Adolescent & Adult Congenital Heart Disease (ACHD)

Redo heart surgery

Many babies and children who undergo heart surgery early in life will require further heart surgery later in life during adolescence or adulthood. If the original heart surgery was performed through the front of the chest using a midline incision called a “median sternotomy”, the subsequent operation(s) will likely be performed by using this same approach. This mildly increases the risk of these subsequent operations as the heart and large blood vessels in the chest become stuck to the back of the sternum due to scarring during the healing process after the first operation. Careful re-opening of the sternum on the second and subsequent operations is usually adequate to safely re-enter the chest however there is always a small risk of injuring the heart or large blood vessels. Sometimes, in teenagers and adults, it is necessary to make a small incision in the groin and use the artery and vein in the groin to go onto the heart-lung machine prior to opening the chest. This can allow the heart and large blood vessels to be partly protected and also allows rapid return of blood to the patient if significant bleeding is created when opening the chest. The risks of redo surgery are specific to each individual and Dr Orr will discuss these with you prior to your operation.

Pulmonary valve replacement

Infants and children who have undergone repair of abnormalities early in life associated with a narrowed or small pulmonary valve will frequently require surgery later in life to replace the pulmonary valve. The pulmonary valve is situated between the right ventricle and pulmonary (lung) artery and allows blood to flow forward into the lungs from the right heart but prevents blood from leaking backwards into the heart. In conditions such as Tetralogy of Fallot, severe pulmonary stenosis and pulmonary atresia with intact ventricular septum the pulmonary valve is often completely removed at the original surgical repair, allowing blood to flow forward freely into the lungs but also resulting in significant leakage of blood backwards from the lung arteries into the right ventricle. As the blood pressure in the lungs is low, this leakage of blood back into the right heart is well tolerated for many years but eventually the right heart will start to stretch and enlarge. Eventually this leads to abnormal heart rhythms, palpitations, occasionally shortness of breath and also a small risk of sudden cardiac death in adult life due to abnormal heart rhythms. Once the right heart has enlarged to a certain amount this is an indicator that it is time to start planning to replace the pulmonary valve. Often a cardiac MRI in addition to regular follow-up echos is useful in determining the optimal timing for pulmonary valve replacement. It is most often required in teenage years and this allows implantation of an adult sized valve. Re-inserting a valve between the right ventricle and lung arteries stops blood leaking back into the right ventricle. This allows the right ventricle to shrink down in size and should relieve any symptoms that have been noticed.

Pulmonary valve replacement is performed using a redo median sternotomy (see section above) and the heart-lung machine is used to support the circulation while the valve is implanted. Although the work of the heart and lungs is performed by the heart-lung machine during the operation, often the heart can remain beating as the valve is implanted into the outlet of the right ventricle and no other chamber of the heart needs to be accessed. If there are additional abnormalities to be fixed such as residual holes inside the heart (atrial septal defect, ventricular septal defect), muscular narrowing of the right ventricular outflow tract or other lesions then is may be necessary to stop the heart for some of the procedure, similar to during the first repair operation.

The surgery for pulmonary valve replacement is usually about 4 hours in duration. Recovery following pulmonary valve replacement usually requires 1-2 nights in the intensive care unit followed by 5-6 days on the post-operative ward.

Aortic valve replacement (AVR)

One of the most common heart abnormalities (1% of individuals) is development of the aortic valve (outlet valve from the left heart) with 2 leaflets (bicuspid aortic valve) rather than the normal 3 leaflets. Sometimes this is associated with other abnormalities such as aortic coarctation that are repaired early in life. Bicuspid aortic valve can be associated with narrowing of the aortic valve that can be very significant at birth or develop throughout life. If the narrowing is significant during early childhood often the aortic valve can be stretched using a balloon inserted via a catheter through an artery in the groin. This may relieve the narrowing but cause some leakage of the aortic valve that can be tolerated for a long period of time. Eventually many individuals with a biscupid aortic valve will require replacement of the valve.

