Complete therapeutical approach in pulmonary arterial hypertension: from vasodilators to lung transplantation - case report

We present the case of a 37 years old patient with idiopathic pulmonary arterial hypertension (PAH). After initial treatment with calcium channel blockers (CCB), based on a positive vasoreactive response at right heart catheterization, due to disease progression, major vasodilator therapies were introduced in a sequential strategy: sildenafil, bosentan and treprostinil. Finally, the patient received double-lung transplantation with eventually favourable evolution despite immediate postoperative significant complications. Markers of disease progression were monitored before the transplant, and after the intervention the patient was screened according to a specific protocol for bronchiolitis obliterans and infections. According to our knowledge, this case represents a premiere in Romanian medicine, being the first lung transplant in a PAH patient. This case represents a model of PAH with a documented evolution of 8 years. We present the progression of the disease and the effective therapeutic strategies according to the current guidelines. This case reinforces the need for upgrading the Romanian National PAH Program with the inclusion of prostanoid therapy, as an option for severe patients.