Tricuspid Valve Disease (TVD)
Tricuspid valve disease occurs when the valve between the right ventricle and right atrium of the heart is not functioning properly.
There are 4 main types of tricuspid valve disease:
Tricuspid valve regurgitation: the tricuspid valve is not working properly, allowing blood to flow from the right ventricle back into the right atrium. This can also be present at birth (congenital).
Tricuspid valve stenosis: the opening of the tricuspid valve is narrowed causing a decrease blood flow to the right ventricle.
Ebstein’s anomaly: also present at birth (congenital). This abnormally formed tricuspid valve sits lower in the right ventricle and thus reduces the size of the right ventricle and causing backflow of blood into the right atrium.
Tricuspid atresia: a congenital defect meaning that it is present at birth. The tricuspid valve is not formed which results in a tissue blocking the blood flow between the right ventricle and right atrium.
Depending on the type and severity of your tricuspid valve defect, the doctor can choose to monitor your condition or treat the defect. If you are symptomatic, the tricuspid valve can either be repaired or replaced by an artificial valve. Tricuspid valve repair or replacement can be accomplished through a traditional surgical or novel transcatheter approach. The symptoms, causes, and treatment of tricuspid valve disease depends on which type of tricuspid valve disease you have. In tricuspid atresia, there is no way to replace the tricuspid valve at the present but there are temporary fixes that the surgeon can perform to alleviate the stress on the baby’s heart. As such, we will be focusing on tricuspid valve regurgitation, stenosis, and Ebstein’s anomaly.
What does this mean for me?
Tricuspid valve disease is rarely an isolated condition unless it is tricuspid atresia or Ebstein’s anomaly, meaning that you may have other pre-existing heart problems. If you have some form of tricuspid valve disease but do not show any symptoms, you may not need treatment aside from going to your cardiologist regularly to monitor your condition. However, if the tricuspid valve shows severe stenosis or regurgitation and you are presenting with signs and symptoms (i.e. fatigue, declining exercise capacity, shortness of breath, palpitations, swelling in abdomen, legs, or neck), the doctor may prescribe medications to treat the irregular heart rhythm and heart failure associated with tricuspid valve disease. The doctor may also consider repairing or replacing your tricuspid valve. Whether it is a repair or replacement of the tricuspid valve, the surgeon will most likely do it in conjunction with another planned cardiac procedure (e.g. mitral valve repair/replacement). The symptoms and treatment options all depend on the underlying cause of the tricuspid valve disease and your overall health.
If you are pregnant or planning to become pregnant and have tricuspid valve disease, it is important to be evaluated by cardiologists that specialize in treating pregnant women with valve disease or congenital valve defects.
Symptoms: How would I know if I have TVD?
Tricuspid valve regurgitation and stenosis can cause the following signs and symptoms:
Shortness of breath (dyspnea)
Declining exercise capacity
Swelling in the abdomen, legs, and neck
Abnormal heart rhythm
These symptoms may also coincide with the symptoms caused by the original condition (e.g. pulmonary hypertension) that resulted in your tricuspid valve disease.
Next Steps: Investigation Plan
Tricuspid valve disease is commonly diagnosed by tests that are performed for other pre-existing conditions. However, if symptoms are present and are indicative of tricuspid valve disease, the doctor will run a battery of tests that measure and reveal the structure and function of your heart. These tests include:
Echocardiogram (transthoracic or transesophageal)
Treatment Options for TVD
If the doctor decides to repair your tricuspid valve, the only option is surgery. The surgeon will repair your valve based on the type of tricuspid defect you have. Repair can be accomplished by:
Closing the holes (fenestrations) in the leaflets of the valve
Separating tethered valve leaflets
Reshaping the valve leaflets so they are in contact with another to prevent backflow of blood
If you have Ebstein’s anomaly, some surgeons will perform a newer procedure called a “cone repair”. The tricuspid leaflets will be separated from the underlying heart muscle and rotated and reattached to form a “leaflet cone”.
Advantages of having your tricuspid valve repaired rather than replaced are that the functioning valve is from your own tissue, which decreases your chance of infection and does not require you to take blood-thinning medication, and increases right ventricle function.
However, if your tricuspid valve is deemed unfit for repair, the tricuspid valve will be replaced. In tricuspid valve replacement, the surgeon will remove parts of the tricuspid valve and replace it with either a mechanical valve or a biological (bioprosthetic) valve. The decision to use either a mechanical or biological valve will be based on many factors such as durability, use of medication, and need for additional surgery. A mechanical valve lasts longer but blood-thinning medication must be taken for life to prevent clots from forming on the device, which can lead to serious events such as heart attack and stroke. A biological valve (which is made from animal tissue) does not require you to take lifelong medication but the probability of needing another valve replacement in the future is higher. You and your doctor can decide on a valve that fits your physical needs and lifestyle.
