Centers of Excellence in Interventional Cardiology and Radiology

Pediatric Angiology

Peripheral Pulmonary Stenosis

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Generalities

Peripheral pulmonary stenosis refers to narrowing in one or several areas of the branches of the pulmonary arteries. This is a different condition from pulmonary (valve) stenosis (PVS, PS) and pulmonary vein stenosis.

Peripheral pulmonary stenosis (PPS) can cause obstruction at the level of the main pulmonary artery, at its bifurcation, or at the more distal branches. PPS may occur at a single level, but multiple sites of obstruction are more common. PPS may be associated with other congenital heart anomalies such as valvular PS, atrial septal defect, ventricular septal defect, or patent ductus arteriosus. 20% of the patients with tetralogy of Fallot have associated PPS. It can also occur if a child is born without a pulmonary valve.

PPS is associated with various inherited and acquired conditions including rubella and the Alagille, cutaneous laxa, Noonan, Ehlers-Danlos, and Williams syndromes.

Poststenotic dilatation occurs with discrete segmental stenosis but is absent if the stenotic segment is long or if the pulmonary artery is diffusely hypoplastic.

Peripheral pulmonary stenosis may be a progressive condition, meaning symptoms can increase and become more obvious and disruptive over time. Many children in the mild stages of peripheral pulmonary stenosis do not show any outward symptoms.

Children with peripheral pulmonary stenosis are at risk of having insufficient oxygen within their blood, placing increased pressure on the heart. This makes the heart have to work harder.

The pulmonary arteries:

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are responsible for carrying blood from the heart to the lungs

are the only arteries in the human body carrying blood that has been de-oxygenated—meaning the oxygen has been delivered throughout the body where it is needed, and has been replaced by carbon dioxide

You can think of the pulmonary arteries as a large tree with the following parts:

the main pulmonary artery, or “trunk,” that reaches from the right ventricle of the heart into the two pulmonary branches

the pulmonary branches—one on the left and one on the right—each of which branches out many times into the corresponding lung

What causes peripheral pulmonary stenosis?

Peripheral pulmonary stenosis is typically a congenital heart defect—something a child is born with, rather than a disease that is acquired later in life. It can occur:

as a complication of Tetralogy of Fallot, a rare condition in which four different heart defects are all present at birth

in conjunction with certain genetic syndromes that affect the heart, like Williams Syndrome, Alagille Syndrome and Noonan Syndrome

if a child is born with only one large vessel that leads out of the heart, causing the oxygen-rich blood coming from the lungs and the de-oxygenated blood going back to the heart to become mixed; this rare condition is called truncus arteriosus

if a child is born without a pulmonary valve; this condition is called pulmonary atresia

if the pulmonary valve is narrowed; this is called pulmonary (valve) stenosis (PVS, PS)

if the opening between the aorta and the pulmonary artery does not close soon enough after birth; this rare condition is called patent ductus arteriosus

as a complication of certain types of infection, like congenital rubella syndrome, that can weaken the heart.

Symptoms

Many children in the mild stages of peripheral pulmonary stenosis do not show any outward symptoms. Usually, the only identifiable symptom in these cases is a pronounced heart murmur.

As peripheral pulmonary stenosis progresses over time, children may display one or more of the following symptoms:

fatigue

shortness of breath

heavy, difficult breathing

rapid, shallow breathing

rapid or fluttering heartbeat

swelling in the feet and ankles

swelling in the face

swelling in the abdomen.

You should seek treatment from a qualified medical professional right away if you notice any of these warning signs in your child.

Diagnosis

Most often, a clinician will notice that your child has a heart murmur—which is a telltale noise blood makes as it flows from the left ventricle to the aorta. Heart murmurs can be detected with a stethoscope during a routine physical examination, and can also emerge during tests or treatment for another condition or problem.

Sometimes, heart defects can be detected when a baby is still in the womb.

