What is Pulmonary Stenosis?Pulmonary stenosis is a very rare condition. It can be diagnosed in utero or shortly after birth. Ultrasound images of the four chambers of the heart in pregnancy may
show isolated pulmonary stenosis due to an abnormally thickened or bicuspid valve, or pulmonary stenosis in association with other congenital anomalies, e.g.
Fallot's tetralogy, Noonan's syndrome.
Pulmonary Stenosis is a heart valve disorder where there is a narrowing or blockage of the pulmonary valve. In a healthy heart the pulmonary valve opens to let blood flow from the right ventricle to the lungs. In a patient with pulmonary stenosis the narrowed pulmonary valve means that the right ventricle has to work harder to get the blood past the blockage and to the lung arteries. In time this can cause the heart muscle to become damaged.
SymptomsSymptoms of pulmonary stenosis vary depending on the severity of the condition.
Usually the only sign of pulmonary stenosis is a heart murmur which may be picked up during a routine medical check (a heart murmur is an unusual sound from the heart, which can be heard through a stethoscope). Other symptoms can include:
- shortness of breath (dyspnea);
- chest pain (angina);
- fainting (syncope), or
- cyanosis (blueness).
The heart on the left is normal showing its pulmonic valve wide open
accepting blood.
The heart on the right has pulmonic stenosis.
Note how little blood flow can get through its pulmonic valve
If significant pulmonary stenosis is present then the child may feel tired when playing or doing any physical activity.
In mild cases the patient may not suffer any symptoms at all.
DiagnosisDiagnosis before birth by an MRI usually means that pulmonary stenosis will be severe and treatment will be needed shortly after birth. However, if Pulmonary Stenosis is not diagnosed before birth, it can be diagnosed by other tests in the form of the following:
TreatmentThe pulmonary valve can be treated to improve the narrowing but it can’t be made normal. Treatment will be required when the pressure in the right ventricle is high.
Balloon ValvoplastyIn many cases the narrowing can be widened by balloon valvoplasty. This is where a balloon is placed into an artery in the groin, advanced to the heart, placed across the valve, and inflated in the hope it will relieve the obstruction caused by the narrowed valve. Valvoplasty is a low risk effective treatment of pulmonary stenosis. Out of every 100 children with the condition, 99 will survive. In a small number of cases, the thickened overworked heart muscle doesn’t return to
its normal size and the muscle itself can obstruct the normal flow of blood. If this causes significant narrowing inside the heart, the child will need to have surgery to remove some of the muscle.
Open Heart SurgeryFor very severe cases of Pulmonary Stenosis surgery will be needed. This is where the heart is stopped and put onto a heart bypass machine. Once the heart has been stopped, the surgeon will open the heart and make a small cut in the valve, to widen the narrowed valve and allow more blood to flow through. The heart is then restarted. If a surgical widening of the valve is not possible then a valve replacement may be done. A homograft valve is usually used to replace the pulmonary valve, which is a valve from another person. This type of valve will need replacing after a few years.
After the operation, your child will have a scar down the middle of the chest, along the breast bone.
After treatmentA good result from a balloon valvoplasty or surgery usually means that further treatment is not required. However, the pulmonary valve will never work normally, and there will be leakage of some degree (this is where some of the blood pumped out from the heart to the lungs flows back into the heart through the valve) This leak is not often very important, but there is a chance that some patients may need further surgery to repair or replace the valve later on in life.