Unlike tricuspid valve repair, your tricuspid valve can be replaced either surgically or with a less invasive approach, known as a transcatheter valve replacement. Although surgery is the more common approach in replacing the tricuspid valve, there is a higher risk of post-procedural complication and mortality in these patients. A transcatheter valve replacement provides a less invasive procedure for patients that are too high risk for open-heart surgery. This is a relatively novel procedure and studies are currently underway in determining the efficacy of a transcatheter approach compared to the surgical approach. Therefore, most transcatheter tricuspid valve replacements are performed if a patient had a previous surgical biological tricuspid valve repair that is no longer functioning. This procedure uses a catheter that is inserted into a blood vessel in your neck or leg and is then guided to your heart using x-ray imaging. The original bioprosthetic valve in the heart will be inflated by a balloon which is attached to the end of the catheter and a new valve will be deployed within it.
There are additional procedures that are also performed to treat the underlying causes and/or symptoms:
Catheter ablation: if you are experiencing fast or irregular heart rhythm, your doctor may perform a catheter ablation in which a catheter is guided through your blood vessels to the heart which then delivers heat, extreme cold, or radiofrequency energy to create a bit of scar tissue in your heart to prevent electrical impulses that are causing the abnormal heart rhythm.
Maze procedure: a surgical approach in treating fast heart rhythms. The surgeon will make small incisions in your heart to produce scar tissue that will block electrical impulses that are causing the fast heart rhythm. This procedure is more invasive and is usually performed during a surgical valve repair or replacement.
Long Term Health Implications
If your tricuspid valve disease and its underlying causes are severe and left untreated, it can result in:
Heart failure: as the right ventricle starts to dilate and weaken because of increased blood volume, it can lead to heart failure over time.
Atrial fibrillation: irregular heart rhythm that can increase the risk of stroke and heart attack.
Causes: Why do I have TVD?
Tricuspid atresia and Ebstein’s anomaly are congenital disorders, meaning the valve defect is formed during fetal development in the womb. The causes are unclear although factors such as genetics and prenatal environment do play a role. Genetic disorders such as Down’s syndrome has been shown to increase risk of developing congenital heart defects.
Tricuspid valve regurgitation is a condition that allows blood from the right atrium to flow back into the right ventricle. Usually, it is secondary to a condition that you already have such as heart failure, pulmonary hypertension, or cardiomyopathy (disease of heart muscle). These conditions cause the right ventricle of the heart to dilate which prevents the tricuspid valve from working properly. Problems of the tricuspid valve can also be caused by:
Ebstein’s anomaly (see above)
Infective endocarditis: infection of the lining of the heart as well as the valves causing damage
Carcinoid syndrome: a rare condition in which tumours develop in your digestive system and lungs which can spread to your lymph nodes or liver. Carcinoid syndrome can lead to carcinoid heart disease in which your heart valves become scarred because of the substances that the tumours secrete.
Pacemakers/implantable cardioverter defibrillators (ICD): injury to the tricuspid valve may occur during placement or removal of the device.
Rheumatic fever: a complication that results from untreated strep throat in children. This can damage the tricuspid valve and cause tricuspid regurgitation later on in life. Although it is rare in North America, it is still common in some other countries.
Marfan syndrome: a connective tissue disorder that is present at birth (congenital) can cause structural abnormalities of the heart leading to tricuspid regurgitation.
Tricuspid valve stenosis is the narrowing of the tricuspid valve opening which restricts the blood flow from the right ventricle to the right atrium. Main causes of tricuspid valve stenosis are rheumatic fever and infective endocarditis. Rarely, it can be caused by a heart tumour or birth defects.
Frequently Asked Questions
Are there any lifestyle restrictions following tricuspid valve repair/replacement?
In general there are no lifestyle restrictions, however activities such as contact sports should be avoided.
Will I need any further treatments following treatment?
Your cardiologist will provide full written guidelines in terms of activities and medications. It is important to follow these guidelines, and adhere to the prescriptions. A follow-up appointment will be scheduled 2-3 months post procedure to ensure a safe and smooth recovery. At that visit you will have an echocardiogram to monitor the device that has been placed and the progress your heart has made in returning to normal. Your doctor will decide at this time whether you should return to your own doctor for follow up of whether further follow up is required.
Can I play sports?
Yes you can play sports, however it is advisable to utilize common sense - for example, physical contact-sports should be avoided.
Will OHIP (public insurance) cover my ASD closure?
How urgent is the treatment?
Once you are symptomatic, it is advisable to receive medical treatment as soon as possible, as your condition will progress.
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Toronto General Hospital
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