Imaging tests:

chest x-ray – produces images of the heart and lungs

electrocardiogram (EKG), which records the heart’s electrical activity and shows any abnormal rhythms or unusual stress

exercise electrocardiogram, which evaluates the heart’s activity during exercise

echocardiogram, which uses sound waves to make a moving picture of the heart’s structure and function

cardiac catheterization, which uses a small, flexible tube threaded up to the heart to give very detailed information about the structures within the heart

cardiovascular magnetic resonance imaging (MRI), which uses magnets to produce detailed, three-dimensional pictures of the heart’s function and blood flow

pulmonary angiography, which uses a special dye to take X-ray images of the pulmonary arteries

lung perfusion scan, or ventilation/perfusion scan, which observes how well air and blood move through your child’s lungs.

Indications

Children with peripheral pulmonary stenosis are at risk of getting insufficient oxygen within their blood, placing increased pressure on the heart. The condition can affect each child differently, so it’s important to talk to your clinician about your child’s individual circumstances and the recommended treatment options.

Children with mild to moderate peripheral pulmonary stenosis may not require any treatment other than routine monitoring in the short term, since they have no noticeable symptoms that affect their daily lives.

The cardiologist will regularly evaluate your child for any sign of further narrowing in the pulmonary branches and related complications. Most often, routine monitoring will involve physical examinations and echocardiograms.

Medication

Medications is not a cure for peripheral pulmonary stenosis, but can be helpful in managing specific symptoms. In some cases, your child’s clinician may prescribe medication to:

1. help the heart maintain healthy function and blood flow

2. control blood pressure

3. prevent abnormal heart rhythms, called arrhythmias.

A child with peripheral pulmonary stenosis may also need to periodically take antibiotics in order to prevent an infection called bacterial endocarditis. Bacterial endocarditis can cause serious damage to the inner lining of the heart and its valves.

Interventional treatment wich use a thin tube called a catheter that is threaded from a vein or artery into the heart. The catheter can be used to fix holes in the heart, open narrowed passageways (like those within the pulmonary branches) and create new passageways if needed. It includes: balloon dilation, balloon dilation and stent placement and the Cutting Balloon device.

Many children need several interventions over time as they grow and age. The good news is that these interventional catheterization procedures are very effective and carry a low incidence of complications. Children who have these procedures are likely to enjoy healthy adult lives with minimal to no restrictions on playing sports or engaging in other activities. Since peripheral pulmonary stenosis can affect each child differently, you should always speak directly to your treating clinician for a specific treatment plan, outlook and recommendations for your child.

Procedure

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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 and then into the affected areas of the pulmonary branches, under fluoroscopic control (X ray control). The interventionist will perform an angiogram, so the stenotic segment is seen and the catheter position is checked. Using this catheter, a deflated balloon at the tip of the catheter is inflated once the tube is in place, and this balloon stretches the constricted area open, reversing the problematic narrowing. The balloon is then removed.

Balloon dilation and stent placement

The effects of balloon dilation can be amplified for some children with peripheral pulmonary stenosis by using a combination of balloon dilation and the placement of a balloon-expandable stent—a small, stainless steel tube. The stent is attached to the balloon dilation catheter as it is fed into the narrowed parts of the pulmonary branches.

The protective covering is removed when the catheter is in place, and the balloon is then inflated. The balloon affixes the stent within the pulmonary branch, stretching the narrowed area and keeping it open.

The Cutting Balloon device

This is a new device used to treat children whose peripheral pulmonary stenosis has not been fixed by standard balloon dilation.

The Cutting Balloon is a balloon with three or four miniature blades attached. These blades are able to make small incisions throughout the narrowed areas of the pulmonary branches, helping to open the problematic narrowings.

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 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

pulmonary valve insufficiency

restenosis

one issue is the growth of patients. Children frecquently require intervention before achieving full adult size and whit later growth, a relative stenosis may occur in the stented vessel wilh time.

Before procedure

The preoperative assessment will establish if the interventional treatment is the best choice for you.

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

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.

It would be prudent to administer antiplatelet drugs such as aspirin and clopidogrel for short periods (3 to 6 months) until full endothelializadon has been accomplished.

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

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.

Physical activity should be normal.

Important!

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Many children need several interventions over time as they grow and age. The good news is that these interventional catheterization procedures are very effective and carry a low incidence of complications. Children who have these procedures are likely to enjoy healthy adult lives with minimal to no restrictions on playing sports or engaging in other activities.

DOCTORS
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