Pulmonary arterial hypertension

Question

Hello,
What are the consequences of pulmonary arterial hypertension (PAH) in patients with cystic fibrosis? When does it occur? Is there an increased risk of mortality? Otherwise, are sports to be banned?

Answer

Hello,
Pulmonary arterial hypertension (PAH) is a consequence of a fall in arterial oxygen tension when respiratory failure worsens. In cystic fibrosis, it often occurs at a stage of advanced lung disease and is an indicator of severity. The pressure of the pulmonary artery is easily measured by echocardiography.
When PAH worsens, it may lead to right heart failure with peripheral edema and an enlarged liver. Ultimately, it results in death from heart failure associated with respiratory failure.
The treatment consists mainly of oxygen that can prevent worsening of PAH and the onset of heart failure. Oxygen treatment is first indicated in the periods when the oxygen pressure decreases as, for example at night or during exercise. At a more advanced stage, oxygen therapy may be needed continuously and will be associated with diuretic treatment. At this stage the indication of lung transplantation must be discussed.
At an early stage of PAH, it is advisable to practice exercise but with oxygen therapy, and at least initially under medical supervision. Sports performed without oxygen are not recommended.
Sincerely,
Dr Dominique Hubert

27.02.2012