Pulmonary Valve Stenosis Diagnosis and Treatment

How common is pulmonary valve stenosis? How to treat pulmonary valve stenosis? Let’s find out about pulmonary valve stenosis diagnosis and treatment!

Pulmonary Valve Stenosis Definition

Pulmonary valve stenosis definition: What is pulmonary valve stenosis? Pulmonary valve stenosis is narrowing the valve between the lower right chamber of the heart (the right ventricle) and the arteries that supply the lungs (pulmonary arteries).

pulmonary valve stenosis diagnosis and treatment - pulmonary valve stenosis newborn

Pulmonary Valve Stenosis Causes

What causes pulmonary valve stenosis? Stenosis of the pulmonary valve or RV infundibulum makes it harder for the RV to let blood out, raises the pressure in the RV, and cuts off blood flow to the lungs. Pulmonic stenosis is often present at birth and is linked to other heart problems.

In valvular pulmonic stenosis, more blood goes to the left lung than to the right. When there are no shunts involved, arterial saturation is normal. Peripheral pulmonic stenosis can happen with valvular pulmonic stenosis and can be part of several clinical syndromes, such as congenital rubella syndrome.

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Patients who have had the Ross procedure for aortic valve disease (transferring the pulmonary valve to the aortic position and putting a pulmonary homograft valve in the pulmonary position) may develop a condition called noncongenital postoperative pulmonic valvular or main pulmonary artery (PA) stenosis because of an immune response in the homograft. RV outflow obstructions can also happen when a passageway from the RV to the pulmonary artery becomes narrowed over time or when a bioprosthetic replacement pulmonary valve loses its shape.

Pulmonary Valve Stenosis Diagnosis

  • In severe cases, the heart may stop working on the right side.
  • P2 late and soft or not there.
  • Pulmonary ejection click is common and gets quieter when you breathe in. This is the only right heart sound that gets quieter when you breathe in; all the others get louder.
  • Echocardiography/Doppler is used to make diagnoses.
  • Patients with an echocardiogram/Doppler peak pulmonic valve gradient of more than 64 mm Hg or a mean of 35 mm Hg should get treatment, even if they don’t have any symptoms. Otherwise, surgery should be done if there are signs or symptoms of right ventricular (RV) dysfunction.
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Pulmonary Valve Stenosis Symptoms

Signs and symptoms of pulmonary valve stenosis: Mild pulmonic stenosis doesn’t cause any symptoms. On the other hand, moderate to severe pulmonic stenosis can cause shortness of breath when you work out, fainting, chest pain, and eventually RV failure.

Right ventricular hypertrophy (RVH) can often be felt as a parasternal lift, and if the PA is bigger, the pulmonary outflow tract may also be felt. In the left second and third interspaces parasternal, there is a loud, harsh systolic murmur and sometimes a strong thrill.

The flow pattern in the main PA makes the murmur move toward the left shoulder, and it gets louder as the person breathes in. You can hear a loud ejection click before the murmur in mild to moderate pulmonic stenosis. This sound gets quieter when you breathe in because the increased RV filling from breathing in opens the valve too early during atrial systole when you breathe in.

Since the valve moves less during systole when you breathe in than when you breathe out, the click is quieter when you breathe in. This is the only right-sided auscultatory event that gets smaller when the person takes a deep breath. All other auscultatory events get bigger when the right heart output increases, which happens when you breathe in. In severe pulmonic stenosis, the murmur makes it hard to hear the second sound, and the pulmonary part of S2 may be weak, late, or not there at all.

When there is RV diastolic dysfunction, there is a right-sided S4 and a big wave in the venous pulse. If there is tricuspid regurgitation, there is a c-v wave in the jugular venous pressure. Pulmonary valve regurgitation is rare in primary pulmonic stenosis. It may be hard to hear because the difference between the lower PA diastolic pressure and the higher RV diastolic pressure may be very small (low-pressure pulmonary valve regurgitation).

