Pulmonary Valve Replacement

Over the past few decades, we’ve made great strides in congenital heart surgery, which has increased survivorship among long-term sufferers of congenital heart disease. While many options for congenital heart disease and defect repairs are available, patients may require future additional surgeries.

Tetrology of Fallot is one of the most common types of congenital heart defects, and is a prime example of a condition that could require pulmonary valve replacement. Tetraology of Fallot (TOF) actually refers to four heart defects present from the time of birth: a ventricular septal defect (hole between the ventricular chambers which allows blue and red blood to mix), pulmonary stenosis (narrowing that makes it difficult for blue blood to reach the lungs), right ventricular hypertrophy (thickening of the heart muscle from pumping blood past the narrowing) and an overriding aorta. These defects often cause an infant to have cyanosis, or blue-tinged color of the skin, resulting from lack of oxygen-rich blood.

Fortunately, the prognosis for children with this condition has greatly improved over the last several decades (assuming proper diagnosis and treatment is administered). During surgical repair of TOF, the hole is closed with a patch, and the narrowing from the diseases pulmonary valve is removed, which can leave patients with a ‘leaky’ pulmonary valve. Although some patients may do well for a decade or more with the leaky valve, the pulmonary valve will eventually need to be replaced in most children born with TOF. Perhaps the most challenging aspect of treatment for patients suffering from this condition is exactly when to time treatment.

Other repairs may be associated with the need for pulmonary valve replacement in the future, including repair for pulmonary atresia with ventricular septal defect (VSD), truncus arteriosus, the Ross procedure for aortic valve disease, and double outlet right ventricle and d-transposition of the great arteries with VSD.

Surgical replacement of the pulmonary valve is the standard treatment for pulmonary valve disease.  Valve replacement surgery involves the replacement of one (or more) of the valves of the heart, typically with an artificial heart valve or a bioprosthesis (a prosthesis consisting of an animal part or animal tissue).

Pulmonary valve replacement is the most common operation performed in the adult congenital heart disease population. This surgery can be performed with extremely low morbidity and mortality. Patients are typically out of the hospital within the first week, and able to perform their normal daily routine. They’re fully recovered within 4 weeks. A surgically placed pulmonary valve is expected to last 10-15 years, or longer.

For very select patients who have already had conduit replacement of the pulmonary valve, transcatheter pulmonary valve replacement may be an option. While this option has a shorter recovery time, the longevity of the valve remains unknown. Early findings show that transcatheter PVR could be used as an alternative to traditional surgical methods in appropriate patients. The percutaneous PVR approach involves the placement of a bovine valve inside a balloon stent.

As physicians, we’re encouraged by the great strides we’re making from both surgical and technological standpoints with pulmonary valve replacement and congenital heart disease in general.

Do you have questions regarding any of these medical advances? If so, please be sure to let me know in the comments section.

About Brian E. Kogon, MD:

Dr. Kogon is an assistant professor of surgery and director of the congenital cardiac surgery fellowship at Emory. Additionally, he’s the surgical director of adult congenital cardiac surgery at Emory University Hospital. His clinical interests include pediatric cardiac surgery, cardiac transplantation, and adult congenital heart surgery. Dr. Kogon has been with Emory as a faculty member since 2004.

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  • Jon B.

    Dr. Kogon,

    I had Tetralogy of Fallot surgery at age 2. I am now in my 30s and on my most recent visit to my doctor he indicated that testing yielded mild to moderate pulmonary regurgitation and a MRI showed that my end right ventricular volume suggested that in time I might need to have a pulmonary valve replacement. It should also be noted that the pulmonary valve was just repaired at age 2 and not replaced. My question for you is “Is pulmonary valve replacement necessarily in my future?” I’ve been told that pulmonary system is a low-pressure system, which indicates to me that the deterioration of the pulmonary valve may be slower than that of other heart valves. Is this thinking correct? Thanks immensely for your response.

