Normal
Video showing normal pulmonary valve opening and closing.it looks like a Mercedes benz sign.
What is a Pulmonary Stenosis?
The pulmonary artery is a large artery (blood vessel) which carries blue deoxygenated blood from the heart to the lungs. in the body. The connection between the heart and the pulmonary artery is guarded by a valve called the pulmonary valve. Narrowing of the pulmonary valve is known as pulmonary stenosis. Pulmonary stenosis increases the work of the heart causing the right ventricle (lower pumping chamber) to thicken (known as hypertrophy) and potentially eventually fail. Pulmonary stenosis can be mild, moderate, severe or critical (the latter is an emergency). The narrowing can be at the level of the valve, or above the valve (supravalvar) or below the valve (subvalvar).
Symptoms
Most babies and children will have no symptoms. The usual form of presentation is the incidental finding of a heart murmur. Severe cases, which are not common, may become breathless, tire more easily, and have slower weight gain with feeding difficulties. Critical stenosis in newborns can lead to collapse. Pulmonary stenosis may also be diagnosed before birth by fetal echocardiography.Diagnosis
A clinical examination will usually reveal a heart murmur. An echocardiogram will confirm the diagnosis. An ECG will also be performed and often a chest X-ray.
Treatment
Mild and moderate pulmonary stenosis is well tolerated and does not require treatment but should be regularly monitored in the outpatient clinic. There is a small possibility of spontaneous improvement with growth. Other cases become more severe with time whilst others remain stable for many years. Pulmonary stenosis can be treated either via a keyhole approach with a balloon cardiac catheter (angioplasty) or less commonly by open heart cardiac surgery. Each child’s heart is individually assessed to decide which treatment will give the best long-term result.
Balloon cardiac catheter involves a general anaesthetic. A tube, with a deflated balloon, is passed from a blood vessel in the leg to the heart, using X ray guidance. Once the balloon is correctly positioned in the narrowed pulmonary artery, it is inflated, thereby stretching the narrow opening to enlarge it. This process is repeated a number of times and then the catheter is removed. After this procedure, there may be some leak across the stretched valve but a leaky valve is better tolerated than a very narrow valve. Hospital stay duration depends on the age of the child and their status prior to the procedure. The simplest cases may only require a 1-2 night admission.
Open heart surgery techniques to repair the pulmonary artery involves being attached to the cardiac bypass machine. Children are usually in hospital for 5-10 days but longer stays can be necessary particularly for babies. Surgical strategies include surgical repair of the valve, and replacement with a mechanical or tissue valve.
Dr Naqvi works with an experienced internationally renowned team of nationally audited congenital cardiac surgeons and interventionalists and if needed will refer your child to the one who is best for your child’s heart.
Prognosis
The outlook for children with Tetralogy Of Fallot is usually excellent. Some patients are left with a leak across the pulmonary artery. This may require further surgery later in life (usually between the teenage years and young adulthood). This may be open heart or via transcather keyhole placement of a Melody valve. These procedures also have excellent success rates. Dr Naqvi has cared for many babies and children with Tetralogy Of Fallot and has followed them up until adulthood. She says “Having Tetralogy Of Fallot does not stop children doing well in sports or from having a happy full life. I know many who are in school teams and even competing at county level. I even know patients who have achieved 10 A stars at GCSE despite having a Fallot repair as a baby.” There are also a number of successful athletes with Tetralogy Of Fallot including Australian cricketer Beau Casson. Shaun White is an inspirational 2 times Gold medallist Olympic snowboarder (net worth $40 million) who had 2 cardiac surgeries for Tetralogy Of Fallot before 1 year of age. You can see his interview with Larry King on CNN on You Tube where he discusses his condition and his career.
For more information about pulmonary stenosis please see the following links:
Disclaimer: The opinions and facts shown in this article are as accurate and up to date as possible, but are provided as general “information resources”, which may not be relevant to individual persons. This article is not a substitute for individual assessment and always take advice from a paediatric cardiologist who is familiar with the particular person.