Pulmonary Valve Stenosis Radiographic Findings

Right axis deviation or RVH is seen, and peaks in the P waves show right atrial (RA) overload. Depending on how bad it is, radiographs may show a normal-sized heart, a large RV and RA, or a gross heart enlargement. The main and left pulmonary arteries often get bigger after they get blocked. Pulmonary vascularity is usually fine, but the left lung gets more blood than the right.

Diagnostic Studies

Echocardiography/Doppler is the best diagnostic tool because it can show if the valve is doming or dysplastic, measure the pressure difference across the valve, and tell if there is subvalvular obstruction or tricuspid or pulmonic valvular regurgitation. By echocardiography or Doppler, pulmonic stenosis is mild if the peak gradient is less than 36 mm Hg.

Pulmonic stenosis is moderate if the peak gradient is between 36 mm Hg and 64 mm Hg. Pulmonic stenosis is severe if the peak gradient is over 64 mm Hg or the mean gradient is over 35 mm Hg. A lower gradient could be a big deal if the RV isn’t working right. Most of the time, catheterization isn’t needed to make a diagnosis. It should only be done if the data aren’t clear or if it’s needed to prepare for percutaneous intervention or surgery.

Pulmonary Valve Stenosis Treatment and Management

Treatment of pulmonary valve stenosis – What is the best treatment for pulmonary valve stenosis?

Even if nothing is done, people with mild pulmonic stenosis live normal lives. In childhood and adolescence, moderate stenosis may not cause symptoms, but as people get older, they often do. Some patients’ stenosis worsens over time, so it’s important to check on them often. Rarely does severe stenosis cause sudden death, but it can lead to right heart failure in people as young as their 20s and 30s. Pregnancy and exercise tend to be well tolerated unless the stenosis is very bad.

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The AHA/ACC guidelines and the ESC guidelines are mostly the same, but the ESC says that severe pulmonic stenosis should be considered if the RV systolic pressure is more than 80 mm Hg. Class I (decisive) indications for intervention include all patients with symptoms and all patients with a resting peak-to-peak gradient of more than 64 mm Hg or a mean of more than 35 mm Hg, no matter if they have symptoms or not.

A patent foramen ovale (PFO) or an atrial septal defect can cause blood to flow from the right side of the heart to the left side. This can cause cyanosis (ASD). Percutaneous balloon valvuloplasty is the best treatment for people with a domed valve because it works very well. Surgical commissurotomy can also be done, or the pulmonary valve can be replaced with a bioprosthetic valve or a homograft if the regurgitation is too bad or the valve is dysplastic. A percutaneously implanted pulmonary valve can often fix a blocked pulmonary outflow tract caused by a blocked RV-to-PA conduit or a stenosed homograft pulmonary valve (both the Medtronic Melody valve and the Edwards Sapien XT valve are FDA approved).

Putting a stent in the pulmonary artery first and then the transcatheter device in this stent can make it easier for these valves to fit. Because the new catheter valve could cause the coronary artery to get smaller, it is a class I required to use temporary balloon inflation to see how the device affects the coronary artery before delivering the device. The FDA also approves percutaneous pulmonary valve replacement for people with conduit stenosis or who have had the Ross procedure. Percutaneous valve replacements have also been done off-label for patients with native pulmonary valve disease, including those who have had tetralogy of Fallot repaired (if the PA root size is small enough to fit a percutaneous valve).

Endocarditis prevention is not needed for native valves, even after valvuloplasty, unless pulmonary valve endocarditis has happened before (an unusual occurrence). It should be used if a valve has been replaced through surgery or percutaneously. After percutaneous pulmonary valve replacement with the Melody valve, there seems to be more pulmonary valve endocarditis than expected. The FDA is keeping a close eye on this.

I hope you understand pulmonary valve stenosis diagnosis and treatment guidelines.

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