    • Dr. Kogon

      Your are correct – the right side of the heart is a lower pressure system, so in general, a leak the pulmonary valve is tolerated longer than a similar problem on the left side of the heart. However, the pulmonary valve does eventually have to be replaced in most adults who have had prior repair of tetralogy of Fallot as the additional workload eventually outstrips the capacity of the right ventricle. Evaluation in a center specializing in the care of adults with congenital heart disease may be useful to answer the question of whether the valve needs to be replaced in your specific situation.

      Thanks for your question,
      Dr. Kogon

  • Nichi

    Hello Dr. Kogon,

    I had my ToF repair nine months after being born [year removed for patient privacy]. I am now [age removed for patient privacy] and just found out that I will need to have the pulmonary valve replaced. I was informed the “trans-valvular gradient” (I think that was the term) is 160. What does that number tell me? I am very active – I run and dance a lot and I hear the muscle in the chambers of the heart are strong, so I wonder why a gradient of 160 is worrisome. Also, do I need to curtail my running and strenuous exercises now?

    Thanks very much.

    • Hey Nichi,

      While I can’t speak to your specific situation without seeing you in-person, I can give you some general information that should help out:

      The initial operation for Tetralogy of Fallot involves: 1) closing a hole in the heart, and 2) enlarging a small pulmonary valve opening. Although this repair works well for many many years, subsequent anatomic and functional problems can arise. Most commonly, the pulmonary valve is leaky (regurgitant), although sometimes it can become narrow (stenotic). If left untreated, this can lead to right heart failure and arrhythmias. The treatment is re-operative surgery and pulmonary valve replacement.

      A transvalvular gradient of 160 mmHg is quite high, and usually means there is severe narrowing at the level of the valve. The right ventricle can typically compensate for a period of time, although it is unhealthy for the heart long-term. I would be somewhat concerned about vigorous exercise with this degree of obstruction.

      You should consult with/see your physician to get a more detailed workup of your condition. He or she will be able to make an assessment on your current exercise levels and make recommendations based on your specific situation.

      Hope this helps,
      Dr. Kogon

  • Tim B.

    Dr. Kogon-

    Thank you for your PVR information – yet another point of data to be considered!

    I have recently been told that my pulmonary valve will need replacement some 40+ years after my TOF repair. This will make me a member of the “triple-zipper” club as I had surgery originally as a toddler and then age 9 (TOF repair). Not something that I am looking forward to and, to be honest, the recent diagnosis of valve replacement was something that I had not anticipated – I guess Mom and Dad didn’t share that little tidbit as I was growing up.

    Now for the $64 question – My cardiologist has indicated that the timing of the surgery is up to me (at least at the moment). Given that organic replacements have a lifespan of 10-15 years, I have been trying to delay the surgery as long as possible. However, since being “diagnosed” I seem to have noted an increase in arrhythmia and have had days where I have been more ‘sluggish’ than normal as well as getting more tired than usual after doing light exercise or chores around the house. In your opinion, what are some of the more ‘critical’ signs that would indicate the need to have the replacement valve surgery performed sooner rather than later? Also, in your experience, how long is recovery (able to resume work/travel) after repeated surgeries (especially those involving opening the sternum multiple times)? I am concerned with the ability to lift suitcases and briefcases that can, at times, be somewhat heavy.

    Many thanks, in advance. I know from reading MANY papers that the timing for valve replacement is not a precise science – but any input you may have would certainly be appreciated as another “voice of experience”.

    Best regards

    • Moderator

      Personal details removed to protect patient privacy

    • Dr. Kogon

      Hi Tim,

      While I can’t speak to your specific situation without seeing you in-person, I can give you some general information that should help out.

      Many patients with Tetralogy of Fallot will indeed need pulmonary valve replacement later in life. The presence of symptoms, as you suggest, are often indications for surgery themselves. For those patients without symptoms, we often resort to echocardiographic findings and EKG changes to dictate timing for pulmonary valve replacement. With these tests, indications for surgery include increased right ventricular size, decrease in right or left ventricular function, signs of tricuspid valve leakage, or prolonged electrical conduction through the ventricle.

