can I expect when I meet with the Surgeon?

Generally, the surgeon will review your case, may discuss the available options, and why he feel the ross procedure is the best option for you. This is usually your best oppurtunity to ask questions question you may have about the procedure.

When you meet your surgeon go in with your questions written down so that you are prepared. Have a family member with you so that they can jot down some notes and so that you can listen to the surgeon so that you can ask additional questions and follow-up questions. If you concentrate to hard on taking notes then you may be missing much of what the surgeon is saying.

Try to limit the questions to those that are really important to you. Most surgeons are not prepared to spend the kind of time it takes to answer 4 pages of hand written questions.

Ask the surgeons office if they have a staff member that does patient education. Many offices will have a Clinical Nurse Specialist, Patient Care Coordinatior or Nurse Specialist. This person will be willing to spend more time with you and answer a lot of questions. It is better that this person is a nurse or physician assistant than a receptionist or secretary. Usually a Nurse Clinician or Coordinator can spend more time with you, and if you get his/her name it gives you someone to call for follow up questions.

Also, make sure you ask about follow up visits. Your surgeon will probably want to see you a few weeks after your Ross, and at least once a year after that. If the surgeon tells you they do not do long term follow up, then they are not tracking their long term statistics. A program, especially with ross procedures, should track their patients long term to make sure that they are problem free. This is often done for publishing but with a surgical procedure like the Ross it is good to be able to say that after so many years the patient's valves are still functioning normally. This usually includes doing an echocardiogram each year. Preferably read by a cardiologist that is very familar with the Ross procedure, as these echos look very different from other valve replacement echos.

[3]

3.3. Is it possible to do a long distance consult?

You can do a long distance consult--to tell if you are a candidate for RP. Actually the surgeon could do that by looking at the echo or cardiac cath film and reading your records. Most of the time you will want to meet the surgeon and know that his/her personality is acceptable to you. Most RP surgeons are used to dealing with patients who are coming in from out of town, or even outside the USA, so the office staff should be able to talk you thru the details of how to set everything up if you are out of state.

[3]

3.4. What can I do to make sure the consult goes well?

If you have had a cardiac cath or an echo done, get the copy of the film itself. The echo will usually be on a video tape and the cardiac cath may be on a CD, video tape or film that is played on a Targano machine. Get the film yourself and bring it to the office or drop it off with the surgeon yourself.

The most frustrating thing for patients and the office staff is that patients or cardiologist assure us the tape will be there and it isn't. No one other than you really cares about that tape, so you be responsible that it gets there.

Get the written reports of echos or cardiac caths, also any other written reports that your cardiologist will give you. Again we hear over and over that don't worry the cardiologist will send that information and the patient shows up and we have no information. Then we are trying to get patients records faxed at the last minute. Some secretary in the cardiologist is not really that focused on whether your records get to the surgeon.

3.5. After my surgery, my surgeon said he wanted to see copies of my annual echos.

Most surgeons should want to see your annual echos. The ones that want to see you back every year may be working on publishing something; that in part is why we see all our RP patients back every year. Most surgeons maintain statistics on their RP patients. If your surgeon does not want to see you back or at least see your yearly echos, I would be a little suspect if he quotes you stats regarding: failure of RP.

[3]

4. Surgery related questions

4.1. How is the heart stopped?

The pumping action of the heart is suspended by infusion of a high potassium solution in the coronary arteries.

4.2. Why is the body temperature of the patient lowered?

The body temperature is decreased to 27 degrees Centigrade. Cooling helps preserve the body tissues and the heart muscle during the physiologic alterations characteristic of heart-lung bypass and open heart surgery. It also reduces the oxygen debt requirements of the body while connected to the heart-lung machine.

4.3. How long does it take to know whether the operation was successful?

In most Ross Procedures, the result can be immediately assessed in the operating room using transesophageal echocardiography. The presence of leakage, blockage, kinking, or other possible distortions of the implanted autograft (or even cadaver pulmonary replacement) can be visualized, and appropriate steps taken if necessary. Once the physiology and anatomy of the result has been confirmed and the patient stabilized, the incision is closed and the patient returned to the intensive care unit.

