Centers of Excellence in Interventional Cardiology and Radiology

Pediatric Angiology

Pulmonary valve stenosis

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Generalities

Pulmonary valve stenosis is a narrowing of the pulmonary valve, which normally lets blood flow from the right ventricle into the lungs.

Adults occasionally have the condition as a complication of another illness (carcinoid syndrome, rheumatic fever), but most of the time, pulmonary valve stenosis develops before birth as a congenital heart defect. A stenotic pulmonary valve may occur without associated congenital abnormalities, although it is most often associated with other structural abnormalities of the heart.

Pulmonary valve stenosis ranges from mild and without symptoms to severe. Mild pulmonary stenosis doesn’t usually worsen over time, but moderate and severe cases may get worse and require surgery. Fortunately, treatment is highly successful, and most people with pulmonary valve stenosis can expect to lead normal lives.

Pulmonary valve stenosis usually occurs when the pulmonary valve doesn’t grow properly during fetal development. Other heart abnormalities also are often present at birth (congenital) in babies who have pulmonary valve stenosis. It’s not known what causes the valve to develop abnormally.

Normal pulmonary valve anatomyFormular 2:

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The pulmonary valve is made up of three thin pieces of tissue called leaflets that are arranged in a circle. With each heartbeat, the valve opens in the direction of blood flow — into the pulmonary artery and continuing to the lungs — and then closes to prevent blood from flowing backward into the right ventricle of the heart.

What happens in pulmonary valve stenosis

In pulmonary valve stenosis, one or more of the leaflets may be defective or too thick, or the leaflets may not separate from each other properly. If this happens, the valve doesn’t open correctly, restricting blood flow.

Complications

Cases of mild to moderate pulmonary stenosis generally don’t cause complications. However, severe pulmonary stenosis may be associated with the following:

1. Infectious endocarditis: people with structural heart problems, such as pulmonary stenosis, have a higher risk of developing bacterial infections in the inner lining of the heart.

2. Heart failure

3. Irregular heartbeat (arrhythmia): unless the stenosis is severe, irregular heartbeats associated with pulmonary stenosis usually aren’t life-threatening.

How Common is Pulmonary Valve Stenosis?

Pulmonary stenosis represents 8-12% of all congenital heart defects in children. In adults, pulmonary stenosis represents approximately 15% of all congenital heart defects. Isolated valvar pulmonary stenosis with an intact ventricular septum is the second most common congenital cardiac defect in children.

Frequency of this defect is not related with race or sex. The patient’s age at presentation is related to the severity of the obstruction. If the stenosis is severe, patients may present in the neonatal period or in infancy. Patients with mild obstruction may present in childhood with asymptomatic murmurs.

Symptoms

Most children with pulmonic stenosis, particularly those with mild pulmonary stenosis, present with asymptomatic cardiac murmurs that are detected during routine examination.

Those with more significant stenosis often first notice symptoms while exercising. Pulmonary valve stenosis signs and symptoms may include:

Heart murmur — an abnormal whooshing sound heard using a stethoscope, caused by turbulent blood flow

Shortness of breath, especially during exertion

Chest pain

Loss of consciousness (fainting)

Fatigue

Palpitations

Of note, many patients with moderate or severe pulmonary stenosis remain asymptomatic.

Diagnosis

Laboratory evaluation is usually not helpful in pulmonary valve stenosis diagnosis.

Imaging studies:

Chest radiography

Echocardiography is very helpful (the sine qua non of diagnosis is 2-dimensional and Doppler echocardiography).

ECG – usually normal in mild pulmonary stenosis.