Aortic valve replacement is performed through an incision in the midline of the chest and sternum (median sternotomy) and requires use of the heart-lung machine to support the circulation during the procedure while the heart is temporarily stopped. The procedure carries a small risk of injury to the conduction tissue that controls the heart rhythm as it passes across the outlet of the left heart beneath the aortic valve and therefore a small risk (1%) of requiring subsequent pacemaker implantation. The procedure usually takes about 4-5 hours and requires 2-3 days in intensive care afterwards followed by about 5-7 days on the ward recovering.

The are several options for replacement of the aortic valve in children, adolescents and young adults:

  • Mechanical AVR
    A mechanical (metal) is often suggested for valve replacement in young individuals as these valves are designed to last longer than a human will live, thereby greatly limiting the risk of requiring further surgery. Mechanical valves make a clicking noise each time they open and close that sounds like a clock ticking. Individuals with mechanical valves are maintained on lifelong warfarin (a blood-thinning medicine) to prevent clots forming on the valve that could cause a stroke or obstruction to valve opening. Warfarin carries a lifelong risk of bleeding particularly if the blood levels are not tightly controlled. Individuals who manage their warfarin levels carefully can have a close to normal life but there are certain limitations in physical activities required to prevent you from having a major bleeding event. Dr Orr will discuss these with you. Unfortunately, Warfarin can cause fetal abnormalities and needs to be avoided during the first trimester of pregnancy so it is useful if it can be avoided in young women planning to become pregnant. There are alternatives to warfarin for use during pregnancy so it is possible for someone with a mechanical valve to become pregnant as long as this is carefully planned and managed.
  • Bioprosthetic AVR
    Bioprosthetic valves are tissue valves that come from either a cow or pig. They are designed to function like a human valve however they gradually wear out over time mostly due to wear and tear but partly because the human body sees them as foreign. In the aortic position a bioprosthetic valve will last for about 10-15 years in a young person and will then need to be re-replaced. The benefits of a bioprosthetic valve are no noise (doesn’t click when opening) and no need for any blood thinning medication so that you can have a normal lifestyle. Bioprosthetic valves may be preferred in young women who are considering pregnancy due to the complex issues associated with taking blood-thinning medication (warfarin) during pregnancy. If you receive a bioprosthetic valve it is important to continue to have regular follow-up with your cardiologist to ensure that the valve is working well and to detect any valve dysfunction early.
  • Ross procedure
    When children, adolescents or young adults require aortic valve replacement another very useful option is to harvest the individual’s pulmonary valve and implant it into the aortic position. The pulmonary valve is then replaced with a donated heart valve (homograft) or a valve from a cow vein (Contegra conduit). The pulmonary valve is the outlet valve to the right heart and is very similar in structure to the aortic valve. It needs to be harvested as a cylinder containing the valve inside so that the valve retains its support mechanism. When it is implanted into the aortic position the coronary arteries (small arteries that supply blood to the heart) also need to be reimplanted as they arise from the walls of the aortic root that were supporting the abnormal aortic valve. This procedure is performed through an incision in the midline of the chest (median sternotomy) and requires use of the heart-lung machine to support the circulation during the operation. It takes about 5-6 hours and requires 2-3 days recovery in intensive care afterwards followed by 5-7 on the ward.

The main benefit of the Ross procedure is that the pulmonary valve, once implanted into the aortic position will grow with the child. In addition, blood-thinning medication is not required and the individual can return to living a normal life. The main disadvantage is that the replaced pulmonary valve will eventually wear out and required re-replacement. This can be done either as an open-heart surgical procedure or frequently as a keyhole procedure by inserting a valve through a large catheter inserted via a leg vein. It usually takes about 15 years for the pulmonary valve to wear out. When leaking of the aortic valve or enlargement (dilatation) of the aorta is the reason for aortic valve replacement the Ross procedure is not advised, as the pulmonary valve implanted in the aortic position is likely to stretch and become leaky (regurgitant) over time.