4.4. Where kind I find a detailed account of the surgery on the internet?

At http://www.hsforum.com/heartsurgery/TLC/Ross/RossTechnique.hsf. This information was put together by the Indiana University Medical Center.

4.5. Why did they shave me from my neck to my toes?

I'm still trying to get an official answer on this one. The chest area is obvious, as there is a large incision there. The groin area is in case the heart/lung machine needs to be hooked up in a hurry. Nowadays the machine is hooked up via the chest opening, but in an emergency, there are major arteries easily assessable in the groin area. The legs, I surmise, are sometimes shaved should they need to harvest a vein for the coronary arteries, though this probably rarely happens.

4.6. How are the homograft valves preserved?

The tissue is soaked in a chemical product known as DMSO. DMSO is the preservation medium. It displaces water from the tissue at the cellular level. Water has to be driven out of the cells, or when it is frozen it will cause ice crystals that will damage the cells (causing cellular rupture). Once the tissue is saturated with DMSO it is placed in liquid nitrogen which is cooled to a temp of 100-130 degrees below zero. This causes the tissue to freeze instantly and minimizes the damage to the tissue. The allograft is then rock hard and has to be handled very carefully. If dropped (and they have been dropped according to the co. rep.) they will shatter into pieces. When the tissue is shipped it is kept frozen and "dry packed". When it gets to the operating room there is a slow procedure of warming and dilution to get rid of the DMSO and unfreeze.

An interesting side note DMSO is a solvent, I'm not sure approved by the FDA for any use other than the above. It is used by Horse vets as an anti-inflamatory agent. People for many years have gone to Mexico and now to vet supply stores to get DMSO to treat arthritis. It is widely popular with atheletes. But still not FDA approved for use as an anti-inflamatory agent.

[3]

4.7. Are there any rejection issues with the homograft valves?

In regard to rejection, my understanding is that the cryo-preservation technique used removes the antigens from the tissue and that you do not have the issue of rejection with this type of tissue. Antigens are small substances on the cell that triggers the production of antibodies and starts the process of destroying or rejecting the tissue. This is the reason why when you get a homograft you do not have to undergo the process of tissue typing and matching tissue types with the homograft.

The homografts are not marked with blood types, etc. like some cyropreserved tissue because the antigens are removed and the tissue can be implanted into anyone regardless of their blood types. This is also true of the Porcine (pig) valves and bovine (cow) pericardial valves. Both use pig and cow tissue which normally the human body would reject but the processing techniques for these valves remove the antigens and thus the risk of rejection.

[3]

Some recent evidence exists for the potential of a rejection process within the pulmonary homograft resulting in pulmonary stenosis. This rejection process may require early explantation of the pulmonary homograft. The rejection process may be more prevalent in patients diagnosed with PPS (PostPericardiotomy Syndrome).

4.8. What issues should I think about if I'm travelling out of town for the surgey?

Check out the hospital's resources. Many large surgical centers have rooms available in the hospital to accomodate your family. Also realize that there will probably be follow up visits with your surgeon. Since you will need to make yearly follow up visits, you may wish to concentrate your surgeon search in a geographic area, such as near family or an area that is easily accessable by air travel. [3]

4.9. What if my insurance limits my choice of surgeons?

A lot of HMOs can be very difficult regarding payment for Ross Procedures.

We had a patient that had his HMO tell him that he had to have his ross done at a particular institution, by a surgeon who was primarily a pediatic surgeon and the surgeon had only done 10 ross procedures in his career. The patient was very upset, needless to say. It took alot of fighting with the HMO and help from the patient's HMO primary care M.D. to get the HMO to agree to pay for the ross procedure by the patient's choice of surgeon.

Cases like the above can happen with HMOs because they often strike relationships with medical institutions based on cost, not performance.

Do not let your insurance dictate which surgeon you should use. It is an elective procedure and this should give you time to work with your insurance on their policies regarding this type of surgery. There are a number of ways to pressure insurance companies, and threats of lawsuits are often effective. The best advice is to be agressive with insurance companies. Often their decision will boil down to potential future liability, and if they know you are dealing with a lawyer already, they may back down.

Talk with your insurance company early in the process, it is hard on patients and their families to have everything all set up a surgeon, only to have the insurance company throw a wrench into the whole process.

