Use of pulmonary arterial hypertensionyspecific therapy in Non-WHO Group I pulmonary hypertension

Adil Shujaat, Jason Bellardini, James D. Cury, Abubakr A. Bajwa

Research output: Contribution to journalArticlepeer-review

3 Scopus citations


Objectives: The development of pulmonary hypertension (PH) in nonYWorld Health Organization group I PH adversely affects exercise capacity. It is unclear whether pulmonary artery hypertension (PAH)Yspecific drugs improve pulmonary hemodynamics and exercise capacity in such patients. Methods: We performed a retrospective chart review of consecutive patients with nonYWorld 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.

Original languageEnglish
Pages (from-to)51-57
Number of pages7
JournalSouthern Medical Journal
Issue number1
StatePublished - 2015
Externally publishedYes


  • Chronic obstructive pulmonary disease
  • Lung diseases
  • Obstructive sleep apnea
  • Pulmonary hypertension
  • Sarcoidosis
  • Therapy


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