Aortic root replacement

The aortic root is the part of the aorta that is attached to the left ventricle and forms part of the outlet from the left heart. It supports the aortic valve (outlet valve to the left ventricle) and the coronary arteries (very important small arteries that supply blood to the heart muscle). Following repair of heart abnormalities that involve the outlets of the right and left hearts, enlargement of the aortic root can occur. This problem is associated with several underlying congenital heart conditions such as repaired Truncus arteriosus, Transposition of the great arteries (treated by arterial switch operation in the newborn period) and repaired of Tetralogy of Fallot. Connective tissue diseases such as Marfan’s syndrome, Ehler’s Danlos syndrome and Loeys-Dietz syndrome are also associated with significant enlargement of the aortic root. In all of these abnormalities, the wall of the aortic root is weaker than normal and has a tendency to stretch and enlarge over time. As the aortic root enlarges it stretches the aortic valve and causes it to leak. In many cases both the aortic root and the aortic valve leaflets need to be replaced using a procedure called an aortic root replacement or Bentall’s procedure. In some cases it is possible to preserve the native aortic valve leaflets and only replace the wall of the aortic root using a procedure called a valve-sparing aortic root replacement or David procedure.

  • Composite aortic root replacement
    The aortic root can be replaced using a Dacron tube containing either a mechanical (metal) aortic valve or a tissue (bioprosthetic ie. cow or pig) aortic valve. Mechanical valves last a long time and are designed to last longer than a human lifespan. Individuals with mechanical valves are maintained on lifelong warfarin (a blood-thinning medicine) to prevent clots forming on the valve that would cause a stroke or obstruction to valve opening. Warfarin carries a lifelong risk of bleeding particularly if the blood levels are not tightly controlled. Bioprosthetic valves eventually wear out and will need to be replaced again once the valve no longer works effectively. This process occurs faster in younger individuals and takes about 10-15 years for aortic bioprosthetic valves. Bioprosthetic valves do not require blood-thinning medication.
  • In general mechanical valves are recommended for younger individuals in order to avoid further operations. However bioprosthetic valves are a reasonable option for people who want to have a normal lifestyle but accept the need for a repeat operation to replace the deteriorated valve in the future.

    The operation for aortic root replacement is performed through an incision in the midline of the sternum and requires use of the heart-lung machine to support the circulation. The heart is temporarily stopped for most of the procedure while the diseased aortic root is removed. The tube containing the new valve is then implanted and then the coronary arteries (which are branches of the aortic root that supply blood to the heart muscle) are then individually implanted into the sides of the tube above the new valve. The heart is then restarted and the patient is weaned from the heart-lung machine. This procedure takes about 6 hours and requires about 2-3 days in intensive care afterwards followed by 5-7 days on the post-operative ward. If a mechanical valve has been implanted then warfarin will be commenced 1-2 days following the operation. It takes several days to obtain adequate blood levels for the warfarin and regular blood tests are required, even after discharge. Your GP will be able to manage this and help you adjust the dose of the warfarin. It is also possible to obtain a machine to monitor the blood levels of the warfarin at home using a fingerprick blood test. Dr Orr can help arrange this for you following the surgery.

    • David procedure
      This procedure involves replacement of the wall of the aortic root using a tailored Dacron tube with re-suspension of the aortic valve within the tube. It requires the aortic valve leaflets to be almost normal and is not applicable in all circumstances. The procedure is performed through an incision in the midline of the sternum and requires use of the heart-lung machine to support the circulation. The heart is temporarily stopped for most of the procedure while the diseased wall of the aortic root is removed. The wall of the aortic root is then replaced with a tailored Dacron tube into which the aortic valve is carefully sewn using a small rim of retained aortic wall. The coronary arteries (which are branches of the aortic root that supply blood to the heart muscle) are then individually implanted into the sides of the tube above the aortic valve. The heart is then restarted and the patient is weaned from the heart-lung machine. This procedure takes about 6 hours and requires about 2-3 days in intensive care afterwards followed by 5-7 days on the post-operative ward. Long term monitoring is important as the aortic valve leaflets may still stretch over time and it is possible that the aortic valve can start to leak later on in life.

