Pulmonary arterial hypertension (PAHT) is a rare and incurable pulmonary vascular disease. Patients usually live 3 to 5 years following diagnosis. The causes of PAHT are multifactorial. The increase in pulmonary pressure is due to the uncontrolled proliferation of vascular cells that obstruct the pulmonary vessels and lead to death by right heart failure.
The therapies currently available are essentially vasodilators that address the consequences of the disease and do nothing to slow its progression.
In the case of therapeutic failure, heart-lung transplant or double lung transplant is the last recourse, but it is only possible for 50% of patients.