CT and MRI may reveal pulmonary valve stenosis, but the state-of-the-art echocardiography and Doppler studies are more useful than CT or MRI in diagnosing and quantitating pulmonary valve obstruction

Cardiac catheterization and selective cineangiography – Catheterization is not indicated in mild pulmonary stenosis, but is essential in severe stenosis and is an integral part of balloon pulmonary valvuloplasty. This procedure is used to confirm the diagnosis; to discern the degree of obstruction; to assess the morphology of the right ventricle, pulmonary outflow tract, and pulmonary arteries; and to exclude other associated cardiac abnormalities. Patients with echocardiographic evidence of clinically significant pulmonary stenosis (50-60 mm Hg) should undergo diagnostic and therapeutic cardiac catheterization with preparation for balloon dilatation of the pulmonary valve.

Angiography – usually reveals thickened and domed leaflets of the pulmonary valve with a thin jet of contrast material across the pulmonary valve.

Indications

Some cases of pulmonary stenosis are mild and don’t require treatment except for routine checkups.

The decision to perform a balloon valvuloplasty or open-heart surgery depends on the extent to which the pulmonary valve is obstructed. Pulmonary stenosis is classified as mild, moderate or severe, depending on a measurement of the blood pressure difference between the right ventricle and pulmonary artery.

1. Patients with mild (gradient < 50 mm Hg) pulmonary stenosis do not need intervention to relieve the obstruction of the pulmonary valve. They should be clinically followed up at periodic intervals, perhaps on a yearly basis. During the period of rapid growth (infancy and adolescence), follow-up more frequent than this may be indicated. Routine well-child care, including immunizations by the primary physician, should be provided. Patients with pulmonary stenosis are candidates for infective endocarditis prophylaxis before they undergo any bacteremia -producing procedures and surgery. Limitations in exercise or activity levels are not needed.

2. Patients with moderate (gradient, 50-79 mm Hg) and severe (gradient, >80 mm Hg) obstruction should undergo intervention to relieve the stenosis of the pulmonary valve. After the obstruction is relieved, recommended routine care, endocarditis prophylaxis, and exercise limitations are the same as those described for trivial and mild stenosis.

3. Patients with signs of right ventricular failure should be promptly treated (heart failure treatment). However, the problem does not resolve until the obstruction is relieved. Therefore, prompt balloon or surgical intervention should be undertaken. Right ventricular function may not recover completely if intervention is withheld for too long and if myocardial damage sets in.

4. A fetus with critical pulmonary stenosis or atresia with intact ventricular septum may benefit from pulmonary balloon valvuloplasty in utero, which promotes growth of the right ventricle.

5. Although the consensus is to offer relief of pulmonary valve obstruction in children with moderate or severe stenoses, this approach is somewhat controversial in adults.

However, a prudent strategy may be to relieve pulmonary valve obstruction in adults with moderate-to-severe pulmonary stenosis, irrespective of their symptoms, because of the potential

for myocardial damage associated with long-term pressure overload of the right ventricle

for generally lowered cardiac indices both before and after exercise in adults compared with children

for exercise-induced hemodynamic abnormalities in adults.

Procedures:

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1. Balloon pulmonary valvuloplasty – minimally invasive, tends to be the first choice for treatment

2. Cardiac surgical intervention : Balloon valvuloplasty can’t be used for cases of pulmonary stenosis that occur above the pulmonary valve (supravalvular) or below the valve (subvalvular). Open-heart surgery is required for these types of stenoses and occasionally for valvular stenosis. Some people with pulmonary stenosis have other congenital heart defects, and these may be repaired at the time of surgery.

Procedure

Indications: In general, indications for balloon pulmonary valvuloplasty are similar to those used in surgical pulmonary valvotomy:

moderate pulmonary valve stenosis with a peak-to-peak gradient >50 mm Hg with a normal cardiac index.

Some cardiologists change this criterion to a gradient of 40 mm Hg or right ventricular pressure of 50 mm Hg.

More recently, the interventional procedures are increasingly performed under general anesthesia and the gradients are usually lower with general anesthesia than with conscious sedation. Consequently, the same criteria should not be applied. Therefore, the Doppler gradients (discussed in echocardiography section) should be used in making the decision regarding balloon pulmonary valvuloplasty.