    Aortic arch surgery following previous aortic coarctation repair

    Individuals who have undergone repair of an aortic coarctation (narrowing of the upper part of the aorta in the left chest) early in life can develop recurrent narrowing or significant enlargement of this area of the aorta throughout their life. Consequently regular monitoring and follow-up is recommended. If recurrent narrowing develops this can often be fixed or improved by a catheter procedure whereby the narrowed area of the aorta is stretched with a balloon and then a stent is implanted into the narrowed region. If enlargement of the aorta (aneurysm formation) occurs this can also be dealt with using covered stents and a keyhole approach through the artery in the groin. However sometimes either recurrent narrowing or aneurysm formation are better managed using an open surgical repair for technical reasons and optimal long-term results. This is most frequently performed through an incision in the left chest under the shoulder blade but can also be performed through an incision in the midline of the front of the chest (median sternotomy). It is frequently necessary to use the heart-lung machine to provide blood flow to the lower body while the abnormal segment of the aorta is replaced with a synthetic graft. Sometimes the repair is so extensive that blood flow to the lower body and parts of the brain need to be interrupted for a period of time to facilitate the repair. During this time the body temperature is maintained at 18oC to protect the brain and other vital organs. It is also quite common to need a blood transfusion and other blood products with these procedures as cooling to 18oC is associated with poor function of the clotting proteins and cells in the blood, even after re-warming. These procedures often take 4-6 hours and require 3-5 days in intensive care afterwards in addition to 5-7 days on the ward recovering.

    Ebstein’s Anomaly

    The tricuspid valve, which is the inlet valve to the right ventricle (right pumping chamber), can sometimes develop very abnormally. This condition is called Ebstein’s Anomaly and can be associated with a hole between the 2 top chambers of the heart (atrial septal defect) and weakness and thinning of the wall of the right ventricle. The tricuspid valve can leak very significantly in Ebstein’s anomaly. This results in stretching of the top chamber of the right heart (right atrium) that can predispose to abnormal, fast and irregular heart rhythms such as supraventricular tachycardia (SVT) and atrial fibrillation (AF) that may be felt as palpitations. The leaking (regurgitant) valve can also cause breathlessness, fatigue and swelling of the legs.

    The tricuspid valve can either be repaired or replaced depending on the extent of the abnormality. The procedure required to repair the valve is called a Cone repair. It is a complex procedure and requires an adequate amount of normal valve leaflet tissue to be available. Therefore it is not always possible to repair the valve and valve replacement may be the preferred option. The tricuspid valve is most often replaced with a bioprosthetic (tissue) valve from either a cow or pig. Mechanical (metal) valves tend to be avoided in the tricuspid position due to a high risk of clotting on the valve and need for lifelong blood thinning medication (warfarin). Bioprosthetic valves wear out with time and will eventually need to be re-replaced. However, in the low pressure right heart this can take 15-20 years.

    If you also have abnormal irregular heart rhythms (atrial fibrillation) it may also be helpful to perform a MAZE procedure at the same time as repairing or replacing the tricuspid valve. The MAZE procedure involves creating lines of scars on the top chambers of the heart using a cryoprobe that freezes the heart muscle. These lines then create a pathway for the normal transmission of the heart rhythm while blocking abnormal electrical currents causing the fast and irregular heart rhythms. The MAZE procedure can reduce the occurrence of your abnormal heart rhythms and may restore normal heart rhythm.

