Use of Pulmonary Arterial Hypertension–Specific Therapy in Non-WHO Group I Pulmonary Hypertension

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Abstract

Objectives

The development of pulmonary hypertension (PH) in non–World Health Organization group I PH adversely affects exercise capacity. It is unclear whether pulmonary artery hypertension (PAH)–specific drugs improve pulmonary hemodynamics and exercise capacity in such patients.

Methods

We performed a retrospective chart review of consecutive patients with non–World Health Organization group I PH treated with PAH-specific therapy.

Results

We identified 24 patients. The mean (standard deviation) age was 48 (14.8) years. Seventeen (71%) patients were women. The 6-minute walk distance improved significantly for the whole group in an initial follow-up period of 4.6 (2.3) months; however, the improvement was seen only in patients with obstructive sleep apnea (OSA) or severe PH and it was not sustained during a longer follow-up period of 11.5 (4.1) months, except in patients with OSA. PH was treated with a variety of PAH-specific drugs, including combination therapy in five patients.

Conclusions

The use of PAH-specific therapy in selected patients with PH secondary to lung diseases, OSA, or sarcoidosis may result in significant improvement in 6-minute walk distance, particularly in patients with OSA or severe PH.

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