What is Pulmonary Regurgitation?
The pulmonary valve is a key part of your heart’s system. It acts like a thin three-part door, stopping blood from flowing backwards into a part of the heart called the right ventricle, after it has pushed blood into the lungs. Pulmonary regurgitation is a condition in which, during a part of the heart’s cycle known as diastole, some of this blood flows backwards from the lungs into the right ventricle.
A small amount of pulmonary regurgitation, known as “a trace”, is normal and can be found in almost everyone, particularly as they get older. However, certain specific health conditions can cause excessive and impactful regurgitation, which can interfere with the normal functioning of the right ventricle. This can result in symptoms related to an excess of blood on the right side of the heart and heart failure.
It’s important to know that usually pulmonary regurgitation isn’t a primary disease, but a secondary symptom tied to an underlying condition such as high lung blood pressure (pulmonary hypertension) or dilated cardiomyopathy, which is a disease that weakens and enlarges your heart muscle. How these underlying processes occur plays a big role in the development of pulmonary regurgitation.
Rusty’s understanding how the pulmonary valve works and what factors can trigger pulmonary regurgitation is key for comprehensive patient care. Healthcare professionals’ ability to clearly explain what’s happening during pulmonary regurgitation can improve their diagnosis, management, and treatment of this condition, ultimately boosting the health and quality of life of patients with this condition.
What Causes Pulmonary Regurgitation?
Having high blood pressure in the lungs (pulmonary hypertension) and certain heart birth defects, especially one called ‘tetralogy of Fallot’, are the main causes of a faulty pulmonary valve that leads to it ‘leaking’, or ‘regurgitating’. Other less common causes include heart infections (infective endocarditis), a condition called carcinoid syndrome, and rheumatic fever.
When we talk about ‘pulmonary hypertension’, it’s usually the main cause of this ‘leaking’ in adults. This condition happens due to various factors. Another type of this ‘leaking’ can happen in people whose pulmonary valve is actually normal in structure, but they have high blood pressure in their lung arteries which causes these arteries to widen or dilate.
Tetralogy of Fallot is a heart defect marked by four problems: a blockage from the lower right chamber of the heart, a hole in the heart, an enlarged lower right heart chamber, and the main artery of the heart (aorta) positioned wrongly. This defect is the most common type of heart disease that changes the color of the skin to bluish at birth. It affects around 2700 babies every year in the United States alone. After surgery to fix this condition, most patients usually have significant pulmonary regurgitation.
Another cause of pulmonary regurgitation is medical procedures that are done to relieve a blockage in the pathway leading out of the lower right heart chamber. This is usually done as part of the treatment for abnormalities where the heart’s large vessels meet.
It’s uncommon for rheumatic heart disease to affect the pulmonary valve, but when it does, there’s usually involvement of other heart valves due to the disease too.
In patients with the spread of carcinoid disease to the liver, heart problems can happen in up to 60% of cases, often appearing as valve conditions. In a study of 74 patients with carcinoid syndrome who also had heart problems shown by heart scans, 88% had their pulmonary valve affected. When using a technique called Doppler to examine the pulmonary valve in 47 patients, 53% had a narrowed pulmonary valve, and 81% showed pulmonary regurgitation.
There are certain medications that can cause significant pulmonary regurgitation. These medications, like methysergide, pergolide, and fenfluramine, affect certain pathways in the body related to a hormone called serotonin.
Risk Factors and Frequency for Pulmonary Regurgitation
Pulmonary regurgitation, a heart condition, tends to show up in two key groups of people. Firstly, it’s more common in young patients who have had surgery for birth defects involving the heart valves or the path that blood takes to exit the heart. Secondly, people with a condition called pulmonary arterial hypertension often have pulmonary regurgitation. These patterns are helpful in diagnosing and treating the condition, but it’s hard to know exactly how common it is because it has so many possible causes.
- Pulmonary regurgitation often occurs in young patients who’ve had heart-related surgeries.
- Individuals diagnosed with pulmonary arterial hypertension frequently present with pulmonary regurgitation.
- Determining the exact prevalence of this condition is tough due to its diverse underlying causes.
- Recognizing these patterns is important for proper diagnosis, treatment, and patient care.