    Procedures on the tricuspid valve (including the MAZE procedure when required) are performed through an incision in the midline of the chest and sternum (median sternotomy) and require use of the heart-lung machine to support the circulation during the procedure while the heart is temporarily stopped. The procedure usually takes about 4-5 hours and requires 2-3 days in intensive care afterwards followed by about 5-7 days on the ward recovering. If the tricuspid valve is repaired your own valve leaflets will be preserved and near-normal valve function is often restored. There is always a possibility that further surgery on your tricuspid valve may be necessary at some point in your life and you should continue to have regular check-ups with your cardiologist.

    Operations to relieve left ventricular outflow tract obstruction (LVOTO)

    Infants and children who have undergone repair of several different types of heart abnormalities early in life can develop recurrent or new narrowing of the outlet to the left side of the heart. Some of these previously repaired problems include atrioventricular septal defects, replacement of the mitral valve early in life, double outlet right ventricle and subaortic stenosis or subaortic membrane. Sometimes left ventricular outflow tract obstruction (LVOTO) or subaortic stenosis can develop in the absence of prior procedures or heart abnormalities. Several different surgical procedures are available to treat left ventricular outflow tract obstruction depending on the type of narrowing and the previous procedure(s) performed. All procedures are performed through an incision in the midline of the chest and sternum (median sternotomy) and require use of the heart-lung machine to support the circulation during the procedure while the heart is temporarily stopped. All of these procedures carry a small risk of injury to the conduction tissue that controls the heart rhythm as it passes across the LVOT and therefore a small risk (1-2%) of requiring subsequent pacemaker implantation. The surgical options for repair of LVOTO include:

    • Subaortic resection and septal myectomy
      This procedure is performed for LVOTO that is localized to the area immediately beneath the aortic valve in the LVOT. It involves removal of muscle and scar tissue in this region to relieve LVOTO while preserving normal aortic valve function. Sometimes the aortic valve leaflets are also scarred with membranous tissue covering the leaflets that also needs to be removed. There is a small risk of injury (1-2%) to the aortic valve with this procedure
    • Modified Konno
      This procedure is performed for LVOTO that is localized to the area immediately beneath the aortic valve in the LVOT. It involves more extensive removal of muscle in the LVOT and creation of an incision in the upper part of the wall (ventricular septum) between the right and left ventricles immediately beneath the aortic valve. A patch is then sutured to the edges of this incision to enlarge the circumference and diameter of the LVOT by allowing it to take up some room in the right ventricular outflow tract (RVOT). A patch is then also placed on the front wall of the right ventricle over the area of the ventricular septal patch to augment the RVOT and prevent any significant narrowing.
    • Konno with bioprosthetic or mechanical aortic valve replacement
      Sometimes the narrowing of the LVOT also involves the aortic valve in addition to the area below the valve. In this case it is necessary to excise muscle in the LVOT, enlarge the LVOT using the Konno procedure but then also to replace the narrowed aortic valve using either a bioprosthetic (cow or pig) valve or a mechanical (metal) valve. This procedure allows for a very extensive repair of LVOTO at multiple levels throughout the left ventricular outflow tract.
    • Ross-Konno procedure
      Sometimes the narrowing of the LVOT also involves the aortic valve in addition to the area below the valve. In this case it is necessary to excise muscle in the LVOT, enlarge the LVOT using the Konno procedure described above and then also to replace the narrowed aortic valve. The aortic valve can be replaced by harvesting the pulmonary valve (outlet valve from the right ventricle) and suturing it into the aortic position after removing the narrowed aortic valve. This approach is mainly used in adolescents and children in whom growth of the aortic valve is beneficial. The disadvantage of this procedure is that the pulmonary valve also needs to be replaced and will eventually require re-replacement when it wears out over the subsequent 15-20 years.

    Mitral valve repair or replacement

    Infants and children who have undergone repair of several different types of heart abnormalities early in life can develop recurrent or new leakage (regurgitation) or narrowing (stenosis) of the mitral valve (inlet valve to the left side of the heart). In some cases abnormalities of the mitral valve develop over the first few years of life and present in teenage or adult years. Some of the underlying heart conditions associated with recurrent or new mitral valve disease include previously repaired atrioventricular septal defects, Shone’s syndrome (small left heart structures) associated with congenital mitral stenosis that may have been previously repaired, rheumatic heart disease and “floppy” or myxomatous mitral valve disease. In some cases the mitral valve has already been replaced early in life and is now too small or does not function normally and needs to be re-replaced.