[3]

As a last note, be aware that most doctors and hospitals are now willing to negotiate prices in this age of managed health care. You may find that you can limit your out of pocket expenses by asking the providers to waive costs that are not covered by you insurance, to accept the UCR (usual and customary rate) or to limit your out of pocket expense to some fixed amount.

4.10. Is'nt there is a less invasive technique, where a small incision is used?

The mini-sternotomy is a possibility, but there are very few surgeons who offer it with RP. RP is a technically difficult surgery and many surgeons want to have a very good view of the operative field. With mini's there are some aspects of the surgery that are done with less than optimal view of the surgical field. We do both minis (not for RP but certain other procedures) and regular sternotomies, I have to say--I do not see any real difference in length of stay in the hospital, complaints of pain or general recovery. This is just personal experience--we are currently tracking the minis and are working on a paper re: length of stay vs regular sternotomies. But I have really not compared the data recently. Personally I would choose the surgeon based on experience with RPs. If your surgeon offers you a mini sternotomy with an RP I would question them as to how many mini sternotomy RPs they have done. There is a learning curve to doing the surgery thru a mini and I certainly would not want to be one of the first few patients that the surgeon has done thru a mini.

Nancy [3]

4.11. How long will my scar be?

The length of the scar or incision depends on body size and the size of the sternal bone. A tall person may have a scar up to 10-11 inches long, a smaller person may have a smaller incision, may be 7-8 inches long. I know that sounds terrible but after a period of time (6 months to a year) the scar fades to the point were it is really hard to see. There typically is not a great deal of pain in the incision area, due to the comparatively few nerve endings in the skin and bone at that area. Most surgeons now use the sutures that dissolve and use the subcutaneous technique that results in a scar that is a nice little thin line.

Another hint to help the healing process: after all the scabs and open areas are gone, put vitamin E oil on the scar. You can get pure vitamin E oil in small vials at pharmacies or use the pills and cut off one end of the gelcap and squeeze out the oil and rub it into the incisions. It seems to help the redness fade and softens the scar so that it is not so raised.

The scar seems to be an issue of great concern for some people prior to the surgery. My personal recommendation is not to worry about the scar. Being alive and healthy is far more important. As someone who has sported one almost my entire life as the result of a valvuplasty at the age of ten and the RP at age 33, I find it more of a source of pride. I remember seeing a news segment a few years ago where a bunch of guys were proudly comparing their bypass scars. The moral of the story is: It's good to be alive, and you can always find someone who has a worse scar(s) that you do.
[3]

4.12. I've heard of patients who go in for the RP and come out with a homograft valve.

Normally a TEE (transesophageal echo) is peformed immediately before the surgery and immediately after the surgery. The TEE provides a much clearer picture of the heart than a normal Echocardiogram. It is possible that during the TEE or during the surgery after the chest is opened, that the surgeon may determine that the RP is not the best option due to the condition of either the aortic or pulmonary valve. This could happen in a situation where the aortic root is extremely dialated for example.

Most surgeons will tell RP candidates that there is a possibility that they will not be able to do an RP, and will discuss what other options (pros/cons and what type of valve) you would prefer if they are unable to do the Ross Procedure? Typically the surgeon is not going to postpone your surgery (especially if he has already invaded the chest and stopped the heart) but you do want to make your non-RP wishes very clear to the surgeon ahead of time. Ask your surgeon how he typically handles the situation. Some surgeons will have a member of the team call or go to the waiting room and explain the situation to your family, so make sure your family members are where of your alternative choices as well. Others will just proceed with the alternative procedure that you have agreed on, or may use an option that they feel is best for your situation.

[3]

5. Recovering From The Surgery

5.1. How long will I be in the hospital?

The average length of hospital stay is about 5 to 6 days.

5.2. When can I start driving a car again?

You'll probably be told to not drive for the first four weeks after the surgery. Going straight ahead is not really a problem, its things like twisting to look to your right or left, and turning around when you back up that are a little painful. It takes about four weeks for the sternum bone to heal. Patients are usually also told not to lift any weight over 5 pounds.

