What is pulmonary atresia?

An ultrasound scan

My daughter was born with pulmonary atresia. At the time I was clueless about it. I still find that most people have never heard of it - please can you explain what it is?

Senior cardiac nurse Christopher Allen says:

Pulmonary atresia is a type of congenital heart disease, which means a condition or defect that develops in the womb before your baby is born. Around 1 in 180 babies are diagnosed with a heart defect at birth - that’s an average of 12 per day in the UK.

This type of congenital heart disease may also include other defects

The word ‘pulmonary’ refers to the pulmonary valve in the heart, which is responsible for allowing blood to flow through the pulmonary arteries from the heart to the lungs to be replenished with oxygen. The word ‘atresia’ usually means a blockage or obstruction, therefore pulmonary atresia means that the pulmonary valve has not formed properly and is blocking the flow of blood from the heart to the lungs.

This type of congenital heart disease may also include other defects, such as a hole in the heart or an abnormally small right ventricle, one of the heart’s chambers. Depending on the structure of the heart itself due to other abnormalities, pulmonary atresia carries very different risks and treatment plans for each baby. Sadly, in severe cases, this condition can be life-limiting and involve many procedures and open heart surgeries from a very young age and into adulthood.

Christopher AllenMeet the expert

Christopher Allen helps manage the BHF’s genetic information service and has extensive specialist experience of working in coronary care.


More useful information