Table 2.
Summary of studies included in systematic review and meta-analysis.
| Study ID | Title | Country | Study Design | Population Description | Treated/Total (n/n) | PH Specific Therapy Used (n) | Outcomes Assessed |
|---|---|---|---|---|---|---|---|
| Abuserewa 2021 [22] | Role of Selexipag in Chronic Obstructive Pulmonary Disease (COPD) Patients With Out-of-Proportion Pulmonary Hypertension | United States | Cohort study | PH-COPD patients treated with selexipag for “out-of-proportion” PH (FEV1 > 50%) | 6 | Selexipag | Hemodynamics, 6MWD, Borg scale |
| Brewis 2015 [23] | Severe pulmonary hypertension in lung disease: phenotypes and response to treatment | UK | Cohort study | Severe PH-COPD who received a minimum of 3 months of PH-targeted therapy. | 71 | ERA (16), prostanoid (4), combination (1) | NYHA, 6MWD, NT-proBNP, survival |
| Calcaianu 2016 [24] | Pulmonary Arterial Hypertension-Specific Drug Therapy in COPD Patients with Severe Pulmonary Hypertension and Mild-to-Moderate Airflow Limitation | France | Cohort study | Severe PH-COPD who were treated with PH-specific therapy. | 28 | ERA (23), PDE5 (1), Combination (2), other (2) | Hemodynamics, survival, NYHA class, BNP |
| Faria-Urbina 2018 [25] | Inhaled Treprostinil in Pulmonary Hypertension Associated with Lung Disease | United States | Cohort study | Severe PH patients evaluated at tertiary PH center; 22/72 patients had Group 3 PH. | 8 | Treprostinil | WHO-FC, echocardiography, 6MWD, SpO2 |
| Fossati 2014 [26] | Long-term effect of vasodilator therapy in pulmonary hypertension due to COPD: a retrospective analysis | Switzerland | Cohort study | Retrospective review of patients seen at pulmonary hypertension clinic who had PH-COPD and received PH target therapy for at least 3 months. | 27 | Prostanoid (15), ERA (15), PDE5I (25) | Survival, NYHA, 6MWD, SpO2 |
| Girard 2015 [27] | Severe pulmonary hypertension associated with COPD: hemodynamic improvement with specific therapy | France | Cohort study | Retrospective review of patients seen at pulmonary hypertension referral center who had PH-COPD. | 26 | ERA (11), PDE5I (11), Mix (3) | 6MWD, NYHA, echocardiography, NT proBNP, SpO2 |
| Hurdman 2013 [28] | Pulmonary hypertension in COPD: Results from the ASPIRE registry | UK | Cohort study | Consecutive patients seen at pulmonary hypertension referral center and diagnosed with PH-COPD were included, subdivided by severity of PH. | 43/59 | PDE-5I (31), ERA (10), Prostanoid (2) | Hemodynamics, survival, 6MWD, WHO-FC |
| Jones 1989 [29] | Pulmonary vasodilation with prostacyclin in primary and secondary pulmonary hypertension | UK | Nonrandomized experimental study | Twenty-three patients with PH underwent vasodilation testing with prostacyclin. Five patients had PH-COPD. | 5 | Prostacyclin IV | Hemodynamics |
| Lange 2014 [30] | Outcome of Patients with Severe PH due to Lung Disease with and without Targeted Therapy | Germany | Cohort study | Consecutive patients with a new diagnosis of Group 3 PH (retrospectively from a database and prospectively from a single center); 29/72 patients had PH-COPD, and 12 patients had severe PH-COPD. | 12 | Various, not reported | Hemodynamics, survival, 6MWD |
| Madden, 2006 [31] | A potential role for sildenafil in the management of pulmonary hypertension in patients with parenchymal lung disease | UK | Cohort study | Consecutive patients seen for Group 3 pulmonary hypertension at the referral center and treated with sildenafil; 4/7 patients had PH-COPD. | 3 | Sildenafil | Hemodynamics, 6MWD |
| Tanabe, 2015 [32] | Multi-institutional retrospective cohort study of patients with severe pulmonary hypertension associated with respiratory diseases | Japan | Cohort study | Patients with Group 3 severe PH; 18/70 patients had PH-COPD. | 14/18 | Beraprost (7), ERA (9), PDE5I (15) |
Hemodynamics, survival |
| Vitulo, 2017 [19] | Sildenafil in severe pulmonary hypertension associated with chronic obstructive pulmonary disease: A randomized controlled multicenter clinical trial | Italy | Randomized controlled trial | Patients with COPD were screened for PH. Patients in whom PH was significantly out of proportion relative to COPD severity were included in the trial. | 28 | Sildenafil | Hemodynamics, BODE, 6MWD, QOL. PaO2 |
| Wang, 2017 [33] | Hemodynamic and gas exchange effects of inhaled iloprost in patients with COPD and pulmonary hypertension | Australia | Nonrandomized experimental study | The efficacy and safety of iloprost inhalation were assessed in patients with COPD and PH; 37/67 patients had severe PH. | 37 | Iloprost | Hemodynamics, gas exchange parameters |
6MWD: six-minute walk distance; BNP: brain-natriuretic peptide; BODE: body mass index, airflow obstruction, dyspnea, and exercise capacity; ERA: endothelin receptor antagonist; NYHA: New York Heart Association; PDE5I: phosphodiesterase type 5 inhibitor; QOL: quality of life questionnaire; SpO2: oxygen saturation; WHO-FC: World Health Organization functional class.