5.3. Why did I have so much back pain after the surgery?

A lot of patients complain about upper back pain, especially between the shoulder blades. The pain is from rolling the shoulders in to try to minimize the pain felt in the sternum (breast bone). Tipping your shoulders forward reduces the pull or pain in the sternum. Heat, massage anything you would do to help a pulled or strained muscle will help. Ultimately what helps the most is when ever you think about it, try to make sure to stand straight and not allow you shoulders to tip forward.

[3]

5.4. What is PPS?

PPS (PostPericardiotomy Syndrome) is also called Post-MI pericarditis and Dressler's Syndrome. It is caused by inflammation of the pericardium (the sac-like covering of the heart) that causes blood to be present in the pericardial sac. It is an inflammatory response to the blood in the pericardial sac. There can be a variety of symptoms, which can include a low grade fever, fatigue, "achy all over" and chest pain. The usual treatment for many post op heart surgery patients is NSAIDS (Ibuprofen).

One method of diagnosing PPS is to draw a blood test called a sed rate, that indicates an inflammatory response in the body if the test is elevated. It is not necessarily definitive but can indicate the possibility of PPS. [3]

5.5. How do I know that healing of the sternum is progressing okay?

Normally nonunion of the sternum is indicated by a popping or clicking sound of the sternal bone when you cough or breath deeply. However, it is possible to have nonunion and be completely painless.

Sometimes the nonunion can be just a small section, often at the bottom of the incision, called the xiphoid area. That is often non-painful, restricted to a limited area, and tolerated well by many people, so often it is not treated.

For nonunion of the whole sternal bone, surgery may be considered. It is called a "rewire" and the incision is opened, sternal wires are cut and the sternum opened, and sternal edges are abraded and rewired. Usually people are in the hospital overnight and are not very painful. Most are up that same evening of surgery walking and eating. The procedure takes about 20 minutes.

In most people the decreasing pain in the sternum is the indicator of healing.

[3]

5.6. I struggled with depression after the surgery.

Apparently this is fairly common. I'm not a clinical phsychologist, but I would suspect that one of the reasons for depression is due to a bit of an emotional backlash. I would guess that this time of one's life is a very intense experience for most people, with a great deal of anxiety before the surgery. It's probably also a time when one is the focus of a great deal of attention from family, friends, co-workers, etc. After the surgery, as you recover, and finally get back to the day to day grind of life, most of that extra attention disappears, and maybe that is a part of the feelings of depression for some people. For a while, a large part of your life involves the surgery in one way or another, and suddenly, all of that is gone about 4-6 weeks after surgery as you really begin to recover and return to work and a normal life.

Someone on the mailing list referenced the following book: "Coping With Heart Surgery and Bypassing Depression: A family's guide to the medical, emotional and practical issues". More information is available at http://www.iup.com/heartbook/.

6. Blood Transfusions

6.1. What are the risks of blood transfusions?

The risks associated with surgery itself are higher than blood related risks.

Acquired Immune Deficiency Syndrome (AIDS) is the most often mentioned fear related to blood transfusions. Human Immunodeficiency Virus (HIV) is the virus associated with AIDS. Blood from persons who have been exposed to HIV can transmit the infection. However, the donor screening and laboratory testing procedures now being used for all blood donations have been very effective in screening out donors who test positive for exposure to HIV. The current risk is estimated at 1:50,000 to 1:104,000 per unit of blood, including screened blood.

Hepatitis B and C: Tests now used on donated blood have markedly reduced Hepatitis B and Hepatitis C associated with blood transfusions. Even though new tests are constantly being researched, there is still a small risk of transfusion-associated hepatitis. It is still possible that the hepatitis virus may be present in a donation and not be detected by the test currently available for use. The risk for Hepatitis C is approximately 1:500 per unit of unscreened blood.

It is also possible for some bacteria and parasites to be transmitted, but these cases are rare.

Fortunately, many Ross Procedure patients do not require blood transfusions, as the procedure can be performed with a minimal loss of blood.

6.2. Can I donate my own blood for transfusions?

An autologous donation is a method by which a patient’s own blood is collected to be stored until transfusion is necessary. Prior to donation, the patient must be evaluated by his or her own physician to be sure it is safe to donate. So if the surgeon reviews your situation and determines that he/she is uncomfortable with you donating your blood, it may be with good reason.

