Sarcoidosis‐associated pulmonary hypertension: acute vasoresponsiveness to inhaled nitric oxide and the relation to long‐term effect of sildenafil

Background: Severe pulmonary sarcoidosis is often complicated by pulmonary hypertension (PH) caused by different pathophysiological mechanisms. Objectives: To assess the acute vasoresponsiveness in patients with sarcoidosis and PH and the relation to the therapeutic effect of sildenafil. Methods: A retrospective chart review of 25 patients with recalcitrant pulmonary sarcoidosis being evaluated for lung transplantation at our centre. Haemodynamics were evaluated by right heart catheterisation in 24 patients of whom 19 had PH. Eight of the 19 patients received vasodilator challenge with inhaled nitric oxide (iNO). Results: The study group of eight patients (seven men) had a median age of 51 years (range 38 years–58 years). During iNO we observed a reduction in all patients’ mean pulmonary arterial pressure (MPAP) of median 9 mmHg (range 1 mmHg–20 mmHg) (P = 0.01) and in all patients’ pulmonary vascular resistance of median 2.0 Wood Units (0.7 Wood Units–5.8 Wood Units) (P = 0.01). Acute vasoresponsiveness defined as reduction in MPAP of ≥10 mmHg to a MPAP of ≤40 mmHg without reduction in cardiac output was achieved in two of eight patients. Seven patients were subsequently started on sildenafil and had haemodynamics measured during treatment. There was no relationship between the acute vasoresponsiveness and the response to treatment. One patient had similar responses, but in six patients, the responses were reversed. Conclusions: Approximately a quarter of the tested patients with end‐stage pulmonary sarcoidosis and PH displayed acute vasoresponsiveness to iNO. However, the degree of vasoresponsiveness appears to be a poor predictor of the response to treatment of PH with sildenafil. Please cite this paper as: Milman N, Svendsen CB, Iversen M, Videbæk R and Carlsen J. Sarcoidosis‐associated pulmonary hypertension: acute vasoresponsiveness to inhaled nitric oxide and the relation to long‐term effect of sildenafil. The Clinical Respiratory Journal 2009; 3: 207–213.