In order for us to understand pulmonary atresia, we need to know the working of the normal heart. The heart is the core pumping organ of the body. Its job is to circulate blood and deliver oxygen and essential nutrients all through the body. It has two sides- the right side gets deoxygenated blood returned from the body, and takes this blood to the lungs and the left side gets oxygenated blood from the lungs in order to supply the body. It consists of four chambers: the right atrium, right ventricle, left atrium, and left ventricle. The right and left atria serve as chambers for return of blood, from the body or lungs, respectively. The right and left ventricles are in charge of pumping blood into the pulmonary artery and the aorta, respectively. The deoxygenated blood flow commences from the right side of the heart, through the pulmonary valve into the pulmonary artery, and from there, it flows to the lungs where it enriches itself with oxygen. This oxygenated blood then flows back from the lungs into the left side of the heart through the pulmonary veins. Specialized atrioventricular (AV) valves (these are the tricuspid valve found on the right side and the mitral valve seen on the left side) divide the upper atria and the lower ventricles and prevent blood from flowing back during cardiac pumping.
So, the flow of blood goes something like this ;
- Deoxygenated blood from body
- Right Atria
- Tricuspid Valve
- Right Ventricles
- Pulmonary Valve
- Pulmonary Artery
- Lungs
- Oxygenation
- Pulmonary Veins
- Flows to Left
- Atria Mitral Valve
- Flows to Left Ventricles
- Aorta
- Blood goes to Body to circulate & supply oxygen
What is pulmonary atresia ?
Pulmonary atresia is a type of Congenital Heart Disease. Congenital heart disease is an abnormality of the heart, seen from birth and it develops in the baby when it is in the womb itself. In pulmonary atresia, the pulmonary valve is malformed/ absent. The deoxygenated blood from the right ventricle can’t flow into the pulmonary artery and on to the lungs. Sometimes, pulmonary atresia can be accompanied with right ventricular disease and valvular defect- that simply means the right ventricle and tricuspid valve are also frequently inadequately developed.
What causes pulmonary atresia ?
In most cases, the cause is unknown. Some children may have some other heart defects besides pulmonary atresia. This combination of congenital cardiac defects is seen in teratology of Fallot (a syndrome of cardiac defects). There are several risk factors in pregnancy- either parent having a history of congenital heart defect, the occurrence of infections (German Measles), smoking or drinking in pregnancy, the usage of certain drugs in pregnancy all predispose to congenital heart disease.
How does pulmonary atresia affect the heart ?
The left ventricle pumps a mixture of oxygen-deprived blood into the aorta, the major blood vessel that supplies the whole body. The newborn baby instead of looking pink and healthy, appears to be blue (cyanotic) since there is inadequate oxygenation of blood. The single resource of lung blood flow is the patent ductus arteriosus (PDA), an open pathway between the pulmonary artery and the aorta.
How does pulmonary atresia affect my child ?
- If the patent ductus arteriosus (PDA) constricts or seals off, the lung blood flow and oxygenation to the body is decreased to significantly poor levels.
- The symptoms include bluish or grayish coloration of skin (also called as cyanosis). The skin may feel clammy and cool to touch.
- Difficulty in breathing.
- Failure or difficulty in breast feeding.
These symptoms may develop soon after birth, or within 24 hours of birth.
How is pulmonary atresia with a ventricular septal defect diagnosed ?
In several cases, pulmonary atresia with or without a ventricular septal defect (VSD) may be detected before birth, but in other cases it remains undiagnosed till the birth of the baby. Classically, an echocardiogram is the diagnostic test. An echocardiogram is an ultrasound scan of the heart that uses high-pitched sound waves to image the heart. Your baby does not feel this at all- it is a totally non-invasive procedure.
How can pulmonary atresia be treated ?
- Provisional treatment includes medicines to keep the patent ductus arteriosus (PDA) from closing. A surgeon can produce a shunt amidst the aorta and the pulmonary artery that may assist augment blood flow to the lungs.
- A permanent repair depends on the dimensions of the pulmonary artery and right ventricle. If the size of the pulmonary artery and right ventricle is too small, it may not be correctable by surgery. In those cases, the surgeon can connect the body veins directly to the pulmonary arteries. In children where the pulmonary artery and right ventricle are better developed, open-heart surgery may help the heart work better.
How much can your child exert himself/ herself ?
This is best decided by your child’s pediatric cardiologist. Children born with pulmonary atresia are requested to keep their physical activities and exercises to certain limits.
How will a child with pulmonary atresia fare in the future ?
Children with pulmonary atresia definitely necessitate routine follow-up with a pediatric cardiologist and, once they have grown up, regular follow-up with a cardiologist for life is recommended. Medicines and more surgery might be recommended, in severe cases.
What are the complications of pulmonary atresia ?
Without treatment, pulmonary atresia could be fatal. Even after surgery, the child will need to be on follow-up and monitoring. People with congenital heart problems, for instance pulmonary atresia, are at an increased risk of infective endocarditis. It is an inflammation of the inner tissues of the heart, the endocardium and the valves. It is brought about by infectious agents, most commonly bacteria. Consult your pediatric cardiologist about the need to take antibiotics prior to dental procedures and surgeries to help avoid endocarditis.
How to find pediatric cardiologists for the treatment of pulmonary atresia ?
Now you can find pediatric cardiologists for the treatment of pulmonary atresia from different hospitals and destinations on a single platform, Hinfoways. You can avail opinions from multiple pediatric cardiologists, get approximate cost for the treatment of pulmonary atresia from various heart hospitals, compare things and then choose a pediatric cardiologist for the treatment of pulmonary atresia.
Find a pediatric cardiologist for the treatment of pulmonary atresia on Hinfoways. Make an informed choice.
Disclaimer: The content provided here is meant for general informational purposes only and hence SHOULD NOT be relied upon as a substitute for sound professional medical advice, care or evaluation by a qualified doctor/physician or other relevantly qualified healthcare provider.