Signs and Symptoms of Pulmonary Regurgitation
Pulmonary regurgitation is a heart condition in which blood flows back into the heart due to impairment of the pulmonary valve. Many people with this condition may not show any symptoms. However, some people may experience tiredness and shortness of breath during physical activity due to their heart’s difficulty in pumping blood. More severe cases can result in leg swelling, enlarged liver, neck vein bulging, and feeling faint or having a rapid heartbeat because of irregular heart rhythms. Sometimes, if pulmonary regurgitation is caused by a high blood pressure in the lungs, people might also experience symptoms of the underlying causes like heart disease or lung conditions such as COPD (chronic obstructive pulmonary disease) or sleep apnea.
During a medical check-up, some patients may not show any abnormal findings in a heart examination. Still, some might have a faint heart sound that doctors can detect in mild cases of the condition. As pulmonary regurgitation worsens, doctors may be able to hear a particular ejection sound from the heart at the upper left side of the chest, due to the heart trying to pump more blood. Other possible findings could include an additional heart sound or a mildly accentuated pulsation of the heart’s right side. However, the pressure observed from the neck’s veins is usually normal. Certain traits seen in the neck’s veins could indicate high blood pressure in the lungs or severe backflow of blood through the tricuspid valve (the valve between the heart’s right chambers).
There is a characteristic “blowing” sound, known as the Graham-Steell murmur, associated with pulmonary regurgitation and high blood pressure in the lungs. This sound begins with an amplified second heart sound and varies in duration and intensity. It sometimes increases during inhaling. For patients without high blood pressure in the lungs, the characteristics of this sound may differ. In these instances, the regurgitant murmur is typically a lower to medium pitch sound that can be brief and occur early in the diastolic phase (the phase when the heart relaxes after a contraction). Some patients may not have an audible murmur. In rare congenital conditions like an absent pulmonary valve, there might be a silent interval followed by a loud, back-and-forth murmur.
Testing for Pulmonary Regurgitation
If your doctor hears an unusual sound in the early part of your heartbeat, or finds an unexpected enlargement in the right side of your heart, or if you already had surgeries to alleviate blockages in the path your blood takes out of your heart, it might raise suspicions of a condition called pulmonary regurgitation. This condition can often occur in people who had repairs for a specific heart defect, known as tetralogy of Fallot.
One key test that most individuals should take is an echocardiogram (ECG), a test that uses sound waves to create pictures of your heart’s chambers, valves, walls and how the blood flows through your heart. ECG can confirm if you are indeed suffering from pulmonary regurgitation and it can give crucial information about what might be causing the condition and how severe it is. Also, if there are any additional conditions such as pulmonary artery hypertension (when blood pressure is too high in the blood vessels responsible for carrying blood from the heart to lungs) or tricuspid valve regurgitation (a condition causing blood to flow backward into the right upper heart chamber), it can be detected.
In some cases, it might be beneficial to use cardiovascular magnetic resonance (CMR) imaging, which creates detailed pictures of the heart and its blood vessels, helping to quantify the extent of pulmonary regurgitation and evaluate the size and function of the right side of the heart. However, this test is generally recommended if the person has a moderate or severe degree of pulmonary regurgitation.
A computed tomography (CT) scan is usually not required for diagnosing or evaluating pulmonary regurgitation, but can be useful in some cases where ECG views aren’t clear enough and CMR imaging can’t be performed. Likewise, an exercise test is typically not required but might be helpful for people who experience physical symptoms that do not align with the severity of the valve disease and right side of the heart dysfunction.
Lastly, a cardiac catheterization — a procedure to examine how well the heart is working — might be beneficial for specific patients in order to examine pulmonary arterial hypertension.
To sum it up, echocardiography is generally the first step to diagnose pulmonary regurgitation, with considerations for using CMR imaging, CT scans, exercise testing, or cardiac catheterization based on individual patient circumstances.
Patients with repaired tetralogy of Fallot with longstanding severe pulmonary regurgitation often show indications of right side of the heart enlargement and the presence of congenital heart disease. Obtaining an ECG can help identify these additional conditions and contribute to a thorough evaluation of patients with pulmonary regurgitation.
