Mariana Faria-Urbina1,2, Rudolf K F Oliveira1,2,3, Manyoo Agarwal4, Aaron B Waxman5,6,7. 1. Pulmonary and Critical Care Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA. 2. Center for Pulmonary Heart Disease, Heart & Vascular Center, Brigham and Women's Hospital, Boston, MA, USA. 3. Division of Respiratory Diseases, Department of Medicine, Federal University of Sao Paulo (UNIFESP), Sao Paulo, Brazil. 4. Department of Medicine, University of Tennessee Health Science Center, Memphis, TN, USA. 5. Pulmonary and Critical Care Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA. abwaxman@bwh.harvard.edu. 6. Center for Pulmonary Heart Disease, Heart & Vascular Center, Brigham and Women's Hospital, Boston, MA, USA. abwaxman@bwh.harvard.edu. 7. Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital, 75 Francis Street, PBB Clinics-3, Boston, MA, 02115, USA. abwaxman@bwh.harvard.edu.
Abstract
PURPOSE: Pulmonary hypertension (PH) in the setting of parenchymal lung disease adversely affects quality of life and survival. However, PH-specific drugs may result in ventilation/perfusion imbalance and currently, there are no approved PH treatments for this patient population. In the present retrospective study, data from 22 patients with PH associated with lung disease treated with inhaled treprostinil (iTre) and followed up clinically for at least 3 months are presented. METHODS: PH was defined by resting right heart catheterization as a mean pulmonary artery pressure (mPAP) ≥ 35 mmHg, or mPAP ≥ 25 mmHg associated with pulmonary vascular resistance ≥ 4 Woods Units. Follow-up evaluation was performed at the discretion of the attending physician. RESULTS: From baseline to follow-up, we observed significant improvement in functional class (n = 22, functional class III-IV 82 vs. 59%, p = 0.041) and 6-min walk distance (n = 11, 243 ± 106 vs. 308 ± 109; p = 0.022), without a deleterious effect on resting peripheral oxygen saturation (n = 22, 92 ± 6 vs. 94 ± 4; p = 0.014). Most of the patients (86%, n = 19/22) were using long-term nasal supplemental oxygen at baseline. During follow-up, only one patient had increased supplemental oxygen requirement. The most common adverse events were cough, headache, and diarrhea. No severe adverse event was reported. CONCLUSIONS: The results suggest that iTre is safe in patients with Group 3 PH and evidence of pulmonary vascular remodeling in terms of functional class, gas exchange, and exercise capacity. Additionally, iTre was well tolerated. The potential role of PH-specific drugs in Group 3 PH should be further assessed in larger prospective studies.
PURPOSE:Pulmonary hypertension (PH) in the setting of parenchymal lung disease adversely affects quality of life and survival. However, PH-specific drugs may result in ventilation/perfusion imbalance and currently, there are no approved PH treatments for this patient population. In the present retrospective study, data from 22 patients with PH associated with lung disease treated with inhaled treprostinil (iTre) and followed up clinically for at least 3 months are presented. METHODS: PH was defined by resting right heart catheterization as a mean pulmonary artery pressure (mPAP) ≥ 35 mmHg, or mPAP ≥ 25 mmHg associated with pulmonary vascular resistance ≥ 4 Woods Units. Follow-up evaluation was performed at the discretion of the attending physician. RESULTS: From baseline to follow-up, we observed significant improvement in functional class (n = 22, functional class III-IV 82 vs. 59%, p = 0.041) and 6-min walk distance (n = 11, 243 ± 106 vs. 308 ± 109; p = 0.022), without a deleterious effect on resting peripheral oxygen saturation (n = 22, 92 ± 6 vs. 94 ± 4; p = 0.014). Most of the patients (86%, n = 19/22) were using long-term nasal supplemental oxygen at baseline. During follow-up, only one patient had increased supplemental oxygen requirement. The most common adverse events were cough, headache, and diarrhea. No severe adverse event was reported. CONCLUSIONS: The results suggest that iTre is safe in patients with Group 3 PH and evidence of pulmonary vascular remodeling in terms of functional class, gas exchange, and exercise capacity. Additionally, iTre was well tolerated. The potential role of PH-specific drugs in Group 3 PH should be further assessed in larger prospective studies.
Authors: Christopher A Thomas; Justin Lee; Roberto J Bernardo; Ryan J Anderson; Vladimir Glinskii; Yon K Sung; Kristina Kudelko; Haley Hedlin; Andrew Sweatt; Steven M Kawut; Rishi Raj; Roham T Zamanian; Vinicio de Jesus Perez Journal: Front Med (Lausanne) Date: 2021-12-03
Authors: Shelsey W Johnson; Lauren Finlay; Stephen C Mathai; Ronald H Goldstein; Bradley A Maron Journal: Pulm Circ Date: 2022-07-01 Impact factor: 2.886