Previous reports have indicated that the addition of bosentan to the treatment regimen of patients with PAH may allow the successful discontinuation of parenteral prostacyclin analogues. There are no reports of long-term outcomes in such patients.
We reviewed the medical records of patients in whom prostacyclin analogues were successfully discontinued after the addition of bosentan. Data collected included demographics, cause of PAH, NYHA class, BNP, echocardiography, and 6 minute walk (6MW).
With the addition of bosentan, prostacyclin analogues were successfully discontinued in eight patients with PAH, mean age 44 yrs. (range 33-61), 7 females/1 male. Etiology of PAH included iPAH (N=4), SLE-PH (N=3), and HIV-PH (N=1). Six patients were taken off intravenous epoprostenol and 2 were taken off subcutaneous remodulin. Mean duration of follow-up after discontinuation of therapy was 27.5 months (range: 12-33). During the course of follow-up 2 patients required the addition of sildenafil. All patients have continued to do very well clinically at last follow-up in NYHA II (N= 7) or NYHA I (N=1), mean 6MW distance 1329 ft, mean BNP 95 pg/ml (range 5-164), and mean RVSP 63 mmHg (range 34-93).
With the addition of bosentan, intravenous or subcutaneous prostacyclin analogues may be successfully discontinued in selected patients with PAH.
In appropriately selected patients, the addition of bosentan helps to the successful discontinuation of prostacyclin analogues without deterioration of the functional capacity over long-term follow up.
Enrique Diaz Guzman Zavala, None.