    Depending on the underlying problem the mitral valve can either be repaired or replaced. These procedures are performed through an incision in the midline of the chest and sternum (median sternotomy) and require use of the heart-lung machine to support the circulation during the procedure while the heart is temporarily stopped. The procedure usually takes about 4-5 hours and requires 2-3 days in intensive care afterwards followed by about 5-7 days on the ward recovering. If the mitral valve is repaired various techniques can be used but importantly your own mitral valve leaflets will be preserved and near-normal valve function is restored. There is always a small possibility that further surgery on your mitral valve may be necessary at some point in your life and you should continue to have regular check-ups with your cardiologist.

    If the mitral valve is replaced either a mechanical (metal) valve or a tissue (bioprosthetic ie. cow or pig) valve is used. A mechanical valve is preferred for mitral valve replacement in young individuals as these valves are designed to last longer than a human will live, thereby limiting the risk of requiring further surgery. Individuals with mechanical valves are maintained on lifelong warfarin (a blood-thinning medicine) to prevent clots forming on the valve that would cause a stroke or obstruction to valve opening. Warfarin carries a lifelong risk of bleeding particularly if the blood levels are not tightly controlled. Individuals who manage their warfarin levels carefully can have a close to normal life but there are certain limitations in physical activities required to prevent you from having a major bleeding event. Dr Orr will discuss these with you. Unfortunately, Warfarin can cause fetal abnormalities and needs to be avoided during the first trimester of pregnancy so it is useful if it can be avoided in young women planning to become pregnant. There are alternatives to warfarin for use during pregnancy so it is possible for someone with a mechanical valve to become pregnant as long as this is carefully planned and managed.

    Bioprosthetic valves in the mitral position are exposed to the high pressure in the left ventricle (pumping chamber) and wear out over time. It takes about 7-10 years for this to occur and then the valve then needs to be replaced again using open-heart surgery. However, bioprosthetic valves do not require the use of lifelong blood thinning medication and are associated with a fairly normal lifestyle. They are a useful option for young women planning pregnancy who need a mitral valve replacement as warfarin can be avoided entirely. However, bioprosthetic valves wear out faster during pregnancy due to the increased workload of the heart and therefore the valve will almost certainly need to be replaced again within 5-8 years.

    Pacemakers and defibrillators

    See Adult section

    MAZE procedure

    Many individuals who have undergone repair of heart abnormalities early in life will develop abnormal heart rhythms that often start in the upper chambers of the heart (atria). The most common of these abnormal rhythms are atrial fibrillation (irregular heart rhythm) and atrial flutter (very fast heart rate due to a very fast abnormal pacemaker beat in the top chamber of the heart). These heart rhythms can sometimes be well-controlled with medications however, if further heart surgery is required to correct structural abnormalities, then it is possible to perform a surgical procedure that can improve these abnormal rhythms and sometimes correct them altogether. The surgical procedure is called a MAZE procedure and involves creating lines of scars on the top chambers of the heart using a cryoprobe that freezes the heart muscle. These lines then create a pathway for the normal transmission of the heart rhythm while blocking the abnormal electrical currents causing the fast and irregular heart rhythms. Sometimes it is also necessary to implant a pacemaker as the underlying heart rhythm can occasionally become too slow after this procedure.

  • The Children's Hospital at Westmead
  • Heart Centre for Children- The Children's Hospital at Westmead
  • Sydney Children's Hospital Randwick
  • Westmead Hospital
  • Westmead Private Hospital
  • Royal Australasian College of Surgeons
  • International Society for Heart and Lung Transplantation
  • Sydney Adventist Hospital