Tehnique:

The procedure is performed in catheterism lab. Usually, it is done under general anesthesia. Thus, after local asepsy, a small inguinal incision made. Through this incision, the physician will introduce a small narrow tube into the femoral vein, then through this tube a catheter will be introduced which will reach the right ventricle, under fluoroscopic control (X ray control). The interventionist will perform an angiogram, so the pulmonary valve stenosis is seen and the catheter position is checked. Using this catheter, an uninflated balloon is placed through the opening of the narrowed pulmonary valve. Your doctor then inflates the balloon, opening up the narrowed pulmonary valve and increasing the area available for blood flow. The balloon is then removed.

When the pulmonary valve annulus is too large to dilate with a single balloon (about 20 mm), valvuloplasty with simultaneous inflation of 2 balloons across the pulmonary valve may be performed, although the current availability of large-diameter balloons make this technique unnecessary. However, the double balloon technique may be more effective and stable in some cases. The Inoue balloon has been used in adults with success. The major advantage of the Inoue balloon over conventional balloons is its adjustable diameter that makes stepwise dilation possible.

Is it painful?

No, usually, the intervention is performed under general anesthesia.

How long does it take?

The duration of the procedure is about 1-2 hours and takes place in the cardiac catheterization laboratory.

Risks

The complications are rare; the complications are reduced by the proper preparation and the continuous surveillance of the patient. Potential complications:

allergic reactions to administered substances, including renal disfunction

reactions to anesthetic compounds

arteriovenous fistulas at the vascular puncture site

minor bleeding at the vascular puncture site

fever

headache, migraine

infection

gaseous embolism

cardiac arrythmias

extremely rare – cardiac perforation and cardiac tamponade (perforation of the cardiac wall and bleeding in the pericardial sac, which compresses the heart)

stroke

myocardial infarction

pulmonary valve insufficiency

neonate with critical pulmonary valve stenosis: nearly 25% patients require reintervention to address related complications, restenosis, and associated defects.

infundibular obstruction

persistent repolarization abnormalities

However, restenosis (gradient >50 mm Hg) is observed in nearly 10% patients. Predictors of restenosis include a balloon-to-annulus ratio of less than 1.2 and a gradient of more than 30 mm Hg immediately after valvuloplasty. Patients with restenosis have been successfully treated with redilatation with balloons larger than those used for initial balloon valvuloplasty. Redilatation is the procedure of choice for managing restenosis after previous balloon dilatation.

Before procedure

The preoperative assessment will establish if the closure of the defect can be done percutaneously or there is an indication of surgical closure of the defect. Physical examination and echocardiography are key elements which help making a decision.

Prior to the intervention, the interventional cardiologist must be prevented about any history of allergic reactions. Blood tests are taken including hemoglobin level, coagulation, renal function, and other specific tests.

The patient is admitted the day before the intervention, and he/she should not eat before the procedure.

After procedure

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You will be connected to monitors that will constantly display your electrocardiogram (ECG or EKG) tracing, blood pressure, other pressure readings, breathing rate, and your oxygen level. You will be given pain medication for incisional pain or you may have had an epidural during surgery which will help with postoperative pain.

Since the procedure is minimally invasive, the postprocedural recovery is usually very fast. The majority of patients can leave the hospital the following day. Indications about recovery and postprocedural treatment will be clearly specified to all patients.

Follow-up evaluation:

Clinical, ECG, and Doppler echocardiographic evaluations are generally recommended at 1 month, 6 months, and 12 months after the procedure and yearly thereafter.

Patients with trivial and mild pulmonary stenosis do not need intervention to relieve the pulmonary valve obstruction. However, they should be clinically followed up at periodic intervals (eg, on a yearly basis).

Routine well-child care, including immunizations, as per the primary physician, is suggested.

Physical activity should be normal. Restriction from highly exertional and competitive sports is recommended only for patients with severe pulmonary stenosis. After successful relief of the obstruction, normal activity may be resumed.

Most patients with pulmonary stenosis are given prophylaxis for subacute bacterial endocarditis (SBE).

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