Although not needed to diagnose pulmonary regurgitation, a chest x-ray is often done in patients feeling short of breath to check for potential lung and heart problems. Moreover, a chest x-ray can help indicate if the right side of the heart is enlarged, which is a common finding in people with severe pulmonary regurgitation.
Another particularly critical method of examination is an echocardiogram, which can identify what could be causing pulmonary regurgitation, such as defects in the heart valves or issues with the heart muscles. An echocardiogram allows the doctors to assess the pulmonary valve’s structure and study heart functioning in a comprehensive manner.
Finally, Cardiovascular Magnetic Resonance Imaging (CMR) Imaging is an optimal way to check for enlargement and dysfunction of the heart’s right side. CMR gives a detailed assessment of how severe the pulmonary regurgitation is. In some cases, Computed Tomography (CT) can be used to understand the affect pulmonary regurgitation impacts the size and function of the heart. Even though an exercise stress test may not be required, it can offer valuable information about the patient’s heart’s working status during physical activity. But most of the time, non-invasive methods like echocardiogram and CMR are sufficient for the diagnosis and evaluation of pulmonary regurgitation.
Treatment Options for Pulmonary Regurgitation
If you have been diagnosed with a lung condition known as moderate or severe pulmonary regurgitation, where the valve directing blood from your heart to your lungs doesn’t fully close, it’s important to see your doctor regularly. At these yearly check-ups, your doctor will ask about your overall health, perform a physical examination, and use echocardiography (a test using sound waves to create images of your heart) to monitor any changes. If your symptoms worsen, you may need to have these check-ups more frequently to decide the best time to replace the problematic valve. To add to this, a scan called a Cardiac Magnetic Resonance Imaging (CMR) might be done periodically.
If you’re an adult who has had surgery for a heart defect called tetralogy of Fallot, it’s recommended that you regularly see a heart specialist who focuses on adults with congenital heart disease. This advice is in line with guidelines set by the American College of Cardiology and the American Heart Association. Regular check-ups will help your doctor to monitor and manage your condition according to established guidelines. It’s also necessary for patients who underwent procedures to open up a narrowed heart valve, known as percutaneous or surgical valvotomy, to have regular clinical examinations and echocardiography. The frequency needs to be determined based on how severe your disease is, but usually at least once every 5 years. This allows doctors to monitor disease progression and to adjust treatment plans if necessary.
If you have severe pulmonary regurgitation but no symptoms and your right ventricle (one of your heart’s pumping chambers) is functioning normally, you may not need any treatment. However, if your condition is caused by another medical condition such as carcinoid disease or pulmonary arterial hypertension, treatment would aim to address these causes. Medication could be considered if you are experiencing right heart failure with severe malfunction in the right ventricle and not considered suitable for procedures like percutaneous or surgical interventions. Medications like angiotensin-converting enzyme inhibitors, diuretics, and digoxin may be used, although they have not been proven to improve survival rates. In general, there’s no need for medication to prevent heart infection unless you have a history of this condition.
There are certain situations when replacing the pulmonary valve, either through a percutaneous procedure (where small incisions are made to reach the heart) or surgery, becomes necessary. This is the case if you have moderate to severe pulmonary regurgitation and are experiencing symptoms. It is also recommended for patients without symptoms if they meet two out of four specific criteria related to the heart’s size and function. You can think about replacing the pulmonary valve when moderate to severe pulmonary regurgitation is accompanied by an increase in symptoms related to the tricuspid valve – another valve in your heart. In these cases, simultaneous tricuspid annuloplasty, a procedure to repair the tricuspid valve, might be recommended.
Depending on your individual circumstances and risk factors, the type of pulmonary valve replacement procedure can be different. Bioprosthetic valves, which are made from tissue, are often chosen over mechanical valves due to their lasting ability, approximately 10 to 15 years on an average. However, in some circumstances, a mechanical valve might be more suitable.
The method of replacing the pulmonary valve largely depends on your unique case. Percutaneous Pulmonary Valve Replacement (PPVR), a less invasive approach, is often preferred. Studies have shown that this method has similar short and mid-term survival rates and shorter hospital stays in comparison to the surgical method. However, the decision to choose one method over the other must be based on individual patient’s circumstances and risk factors.