6.3. Can friends or relatives donate blood?

Directed donations, that is donations from relatives or friends specified for a certain patient, may be supported by your local blood agency.

Directed donations is not supported in some areas, because such directed donations have not been shown to be safer. One reason is because of the spread of HIV and AIDS. Even among married couples, for example, the percentages of infidelity are high. People will generally donate blood for someone when asked, even though they know they live a lifestyle that would put them at a higher risk of diseases such as AIDS or Hepatitis. Furthermore, blood donated in this manner may not be screened as closely as that from anonymous donors.

6.4. When would I not be allowed to donate my own blood?

It can be dangerous for some people with Aortic Stenosis to donate their own blood, as decreasing the body's blood volume puts too much strain on a heart that is already stressed.

6.5. How do I donate my own blood?

Start by checking with your surgeon's office and with the hospital where the surgery is being performed. You can call the hospital and ask for the "Blood Bank" and check on their policy. Some hospitals handle autologous blood donations themselves. You should also ask about how long prior to the surgery you should begin donating blood. Directed donations can take 3-5 working days to process, test and transport to your hospital.

If you are looking at directed donations (family and friends donate blood for your surgery), you usually have to go thru the Red Cross or a commercial blood bank that adheres to red Cross standards.

[3]

6.6. I found the Red Cross difficult to deal with.

Yes, the Red Cross can tough to deal with and they will not flex in the least little bit.

The Red Cross has some very strict standards mainly for the patient's protection, and the increased popularity of directed donations means that you or the surgeon's office need to be organized in terms of getting all the proper paperwork to the Red Cross and getting potential donors to make appointments in a timely fashion. If you delay there is no way that a surgeon's office can "make" the Red Cross hurry the process along.

The Red Cross is a large bureaucracy that works within their own standards, practices and protocols and if your friend or family's blood is not acceptable to them, there is no way that you or the surgeon can intervene. Again it is for your protection. You might think the world of Aunt Jane, but if anything comes up in her interview or in testing then the Red Cross will not allow her blood to be processed and sent to the hospital.

[3]

6.7. Are there any costs associated with donated blood?.

Typically there are costs related to processing, testing, transportation and storage of the blood, regardless of whether it was a Directed/Autologous donation or is coming from a "blood blank".

6.8. How many RP patients need a blood transfusion at the time of operation?

I doubt there is any definitive study on this, that can actually quote percentages. But whenever you are working near a high pressure structure like the aorta--there is the potential for bleeding. Most heart surgery centers will use a process during surgery called cell saver. The blood from the operative field is salvaged and spunned down and filtered and the red cells are returned to the patient. The other problem with RP is that it is a long procedure, and the patient is on the heart lung machine for a longer period of time, that causes damage to platelets. The less functional platelets around in the blood stream there is an increased risk of some generalized oozing or slow bleeding.

We always have 4 units of packed red blood cells and 1 unit of platelet pheresis set up for all our RP patients. If the patient or family does not donate then, that blood comes from the Red Cross. I would say that it is infrequent that we give blood to RP patients, part of the reason is that the PR patients are usually younger and they can usually tolerate lower blood counts. My guess would be 1 in 5 RP patients receive some type of blood product, either red cells, platelets or fresh frozen plasma.

Nancy [3]

7. Long Term Results

This information primarily comes from the Heart Surgery Forum.

7.1. Overall Results

The first Ross Procedure (pulmonary autotransplant) was performed in 1967. However, the current surgical techniques were not refined until at least 1976. Since that time, the results have improved dramatically. Postoperative patient survival at 20 years is an impressive 80% overall. Of those, 85% had not required reoperation, and 75% were free from any other event, including endocarditis.

[1],[2]

7.2. Reoperation Rates

Ross and colleagues followed 339 patients for up to 24 years following surgery. Only 15% of surviving patients required any additional valve procedures and most of these were for replacement of the homograft valve used to replace the patients pulmonary valve, not the aortic substitute. Fortunately, it is easier to replace the right ventricular substitute (the pulmonary valve). Follow-up of recent Ross cases where a human pulmonary artery homograft was initially used to reconstruct the right ventricular outflow tract has shown a remarkable freedom from failure (94% at 5 years, 83% at 20 years). Although pioneered solely by Mr. Donald Ross of London, England, surgeons in many other centers throughout the world are able to reproduce similar excellent results with the Ross Procedure. Reoperation rates for failure of the autotransplant and/or the right ventricular homograft are neglible in most centers (less than 10% at 10 years).

