How is atrial septal defect with pulmonary hypertension treated?

Q: My friend has been diagnosed with ASD (atrial septal defect) with severe pulmonary hypertension. Her pulmonary artery pressure is 140 mm/Hg. She is not on any drugs. Since last week she is feeling a slight pain in the chest while sleeping and lifting the left arm. Her congenital heart disease was diagnosed at the age of 28. What is the cause of recent pain? Should she consult a doctor? How long can a person with PA pressure of 140 mm/Hg survive? What is the severity of the problem? She feels breathlessness and fatigue often. How can I take care of her?

A:A birth defect like ASD with severely elevated pressures in the pulmonary arteries is basically an inoperable situation. This because irreversible changes have occurred in the lung vasculature. These patients are invariably blue because of un-oxygenated blood getting mixed with the oxygenated blood.The long term prognosis of such patients is not good although the defect acts as an outlet for right heart to decompress. Drugs have a very limited value besides symptom relief. Inspite of these adverse problems many patients can live a long life. These patients should avoid getting dehydrated and infections any where in the body should be treated aggressively. If the haemoglobin is very high, periodic blood letting helps in relieving some symptoms.Heart lung transplant is a method of treatment but the procedure has several difficulties because of availability of donor and several procedural problems.