Pulmonary hypertension
Pulmonary hypertension is a possible complication of advanced cystic fibrosis, caused by a chronic lack of oxygen, which can lead to right heart failure, while it remains rare after a lung transplant unless there is a severe respiratory problem.
Dr. Lara Bilodeau Pulmonologist
Quebec University Institute of Cardiology and Pulmonology (Laval Hospital)
Quebec (Quebec) Canada
PULMONARY HYPERTENSION
Q: Can you explain to me what pulmonary hypertension is? What causes it? How do you recognize the symptoms? Are lung transplant patients equally at risk of suffering from pulmonary hypertension one day or another?
R : The term pulmonary hypertension means that blood pressure is abnormally high in the arteries of the lungs. This phenomenon can be caused by several lung and heart diseases. In the case of lung diseases, it is the chronic lack of oxygen that causes pulmonary hypertension. This complication can therefore occur in people with cystic fibrosis when the disease is at an advanced stage.
In the long term, pulmonary hypertension can lead to right heart failure. The heart is divided into two parts: the right and the left. The right part receives venous blood (blood with low oxygen content) from all organs and sends it to the lungs to be oxygenated. The left part receives oxygenated blood from the lungs and propels it to all organs. If the pressure in the arteries in the lungs is abnormally high, the right heart must pump harder to direct blood to the lungs. Over time, it can get tired and become less effective. This is called right heart failure, which means that the right heart can no longer pump enough blood to the lungs. Blood then flows back into the veins, which can be manifested by swelling (accumulation of fluid) in the legs and sometimes even in the belly.
If pulmonary hypertension is suspected, an ultrasound of the heart should be performed to make the diagnosis. This test measures the pressure in the arteries of the lungs and checks whether the heart is pumping normally. There is no specific treatment for pulmonary hypertension secondary to cystic fibrosis. Treatment consists of eliminating excess fluid when the heart no longer pumps enough. To do this, diuretics must be taken, that is, medications that cause urination. The best treatment is prevention, by giving oxygen to people who need it.
For people who have had a lung transplant, they should not have pulmonary hypertension unless they develop a respiratory problem that causes a lack of oxygen. For example, a person who experiences severe chronic rejection may have a lowered oxygen level and develop pulmonary hypertension.
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