There is growing evidence that a pulmonary autograft properly implanted into the aortic position will continue functioning indefinately. The tissues of the patients own pulmonary valve have not shown a tendency to calcify, degenerate, perforate, or develop leakage over time even when transplanted into the higher pressure aortic position. There have been a few reports of late occurring dilation of the aortic root causing central leakage of the autotransplanted valve. However, this problem occurred before the technique of total root replacement was widely adopted. It is now known that secondary root dilatation can be prevented by reinforcing the aortic diameter with a cuff of Dacron, Teflon, or native pericardium during implantation of the pulmonary autograft. Post-operative studies now confirm that leakage is present in only 10% of modern cases, and usually will not progress.

Dr. Ronald Elkins from Oklahoma City has reported about 15% of pulmonary homografts will contract or shrink within 6 months of implantation. However, this does not appear to make a major difference in function of the homograft. Also, now that a pulmonary homograft is seen as a much better replacement for the pulmonary valve, the overall results of the Ross procedure have improved. When the right ventricular reconstruction is done with a pulmonary homograft, the freedom from degeneration is 94% at 5 years and still an admirable 83% at 18 years[Ross 1996].

[1]

7.3. Ross Registry Data

The International Ross Registry includes data on over 2,000 Ross procedures performed by 126 surgeons throughout the world. The average age of the patient population at the time of surgery was 28.7 years. Seventy-three percent of the patients were males. Over half of these cases were operated for congenital aortic valve disease, such as bicuspid aortic stenosis. Over 30% of these patients had undergone heart surgery at some time before the Ross procedure was performed. The combined operative mortality rate for the over 2,000 patients in the Registry (including small babies) was 5.4%. However, in stable adult patients undergoing elective operations, the mortality rate is now below 1%[Ross 1991]. The incidence of post-operative bleeding requiring reoperation was an admirable 0.9%. The symptoms or findings of aortic valve disease were relieved or greatly improved in all patients. Follow-up echocardiograms revealed only trivial or mild valve leakage in the vast majority.

In the "classic" era of the Ross procedure (prior to 1986), there were some late failures. Of these, 73% initially had the pulmonary valve implanted using the subcoronary ("freehand") technique. Due to this higher rate of surgical failure, this technique has been abandoned by most surgeons. Only 16% of the failures occured in patients who had full root replacement with the entire sino-tubular mechanism of the pulmonary valve.

[1],[2]

8. Pediatrics and the Ross Procedure

8.1. What are the problems of valve replacement in children?

The most common valve problem in children is aortic stenosis. The small, malformed aortic valves in these children are very difficult to repair. Stenotic valves can either be dilated with balloon catheters or surgically incised to increase the opening. These solutions often require repeated surgery, as the valve can calcify and restenose.

The use of artificial valve in small children is a problem, since they do not perform well, and very young children cannot fit the smallest artificial valve. Pediatric patients receiving artificial valves will usually require further surgery as they outgrow the implant. Furthermore, the issue of reliable anticoagulation administration is troublesome in small children and adolescents. Animal and human tissue valves used in small infants tend to calcify and degenerate before the patient reaches adult size.

[1]

8.2. How has the Ross Procedure benefited pediatric patients?

To overcome the significant problems with surgical treatment of congenital aortic valve disease, the Ross procedure has been proposed as a desirable alternative. Over the last decade, Dr. Ronald Elkins at the University of Oklahoma has shown that the Ross procedure is truely the best long term treatment for children with aortic valve and root abnormalities. Notably, Dr. Elkins was the first to demonstrate that the autotransplant grew along with the child, making this operation the only aortic valve replacement with growth potential. In addition, the native tissue reconstruction provided by the Ross makes it possible to eliminate the burden of anticoagulation altogether.

Dr. Elkins has shown excellent results. Actuarial survival was 96.5% at seven years from the date of surgery . Freedom from failure in the transplanted pulmonary graft was 92%, and as high as 96% when used as a full root replacement. [1]