Inhaled iloprost as an add-on therapy for advanced pulmonary arterial hypertension: An Indian perspective.

Background Pulmonary arterial hypertension (PAH) is a progressive disease with high morbidity and mortality. Risk stratification and initiation of dual or triple combination therapy has a better clinical response, especially in high-risk patients. Unfortunately, prostacyclin analogues are not marketed in India; hence, the use of these medications is limited. We report the benefits and difficulties of using iloprost inhalation in patients with advanced PAH in India. Methods In this prospective observational study, we included patients with group 1 PAH. Inhaled iloprost was initiated as an add-on therapy for patients who had clinical, echocardiographic or laboratory deterioration on dual oral medications. Patients with clinical instability were excluded. All patients underwent thorough clinical evaluation, detailed echocardiogram and laboratory investigations. Patients were started on inhaled iloprost 2.5 μg six times daily and closely followed up. The dose was escalated if necessary. On follow-up, clinical echocardiographic and laboratory evaluation was done on all patients. Results Fourteen patients (11 women) with a median age of 32 years (2-66 years) with group 1 PAH were started on inhaled iloprost as an add-on therapy. Improvement in clinical parameters, WHO functional class, echocardiographic-derived right ventricular function, and N-terminal pro-brain natriuretic peptide (NT-pro-BNP) levels were observed in 10 of 14 patients. A median increase of 31% (4.2, 106%) in the distance travelled during 6-minute walk test, a median increase of 45% (-20, 120%) in right ventricular fractional area change, a median increase of 27% (-16.7, 60%) in tricuspid annular peak systolic excursion and a median decrease of 36.7% (-69.6, 17.2%) in NT-pro-BNP levels were observed after initiation of medication. Three patients had progression of symptoms and were then referred for lung/heart-lung transplant. One patient developed progression of symptoms after an excellent initial response and transitioned to subcutaneous treprostinil. Improvement in clinical, echocardiographic and laboratory features allowed us to successfully perform surgical Potts shunt in 2 patients. The medications were well tolerated with minimal and transient side-effects. There were no deaths. Conclusion Inhaled iloprost can be used with acceptable benefits and minimal side-effects in patients with PAH.