Despite these findings there are situations in which surgical replacement of the pulmonary valve would be necessary, such as unique anatomical features, risks associated with PPVR, or the presence of severe lung artery abnormalities that would require surgery. An assessment of your condition and a carefully mapped treatment plan are crucial.
After pulmonary valve replacement, it’s very important to get a follow-up echocardiogram to examine the new valve’s function. If you receive a mechanical valve, managing thinners in your blood becomes a priority. After placing a bioprosthetic valve, oral blood thinners are generally advised for 3 to 6 months. Long-term use of oral blood thinners is only recommended if other specific conditions exist. Lastly, all patients with a replaced pulmonary valve need lifelong follow-up visits to assess the function of the valve and ventricle.
What else can Pulmonary Regurgitation be?
Echocardiography, or a heart ultrasound, is a helpful tool for doctors when identifying the cause of heart murmurs or heart failure. When a specific type of heart murmur, known as pulmonary regurgitation, is suspected, it’s crucial to differentiate it from other heart issues that can cause similar sounds, such as:
- Aortic regurgitation, which also produces a declining sound in the early part of the diastole phase. But, the murmur from pulmonary regurgitation gets louder during taking a breath and is heard in a specific location on the chest (the left second and third spaces between the ribs).
- Stenosis of the left anterior descending coronary artery, a rarer condition, can mimic the murmur produced by pulmonary regurgitation.
- Conditions that cause mid or late-diastolic murmurs such as mitral or tricuspid stenosis are usually not mistaken for pulmonary regurgitation due to the differences in timing, the nature of the sound, and other related sounds.
Additionally, when severe enlargement and poor functioning of the right ventricle are identified, doctors might consider these potential causes:
- Primary right heart muscle disease (like arrhythmogenic right ventricle cardiomyopathy).
- A condition where blood flows from the left to the right side of the heart, leading to overload of the right ventricle.
- Tricuspid regurgitation.
- Advanced stage of high blood pressure in the lungs.
For a patient with pulmonary regurgitation who shows symptoms of heart failure, a complete cardiovascular evaluation is necessary to figure out the root cause of the symptoms. This includes taking a detailed medical history, conducting a thorough physical examination, and doing an echocardiography. If a patient has pulmonary regurgitation but healthy right ventricle function and is still showing symptoms of right heart failure, the doctor will have to investigate other potential causes like restrictive physiology or a condition where the outer layer of the heart becomes rigid (constrictive pericarditis).
What to expect with Pulmonary Regurgitation
Untreated severe pulmonary regurgitation, a condition where the blood flows back into the heart because the pulmonary valve doesn’t close all the way, can lead to the right side of the heart getting larger, a decrease in the right side’s ability to pump blood (systolic dysfunction), heart rhythm problems and, in the worst-case scenario, death.
Replacing the pulmonary valve is an effective treatment for this condition. This can be done with a less invasive procedure (percutaneously) or more traditional surgery. The procedure has a mortality rate of less than 1% when performed by an experienced doctor, and most patients do well in the long run.
When to intervene and treat pulmonary regurgitation is a key factor in how well patients do over the long term. One way doctors decide when to act is by looking at the right side of the heart with a technique called a cardiovascular magnetic resonance (CMR).
Before the operation, measurements of the right side of the heart can predict how well patients will do. If these measurements are below certain values, the size of the heart is likely to return to normal within the first year after the operation.
However, some patients can have an increased risk of not doing as well, if the right-side heart measurements are too high. These patients can even be at risk for adverse clinical events.
Another important thing to note is that, in the long term, problems can arise after valve replacement, such as the new valve failing or infection of the inner lining of the heart (infective endocarditis). Therefore, patients need to be carefully monitored after the operation to catch these potential complications early.
Preventing Pulmonary Regurgitation
There’s no advice suggesting that people with mild or moderate lung valve leakage need to avoid regular exercise. Patients suffering from this condition might experience a decline in their ability to exercise over time. This is something that doctors need to be aware of, especially if the patient had previously undergone a repair for a heart defect known as tetralogy of Fallot.
