17399 Pulmonary Artery Stenosis

Overview

Pulmonary artery stenosis is a condition where the artery that carries blood from the heart to the lungs is narrower than it should be. This narrowing can make it harder for blood to reach the lungs to get oxygen. It can be present at birth or develop later in life.

It is a rare condition. When it happens, it is often seen in babies born with other heart problems. It can also appear in adults who have had heart surgery or certain infections.

People of any age can have it. It may be diagnosed in infants and children, often together with other heart conditions. Adults can also develop it later in life, especially after heart surgery or due to certain diseases.

Symptoms

Common symptoms

• No symptoms at all, especially if the narrowing is mild
• Shortness of breath during activity
• Feeling tired or weak
• Chest pain or tightness
• A heart murmur (an extra sound your doctor can hear with a stethoscope)

Symptoms in children

• Difficulty feeding or getting tired while eating
• Poor weight gain or slow growth
• Skin that looks blue or pale, especially around the lips
• Faster breathing than normal

Symptoms in older adults

• Breathlessness with everyday activities
• Palpitations (feeling like your heart is racing or skipping beats)
• Swelling in the legs or ankles
• Dizziness or light-headedness

Causes

Main causes

• Present at birth (congenital) – the artery does not form normally before birth
• After heart surgery or a medical procedure that affects the artery
• Inflammation of the blood vessels (for example, a condition called vasculitis)
• An injury or tumour pressing on the artery

Risk factors

• A congenital heart defect
• A genetic condition such as Williams syndrome
• A mother who had rubella during pregnancy
• Having had certain heart surgeries or catheter procedures
• Infections that affect the heart valves

When to see a doctor

See a doctor urgently if:

• You have symptoms such as fainting, severe chest pain, or trouble catching your breath at rest.
• Your child is feeding very poorly, having blue spells, or breathing fast.

Book a routine appointment if:

• You notice a new heart murmur or a change in your usual exercise tolerance.
• You have been told you have a narrowed pulmonary artery and you have not had a check-up in more than a year.
• You are having follow-up for another heart condition and notice new symptoms.

Diagnosis

A doctor may suspect pulmonary artery stenosis after listening to your chest and hearing a heart murmur. They will usually arrange an ultrasound scan of the heart (echocardiogram) to see how blood is flowing through the pulmonary artery. If the scan is not clear enough, other imaging tests can provide a more detailed picture.

Tests that may be done

• Echocardiogram (ultrasound of the heart)
• Electrocardiogram (ECG) to check the heart’s rhythm
• Chest X-ray
• Magnetic resonance imaging (MRI) or CT scan of the heart and lungs
• Cardiac catheterisation – a thin tube is inserted into a blood vessel to measure pressures in the heart directly

What to expect at your appointment

If you are referred to a cardiologist (a heart specialist), you will have a physical examination and one or more of the tests above. The tests are usually painless. A cardiac catheterisation is done with a numbing medicine and a sedative, and you will likely go home the same day or the next morning.

Treatment

Treatment depends on how narrow the artery is and how much it affects your heart. Mild stenosis may need no treatment at all, only regular check-ups. If the narrowing is significant, a specialist can open it with a balloon or place a small metal tube (stent). In some cases, surgery may be needed.

Self-care at home

• Attend all follow-up appointments with your heart specialist.
• Tell your doctor if you develop any new symptoms.
• Keep a healthy weight and stay physically active as advised by your healthcare team.
• Take all medicines exactly as prescribed.

Medical treatments

Doctors may prescribe medicines to help the heart pump more efficiently or to lower blood pressure in the lungs. These medicines do not widen the narrowed artery, but they can help with symptoms and reduce strain on the heart. Always talk to your doctor or pharmacist about the medicines you are taking.

When is surgery considered?

Surgery may be recommended when the narrowing is severe, when it affects both main branches of the pulmonary artery, or when a balloon or stent is not effective. The type of operation varies and your specialist will explain the options in detail.

Living with this condition

Living with pulmonary artery stenosis usually means keeping in touch with your cardiology team and looking after your heart health. You can live a full and active life, but you may need to take a few precautions, such as avoiding vigorous exercise if your doctor advises so.

Lifestyle tips

• Do not smoke or use e-cigarettes.
• Avoid illegal drugs and limit alcohol.
• Get plenty of rest and manage stress in healthy ways.
• Let all healthcare providers know you have a heart condition.

Diet and exercise

Follow a heart-healthy diet with plenty of vegetables, fruits, wholegrains, and lean protein. Your doctor or a dietitian may suggest limiting salt. For exercise, most people can do moderate activity such as walking, swimming, or cycling. Talk to your doctor about what level of activity is safe for you.

Mental health and emotional wellbeing

Living with a heart condition can sometimes cause worry, stress, or low mood. It is normal to feel anxious, especially after a new diagnosis. Talk to your healthcare team about how you are feeling. They can offer support or refer you to a counsellor or psychologist.

Prevention

Pulmonary artery stenosis that is present at birth cannot be prevented. If it develops later in life, treating underlying conditions early and avoiding infections that can affect the heart may help reduce the risk.

Vaccines

Stay up-to-date with recommended vaccines, including the flu vaccine and any vaccinations your doctor advises for heart health. Vaccines can prevent illnesses that might put extra strain on your heart.

Screening programmes

Newborns are routinely checked for heart defects in most countries. If you have a family history of congenital heart disease or a genetic condition linked to it, a specialist may recommend screening with an ultrasound scan during pregnancy.

Complications

If left untreated

• The right side of the heart can become enlarged and weak.
• High blood pressure in the arteries of the lungs (pulmonary hypertension).
• Irregular heart rhythms (arrhythmias).
• A higher risk of developing blood clots in the lungs.

Long-term outlook

The outlook for people with pulmonary artery stenosis is generally good. Many people with a mild narrowing need no treatment and live normal lives. Even when treatment is needed, balloon procedures or surgery are very successful at improving blood flow to the lungs and reducing symptoms. With the right care, you can manage this condition and stay active for many years to come.

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