      In general and on a positive note, if the shunt was done through a thoracotomy (side or back incision), rather than the front, then you are only in the “double” zipper club. Only if the shunt was done through a sternotomy (front) incision, would you be a member of the “triple” zipper club.

      In general I tell patients to anticipate the new bioprosthetic valve would last closer to 20-30 years rather than 10-15. Surgery is typically 4-5 hours. The total hospital stay is 5-6 days. At the time of discharge, patients are doing a “normal” routine. However, there are restrictions for 4-6 weeks related to heavy lifting and driving. At 8 weeks, the breastbone is completely healed and the restrictions are lifted.

      If you need more information feel free to call my office and set-up an appointment (404) 778-5036

      Hope this helps,
      Dr. Kogon

  • karen

    Dear Dr. Kogon,
    I had pulmonary stenosis as a child and had 90 percent stenosis when they discovered it at the age of 12. I am now in my 40’s and have shortness of breath and a mild to moderate pulmonary regurgitation with dilated right atrium and ventricle. My doctor is urging me to have the transvascular PVR. Can I expect a significant increase in function? I would also like to know if I will be on blood thinners like Coumadin.

  • karen

    I am sorry. I forgot to add that I had the valvulotomy to correct the stenosis when I was 12.

  • Pat W.

    Hi Dr. Kogon,
    Does Emory Adult Congenital Heart Center currently perform the transcatheter pulmonary valve replacement procedure, to those that qualify? If so, can you tell me which physician is doing that procedure?

    Thank you,

    • Hello Pat,

      Thanks for your question. Yes. Our program does offer transcatheter pulmonary valve replacement. You can find more information on the procedure and the program here: http://www.emoryhealthcare.org/congenital-heart/treatments-services.html

      Physicians at Emory performing this procedure are:

      Dr. Vasilis Babiliaros
      Dr. Dennis Kim
      Dr. Robert Vincent
      Dr. Peter Block

      Hope this helps and thank you for your question!
      Dr. Kogon

  • Laura

    Hi, Dr. Kogon, My daughter had full heart repair for tetralogy of fallot this year at 5 months. She is doing really well now but I was wondering how her heart will cope without her heart valve as she had it removed.


    • Hi Laura,

      The majority of repairs for Tetralogy of Fallot involve removing the pulmonary valve. Fortunately, this is very well tolerated. Patients can “grow up” through childhood, teenage years, and even early adulthood with few symptoms and limitations. At some point, however, the right heart begins to dilate as a result of the leaking or absent pulmonary valve. At that time, it may be reasonable to undergo subsequent pulmonary valve replacement. On average, the typical time frame at which this becomes necessary is 25-40 years of age. Hope the information was helpful.
      -Dr. Kogon

  • Anne S.

    I had pulmonic valve placement in 2004 for TOF. Original surgery done in 40 years prior. My Dr. states the pig valve should last 7 to 10 years. I think you are saying more like 10-15 years. What would make the difference in the longevity of this type of valve. I am not overweight, do not smoke and in good health. Thanks for your help.

    • Hi Anne,

      Thank you for your questions. The various types of tissue valves that can be used in the pulmonary position include “pig valves”, “cow pericardial valves”, and “ cadaver homografts”. There is no data to show any major differences between them with respect to longevity. Tissue valves placed on the left side of the heart in the aortic and mitral positions typically last 10-15 years. Due to decreased pressure and stress on the right side of the heart, those placed in the pulmonary position should last even longer. Hope this helped.

      – Dr. Kogon

  • eman

    my son had tof repair on age of 4 month after one year his dr told us he is having moderate regargetation and dilation on right ventcular and he needs pulmonary valve replacement he is now 16 month old it is correct to have this operation on his age and what is the out come of this operation.